Materiały źródłowe

• #1 Total anomalous pulmonary venous connection – UpToDate

  https://www.uptodate.com/contents/total-anomalous-pulmonary-venous-connection

  Total anomalous pulmonary venous connection (TAPVC), also referred to as total anomalous pulmonary venous return (TAPVR), is a cyanotic congenital defect in which all four pulmonary veins fail to make their normal connection to the left atrium. This results in drainage of all pulmonary venous return into the systemic venous circulation. […] The reported prevalence of TAPVC ranges from 0.6 to 1.2 per 10,000 live births. TAPVC accounts for approximately ranges between 0.7 and 1.5 percent of all congenital heart defects in newborns. […] TAPVC arises from the failure of the left atrium to link with the pulmonary venous plexus, which results in the retention of connections through the primitive cardinal and umbilicovitelline drainage pathways.

• #1 About Total Anomalous Pulmonary Venous Return | Congenital Heart Defects (CHDs) | CDC

  https://www.cdc.gov/heart-defects/about/tapvr.html

  Total anomalous pulmonary venous return (pronounced TOHT-l uh-NOM-uh-luh-s PUHL-muh-ner-ee VEE-nuh-s ri-TURN) or T-A-P-V-R is a congenital heart defect. […] About 1 in every 7,809 babies in the United States are born with TAPVR. This means that about 504 babies are born with TAPVR each year. […] TAPVR and PAPVR might be diagnosed during pregnancy, but more often these defects are diagnosed soon after a baby is born. […] Babies with TAPVR will need surgery to repair the defect. […] Surgical repairs for TAPVR are not a cure. People living with TAPVR may have lifelong complications. Routine checkups with a heart doctor are needed to monitor progress, avoid complications, and check for other health conditions that might develop over time.

• #1 Total anomalous pulmonary venous return (TAPVR) – Children’s Health Cardiology

  https://www.childrens.com/specialties-services/conditions/total-anomalous-pulmonary-venous-return

  Surgery is the only way to fix a total anomalous pulmonary venous return, a rare congenital birth defect of the heart. […] Total anomalous pulmonary venous return (TAPVR) makes it hard for the heart to pump oxygen-rich blood to the rest of the body. […] TAPVR develops during the first four to six weeks of pregnancy because the child’s heart doesnt develop normally. […] In some cases, signs of TAPVR show up during prenatal tests, such as a fetal ultrasound. Children can develop symptoms within the first weeks, months or year of their life. […] We dont know what causes TAPVR, but we do know the defect develops during the first four to six weeks of pregnancy. Its not caused by anything a mother or father did or did not do. […] Children with TAPVR need surgery to restore normal blood flow to and through the heart. Your childs surgeon will discuss which surgery is right for your child. […] Yes. The only effective treatment for TAPVR is surgery. The good news is that after your child recovers, he or she can expect to live a healthy, active life.

• #1 Total Anomalous Pulmonary Venous Return (TAPVR) | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/total-anomalous-pulmonary-venous-return-tapvr

  Total anomalous pulmonary venous return is a congenital (meaning it’s present from birth) heart disease that affects the pulmonary veins. […] Total anomalous pulmonary venous return (TAPVR) may be diagnosed before birth, with fetal echocardiogram (ultrasound). […] Total anomalous pulmonary venous return requires open heart surgery in all cases. […] Because of enormous strides in medicine and technology, today most children born with total anomalous pulmonary venous return go on to lead productive lives as adults. […] Children who were born with TAPVR and had it repaired must continue to see a pediatric cardiologist regularly. […] It is important that children who were born with total anomalous pulmonary venous return continue to see a cardiologist.

• #1 Partial and Total Anomalous Pulmonary Venous Connection – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560707/

  TAPVC and PAPVC are uncommon congenital anomalies accounting for 1% of all congenital heart diseases. […] Total anomalous pulmonary venous connections (TAPVC) is a cyanotic congenital heart disease where all of the pulmonary veins drain directly or indirectly into the right atrium. […] TAPVC implies that all the pulmonary veins connect to the systemic veins or right atrium, either by a common vertical vein or individually. Anatomically TAPVC can be divided into four subtypes based on the level of the anomalous connection as supracardiac, cardiac, infracardiac, and mixed. […] The supracardiac variety is the most common of the TAPVC. […] In untreated patients, TAPVC is almost always fatal within the first few weeks of life. The long term survival in patients has increased with the improvement in medical management and surgical techniques.

• #1 Supracardiac Total Anomalous Pulmonary Venous Return | Pediatric Echocardiography

  https://pedecho.org/library/chd/tapvr

  Total anomalous pulmonary venous connection is when all of the pulmonary veins returning from the lungs have an abnormal connection and drain anomalously to the right side of the heart instead of the left atrium. Supracardiac TAPVC is the most common type of anomalous pulmonary venous connection. In supracardiac TAPVC, the common pulmonary vein drains superiorly via a vertical vein into the left innominate vein, the right superior vena cava, or the right azygos vein by way of an ascending vertical vein. […] TAPVC has been described in 9 in 100,000 of the population. Supracardiac TAPVC is the most common type (47% in the largest published series) and in this group the most common site of connection is to the left innominate vein (36% of all cases). […] The mortality of untreated patients is 75-85% by 1st year of life.

• #1 Neonatal Outcomes in Total Anomalous Pulmonary Venous Return: The Role of Prenatal Diagnosis and Pulmonary Venous Obstruction – PubMed

  https://pubmed.ncbi.nlm.nih.gov/29796693/

  The objective of this study is to evaluate neonatal outcomes of total anomalous pulmonary venous return (TAPVR) and identify fetal echocardiography findings associated with preoperative pulmonary venous obstruction (PPVO). This retrospective study evaluated TAPVR cases from 2005 to 2014 for preoperative and postoperative outcomes based on prenatal diagnosis, PPVO, and heterotaxy syndrome. Of 137 TAPVR cases, 12% were prenatally diagnosed; 60% had PPVO, and 21% had heterotaxy. TAPVR repair was performed in 135 cases and survival to discharge was 82% (112/137). Heterotaxy was the only independent predictor of mortality on multiple regression analysis [OR 5.5 (CI 1.3-16.7), p = 0.02]. TAPVR has severe neonatal morbidity and mortality with low prenatal diagnosis rates in the absence of heterotaxy. […] Patients with obstructed TAPVR had greater preoperative morbidity, but only heterotaxy was independently associated with increased postoperative mortality. Vertical vein velocity helped prenatally identify those at risk of PPVO.

• #1 TAPVR | Types, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/t/tapvr

  Because of the abnormal pulmonary vein connection red (oxygenated) blood returning from the lungs mixes with the blue (lower oxygenated) blood returning from the body. […] The outcome of surgical repair for total anomalous pulmonary venous return is excellent. The surgical mortality is less than 5% when repair is performed electively. This is in healthy children without obstructed pulmonary veins. […] Patients who have surgery to repair total anomalous pulmonary venous return early in their lives often do well. Most do not need expert follow-up care as adults.

• #1 Anesthetic Management During Stenting of Obstructed Total Anomalous Pulmonary Return in a Neonate With Single Ventricle Anatomy | Hakim | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/2724/2078

  Total anomalous pulmonary venous return (TAPVR) is a relatively rare congenital cardiac abnormality accounting for 1.5-3% of cases of congenital heart disease (CHD). […] Morbidity and mortality associated with surgical repair of TAPVR increase dramatically when there are other associated cardiac defects, particularly single ventricle anatomy. […] Total anomalous pulmonary venous return (TAPVR) is a relatively rare type of congenital heart disease (CHD) accounting for 1.5-3% of such defects. […] Morbidity and mortality associated with TAPVR repair increase dramatically when another cardiac anomaly is present, particularly when combined with single ventricle anatomy. […] TAPVR remains one of the most challenging CHDs in newborns and young infants. […] The surgical results of TAPVR repair have improved significantly in the modern era as a result of earlier diagnosis and treatment, improvements in surgical technique, cardiac anesthesia, neonatal myocardial preservation, and postoperative care.

• #1 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  Overall TAPVR mortality may be decreasing with time, which may be partially due to earlier recognition and a change in preoperative diagnostic strategies over recent decades. Complex mixed-type TAPVR has now been demonstrated in fetal ultrasound as early as 18 weeks. There has been a move towards more advanced post-natal imaging including the use of angiography. […] While TAPVR literature has continued to compound, there remains relatively limited mixed-type TAPVR-specific case series. The following is a retrospective clinical review. We aim to describe and display the anatomic variance and surgical technique used in the operative repair of one surgical groups experience with 14 mixed-type TAPVR patients between 2004-2018 in an attempt to add to the comprehensive available data with which clinicians can use to optimize decision-making in a rare congenital lesion bearing significant mortality.

• #1 Surgical repair of neonatal total anomalous pulmonary venous connection: A single institutional experience with 241 cases – JTCVS Open

  https://www.jtcvsopen.org/article/S2666-2736(23)00210-3/fulltext

  Challenges persist in surgery for neonatal total anomalous pulmonary venous connection (neoTAPVC), with the high mortality risk not mitigated over time. A prospectively collected single-center database containing all neonates with TAPVC undergoing biventricular repair in 2012 to 2020 was retrospectively reviewed. The primary outcome was death or postoperative pulmonary venous obstruction (PPVO). Overall, 241 patients with a median age at surgery of 14 days (interquartile range [IQR], 9-21 days) were included. Anomalous return was supracardiac in 38.6%, cardiac in 26.1%, infracardiac in 28.6%, and mixed in 6.6%. Patients receiving DPC had better survival (96.3% vs 84.3%; P= .0028) and lower incidence of PPVO (15.2% vs 28.6%; P= .011) compared with those without DPC. Access to DPC potentially improves outcomes in the neoTAPVC setting; freedom from PPVO were similar using conventional versus sutureless repair. Dedicated preoperative care is associated with better survival and lower restenosis rate following surgical repair of total anomalous pulmonary venous connection (TAPVC) in the neonatal patients. The rationale for the requirement of such specialist care is mainly based on the previous finding that patient-specific preoperative status has a strong association with mortality and morbidity, which can be influenced by preoperative management strategy. However, to what extent dedicated presurgical care (DPC) would influence the outcomes after neonatal TAPVC (neoTAPVC) surgery has not been well investigated. This study first provides an insight into the impact of presurgical care models on outcomes in the neoTAPVC setting. It is noteworthy that the access to DPC conferred an approximately 4-fold decrease in death, particularly resulting in the postoperative 1-year mortality of 3.7% that was remarkably lower than previously published data. Findings from this study suggest that care by a dedicated cardiac team can allow for tiered patient triage and helps to optimize presurgical clinical status and bridge the time to surgery in the selected patients.

• #1 Surgery-related complications and their management in total anomalous pulmonary venous return during intensive care unit stay – Turkish Journal of Thoracic and Cardiovascular Surgery

  https://tgkdc.dergisi.org/text.php3?id=2188

  Pulmonary venous obstruction is crucial for both pre- and postoperative assessment in the ICU. Several studies have shown that patients with preoperative PVO had lower intubation rates, a higher number of postoperative pulmonary hypertensive crises and episodes, and a greater amount of positive inotropic support during their ICU stays. […] Following TAPVR corrective surgery, the mortality rate in recent years has decreased from 80% to below 10%, with early and accurate diagnoses, new developments in surgical techniques, and effective and improved ICU support being responsible for this dramatic improvement. […] Our findings revealed that postoperative complications such as low cardiac output, pulmonary hypertensive crises, and arrhythmia can be prevented by advanced ICU monitorization, especially for patients without the need for mechanical ventilation, those without metabolic acidosis, those with TAPVR who also have left ventricular hypoplasia, and those who have PVO. Furthermore, early and effective treatment with NO drugs or ECMO support to counteract these complications could lead to an improvement in the morbidity and mortality rates of these patients.

• #1 Whole-exome sequencing identifies SGCD and ACVRL1 mutations associated with total anomalous pulmonary venous return (TAPVR) in Chinese population | Oncotarget

  https://www.oncotarget.com/article/15434/text/

  As a rare type of Congenital Heart Defects (CHD), the genetic mechanism of Total Anomalous Pulmonary Venous Return (TAPVR) remains unknown, although previous studies have revealed potential disease-driving regions/genes. […] TAPVR characterized by cardiac function deterioration including cyanosis, pulmonary hypertension, dyspnea, pulmonary edema, congestive heart failure affects 1 in 15,000 live births and 48.8% of them will die without surgery before the age of 1. […] We provided evidence for ACVRL1 as a known causative gene and for SGCD as candidate genes for TAPVR. […] A rare missense mutation in ACVRL1 (c.C652T, p.R218W) was identified as a causal mutation for TAPVR. […] A recurrent mutation in SGCD (c.C717G, p.D239E) was detected, which coded for the dystrophin-glycoprotein complex (DGC). […] In conclusion, our study was the first attempt to dissect the etiology of TAPVR using whole-exome sequencing strategy in Chinese population.

• #1 Anomalous Pulmonary Venous Return

  https://fpnotebook.com/CV/Peds/AnmlsPlmnryVnsRtrn.htm

  Accounts for 0.7 to 1.5% of Congenital Heart Disease cases (5th most common cyanotic cause) […] TAPVR is frequently misdiagnosed initially.

• #1 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  This study outlines the complex variance in mixed type TAPVR patients, and to our knowledge only 5 other mixed type-specific retrospective case series exist in the literature, though we acknowledge mixed type data can be found within generalized TAPVR studies. The intent of this data was to add to the literature for purposes of future meta and systematic analyses to improve surgical approach, as well as serve educational purposes regarding a rare and intricate lesion. […] Mixed type TAPVR presents a wide variety of connections, lending to the complexity in planning and execution of surgical correction. Our study contributes further to the available data on this more rare form of TAPVR, which may be important as more cohorts emerge with varying success rates. More widespread studies, including systematic review may be beneficial to further characterize morphology, intraoperative technique, outcome data, and perhaps most importantly, specific indices for predicting higher survival or mortality rates.

• #2 Partial and Total Anomalous Pulmonary Venous Connection – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560707/

  TAPVC and PAPVC are uncommon congenital anomalies accounting for 1% of all congenital heart diseases. […] Total anomalous pulmonary venous connections (TAPVC) is a cyanotic congenital heart disease where all of the pulmonary veins drain directly or indirectly into the right atrium. […] TAPVC implies that all the pulmonary veins connect to the systemic veins or right atrium, either by a common vertical vein or individually. Anatomically TAPVC can be divided into four subtypes based on the level of the anomalous connection as supracardiac, cardiac, infracardiac, and mixed. […] The supracardiac variety is the most common of the TAPVC. […] In untreated patients, TAPVC is almost always fatal within the first few weeks of life. The long term survival in patients has increased with the improvement in medical management and surgical techniques.

• #2 Total Anomalous Pulmonary Venous Return (TAPVR)

  https://my.clevelandclinic.org/health/diseases/23069-total-anomalous-pulmonary-venous-return

  Total anomalous pulmonary venous return is a rare condition. It affects 1 in about 7,500 newborns in the U.S. each year. […] Total anomalous pulmonary venous return (TAPVR) is an issue where veins from your baby’s lungs connect to the right side of their heart instead of the left. […] TAPVR is a life-threatening heart condition. Its congenital, which means it’s present at birth. […] Without surgery, some forms of total anomalous pulmonary venous return are typically fatal a few weeks after birth. With early diagnosis and surgical treatment, the outlook for babies with TAPVR is very good. The survival rate after surgery is around 97%.

• #2 Total Anomalous Pulmonary Venous Return (TAPVR)

  https://healthlibrary.brighamandwomens.org/SummerHeat/90,P01820

  Most of the time, the cause of TAPVR isnt known. It may occur with other heart or body system problems. […] The symptoms of TAPVR may seem like symptoms of other health conditions. Your childs healthcare provider must diagnose them with this condition. […] Newborns with TAPVR will have blue coloring of their skin, lips, and nailbeds (cyanosis). This happens in the first hours or days of their life. How severe your childs condition is depends on how much blood is able to get to their body. […] A healthcare provider may first spot TAPVR in a baby during an ultrasound in pregnancy. After birth, if your child has signs of TAPVR, a pediatric cardiologist or neonatologist will check them. […] Treatment will depend on your childs symptoms, age, and overall health. It will also depend on how severe the condition is.

• #2 About Total Anomalous Pulmonary Venous Return | Congenital Heart Defects (CHDs) | CDC

  https://www.cdc.gov/heart-defects/about/tapvr.html

  Total anomalous pulmonary venous return (pronounced TOHT-l uh-NOM-uh-luh-s PUHL-muh-ner-ee VEE-nuh-s ri-TURN) or T-A-P-V-R is a congenital heart defect. […] About 1 in every 7,809 babies in the United States are born with TAPVR. This means that about 504 babies are born with TAPVR each year. […] TAPVR and PAPVR might be diagnosed during pregnancy, but more often these defects are diagnosed soon after a baby is born. […] Babies with TAPVR will need surgery to repair the defect. […] Surgical repairs for TAPVR are not a cure. People living with TAPVR may have lifelong complications. Routine checkups with a heart doctor are needed to monitor progress, avoid complications, and check for other health conditions that might develop over time.

• #2 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  Total anomalous pulmonary venous return (TAPVR), also referred in literature as total anomalous pulmonary venous connection (TAPVC), provides a wide spectrum of complex anatomic and therapeutic challenges amongst congenital heart disease. An estimated 400 infants (1/10.000 births) are born annually in the United States with TAPVR, an incidence among congenital heart disease of 0.7-1.5%. While there may be rare survival to adulthood if un-repaired, typical median survival if unrepaired is 2 months with 50% mortality in first 3 months of life, thereby demonstrating the urgency of recognition and intervention. […] TAPVR is classified according to the Darling system developed in 1957 into cardiac, supracardiac, infracardiac, or mixed-type depending on the pattern of pulmonary venous (PV) drainage to the systemic venous circuitry. Mixed-type TAPVR in itself has a higher mortality, with univariate analysis of mixed type versus cardiac type (lowest mortality of TAPVR variants) demonstrating statistically significant hazard ratios of 2.88 in previous study, and mortality as high as 42-50%. Other determinants of increased mortality or transplant in this study include complex TAPVC and postoperative length of stay, while older age, weight greater than 2.5 kg, non-emergent operation, lack of PV obstruction, cardiac and supracardiac types were associated with increased survival to discharge.

• #2 Total Anomalous Pulmonary Venous Return (TAPVR) | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/total-anomalous-pulmonary-venous-return-tapvr

  Total anomalous pulmonary venous return is a congenital (meaning it’s present from birth) heart disease that affects the pulmonary veins. […] Total anomalous pulmonary venous return (TAPVR) may be diagnosed before birth, with fetal echocardiogram (ultrasound). […] Total anomalous pulmonary venous return requires open heart surgery in all cases. […] Because of enormous strides in medicine and technology, today most children born with total anomalous pulmonary venous return go on to lead productive lives as adults. […] Children who were born with TAPVR and had it repaired must continue to see a pediatric cardiologist regularly. […] It is important that children who were born with total anomalous pulmonary venous return continue to see a cardiologist.

• #2 Total Anomalous Pulmonary Venous Return (TAPVR) | Nationwide Children’s

  https://www.nationwidechildrens.org/conditions/total-anomalous-pulmonary-venous-return

  Total anomalous pulmonary venous return is a heart problem where veins connect to the wrong place. It needs surgery to fix. […] Total anomalous pulmonary venous return (TAPVR) is a condition where the blood vessels in the lungs, called the pulmonary veins, are not attached to the heart in the right place. […] All children born with TAPVR will need surgery to connect the pulmonary veins to the left atrium and close the atrial septal defect. […] At Nationwide Childrens, we provide care for TAPVR across all stages of life, from prenatal to adulthood. […] Your cardiologist will follow up with your babys heart needs even after they are discharged from the hospital.

• #2 TAPVR | Norton Children’s Louisville, Ky.

  https://nortonchildrens.com/services/cardiology/conditions/congenital-heart-disease/tapvr/

  Total anomalous pulmonary venous return (TAPVR) is a critical birth defect of the pulmonary veins that causes oxygen-rich blood from the lungs to return to the wrong side of the heart. […] All babies born with TAPVR likely will need surgery in the first year of life. […] As the leading providers of pediatric heart care in Louisville and Southern Indiana, the board-certified and fellowship-trained cardiothoracic surgeons at Norton Children’s Heart Institute are experienced with successfully repairing TAPVR. […] TAPVR is a rare heart defect, and in most cases the cause is unknown. […] TAPVR also can be found after a baby is born. […] Babies born with TAPVR will need surgery to restore normal blood flow through the heart. […] Approximately 5% to 8% of children who have the procedure do not survive. […] Children with surgically repaired TAPVR may have lifelong complications.

• #2 Surgery-related complications and their management in total anomalous pulmonary venous return during intensive care unit stay – Turkish Journal of Thoracic and Cardiovascular Surgery

  https://tgkdc.dergisi.org/text.php3?id=2188

  Pulmonary venous obstruction is crucial for both pre- and postoperative assessment in the ICU. Several studies have shown that patients with preoperative PVO had lower intubation rates, a higher number of postoperative pulmonary hypertensive crises and episodes, and a greater amount of positive inotropic support during their ICU stays. […] Following TAPVR corrective surgery, the mortality rate in recent years has decreased from 80% to below 10%, with early and accurate diagnoses, new developments in surgical techniques, and effective and improved ICU support being responsible for this dramatic improvement. […] Our findings revealed that postoperative complications such as low cardiac output, pulmonary hypertensive crises, and arrhythmia can be prevented by advanced ICU monitorization, especially for patients without the need for mechanical ventilation, those without metabolic acidosis, those with TAPVR who also have left ventricular hypoplasia, and those who have PVO. Furthermore, early and effective treatment with NO drugs or ECMO support to counteract these complications could lead to an improvement in the morbidity and mortality rates of these patients.

• #2 Total Anomalous Pulmonary Venous Return | Thoracic Key

  https://thoracickey.com/total-anomalous-pulmonary-venous-return/

  In the series by St. Louis etal., 27% of patients with TAPVR had an associated cardiac lesion. […] The population-based study reported by Seale showed that in patients with TAPVR, excluding those with heterotaxy and complex single-ventricle anatomy, 14% had associated cardiac lesions. […] The diagnosis of TAPVR can be difficult on prenatal ultrasonography. […] Echocardiography is an important initial diagnostic tool for patients with suspected TAPVR. […] With the advent of multidetector computed tomography (CT) scanning, detailed images of the pulmonary venous drainage, including inflow and runoff vessels from the confluence, can be achieved. […] Diagnostic cardiac catheterization is reserved for patients in whom echocardiography or CT imaging is not satisfactory or when associated lesions need to be further defined. […] The level of preoperative support required will reflect the pathophysiologic state of the patient and depends on the degree of PVO and pulmonary blood flow.

• #2 Surgical repair of neonatal total anomalous pulmonary venous connection: A single institutional experience with 241 cases – JTCVS Open

  https://www.jtcvsopen.org/article/S2666-2736(23)00210-3/fulltext

  Challenges persist in surgery for neonatal total anomalous pulmonary venous connection (neoTAPVC), with the high mortality risk not mitigated over time. A prospectively collected single-center database containing all neonates with TAPVC undergoing biventricular repair in 2012 to 2020 was retrospectively reviewed. The primary outcome was death or postoperative pulmonary venous obstruction (PPVO). Overall, 241 patients with a median age at surgery of 14 days (interquartile range [IQR], 9-21 days) were included. Anomalous return was supracardiac in 38.6%, cardiac in 26.1%, infracardiac in 28.6%, and mixed in 6.6%. Patients receiving DPC had better survival (96.3% vs 84.3%; P= .0028) and lower incidence of PPVO (15.2% vs 28.6%; P= .011) compared with those without DPC. Access to DPC potentially improves outcomes in the neoTAPVC setting; freedom from PPVO were similar using conventional versus sutureless repair. Dedicated preoperative care is associated with better survival and lower restenosis rate following surgical repair of total anomalous pulmonary venous connection (TAPVC) in the neonatal patients. The rationale for the requirement of such specialist care is mainly based on the previous finding that patient-specific preoperative status has a strong association with mortality and morbidity, which can be influenced by preoperative management strategy. However, to what extent dedicated presurgical care (DPC) would influence the outcomes after neonatal TAPVC (neoTAPVC) surgery has not been well investigated. This study first provides an insight into the impact of presurgical care models on outcomes in the neoTAPVC setting. It is noteworthy that the access to DPC conferred an approximately 4-fold decrease in death, particularly resulting in the postoperative 1-year mortality of 3.7% that was remarkably lower than previously published data. Findings from this study suggest that care by a dedicated cardiac team can allow for tiered patient triage and helps to optimize presurgical clinical status and bridge the time to surgery in the selected patients.

• #2

  https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx

  The goal of surgery is to redirect pulmonary vein flow entirely to the left atrium. […] Corrective surgery is necessary for all patients with this condition. […] Continuous monitoring and surveillance of the clinical data are mandatory to direct further treatment following cardiac surgery. […] Management of a neonate or infant with TAPVD involves a major perioperative connect between the referring physician and the pediatric cardiologist, pediatric cardiac surgeon and intensivist. […] The outcome in major centre is entirely dependent upon early detection rapid perioperative stabilization and timely surgical interventions.

• #2 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  This study outlines the complex variance in mixed type TAPVR patients, and to our knowledge only 5 other mixed type-specific retrospective case series exist in the literature, though we acknowledge mixed type data can be found within generalized TAPVR studies. The intent of this data was to add to the literature for purposes of future meta and systematic analyses to improve surgical approach, as well as serve educational purposes regarding a rare and intricate lesion. […] Mixed type TAPVR presents a wide variety of connections, lending to the complexity in planning and execution of surgical correction. Our study contributes further to the available data on this more rare form of TAPVR, which may be important as more cohorts emerge with varying success rates. More widespread studies, including systematic review may be beneficial to further characterize morphology, intraoperative technique, outcome data, and perhaps most importantly, specific indices for predicting higher survival or mortality rates.

• #2 Total Anomalous Pulmonary Venous Return – OpenAnesthesia

  https://www.openanesthesia.org/keywords/total-anomalous-pulmonary-venous-return/

  TAPVR most often occurs without a family history of congenital heart disease and has a low recurrence rate. However, several genetic abnormalities have been isolated in patients with TAPVR. […] TAPVR consists of a spectrum of disease in which the pulmonary veins (PV) fail to form a connection to the left atrium (LA). Pulmonary venous blood returns to the systemic venous circulation via systemic veins or by a direct connection to the right atrium.

• #2 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  Overall TAPVR mortality may be decreasing with time, which may be partially due to earlier recognition and a change in preoperative diagnostic strategies over recent decades. Complex mixed-type TAPVR has now been demonstrated in fetal ultrasound as early as 18 weeks. There has been a move towards more advanced post-natal imaging including the use of angiography. […] While TAPVR literature has continued to compound, there remains relatively limited mixed-type TAPVR-specific case series. The following is a retrospective clinical review. We aim to describe and display the anatomic variance and surgical technique used in the operative repair of one surgical groups experience with 14 mixed-type TAPVR patients between 2004-2018 in an attempt to add to the comprehensive available data with which clinicians can use to optimize decision-making in a rare congenital lesion bearing significant mortality.

• #2 Anomalous Pulmonary Venous Return | UK Healthcare

  https://ukhealthcare.uky.edu/gill-heart-vascular-institute/conditions/adult-congenital-heart-disease/anomalous-pulmonary-venous-return

  Total anomalous pulmonary venous return (TAPVR), also called total anomalous pulmonary venous connection, affects all the pulmonary veins. […] In rare cases, patients with TAPVR or PAPVR may reach adulthood before diagnosis. […] Most children who have successful surgeries to treat TAPVR or PAPVR go on to lead healthy lives as adults. […] Overall, the prognosis for the majority of patients is excellent, as long as they keep up with follow-up care. […] Research is ongoing as to which specific risk factors could ultimately lead to TAPVR or PAPVR.

• #2 Surgical correction of total anomalous pulmonary venous return in an adult patient | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/s13019-022-01990-9

  Total anomalous pulmonary venous return (TAPVR) is rare congenital heart disease. Most TAPVRs require surgical corrections in the neonatal period and survival to adulthood without surgical correction is extremely rare. […] TAPVR is a rare anomaly accounting for only 1.5% to 2.2% of congenital heart disease. TAPVR is a cardiac malformation in which all pulmonary veins on either side are not connected to the left atrium, but directly to the somatic vein or the right atrium. Almost all patients with TAPVR present with symptoms of heart failure in the neonatal period, which require early surgical correction. It is unusual to encounter a adult patient with TAPVR due to low survival rate in untreated cases. […] There are few adult TAPVR patients worldwide. The cases reported previously showed a favorable prognosis in the postoperative phase. However, approximately 10% to 15% of the patients have evidence of late pulmonary vein obstruction, which tends to be recurrent and progressive. […] However, long-term surveillance and monitoring are required.

• #3 About Total Anomalous Pulmonary Venous Return | Congenital Heart Defects (CHDs) | CDC

  https://www.cdc.gov/heart-defects/about/tapvr.html

  Total anomalous pulmonary venous return (pronounced TOHT-l uh-NOM-uh-luh-s PUHL-muh-ner-ee VEE-nuh-s ri-TURN) or T-A-P-V-R is a congenital heart defect. […] About 1 in every 7,809 babies in the United States are born with TAPVR. This means that about 504 babies are born with TAPVR each year. […] TAPVR and PAPVR might be diagnosed during pregnancy, but more often these defects are diagnosed soon after a baby is born. […] Babies with TAPVR will need surgery to repair the defect. […] Surgical repairs for TAPVR are not a cure. People living with TAPVR may have lifelong complications. Routine checkups with a heart doctor are needed to monitor progress, avoid complications, and check for other health conditions that might develop over time.

• #3 Total anomalous pulmonary venous return (TAPVR) – Children’s Health Cardiology

  https://www.childrens.com/specialties-services/conditions/total-anomalous-pulmonary-venous-return

  Surgery is the only way to fix a total anomalous pulmonary venous return, a rare congenital birth defect of the heart. […] Total anomalous pulmonary venous return (TAPVR) makes it hard for the heart to pump oxygen-rich blood to the rest of the body. […] TAPVR develops during the first four to six weeks of pregnancy because the child’s heart doesnt develop normally. […] In some cases, signs of TAPVR show up during prenatal tests, such as a fetal ultrasound. Children can develop symptoms within the first weeks, months or year of their life. […] We dont know what causes TAPVR, but we do know the defect develops during the first four to six weeks of pregnancy. Its not caused by anything a mother or father did or did not do. […] Children with TAPVR need surgery to restore normal blood flow to and through the heart. Your childs surgeon will discuss which surgery is right for your child. […] Yes. The only effective treatment for TAPVR is surgery. The good news is that after your child recovers, he or she can expect to live a healthy, active life.

• #3 Total Anomalous Pulmonary Venous Return – OpenAnesthesia

  https://www.openanesthesia.org/keywords/total-anomalous-pulmonary-venous-return/

  Total anomalous pulmonary venous return (TAPVR) is uncommon with an incidence of 7 per 100,000 live births and accounting for less than 3% of congenital anomalies. It is the fifth most common cause of cyanotic congenital heart disease. […] TAPVR is associated with other cardiac anomalies (e.g., complete atrioventricular canal defect, single ventricle lesions, transposition of the great arteries, and/or PDA) in ~30% of patients. Abnormalities of atrial and visceral situs are also common. This is especially true for heterotaxy with right-sided isomerism. […] Prenatal diagnosis: TAPVR has the lowest prenatal detection rate of any critical cardiac disease. Screening ultrasonography only detects 2-10% of cases. […] All TAPVR is assumed to have some degree of obstruction, with the clinical presentation depending on the degree of pulmonary vein obstruction (PVO) and the presence or absence of a nonrestrictive atrial communication.

• #3 Supracardiac Total Anomalous Pulmonary Venous Return | Pediatric Echocardiography

  https://pedecho.org/library/chd/tapvr

  Total anomalous pulmonary venous connection is when all of the pulmonary veins returning from the lungs have an abnormal connection and drain anomalously to the right side of the heart instead of the left atrium. Supracardiac TAPVC is the most common type of anomalous pulmonary venous connection. In supracardiac TAPVC, the common pulmonary vein drains superiorly via a vertical vein into the left innominate vein, the right superior vena cava, or the right azygos vein by way of an ascending vertical vein. […] TAPVC has been described in 9 in 100,000 of the population. Supracardiac TAPVC is the most common type (47% in the largest published series) and in this group the most common site of connection is to the left innominate vein (36% of all cases). […] The mortality of untreated patients is 75-85% by 1st year of life.

• #3 Neonatal Outcomes in Total Anomalous Pulmonary Venous Return: The Role of Prenatal Diagnosis and Pulmonary Venous Obstruction – PubMed

  https://pubmed.ncbi.nlm.nih.gov/29796693/

  The objective of this study is to evaluate neonatal outcomes of total anomalous pulmonary venous return (TAPVR) and identify fetal echocardiography findings associated with preoperative pulmonary venous obstruction (PPVO). This retrospective study evaluated TAPVR cases from 2005 to 2014 for preoperative and postoperative outcomes based on prenatal diagnosis, PPVO, and heterotaxy syndrome. Of 137 TAPVR cases, 12% were prenatally diagnosed; 60% had PPVO, and 21% had heterotaxy. TAPVR repair was performed in 135 cases and survival to discharge was 82% (112/137). Heterotaxy was the only independent predictor of mortality on multiple regression analysis [OR 5.5 (CI 1.3-16.7), p = 0.02]. TAPVR has severe neonatal morbidity and mortality with low prenatal diagnosis rates in the absence of heterotaxy. […] Patients with obstructed TAPVR had greater preoperative morbidity, but only heterotaxy was independently associated with increased postoperative mortality. Vertical vein velocity helped prenatally identify those at risk of PPVO.

• #3 Total Anomalous Pulmonary Venous Return (TAPVR)

  https://my.clevelandclinic.org/health/diseases/23069-total-anomalous-pulmonary-venous-return

  Total anomalous pulmonary venous return is a rare condition. It affects 1 in about 7,500 newborns in the U.S. each year. […] Total anomalous pulmonary venous return (TAPVR) is an issue where veins from your baby’s lungs connect to the right side of their heart instead of the left. […] TAPVR is a life-threatening heart condition. Its congenital, which means it’s present at birth. […] Without surgery, some forms of total anomalous pulmonary venous return are typically fatal a few weeks after birth. With early diagnosis and surgical treatment, the outlook for babies with TAPVR is very good. The survival rate after surgery is around 97%.

• #3 Total Anomalous Pulmonary Venous Return (TAPVR)

  https://healthlibrary.uwmedicine.org/library/testsprocedures/urology/90,P01820

  Total anomalous pulmonary venous return (TAPVR) is a congenital heart defect. This means that your child is born with it. It happens as the babys heart develops during the first 8 weeks of pregnancy. […] There are different types of TAPVR. The type your child has depends on where the pulmonary veins are connected. […] This condition causes oxygen-rich blood from the lungs to mix with oxygen-poor (blue) blood from the body. This keeps oxygen-rich blood from being carried all over the body. Your child can’t live with TAPVR long-term. They need oxygen-rich blood all over the body. […] Most of the time, the cause of TAPVR isnt known. It may occur with other heart or body system problems. […] All children with a TAPVR will need to have surgery to fix it. […] Your child can’t live with TAPVR long-term because people need oxygen-rich blood all over their body. […] All children with TAPVR will need surgery. This is done to restore normal blood flow through the heart. […] Many children with TAPVR will grow and develop normally after they have surgery. […] Your child will need to see a heart doctor for regular checkups for the rest of their life.

• #3 Surgical correction of total anomalous pulmonary venous return in an adult patient | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/s13019-022-01990-9

  Total anomalous pulmonary venous return (TAPVR) is rare congenital heart disease. Most TAPVRs require surgical corrections in the neonatal period and survival to adulthood without surgical correction is extremely rare. […] TAPVR is a rare anomaly accounting for only 1.5% to 2.2% of congenital heart disease. TAPVR is a cardiac malformation in which all pulmonary veins on either side are not connected to the left atrium, but directly to the somatic vein or the right atrium. Almost all patients with TAPVR present with symptoms of heart failure in the neonatal period, which require early surgical correction. It is unusual to encounter a adult patient with TAPVR due to low survival rate in untreated cases. […] There are few adult TAPVR patients worldwide. The cases reported previously showed a favorable prognosis in the postoperative phase. However, approximately 10% to 15% of the patients have evidence of late pulmonary vein obstruction, which tends to be recurrent and progressive. […] However, long-term surveillance and monitoring are required.

• #3 Total Anomalous Pulmonary Venous Return | Thoracic Key

  https://thoracickey.com/total-anomalous-pulmonary-venous-return/

  In the series by St. Louis etal., 27% of patients with TAPVR had an associated cardiac lesion. […] The population-based study reported by Seale showed that in patients with TAPVR, excluding those with heterotaxy and complex single-ventricle anatomy, 14% had associated cardiac lesions. […] The diagnosis of TAPVR can be difficult on prenatal ultrasonography. […] Echocardiography is an important initial diagnostic tool for patients with suspected TAPVR. […] With the advent of multidetector computed tomography (CT) scanning, detailed images of the pulmonary venous drainage, including inflow and runoff vessels from the confluence, can be achieved. […] Diagnostic cardiac catheterization is reserved for patients in whom echocardiography or CT imaging is not satisfactory or when associated lesions need to be further defined. […] The level of preoperative support required will reflect the pathophysiologic state of the patient and depends on the degree of PVO and pulmonary blood flow.

• #3 Anesthetic Management During Stenting of Obstructed Total Anomalous Pulmonary Return in a Neonate With Single Ventricle Anatomy | Hakim | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/2724/2078

  Total anomalous pulmonary venous return (TAPVR) is a relatively rare congenital cardiac abnormality accounting for 1.5-3% of cases of congenital heart disease (CHD). […] Morbidity and mortality associated with surgical repair of TAPVR increase dramatically when there are other associated cardiac defects, particularly single ventricle anatomy. […] Total anomalous pulmonary venous return (TAPVR) is a relatively rare type of congenital heart disease (CHD) accounting for 1.5-3% of such defects. […] Morbidity and mortality associated with TAPVR repair increase dramatically when another cardiac anomaly is present, particularly when combined with single ventricle anatomy. […] TAPVR remains one of the most challenging CHDs in newborns and young infants. […] The surgical results of TAPVR repair have improved significantly in the modern era as a result of earlier diagnosis and treatment, improvements in surgical technique, cardiac anesthesia, neonatal myocardial preservation, and postoperative care.

• #3 Surgical repair of neonatal total anomalous pulmonary venous connection: A single institutional experience with 241 cases – JTCVS Open

  https://www.jtcvsopen.org/article/S2666-2736(23)00210-3/fulltext

  Challenges persist in surgery for neonatal total anomalous pulmonary venous connection (neoTAPVC), with the high mortality risk not mitigated over time. A prospectively collected single-center database containing all neonates with TAPVC undergoing biventricular repair in 2012 to 2020 was retrospectively reviewed. The primary outcome was death or postoperative pulmonary venous obstruction (PPVO). Overall, 241 patients with a median age at surgery of 14 days (interquartile range [IQR], 9-21 days) were included. Anomalous return was supracardiac in 38.6%, cardiac in 26.1%, infracardiac in 28.6%, and mixed in 6.6%. Patients receiving DPC had better survival (96.3% vs 84.3%; P= .0028) and lower incidence of PPVO (15.2% vs 28.6%; P= .011) compared with those without DPC. Access to DPC potentially improves outcomes in the neoTAPVC setting; freedom from PPVO were similar using conventional versus sutureless repair. Dedicated preoperative care is associated with better survival and lower restenosis rate following surgical repair of total anomalous pulmonary venous connection (TAPVC) in the neonatal patients. The rationale for the requirement of such specialist care is mainly based on the previous finding that patient-specific preoperative status has a strong association with mortality and morbidity, which can be influenced by preoperative management strategy. However, to what extent dedicated presurgical care (DPC) would influence the outcomes after neonatal TAPVC (neoTAPVC) surgery has not been well investigated. This study first provides an insight into the impact of presurgical care models on outcomes in the neoTAPVC setting. It is noteworthy that the access to DPC conferred an approximately 4-fold decrease in death, particularly resulting in the postoperative 1-year mortality of 3.7% that was remarkably lower than previously published data. Findings from this study suggest that care by a dedicated cardiac team can allow for tiered patient triage and helps to optimize presurgical clinical status and bridge the time to surgery in the selected patients.

• #3 Surgery-related complications and their management in total anomalous pulmonary venous return during intensive care unit stay – Turkish Journal of Thoracic and Cardiovascular Surgery

  https://tgkdc.dergisi.org/text.php3?id=2188

  Pulmonary venous obstruction is crucial for both pre- and postoperative assessment in the ICU. Several studies have shown that patients with preoperative PVO had lower intubation rates, a higher number of postoperative pulmonary hypertensive crises and episodes, and a greater amount of positive inotropic support during their ICU stays. […] Following TAPVR corrective surgery, the mortality rate in recent years has decreased from 80% to below 10%, with early and accurate diagnoses, new developments in surgical techniques, and effective and improved ICU support being responsible for this dramatic improvement. […] Our findings revealed that postoperative complications such as low cardiac output, pulmonary hypertensive crises, and arrhythmia can be prevented by advanced ICU monitorization, especially for patients without the need for mechanical ventilation, those without metabolic acidosis, those with TAPVR who also have left ventricular hypoplasia, and those who have PVO. Furthermore, early and effective treatment with NO drugs or ECMO support to counteract these complications could lead to an improvement in the morbidity and mortality rates of these patients.

• #3 Whole-exome sequencing identifies SGCD and ACVRL1 mutations associated with total anomalous pulmonary venous return (TAPVR) in Chinese population | Oncotarget

  https://www.oncotarget.com/article/15434/text/

  As a rare type of Congenital Heart Defects (CHD), the genetic mechanism of Total Anomalous Pulmonary Venous Return (TAPVR) remains unknown, although previous studies have revealed potential disease-driving regions/genes. […] TAPVR characterized by cardiac function deterioration including cyanosis, pulmonary hypertension, dyspnea, pulmonary edema, congestive heart failure affects 1 in 15,000 live births and 48.8% of them will die without surgery before the age of 1. […] We provided evidence for ACVRL1 as a known causative gene and for SGCD as candidate genes for TAPVR. […] A rare missense mutation in ACVRL1 (c.C652T, p.R218W) was identified as a causal mutation for TAPVR. […] A recurrent mutation in SGCD (c.C717G, p.D239E) was detected, which coded for the dystrophin-glycoprotein complex (DGC). […] In conclusion, our study was the first attempt to dissect the etiology of TAPVR using whole-exome sequencing strategy in Chinese population.

• #3 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  This study outlines the complex variance in mixed type TAPVR patients, and to our knowledge only 5 other mixed type-specific retrospective case series exist in the literature, though we acknowledge mixed type data can be found within generalized TAPVR studies. The intent of this data was to add to the literature for purposes of future meta and systematic analyses to improve surgical approach, as well as serve educational purposes regarding a rare and intricate lesion. […] Mixed type TAPVR presents a wide variety of connections, lending to the complexity in planning and execution of surgical correction. Our study contributes further to the available data on this more rare form of TAPVR, which may be important as more cohorts emerge with varying success rates. More widespread studies, including systematic review may be beneficial to further characterize morphology, intraoperative technique, outcome data, and perhaps most importantly, specific indices for predicting higher survival or mortality rates.

• #4 Total anomalous pulmonary venous connection – UpToDate

  https://www.uptodate.com/contents/total-anomalous-pulmonary-venous-connection

  Total anomalous pulmonary venous connection (TAPVC), also referred to as total anomalous pulmonary venous return (TAPVR), is a cyanotic congenital defect in which all four pulmonary veins fail to make their normal connection to the left atrium. This results in drainage of all pulmonary venous return into the systemic venous circulation. […] The reported prevalence of TAPVC ranges from 0.6 to 1.2 per 10,000 live births. TAPVC accounts for approximately ranges between 0.7 and 1.5 percent of all congenital heart defects in newborns. […] TAPVC arises from the failure of the left atrium to link with the pulmonary venous plexus, which results in the retention of connections through the primitive cardinal and umbilicovitelline drainage pathways.

• #4 Pediatric Total Anomalous Pulmonary Venous Return (TAPVR) – Conditions and Treatments | Children’s National Hospital

  https://www.childrensnational.org/get-care/health-library/total-anomalous-pulmonary-venous-return-tapvr

  Total anomalous pulmonary venous return (TAPVR) is a congenital (present at birth) heart defect. Total anomalous pulmonary venous return occurs equally in boys and in girls. Babies with this heart defect cannot supply oxygen-rich (red) blood to the body after birth. Without an additional heart defect that allows mixing of oxygen-poor (blue) and oxygen-rich (red) blood, such as an atrial septal defect, infants with TAPVR will have a mixture of oxygen-rich (red) and oxygen-poor (blue) blood circulating through the right heart and back to the lungs a situation that is fatal. Many infants who have had TAPVR surgical repair will grow and develop normally. Regular follow-up care at a center offering pediatric or adult congenital cardiac care should continue throughout the individuals lifespan. […] The heart is forming during the first eight weeks of prenatal development. The problem occurs during this time, allowing the pulmonary veins to be attached incorrectly.

• #4 Total Anomalous Pulmonary Venous Return (TAPVR) | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/total-anomalous-pulmonary-venous-return

  Total anomalous pulmonary venous return (TAPVR), also known as total anomalous pulmonary venous connection (TAPVC), is a rare heart defect in which the blood vessels that drain the lungs (pulmonary veins) are not connected normally to the heart. Instead, the pulmonary veins are redirected abnormally to other chambers of the heart. About 1 in every 20,000 babies is born with TAPVR. […] TAPVR can occur with obstruction, meaning that some of the draining blood vessels are obstructed. This can cause high blood pressure in the lungs (pulmonary hypertension) and can be a surgical emergency. […] Children with TAPVR will need surgery in infancy to repair the problem. […] In some cases, total anomalous pulmonary venous return is found before birth on a fetal echocardiogram. […] Most babies with total anomalous pulmonary venous return (TAPVR) have symptoms on the day they’re born. Severe TAPVR can usually be diagnosed promptly based on symptoms and tests, including chest X-ray and cardiac ultrasound.

• #4 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  Total anomalous pulmonary venous return (TAPVR), also referred in literature as total anomalous pulmonary venous connection (TAPVC), provides a wide spectrum of complex anatomic and therapeutic challenges amongst congenital heart disease. An estimated 400 infants (1/10.000 births) are born annually in the United States with TAPVR, an incidence among congenital heart disease of 0.7-1.5%. While there may be rare survival to adulthood if un-repaired, typical median survival if unrepaired is 2 months with 50% mortality in first 3 months of life, thereby demonstrating the urgency of recognition and intervention. […] TAPVR is classified according to the Darling system developed in 1957 into cardiac, supracardiac, infracardiac, or mixed-type depending on the pattern of pulmonary venous (PV) drainage to the systemic venous circuitry. Mixed-type TAPVR in itself has a higher mortality, with univariate analysis of mixed type versus cardiac type (lowest mortality of TAPVR variants) demonstrating statistically significant hazard ratios of 2.88 in previous study, and mortality as high as 42-50%. Other determinants of increased mortality or transplant in this study include complex TAPVC and postoperative length of stay, while older age, weight greater than 2.5 kg, non-emergent operation, lack of PV obstruction, cardiac and supracardiac types were associated with increased survival to discharge.

• #4 Surgery-related complications and their management in total anomalous pulmonary venous return during intensive care unit stay – Turkish Journal of Thoracic and Cardiovascular Surgery

  https://tgkdc.dergisi.org/text.php3?id=2188

  Of patients with TAPVR, 58% were supracardiac, 19% were infracardiac, 13% were cardiac, and 10% were mixed type. Pulmonary venous obstruction was present in 10 patients (32%). While pulmonary hypertension crisis developed in eight patients (25%), low cardiac output was detected in six patients (19%). Rhythm problems were observed in 11 patients (35%). Pulmonary venous obstruction was more common in patients with infracardiac drainage TAPVR, low weight and small age. […] The mortality rate of TAPVR may decrease significantly with early diagnosis and effective, advanced, and suitable intensive care unit follow-up. […] Total anomalous pulmonary venous return (TAPVR) is a heart disease in which the pulmonary vein flows enter the systemic veins, right atrium, or coronary sinus instead of the left atrium, and it accounts for less than 1% of all congenital heart pathologies. The signs and symptoms of TAPVR vary according to the sufficiency of the interatrial connection and the presence of pulmonary venous obstruction (PVO). The severity of these symptoms range from mild (e.g., a murmur and tachypnea) to severe (e.g., cyanosis, metabolic acidosis, cardiac insufficiency, or low cardiac output). Surgery is the definitive treatment, and thanks to early, accurate diagnostic methods, improved surgical techniques, myocardial protection during surgery, and improved postoperative care in the intensive care unit (ICU), the mortality rate has now been reduced to less than 10%.

• #4 TAPVR | Types, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/t/tapvr

  Because of the abnormal pulmonary vein connection red (oxygenated) blood returning from the lungs mixes with the blue (lower oxygenated) blood returning from the body. […] The outcome of surgical repair for total anomalous pulmonary venous return is excellent. The surgical mortality is less than 5% when repair is performed electively. This is in healthy children without obstructed pulmonary veins. […] Patients who have surgery to repair total anomalous pulmonary venous return early in their lives often do well. Most do not need expert follow-up care as adults.

• #4 Total Anomalous Pulmonary Venous Return (TAPVR) | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/total-anomalous-pulmonary-venous-return-tapvr

  Total anomalous pulmonary venous return is a congenital (meaning it’s present from birth) heart disease that affects the pulmonary veins. […] Total anomalous pulmonary venous return (TAPVR) may be diagnosed before birth, with fetal echocardiogram (ultrasound). […] Total anomalous pulmonary venous return requires open heart surgery in all cases. […] Because of enormous strides in medicine and technology, today most children born with total anomalous pulmonary venous return go on to lead productive lives as adults. […] Children who were born with TAPVR and had it repaired must continue to see a pediatric cardiologist regularly. […] It is important that children who were born with total anomalous pulmonary venous return continue to see a cardiologist.

• #4 Content – Health Encyclopedia – University of Rochester Medical Center

  https://www.urmc.rochester.edu/encyclopedia/content?contenttypeid=90&contentid=p01820

  Total anomalous pulmonary venous return (TAPVR) is a congenital heart defect. This condition causes oxygen-rich blood from the lungs to mix with oxygen-poor (blue) blood from the body. Your child can’t live with TAPVR long-term. All children with a TAPVR will need to have surgery to fix it. Many children with TAPVR will grow and develop normally after they have surgery. Your child will need to see a heart doctor for regular checkups for the rest of their life. […] The complications of TAPVR include: enlarged heart, lung problems, including respiratory failure and high blood pressure in the lungs, abnormal heart rhythms, shock, heart failure, slowed growth and development, enlarged liver, need for another surgery, infection, and death. […] In TAPVR, the 4 blood vessels that carry oxygen-rich blood to the heart from the lungs aren’t connected correctly. This condition prevents oxygen-rich blood from being pumped to the body. Your child can’t live with TAPVR long-term because people need oxygen-rich blood all over their body.

• #4 Total Anomalous Pulmonary Venous Return (TAPVR)

  https://healthlibrary.ecuhealth.org/Library/DiseasesConditions/Adult/NervousSystem/90,P01820

  The complications of TAPVR include: […] Your child will likely stay in the hospital until TAPVR is repaired. […] Many children with TAPVR will grow and develop normally after they have surgery. […] In TAPVR, the 4 blood vessels that carry oxygen-rich blood to the heart from the lungs arent connected correctly. […] This condition prevents oxygen-rich blood from being pumped to the body. […] Your child can’t live with TAPVR long-term because people need oxygen-rich blood all over their body. […] All children with TAPVR will need surgery. This is done to restore normal blood flow through the heart. […] Your child will need to see a heart doctor for regular checkups for the rest of their life.

• #4 Total Anomalous Pulmonary Venous Return | Thoracic Key

  https://thoracickey.com/total-anomalous-pulmonary-venous-return/

  In the series by St. Louis etal., 27% of patients with TAPVR had an associated cardiac lesion. […] The population-based study reported by Seale showed that in patients with TAPVR, excluding those with heterotaxy and complex single-ventricle anatomy, 14% had associated cardiac lesions. […] The diagnosis of TAPVR can be difficult on prenatal ultrasonography. […] Echocardiography is an important initial diagnostic tool for patients with suspected TAPVR. […] With the advent of multidetector computed tomography (CT) scanning, detailed images of the pulmonary venous drainage, including inflow and runoff vessels from the confluence, can be achieved. […] Diagnostic cardiac catheterization is reserved for patients in whom echocardiography or CT imaging is not satisfactory or when associated lesions need to be further defined. […] The level of preoperative support required will reflect the pathophysiologic state of the patient and depends on the degree of PVO and pulmonary blood flow.

• #4 Surgical repair of neonatal total anomalous pulmonary venous connection: A single institutional experience with 241 cases – JTCVS Open

  https://www.jtcvsopen.org/article/S2666-2736(23)00210-3/fulltext

  Challenges persist in surgery for neonatal total anomalous pulmonary venous connection (neoTAPVC), with the high mortality risk not mitigated over time. A prospectively collected single-center database containing all neonates with TAPVC undergoing biventricular repair in 2012 to 2020 was retrospectively reviewed. The primary outcome was death or postoperative pulmonary venous obstruction (PPVO). Overall, 241 patients with a median age at surgery of 14 days (interquartile range [IQR], 9-21 days) were included. Anomalous return was supracardiac in 38.6%, cardiac in 26.1%, infracardiac in 28.6%, and mixed in 6.6%. Patients receiving DPC had better survival (96.3% vs 84.3%; P= .0028) and lower incidence of PPVO (15.2% vs 28.6%; P= .011) compared with those without DPC. Access to DPC potentially improves outcomes in the neoTAPVC setting; freedom from PPVO were similar using conventional versus sutureless repair. Dedicated preoperative care is associated with better survival and lower restenosis rate following surgical repair of total anomalous pulmonary venous connection (TAPVC) in the neonatal patients. The rationale for the requirement of such specialist care is mainly based on the previous finding that patient-specific preoperative status has a strong association with mortality and morbidity, which can be influenced by preoperative management strategy. However, to what extent dedicated presurgical care (DPC) would influence the outcomes after neonatal TAPVC (neoTAPVC) surgery has not been well investigated. This study first provides an insight into the impact of presurgical care models on outcomes in the neoTAPVC setting. It is noteworthy that the access to DPC conferred an approximately 4-fold decrease in death, particularly resulting in the postoperative 1-year mortality of 3.7% that was remarkably lower than previously published data. Findings from this study suggest that care by a dedicated cardiac team can allow for tiered patient triage and helps to optimize presurgical clinical status and bridge the time to surgery in the selected patients.

• #4 Surgery-related complications and their management in total anomalous pulmonary venous return during intensive care unit stay – Turkish Journal of Thoracic and Cardiovascular Surgery

  https://tgkdc.dergisi.org/text.php3?id=2188

  Pulmonary venous obstruction is crucial for both pre- and postoperative assessment in the ICU. Several studies have shown that patients with preoperative PVO had lower intubation rates, a higher number of postoperative pulmonary hypertensive crises and episodes, and a greater amount of positive inotropic support during their ICU stays. […] Following TAPVR corrective surgery, the mortality rate in recent years has decreased from 80% to below 10%, with early and accurate diagnoses, new developments in surgical techniques, and effective and improved ICU support being responsible for this dramatic improvement. […] Our findings revealed that postoperative complications such as low cardiac output, pulmonary hypertensive crises, and arrhythmia can be prevented by advanced ICU monitorization, especially for patients without the need for mechanical ventilation, those without metabolic acidosis, those with TAPVR who also have left ventricular hypoplasia, and those who have PVO. Furthermore, early and effective treatment with NO drugs or ECMO support to counteract these complications could lead to an improvement in the morbidity and mortality rates of these patients.

• #4 Whole-exome sequencing identifies SGCD and ACVRL1 mutations associated with total anomalous pulmonary venous return (TAPVR) in Chinese population | Oncotarget

  https://www.oncotarget.com/article/15434/text/

  As a rare type of Congenital Heart Defects (CHD), the genetic mechanism of Total Anomalous Pulmonary Venous Return (TAPVR) remains unknown, although previous studies have revealed potential disease-driving regions/genes. […] TAPVR characterized by cardiac function deterioration including cyanosis, pulmonary hypertension, dyspnea, pulmonary edema, congestive heart failure affects 1 in 15,000 live births and 48.8% of them will die without surgery before the age of 1. […] We provided evidence for ACVRL1 as a known causative gene and for SGCD as candidate genes for TAPVR. […] A rare missense mutation in ACVRL1 (c.C652T, p.R218W) was identified as a causal mutation for TAPVR. […] A recurrent mutation in SGCD (c.C717G, p.D239E) was detected, which coded for the dystrophin-glycoprotein complex (DGC). […] In conclusion, our study was the first attempt to dissect the etiology of TAPVR using whole-exome sequencing strategy in Chinese population.

• #4 Anomalous Pulmonary Venous Return

  https://fpnotebook.com/CV/Peds/AnmlsPlmnryVnsRtrn.htm

  Accounts for 0.7 to 1.5% of Congenital Heart Disease cases (5th most common cyanotic cause) […] TAPVR is frequently misdiagnosed initially.

• #4 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  This study outlines the complex variance in mixed type TAPVR patients, and to our knowledge only 5 other mixed type-specific retrospective case series exist in the literature, though we acknowledge mixed type data can be found within generalized TAPVR studies. The intent of this data was to add to the literature for purposes of future meta and systematic analyses to improve surgical approach, as well as serve educational purposes regarding a rare and intricate lesion. […] Mixed type TAPVR presents a wide variety of connections, lending to the complexity in planning and execution of surgical correction. Our study contributes further to the available data on this more rare form of TAPVR, which may be important as more cohorts emerge with varying success rates. More widespread studies, including systematic review may be beneficial to further characterize morphology, intraoperative technique, outcome data, and perhaps most importantly, specific indices for predicting higher survival or mortality rates.

• #5 Total Anomalous Pulmonary Venous Return (TAPVR) | Concise Medical Knowledge

  https://www.lecturio.com/concepts/total-anomalous-pulmonary-venous-return-tapvr/

  Total anomalous pulmonary venous return (TAPVR) is a cyanotic congenital heart defect wherein the pulmonary veins drain into anatomical sites other than the left atrium. Patients are usually cyanotic from birth and present with respiratory and heart failure right after birth. […] Total anomalous pulmonary venous return (TAPVR) is a cyanotic congenital heart defect that results in the drainage of the 4 pulmonary veins into the systemic venous circulation, rather than into the left atrium, resulting in mixing of oxygenated and deoxygenated blood. […] Rare condition: 0.7%1.5% of all CHD. Prevalence: 1 per 7,800 births in the United States. […] All patients require surgery for survival and medical management is used to bridge the gap to surgery.

• #5 Total anomalous pulmonary venous connection – UpToDate

  https://www.uptodate.com/contents/total-anomalous-pulmonary-venous-connection

  Total anomalous pulmonary venous connection (TAPVC), also referred to as total anomalous pulmonary venous return (TAPVR), is a cyanotic congenital defect in which all four pulmonary veins fail to make their normal connection to the left atrium. This results in drainage of all pulmonary venous return into the systemic venous circulation. […] The reported prevalence of TAPVC ranges from 0.6 to 1.2 per 10,000 live births. TAPVC accounts for approximately ranges between 0.7 and 1.5 percent of all congenital heart defects in newborns. […] TAPVC arises from the failure of the left atrium to link with the pulmonary venous plexus, which results in the retention of connections through the primitive cardinal and umbilicovitelline drainage pathways.

• #5 Total Anomalous Pulmonary Venous Connection (TAPVC) Diagnosis & Treatments | Mount Sinai – New York

  https://www.mountsinai.org/locations/childrens-heart/conditions/pulmonary-venous-connection

  Total anomalous pulmonary venous connection (TAPVC), also referred to as total anomalous pulmonary venous return (TAPVR), disrupts the normal flow of blood between your baby’s lungs and heart. […] The diagnosis of this condition most often occurs after birth, but occasionally is made during pregnancy. […] Our doctors and staff specialize in providing outstanding personalized care for your child with congenital heart disease, such as total anomalous pulmonary venous connection.

• #5 Partial and Total Anomalous Pulmonary Venous Connection – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560707/

  TAPVC and PAPVC are uncommon congenital anomalies accounting for 1% of all congenital heart diseases. […] Total anomalous pulmonary venous connections (TAPVC) is a cyanotic congenital heart disease where all of the pulmonary veins drain directly or indirectly into the right atrium. […] TAPVC implies that all the pulmonary veins connect to the systemic veins or right atrium, either by a common vertical vein or individually. Anatomically TAPVC can be divided into four subtypes based on the level of the anomalous connection as supracardiac, cardiac, infracardiac, and mixed. […] The supracardiac variety is the most common of the TAPVC. […] In untreated patients, TAPVC is almost always fatal within the first few weeks of life. The long term survival in patients has increased with the improvement in medical management and surgical techniques.

• #5 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  Total anomalous pulmonary venous return (TAPVR), also referred in literature as total anomalous pulmonary venous connection (TAPVC), provides a wide spectrum of complex anatomic and therapeutic challenges amongst congenital heart disease. An estimated 400 infants (1/10.000 births) are born annually in the United States with TAPVR, an incidence among congenital heart disease of 0.7-1.5%. While there may be rare survival to adulthood if un-repaired, typical median survival if unrepaired is 2 months with 50% mortality in first 3 months of life, thereby demonstrating the urgency of recognition and intervention. […] TAPVR is classified according to the Darling system developed in 1957 into cardiac, supracardiac, infracardiac, or mixed-type depending on the pattern of pulmonary venous (PV) drainage to the systemic venous circuitry. Mixed-type TAPVR in itself has a higher mortality, with univariate analysis of mixed type versus cardiac type (lowest mortality of TAPVR variants) demonstrating statistically significant hazard ratios of 2.88 in previous study, and mortality as high as 42-50%. Other determinants of increased mortality or transplant in this study include complex TAPVC and postoperative length of stay, while older age, weight greater than 2.5 kg, non-emergent operation, lack of PV obstruction, cardiac and supracardiac types were associated with increased survival to discharge.

• #5 Partial and Total Anomalous Pulmonary Venous Connections and Associated Defects | SpringerLink

  https://link.springer.com/10.1007/978-1-4471-4619-3_43?fromPaywallRec=true

  Anomalous pulmonary venous return is a relatively rare congenital cardiac malformation in which the pulmonary veins do not return to the left atrium. If all four pulmonary veins drain anomalously, the designation of total anomalous pulmonary venous return (TAPVR) is applied; if only one to three pulmonary veins drain anomalously, the designation of partial anomalous pulmonary venous return (PAPVR) is applied. TAPVR is classified into supracardiac, infracardiac, cardiac, and mixed types according to the location of the anomalous drainage. The prognosis of patients with TAPVR depends on the degree of pulmonary venous obstruction and associated defects. TAPVR generally requires neonatal surgical correction. Early and late mortality for simple TAPVR is 10 % and 4 %, respectively. Patients with TAPVR and pulmonary venous obstruction have a higher early and late mortality (17 % and 11 %, respectively) than unobstructed patients (4 % and 6 %, respectively). TAPVR associated with other cardiac anomalies (other than an ASD or PDA) have worse early and late mortality (14 % and 18 %, respectively). The prognosis for all anomalous venous return patients has improved since the first surgical corrections were described. The management of pulmonary vein obstruction continues to be the largest obstacle to successful management of anomalous pulmonary venous return.

• #5 About Total Anomalous Pulmonary Venous Return | Congenital Heart Defects (CHDs) | CDC

  https://www.cdc.gov/heart-defects/about/tapvr.html

  Total anomalous pulmonary venous return (pronounced TOHT-l uh-NOM-uh-luh-s PUHL-muh-ner-ee VEE-nuh-s ri-TURN) or T-A-P-V-R is a congenital heart defect. […] About 1 in every 7,809 babies in the United States are born with TAPVR. This means that about 504 babies are born with TAPVR each year. […] TAPVR and PAPVR might be diagnosed during pregnancy, but more often these defects are diagnosed soon after a baby is born. […] Babies with TAPVR will need surgery to repair the defect. […] Surgical repairs for TAPVR are not a cure. People living with TAPVR may have lifelong complications. Routine checkups with a heart doctor are needed to monitor progress, avoid complications, and check for other health conditions that might develop over time.

• #5 Content – Health Encyclopedia – University of Rochester Medical Center

  https://www.urmc.rochester.edu/encyclopedia/content?contenttypeid=90&contentid=P01820

  Total anomalous pulmonary venous return (TAPVR) is a congenital heart defect. This condition causes oxygen-rich blood from the lungs to mix with oxygen-poor (blue) blood from the body. Your child can’t live with TAPVR long-term. All children with a TAPVR will need to have surgery to fix it. Many children with TAPVR will grow and develop normally after they have surgery. Your child will need to see a heart doctor for regular checkups for the rest of their life. […] The complications of TAPVR include: enlarged heart, lung problems, including respiratory failure and high blood pressure in the lungs, abnormal heart rhythms, shock, heart failure, slowed growth and development, enlarged liver, need for another surgery, infection, and death.

• #5 Total Anomalous Pulmonary Venous Return (TAPVR) | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/total-anomalous-pulmonary-venous-return-tapvr

  Total anomalous pulmonary venous return is a congenital (meaning it’s present from birth) heart disease that affects the pulmonary veins. […] Total anomalous pulmonary venous return (TAPVR) may be diagnosed before birth, with fetal echocardiogram (ultrasound). […] Total anomalous pulmonary venous return requires open heart surgery in all cases. […] Because of enormous strides in medicine and technology, today most children born with total anomalous pulmonary venous return go on to lead productive lives as adults. […] Children who were born with TAPVR and had it repaired must continue to see a pediatric cardiologist regularly. […] It is important that children who were born with total anomalous pulmonary venous return continue to see a cardiologist.

• #5 Total Anomalous Pulmonary Venous Return | Thoracic Key

  https://thoracickey.com/total-anomalous-pulmonary-venous-return/

  In the series by St. Louis etal., 27% of patients with TAPVR had an associated cardiac lesion. […] The population-based study reported by Seale showed that in patients with TAPVR, excluding those with heterotaxy and complex single-ventricle anatomy, 14% had associated cardiac lesions. […] The diagnosis of TAPVR can be difficult on prenatal ultrasonography. […] Echocardiography is an important initial diagnostic tool for patients with suspected TAPVR. […] With the advent of multidetector computed tomography (CT) scanning, detailed images of the pulmonary venous drainage, including inflow and runoff vessels from the confluence, can be achieved. […] Diagnostic cardiac catheterization is reserved for patients in whom echocardiography or CT imaging is not satisfactory or when associated lesions need to be further defined. […] The level of preoperative support required will reflect the pathophysiologic state of the patient and depends on the degree of PVO and pulmonary blood flow.

• #5

  https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx

  The goal of surgery is to redirect pulmonary vein flow entirely to the left atrium. […] Corrective surgery is necessary for all patients with this condition. […] Continuous monitoring and surveillance of the clinical data are mandatory to direct further treatment following cardiac surgery. […] Management of a neonate or infant with TAPVD involves a major perioperative connect between the referring physician and the pediatric cardiologist, pediatric cardiac surgeon and intensivist. […] The outcome in major centre is entirely dependent upon early detection rapid perioperative stabilization and timely surgical interventions.

• #5 Surgical repair of neonatal total anomalous pulmonary venous connection: A single institutional experience with 241 cases – JTCVS Open

  https://www.jtcvsopen.org/article/S2666-2736(23)00210-3/fulltext

  Challenges persist in surgery for neonatal total anomalous pulmonary venous connection (neoTAPVC), with the high mortality risk not mitigated over time. A prospectively collected single-center database containing all neonates with TAPVC undergoing biventricular repair in 2012 to 2020 was retrospectively reviewed. The primary outcome was death or postoperative pulmonary venous obstruction (PPVO). Overall, 241 patients with a median age at surgery of 14 days (interquartile range [IQR], 9-21 days) were included. Anomalous return was supracardiac in 38.6%, cardiac in 26.1%, infracardiac in 28.6%, and mixed in 6.6%. Patients receiving DPC had better survival (96.3% vs 84.3%; P= .0028) and lower incidence of PPVO (15.2% vs 28.6%; P= .011) compared with those without DPC. Access to DPC potentially improves outcomes in the neoTAPVC setting; freedom from PPVO were similar using conventional versus sutureless repair. Dedicated preoperative care is associated with better survival and lower restenosis rate following surgical repair of total anomalous pulmonary venous connection (TAPVC) in the neonatal patients. The rationale for the requirement of such specialist care is mainly based on the previous finding that patient-specific preoperative status has a strong association with mortality and morbidity, which can be influenced by preoperative management strategy. However, to what extent dedicated presurgical care (DPC) would influence the outcomes after neonatal TAPVC (neoTAPVC) surgery has not been well investigated. This study first provides an insight into the impact of presurgical care models on outcomes in the neoTAPVC setting. It is noteworthy that the access to DPC conferred an approximately 4-fold decrease in death, particularly resulting in the postoperative 1-year mortality of 3.7% that was remarkably lower than previously published data. Findings from this study suggest that care by a dedicated cardiac team can allow for tiered patient triage and helps to optimize presurgical clinical status and bridge the time to surgery in the selected patients.

• #5 Total Anomalous Pulmonary Venous Return – OpenAnesthesia

  https://www.openanesthesia.org/keywords/total-anomalous-pulmonary-venous-return/

  TAPVR most often occurs without a family history of congenital heart disease and has a low recurrence rate. However, several genetic abnormalities have been isolated in patients with TAPVR. […] TAPVR consists of a spectrum of disease in which the pulmonary veins (PV) fail to form a connection to the left atrium (LA). Pulmonary venous blood returns to the systemic venous circulation via systemic veins or by a direct connection to the right atrium.

• #5 Total Anomalous Pulmonary Venous Return – OpenAnesthesia

  https://www.openanesthesia.org/keywords/total-anomalous-pulmonary-venous-return/

  Total anomalous pulmonary venous return (TAPVR) is uncommon with an incidence of 7 per 100,000 live births and accounting for less than 3% of congenital anomalies. It is the fifth most common cause of cyanotic congenital heart disease. […] TAPVR is associated with other cardiac anomalies (e.g., complete atrioventricular canal defect, single ventricle lesions, transposition of the great arteries, and/or PDA) in ~30% of patients. Abnormalities of atrial and visceral situs are also common. This is especially true for heterotaxy with right-sided isomerism. […] Prenatal diagnosis: TAPVR has the lowest prenatal detection rate of any critical cardiac disease. Screening ultrasonography only detects 2-10% of cases. […] All TAPVR is assumed to have some degree of obstruction, with the clinical presentation depending on the degree of pulmonary vein obstruction (PVO) and the presence or absence of a nonrestrictive atrial communication.

• #5 Anomalous Pulmonary Venous Return | UK Healthcare

  https://ukhealthcare.uky.edu/gill-heart-vascular-institute/conditions/adult-congenital-heart-disease/anomalous-pulmonary-venous-return

  Total anomalous pulmonary venous return (TAPVR), also called total anomalous pulmonary venous connection, affects all the pulmonary veins. […] In rare cases, patients with TAPVR or PAPVR may reach adulthood before diagnosis. […] Most children who have successful surgeries to treat TAPVR or PAPVR go on to lead healthy lives as adults. […] Overall, the prognosis for the majority of patients is excellent, as long as they keep up with follow-up care. […] Research is ongoing as to which specific risk factors could ultimately lead to TAPVR or PAPVR.

• #5 Surgical correction of total anomalous pulmonary venous return in an adult patient | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/s13019-022-01990-9

  Total anomalous pulmonary venous return (TAPVR) is rare congenital heart disease. Most TAPVRs require surgical corrections in the neonatal period and survival to adulthood without surgical correction is extremely rare. […] TAPVR is a rare anomaly accounting for only 1.5% to 2.2% of congenital heart disease. TAPVR is a cardiac malformation in which all pulmonary veins on either side are not connected to the left atrium, but directly to the somatic vein or the right atrium. Almost all patients with TAPVR present with symptoms of heart failure in the neonatal period, which require early surgical correction. It is unusual to encounter a adult patient with TAPVR due to low survival rate in untreated cases. […] There are few adult TAPVR patients worldwide. The cases reported previously showed a favorable prognosis in the postoperative phase. However, approximately 10% to 15% of the patients have evidence of late pulmonary vein obstruction, which tends to be recurrent and progressive. […] However, long-term surveillance and monitoring are required.

• #6 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  Total anomalous pulmonary venous return (TAPVR), also referred in literature as total anomalous pulmonary venous connection (TAPVC), provides a wide spectrum of complex anatomic and therapeutic challenges amongst congenital heart disease. An estimated 400 infants (1/10.000 births) are born annually in the United States with TAPVR, an incidence among congenital heart disease of 0.7-1.5%. While there may be rare survival to adulthood if un-repaired, typical median survival if unrepaired is 2 months with 50% mortality in first 3 months of life, thereby demonstrating the urgency of recognition and intervention. […] TAPVR is classified according to the Darling system developed in 1957 into cardiac, supracardiac, infracardiac, or mixed-type depending on the pattern of pulmonary venous (PV) drainage to the systemic venous circuitry. Mixed-type TAPVR in itself has a higher mortality, with univariate analysis of mixed type versus cardiac type (lowest mortality of TAPVR variants) demonstrating statistically significant hazard ratios of 2.88 in previous study, and mortality as high as 42-50%. Other determinants of increased mortality or transplant in this study include complex TAPVC and postoperative length of stay, while older age, weight greater than 2.5 kg, non-emergent operation, lack of PV obstruction, cardiac and supracardiac types were associated with increased survival to discharge.

• #6 Total Anomalous Pulmonary Venous Return (TAPVR)

  https://healthlibrary.ecuhealth.org/Library/DiseasesConditions/Adult/NervousSystem/90,P01820

  Total anomalous pulmonary venous return (TAPVR) is a congenital heart defect. This means that your child is born with it. It happens as the babys heart develops during the first 8 weeks of pregnancy. […] Most of the time, the cause of TAPVR isnt known. It may occur with other heart or body system problems. […] The symptoms of TAPVR may seem like symptoms of other health conditions. Your childs healthcare provider must diagnose them with this condition. […] A healthcare provider may first spot TAPVR in a baby during an ultrasound in pregnancy. After birth, if your child has signs of TAPVR, a pediatric cardiologist or neonatologist will check them. […] Treatment will depend on your childs symptoms, age, and overall health. It will also depend on how severe the condition is. […] All children with a TAPVR will need to have surgery to fix it.

• #6 Total Anomalous Pulmonary Venous Returns | TAPVR | Children’s Wisconsin

  https://childrenswi.org/medical-care/herma-heart/conditions/total-anomalous-pulmonary-venous-return

  Total anomalous pulmonary venous return (TAPVR) is a congenital (present at birth) heart defect. […] TAPVR occurs in one out of every 15,000 live births, and is seen in boys just as often as in girls. […] Babies with TAPVR cannot supply oxygen-rich (red) blood to the body after birth. […] Without an additional heart defect that allows mixing of oxygen-poor (blue) and oxygen-rich (red) blood, infants with TAPVR will have a mixture of oxygen-rich (red) and oxygen-poor (blue) blood circulating through the right heart and back to the lungs – a situation that is fatal. […] The cause of TAPVR is not known. […] The obvious indication of total anomalous pulmonary venous return is a newborn who becomes cyanotic (blue coloring of the skin, lips, and nailbeds) in the first days of life after the maternal source of oxygen (from the placenta) is removed. […] TAPVR treatment will be determined by your child’s physician based on the extent of the disease. […] Treatment for total anomalous pulmonary venous return requires open heart surgery. […] Many infants who have had TAPVR surgery will grow and develop normally.

• #6 Surgical correction of total anomalous pulmonary venous return in an adult patient | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/s13019-022-01990-9

  Total anomalous pulmonary venous return (TAPVR) is rare congenital heart disease. Most TAPVRs require surgical corrections in the neonatal period and survival to adulthood without surgical correction is extremely rare. […] TAPVR is a rare anomaly accounting for only 1.5% to 2.2% of congenital heart disease. TAPVR is a cardiac malformation in which all pulmonary veins on either side are not connected to the left atrium, but directly to the somatic vein or the right atrium. Almost all patients with TAPVR present with symptoms of heart failure in the neonatal period, which require early surgical correction. It is unusual to encounter a adult patient with TAPVR due to low survival rate in untreated cases. […] There are few adult TAPVR patients worldwide. The cases reported previously showed a favorable prognosis in the postoperative phase. However, approximately 10% to 15% of the patients have evidence of late pulmonary vein obstruction, which tends to be recurrent and progressive. […] However, long-term surveillance and monitoring are required.

• #6 Neonatal Outcomes in Total Anomalous Pulmonary Venous Return: The Role of Prenatal Diagnosis and Pulmonary Venous Obstruction – PubMed

  https://pubmed.ncbi.nlm.nih.gov/29796693/

  The objective of this study is to evaluate neonatal outcomes of total anomalous pulmonary venous return (TAPVR) and identify fetal echocardiography findings associated with preoperative pulmonary venous obstruction (PPVO). This retrospective study evaluated TAPVR cases from 2005 to 2014 for preoperative and postoperative outcomes based on prenatal diagnosis, PPVO, and heterotaxy syndrome. Of 137 TAPVR cases, 12% were prenatally diagnosed; 60% had PPVO, and 21% had heterotaxy. TAPVR repair was performed in 135 cases and survival to discharge was 82% (112/137). Heterotaxy was the only independent predictor of mortality on multiple regression analysis [OR 5.5 (CI 1.3-16.7), p = 0.02]. TAPVR has severe neonatal morbidity and mortality with low prenatal diagnosis rates in the absence of heterotaxy. […] Patients with obstructed TAPVR had greater preoperative morbidity, but only heterotaxy was independently associated with increased postoperative mortality. Vertical vein velocity helped prenatally identify those at risk of PPVO.

• #6 Partial and Total Anomalous Pulmonary Venous Connections and Associated Defects | SpringerLink

  https://link.springer.com/10.1007/978-1-4471-4619-3_43?fromPaywallRec=true

  Anomalous pulmonary venous return is a relatively rare congenital cardiac malformation in which the pulmonary veins do not return to the left atrium. If all four pulmonary veins drain anomalously, the designation of total anomalous pulmonary venous return (TAPVR) is applied; if only one to three pulmonary veins drain anomalously, the designation of partial anomalous pulmonary venous return (PAPVR) is applied. TAPVR is classified into supracardiac, infracardiac, cardiac, and mixed types according to the location of the anomalous drainage. The prognosis of patients with TAPVR depends on the degree of pulmonary venous obstruction and associated defects. TAPVR generally requires neonatal surgical correction. Early and late mortality for simple TAPVR is 10 % and 4 %, respectively. Patients with TAPVR and pulmonary venous obstruction have a higher early and late mortality (17 % and 11 %, respectively) than unobstructed patients (4 % and 6 %, respectively). TAPVR associated with other cardiac anomalies (other than an ASD or PDA) have worse early and late mortality (14 % and 18 %, respectively). The prognosis for all anomalous venous return patients has improved since the first surgical corrections were described. The management of pulmonary vein obstruction continues to be the largest obstacle to successful management of anomalous pulmonary venous return.

• #6 Pediatric Total Anomalous Pulmonary Venous Return (TAPVR) | Memorial Hermann

  https://memorialhermann.org/services/conditions/pediatric-total-anomalous-pulmonary-venous-return

  Treatment of TAPVR is surgery, which consists of placing the patient on the heart-lung machine (cardiopulmonary bypass) and making the connection between the pulmonary veins and the left atrium. […] Recovery from surgery is most closely related to the health of the lungs going into surgery. […] The pediatric cardiologist will monitor the child long-term and may prescribe medication to prevent an infection of the heart’s lining (endocarditis) or an odd heartbeat (arrhythmia).

• #6 About Total Anomalous Pulmonary Venous Return | Congenital Heart Defects (CHDs) | CDC

  https://www.cdc.gov/heart-defects/about/tapvr.html

  Total anomalous pulmonary venous return (pronounced TOHT-l uh-NOM-uh-luh-s PUHL-muh-ner-ee VEE-nuh-s ri-TURN) or T-A-P-V-R is a congenital heart defect. […] About 1 in every 7,809 babies in the United States are born with TAPVR. This means that about 504 babies are born with TAPVR each year. […] TAPVR and PAPVR might be diagnosed during pregnancy, but more often these defects are diagnosed soon after a baby is born. […] Babies with TAPVR will need surgery to repair the defect. […] Surgical repairs for TAPVR are not a cure. People living with TAPVR may have lifelong complications. Routine checkups with a heart doctor are needed to monitor progress, avoid complications, and check for other health conditions that might develop over time.

• #6 TAPVR | Types, Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/t/tapvr

  Because of the abnormal pulmonary vein connection red (oxygenated) blood returning from the lungs mixes with the blue (lower oxygenated) blood returning from the body. […] The outcome of surgical repair for total anomalous pulmonary venous return is excellent. The surgical mortality is less than 5% when repair is performed electively. This is in healthy children without obstructed pulmonary veins. […] Patients who have surgery to repair total anomalous pulmonary venous return early in their lives often do well. Most do not need expert follow-up care as adults.

• #6 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  Overall TAPVR mortality may be decreasing with time, which may be partially due to earlier recognition and a change in preoperative diagnostic strategies over recent decades. Complex mixed-type TAPVR has now been demonstrated in fetal ultrasound as early as 18 weeks. There has been a move towards more advanced post-natal imaging including the use of angiography. […] While TAPVR literature has continued to compound, there remains relatively limited mixed-type TAPVR-specific case series. The following is a retrospective clinical review. We aim to describe and display the anatomic variance and surgical technique used in the operative repair of one surgical groups experience with 14 mixed-type TAPVR patients between 2004-2018 in an attempt to add to the comprehensive available data with which clinicians can use to optimize decision-making in a rare congenital lesion bearing significant mortality.

• #6

  https://journals.lww.com/jpcr/fulltext/2014/01030/perioperative_management_of_total_anomalous.8.aspx

  The goal of surgery is to redirect pulmonary vein flow entirely to the left atrium. […] Corrective surgery is necessary for all patients with this condition. […] Continuous monitoring and surveillance of the clinical data are mandatory to direct further treatment following cardiac surgery. […] Management of a neonate or infant with TAPVD involves a major perioperative connect between the referring physician and the pediatric cardiologist, pediatric cardiac surgeon and intensivist. […] The outcome in major centre is entirely dependent upon early detection rapid perioperative stabilization and timely surgical interventions.

• #6 Anesthetic Management During Stenting of Obstructed Total Anomalous Pulmonary Return in a Neonate With Single Ventricle Anatomy | Hakim | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/2724/2078

  However, increased mortality occurs in patients with obstructed TAPVR, associated CHD, and preoperative decompensation with the need for emergency intervention. […] The care of these critically ill neonates with CHD is compounded by the fact that direct measurements of cardiac output are not possible. […] One of the problems when there is more than one associated CHD lesion is that the anesthetic and medical therapies of one condition may directly hinder care of the other. […] For these lesions, the primary intervention requires relief of the obstruction to pulmonary venous return by either a surgical or cardiac catheterization procedure. […] Following these interventions, a balance must be achieved between treatment of pulmonary hypertension, and optimizing systemic cardiac output while maintaining adequate oxygen saturation.

• #6 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  This study outlines the complex variance in mixed type TAPVR patients, and to our knowledge only 5 other mixed type-specific retrospective case series exist in the literature, though we acknowledge mixed type data can be found within generalized TAPVR studies. The intent of this data was to add to the literature for purposes of future meta and systematic analyses to improve surgical approach, as well as serve educational purposes regarding a rare and intricate lesion. […] Mixed type TAPVR presents a wide variety of connections, lending to the complexity in planning and execution of surgical correction. Our study contributes further to the available data on this more rare form of TAPVR, which may be important as more cohorts emerge with varying success rates. More widespread studies, including systematic review may be beneficial to further characterize morphology, intraoperative technique, outcome data, and perhaps most importantly, specific indices for predicting higher survival or mortality rates.

• #6 Whole-exome sequencing identifies SGCD and ACVRL1 mutations associated with total anomalous pulmonary venous return (TAPVR) in Chinese population | Oncotarget

  https://www.oncotarget.com/article/15434/text/

  As a rare type of Congenital Heart Defects (CHD), the genetic mechanism of Total Anomalous Pulmonary Venous Return (TAPVR) remains unknown, although previous studies have revealed potential disease-driving regions/genes. […] TAPVR characterized by cardiac function deterioration including cyanosis, pulmonary hypertension, dyspnea, pulmonary edema, congestive heart failure affects 1 in 15,000 live births and 48.8% of them will die without surgery before the age of 1. […] We provided evidence for ACVRL1 as a known causative gene and for SGCD as candidate genes for TAPVR. […] A rare missense mutation in ACVRL1 (c.C652T, p.R218W) was identified as a causal mutation for TAPVR. […] A recurrent mutation in SGCD (c.C717G, p.D239E) was detected, which coded for the dystrophin-glycoprotein complex (DGC). […] In conclusion, our study was the first attempt to dissect the etiology of TAPVR using whole-exome sequencing strategy in Chinese population.

• #7 Total Anomalous Pulmonary Venous Return in the Time of SARS-CoV-2—Case Report

  https://www.mdpi.com/2227-9067/10/2/387

  Total anomalous pulmonary venous return (TAPVR) is a congenital heart disease, accounting for up to 3% of all birth defects; it can be total, with all four pulmonary veins connected to the right atrium either directly or via systemic venous circulation, or partial. […] Totally aberrant pulmonary venous return is a cyanotic heart defect in which all four pulmonary veins are drained into the systemic venous circulatory system, either in the right atrium or in the tributary veins. It is identified with an incidence of 0.6–1.2 per 10,000 live births, accounting for 0.7–1.5% of congenital heart defects. […] The selection of the appropriate time for surgical intervention is based on the magnitude of the pulmonary blood flow compared to the systemic blood flow. […] The surgical management techniques are highly dependent on the type of TAPVR and the presence of other cardiac malformations. […] The surgical repair of TAPVR is associated with various degrees of mortality and morbidity, depending on the presence of concurrent cardiac anomalies. […] Our patient required urgent medical therapy and early surgical management, despite an initial asymptomatic status.

• #7 Total anomalous pulmonary venous return (TAPVR) – Children’s Health Cardiology

  https://www.childrens.com/specialties-services/conditions/total-anomalous-pulmonary-venous-return

  Surgery is the only way to fix a total anomalous pulmonary venous return, a rare congenital birth defect of the heart. […] Total anomalous pulmonary venous return (TAPVR) makes it hard for the heart to pump oxygen-rich blood to the rest of the body. […] TAPVR develops during the first four to six weeks of pregnancy because the child’s heart doesnt develop normally. […] In some cases, signs of TAPVR show up during prenatal tests, such as a fetal ultrasound. Children can develop symptoms within the first weeks, months or year of their life. […] We dont know what causes TAPVR, but we do know the defect develops during the first four to six weeks of pregnancy. Its not caused by anything a mother or father did or did not do. […] Children with TAPVR need surgery to restore normal blood flow to and through the heart. Your childs surgeon will discuss which surgery is right for your child. […] Yes. The only effective treatment for TAPVR is surgery. The good news is that after your child recovers, he or she can expect to live a healthy, active life.

• #7 Total anomalous pulmonary venous return (TAPVR) – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/total-anomalous-pulmonary-venous-return/cdc-20385613

  Total anomalous pulmonary venous return (TAPVR) is a rare heart condition that’s present at birth. That means it’s a congenital heart defect. […] The type of TAPVR depends on where the veins connect. Most children born with TAPVR have no family history of congenital heart disease. […] Possible risk factors for congenital heart defects, including total anomalous pulmonary venous return (TAPVR), may include: […] To diagnose total anomalous pulmonary venus return (TAPVR), a healthcare professional listens to the heart and lungs. A whooshing sound, called a heart murmur, may be heard. […] Total anomalous pulmonary venous return (TAPVR) is treated with surgery. The surgery usually is done when a child is a baby. The timing of surgery depends on whether there’s a blockage.

• #7 Partial and Total Anomalous Pulmonary Venous Connections and Associated Defects | SpringerLink

  https://link.springer.com/10.1007/978-1-4471-4619-3_43?fromPaywallRec=true

  Anomalous pulmonary venous return is a relatively rare congenital cardiac malformation in which the pulmonary veins do not return to the left atrium. If all four pulmonary veins drain anomalously, the designation of total anomalous pulmonary venous return (TAPVR) is applied; if only one to three pulmonary veins drain anomalously, the designation of partial anomalous pulmonary venous return (PAPVR) is applied. TAPVR is classified into supracardiac, infracardiac, cardiac, and mixed types according to the location of the anomalous drainage. The prognosis of patients with TAPVR depends on the degree of pulmonary venous obstruction and associated defects. TAPVR generally requires neonatal surgical correction. Early and late mortality for simple TAPVR is 10 % and 4 %, respectively. Patients with TAPVR and pulmonary venous obstruction have a higher early and late mortality (17 % and 11 %, respectively) than unobstructed patients (4 % and 6 %, respectively). TAPVR associated with other cardiac anomalies (other than an ASD or PDA) have worse early and late mortality (14 % and 18 %, respectively). The prognosis for all anomalous venous return patients has improved since the first surgical corrections were described. The management of pulmonary vein obstruction continues to be the largest obstacle to successful management of anomalous pulmonary venous return.

• #7 Surgery-related complications and their management in total anomalous pulmonary venous return during intensive care unit stay – Turkish Journal of Thoracic and Cardiovascular Surgery

  https://tgkdc.dergisi.org/text.php3?id=2188

  Pulmonary venous obstruction is crucial for both pre- and postoperative assessment in the ICU. Several studies have shown that patients with preoperative PVO had lower intubation rates, a higher number of postoperative pulmonary hypertensive crises and episodes, and a greater amount of positive inotropic support during their ICU stays. […] Following TAPVR corrective surgery, the mortality rate in recent years has decreased from 80% to below 10%, with early and accurate diagnoses, new developments in surgical techniques, and effective and improved ICU support being responsible for this dramatic improvement. […] Our findings revealed that postoperative complications such as low cardiac output, pulmonary hypertensive crises, and arrhythmia can be prevented by advanced ICU monitorization, especially for patients without the need for mechanical ventilation, those without metabolic acidosis, those with TAPVR who also have left ventricular hypoplasia, and those who have PVO. Furthermore, early and effective treatment with NO drugs or ECMO support to counteract these complications could lead to an improvement in the morbidity and mortality rates of these patients.

• #7 Total Anomalous Pulmonary Venous Return (TAPVR) | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/total-anomalous-pulmonary-venous-return

  Newborns with severe TAPVR will need emergency surgery shortly after birth. […] The goal of surgery for TAPVR is to restore normal connections of the pulmonary veins to the heart, alleviate any obstructions or narrowing of the pulmonary veins, to tie up any vessels that have developed and to close the atrial septal defect (ASD). […] Thanks to updates in surgical techniques for repairing TAPVR, the long-term outlook is continually improving. […] Our team in the Boston Children’s Department of Cardiac Surgery treat some of the most complex pediatric heart conditions in the world.

• #7 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  Overall TAPVR mortality may be decreasing with time, which may be partially due to earlier recognition and a change in preoperative diagnostic strategies over recent decades. Complex mixed-type TAPVR has now been demonstrated in fetal ultrasound as early as 18 weeks. There has been a move towards more advanced post-natal imaging including the use of angiography. […] While TAPVR literature has continued to compound, there remains relatively limited mixed-type TAPVR-specific case series. The following is a retrospective clinical review. We aim to describe and display the anatomic variance and surgical technique used in the operative repair of one surgical groups experience with 14 mixed-type TAPVR patients between 2004-2018 in an attempt to add to the comprehensive available data with which clinicians can use to optimize decision-making in a rare congenital lesion bearing significant mortality.

• #8 Total Anomalous Pulmonary Venous Drainage – TeachMePaeds

  https://teachmepaediatrics.com/cardiology/congenital-heart-defects/total-anomalous-pulmonary-venous-drainage/

  It occurs in about 0.6 to 1.2 per 10,000 live births [1,2] […] It accounts for about 1-1.5% of all congenital cardiac malformations [3] […] There is a marked male preponderance – Male to female ratio 4:1 (especially for the infra-cardiac type) [4]

• #8 Whole-exome sequencing identifies SGCD and ACVRL1 mutations associated with total anomalous pulmonary venous return (TAPVR) in Chinese population | Oncotarget

  https://www.oncotarget.com/article/15434/text/

  As a rare type of Congenital Heart Defects (CHD), the genetic mechanism of Total Anomalous Pulmonary Venous Return (TAPVR) remains unknown, although previous studies have revealed potential disease-driving regions/genes. […] TAPVR characterized by cardiac function deterioration including cyanosis, pulmonary hypertension, dyspnea, pulmonary edema, congestive heart failure affects 1 in 15,000 live births and 48.8% of them will die without surgery before the age of 1. […] We provided evidence for ACVRL1 as a known causative gene and for SGCD as candidate genes for TAPVR. […] A rare missense mutation in ACVRL1 (c.C652T, p.R218W) was identified as a causal mutation for TAPVR. […] A recurrent mutation in SGCD (c.C717G, p.D239E) was detected, which coded for the dystrophin-glycoprotein complex (DGC). […] In conclusion, our study was the first attempt to dissect the etiology of TAPVR using whole-exome sequencing strategy in Chinese population.

• #8 Total Anomalous Pulmonary Venous Return | Thoracic Key

  https://thoracickey.com/total-anomalous-pulmonary-venous-return/

  In the series by St. Louis etal., 27% of patients with TAPVR had an associated cardiac lesion. […] The population-based study reported by Seale showed that in patients with TAPVR, excluding those with heterotaxy and complex single-ventricle anatomy, 14% had associated cardiac lesions. […] The diagnosis of TAPVR can be difficult on prenatal ultrasonography. […] Echocardiography is an important initial diagnostic tool for patients with suspected TAPVR. […] With the advent of multidetector computed tomography (CT) scanning, detailed images of the pulmonary venous drainage, including inflow and runoff vessels from the confluence, can be achieved. […] Diagnostic cardiac catheterization is reserved for patients in whom echocardiography or CT imaging is not satisfactory or when associated lesions need to be further defined. […] The level of preoperative support required will reflect the pathophysiologic state of the patient and depends on the degree of PVO and pulmonary blood flow.

• #8 Anesthetic Management During Stenting of Obstructed Total Anomalous Pulmonary Return in a Neonate With Single Ventricle Anatomy | Hakim | Journal of Medical Cases

  https://www.journalmc.org/index.php/JMC/article/view/2724/2078

  Total anomalous pulmonary venous return (TAPVR) is a relatively rare congenital cardiac abnormality accounting for 1.5-3% of cases of congenital heart disease (CHD). […] Morbidity and mortality associated with surgical repair of TAPVR increase dramatically when there are other associated cardiac defects, particularly single ventricle anatomy. […] Total anomalous pulmonary venous return (TAPVR) is a relatively rare type of congenital heart disease (CHD) accounting for 1.5-3% of such defects. […] Morbidity and mortality associated with TAPVR repair increase dramatically when another cardiac anomaly is present, particularly when combined with single ventricle anatomy. […] TAPVR remains one of the most challenging CHDs in newborns and young infants. […] The surgical results of TAPVR repair have improved significantly in the modern era as a result of earlier diagnosis and treatment, improvements in surgical technique, cardiac anesthesia, neonatal myocardial preservation, and postoperative care.

• #9 Partial and Total Anomalous Pulmonary Venous Connection – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560707/

  TAPVC and PAPVC are uncommon congenital anomalies accounting for 1% of all congenital heart diseases. […] Total anomalous pulmonary venous connections (TAPVC) is a cyanotic congenital heart disease where all of the pulmonary veins drain directly or indirectly into the right atrium. […] TAPVC implies that all the pulmonary veins connect to the systemic veins or right atrium, either by a common vertical vein or individually. Anatomically TAPVC can be divided into four subtypes based on the level of the anomalous connection as supracardiac, cardiac, infracardiac, and mixed. […] The supracardiac variety is the most common of the TAPVC. […] In untreated patients, TAPVC is almost always fatal within the first few weeks of life. The long term survival in patients has increased with the improvement in medical management and surgical techniques.

• #9

  https://journals.lww.com/epidem/fulltext/2004/07000/paternal_occupational_lead_exposure_and_total.497.aspx

  Paternal lead exposure has been associated with congenital malformations, spontaneous abortions and decreased sperm quality and quantity in previous studies; however, results have been inconsistent. […] the authors investigated the association between paternal occupational lead exposure and total anomalous pulmonary venous return (TAPVR), a rare cardiovascular malformation, within the Baltimore Washington Infant Study. […] After adjusting for infant race, maternal employment, and maternal pesticide exposure, the odds of paternal occupational lead exposure for TAPVR was 1.83 (95% confidence interval (CI) 0.96-3.50) for industrial hygienist 1 (IH 1), 1.57 (95% CI 0.71-3.47) for IH 2, 1.37 (95% CI 0.70-2.66) for IH 3. […] After correcting for potential measurement error, there appeared to be a possible association between paternal occupational lead exposure and TAPVR. Given the high environmental lead levels that persist in developing countries, it is important to further assess the association between lead and TAPVR, as well as other reproductive and perinatal outcomes.

• #9 Anomalous Pulmonary Venous Return

  https://fpnotebook.com/CV/Peds/AnmlsPlmnryVnsRtrn.htm

  Accounts for 0.7 to 1.5% of Congenital Heart Disease cases (5th most common cyanotic cause) […] TAPVR is frequently misdiagnosed initially.

• #9 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  Total anomalous pulmonary venous return (TAPVR), also referred in literature as total anomalous pulmonary venous connection (TAPVC), provides a wide spectrum of complex anatomic and therapeutic challenges amongst congenital heart disease. An estimated 400 infants (1/10.000 births) are born annually in the United States with TAPVR, an incidence among congenital heart disease of 0.7-1.5%. While there may be rare survival to adulthood if un-repaired, typical median survival if unrepaired is 2 months with 50% mortality in first 3 months of life, thereby demonstrating the urgency of recognition and intervention. […] TAPVR is classified according to the Darling system developed in 1957 into cardiac, supracardiac, infracardiac, or mixed-type depending on the pattern of pulmonary venous (PV) drainage to the systemic venous circuitry. Mixed-type TAPVR in itself has a higher mortality, with univariate analysis of mixed type versus cardiac type (lowest mortality of TAPVR variants) demonstrating statistically significant hazard ratios of 2.88 in previous study, and mortality as high as 42-50%. Other determinants of increased mortality or transplant in this study include complex TAPVC and postoperative length of stay, while older age, weight greater than 2.5 kg, non-emergent operation, lack of PV obstruction, cardiac and supracardiac types were associated with increased survival to discharge.

• #10 Anomalous Pulmonary Venous Return: Pearls and Pitfalls in multimodal imaging.

  https://epos.myesr.org/poster/esr/ecr2022/C-10696

  1. Review the epidemiology, embryology, and classification of anomalous pulmonary venous connection. […] TAPVR consists in all four pulmonary veins draining into systemic veins of the right atrium with or without pulmonary venous obstruction. […] TAPVR is generally classified into 4 anatomical subtypes: supracardiac, cardiac, infracardiac, or… […] Anomalous venous drainage of the pulmonary veins is an infrequent condition, which can be detected in pediatric patients and treated appropriately, or it can be diagnosed in adult life in patients with symptoms suggestive of pulmonary hypertension.

• #11 Supracardiac Total Anomalous Pulmonary Venous Return | Pediatric Echocardiography

  https://pedecho.org/library/chd/tapvr

  Total anomalous pulmonary venous connection is when all of the pulmonary veins returning from the lungs have an abnormal connection and drain anomalously to the right side of the heart instead of the left atrium. Supracardiac TAPVC is the most common type of anomalous pulmonary venous connection. In supracardiac TAPVC, the common pulmonary vein drains superiorly via a vertical vein into the left innominate vein, the right superior vena cava, or the right azygos vein by way of an ascending vertical vein. […] TAPVC has been described in 9 in 100,000 of the population. Supracardiac TAPVC is the most common type (47% in the largest published series) and in this group the most common site of connection is to the left innominate vein (36% of all cases). […] The mortality of untreated patients is 75-85% by 1st year of life.

• #12 Outcomes of Complete Repair of Mixed-Type Total Anomalous Pulmonary Venous Return – Journal of Updates in Cardiovascular Medicine

  https://www.jucvm.com/articles/outcomes-of-complete-repair-of-mixed-type-total-anomalous-pulmonary-venous-return/doi/jucvm.galenos.2024.2024.32.87

  Total anomalous pulmonary venous return (TAPVR), also referred in literature as total anomalous pulmonary venous connection (TAPVC), provides a wide spectrum of complex anatomic and therapeutic challenges amongst congenital heart disease. An estimated 400 infants (1/10.000 births) are born annually in the United States with TAPVR, an incidence among congenital heart disease of 0.7-1.5%. While there may be rare survival to adulthood if un-repaired, typical median survival if unrepaired is 2 months with 50% mortality in first 3 months of life, thereby demonstrating the urgency of recognition and intervention. […] TAPVR is classified according to the Darling system developed in 1957 into cardiac, supracardiac, infracardiac, or mixed-type depending on the pattern of pulmonary venous (PV) drainage to the systemic venous circuitry. Mixed-type TAPVR in itself has a higher mortality, with univariate analysis of mixed type versus cardiac type (lowest mortality of TAPVR variants) demonstrating statistically significant hazard ratios of 2.88 in previous study, and mortality as high as 42-50%. Other determinants of increased mortality or transplant in this study include complex TAPVC and postoperative length of stay, while older age, weight greater than 2.5 kg, non-emergent operation, lack of PV obstruction, cardiac and supracardiac types were associated with increased survival to discharge.

• #13 Surgical repair of neonatal total anomalous pulmonary venous connection: A single institutional experience with 241 cases – JTCVS Open

  https://www.jtcvsopen.org/article/S2666-2736(23)00210-3/fulltext

  Challenges persist in surgery for neonatal total anomalous pulmonary venous connection (neoTAPVC), with the high mortality risk not mitigated over time. A prospectively collected single-center database containing all neonates with TAPVC undergoing biventricular repair in 2012 to 2020 was retrospectively reviewed. The primary outcome was death or postoperative pulmonary venous obstruction (PPVO). Overall, 241 patients with a median age at surgery of 14 days (interquartile range [IQR], 9-21 days) were included. Anomalous return was supracardiac in 38.6%, cardiac in 26.1%, infracardiac in 28.6%, and mixed in 6.6%. Patients receiving DPC had better survival (96.3% vs 84.3%; P= .0028) and lower incidence of PPVO (15.2% vs 28.6%; P= .011) compared with those without DPC. Access to DPC potentially improves outcomes in the neoTAPVC setting; freedom from PPVO were similar using conventional versus sutureless repair. Dedicated preoperative care is associated with better survival and lower restenosis rate following surgical repair of total anomalous pulmonary venous connection (TAPVC) in the neonatal patients. The rationale for the requirement of such specialist care is mainly based on the previous finding that patient-specific preoperative status has a strong association with mortality and morbidity, which can be influenced by preoperative management strategy. However, to what extent dedicated presurgical care (DPC) would influence the outcomes after neonatal TAPVC (neoTAPVC) surgery has not been well investigated. This study first provides an insight into the impact of presurgical care models on outcomes in the neoTAPVC setting. It is noteworthy that the access to DPC conferred an approximately 4-fold decrease in death, particularly resulting in the postoperative 1-year mortality of 3.7% that was remarkably lower than previously published data. Findings from this study suggest that care by a dedicated cardiac team can allow for tiered patient triage and helps to optimize presurgical clinical status and bridge the time to surgery in the selected patients.

• #14 Total anomalous pulmonary venous return (type II) | Radiology Case | Radiopaedia.org

  https://radiopaedia.org/cases/total-anomalous-pulmonary-venous-return-type-ii?lang=us

  A case of complex congenital heart disease with single ventricle and atrial septal defect. All pulmonary veins drain into the right atrium indicative of type II (cardiac) total anomalous pulmonary venous return. […] This case is an example of type II (cardiac) total anomalous pulmonary venous return (TAPVR), in which the common pulmonary venous sinus joins either the coronary sinus or the right atrium. It accounts for up to 30% of TAPVR cases.

• #15 Surgical repair of neonatal total anomalous pulmonary venous connection: A single institutional experience with 241 cases – JTCVS Open

  https://www.jtcvsopen.org/article/S2666-2736(23)00210-3/fulltext

  Challenges persist in surgery for neonatal total anomalous pulmonary venous connection (neoTAPVC), with the high mortality risk not mitigated over time. A prospectively collected single-center database containing all neonates with TAPVC undergoing biventricular repair in 2012 to 2020 was retrospectively reviewed. The primary outcome was death or postoperative pulmonary venous obstruction (PPVO). Overall, 241 patients with a median age at surgery of 14 days (interquartile range [IQR], 9-21 days) were included. Anomalous return was supracardiac in 38.6%, cardiac in 26.1%, infracardiac in 28.6%, and mixed in 6.6%. Patients receiving DPC had better survival (96.3% vs 84.3%; P= .0028) and lower incidence of PPVO (15.2% vs 28.6%; P= .011) compared with those without DPC. Access to DPC potentially improves outcomes in the neoTAPVC setting; freedom from PPVO were similar using conventional versus sutureless repair. Dedicated preoperative care is associated with better survival and lower restenosis rate following surgical repair of total anomalous pulmonary venous connection (TAPVC) in the neonatal patients. The rationale for the requirement of such specialist care is mainly based on the previous finding that patient-specific preoperative status has a strong association with mortality and morbidity, which can be influenced by preoperative management strategy. However, to what extent dedicated presurgical care (DPC) would influence the outcomes after neonatal TAPVC (neoTAPVC) surgery has not been well investigated. This study first provides an insight into the impact of presurgical care models on outcomes in the neoTAPVC setting. It is noteworthy that the access to DPC conferred an approximately 4-fold decrease in death, particularly resulting in the postoperative 1-year mortality of 3.7% that was remarkably lower than previously published data. Findings from this study suggest that care by a dedicated cardiac team can allow for tiered patient triage and helps to optimize presurgical clinical status and bridge the time to surgery in the selected patients.

• #16 Infracardiac Total Anomalous Pulmonary Venous Return | Pediatric Echocardiography

  https://pedecho.org/library/chd/tapvr-infracardiac

  Total anomalous pulmonary venous return (TAPVR) is a rare congenital malformation in which all four pulmonary veins do not connect normally to the left atrium. Instead, the four pulmonary veins drain abnormally to the right atrium by way of an anomalous venous connection. […] Infracardiac TAPVR is a subtype of TAPVR in which the pulmonary veins connect via the umbilicovitelline system. The pulmonary veins drain to a confluence which in turn drains inferiorly, most commonly via a descending vertical vein which descends anterior to the esophagus through the diaphragm into the portal venous system (most common), ductus venosus, hepatic veins or IVC. […] This subtype of TAPVR can result in pulmonary venous obstruction as the descending vertical vein drains within the portal venous system. Obstruction may be secondary to multiple etiologies which include intrinsic narrowing of the connecting vessel, the interposition of the hepatic sinusoids between the pulmonary veins and the heart and constriction of the ductus venosus. An atrial septal defect is necessary to allow pulmonary venous return to reach the left heart and systemic circulation.

• #17 Surgical repair of neonatal total anomalous pulmonary venous connection: A single institutional experience with 241 cases – JTCVS Open

  https://www.jtcvsopen.org/article/S2666-2736(23)00210-3/fulltext

  Challenges persist in surgery for neonatal total anomalous pulmonary venous connection (neoTAPVC), with the high mortality risk not mitigated over time. A prospectively collected single-center database containing all neonates with TAPVC undergoing biventricular repair in 2012 to 2020 was retrospectively reviewed. The primary outcome was death or postoperative pulmonary venous obstruction (PPVO). Overall, 241 patients with a median age at surgery of 14 days (interquartile range [IQR], 9-21 days) were included. Anomalous return was supracardiac in 38.6%, cardiac in 26.1%, infracardiac in 28.6%, and mixed in 6.6%. Patients receiving DPC had better survival (96.3% vs 84.3%; P= .0028) and lower incidence of PPVO (15.2% vs 28.6%; P= .011) compared with those without DPC. Access to DPC potentially improves outcomes in the neoTAPVC setting; freedom from PPVO were similar using conventional versus sutureless repair. Dedicated preoperative care is associated with better survival and lower restenosis rate following surgical repair of total anomalous pulmonary venous connection (TAPVC) in the neonatal patients. The rationale for the requirement of such specialist care is mainly based on the previous finding that patient-specific preoperative status has a strong association with mortality and morbidity, which can be influenced by preoperative management strategy. However, to what extent dedicated presurgical care (DPC) would influence the outcomes after neonatal TAPVC (neoTAPVC) surgery has not been well investigated. This study first provides an insight into the impact of presurgical care models on outcomes in the neoTAPVC setting. It is noteworthy that the access to DPC conferred an approximately 4-fold decrease in death, particularly resulting in the postoperative 1-year mortality of 3.7% that was remarkably lower than previously published data. Findings from this study suggest that care by a dedicated cardiac team can allow for tiered patient triage and helps to optimize presurgical clinical status and bridge the time to surgery in the selected patients.

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