Materiały źródłowe

• #1 Partial anomalous pulmonary venous return – UpToDate

  https://www.uptodate.com/contents/partial-anomalous-pulmonary-venous-return

  Partial anomalous pulmonary venous return (PAPVR; also known as partial anomalous pulmonary venous connection [PAPVC]), encompasses a spectrum of congenital cardiovascular anomalies. Blood from one or more pulmonary veins returns abnormally to the right atrium, either directly or indirectly through a variety of systemic venous pathways that connect with the anomalous pulmonary vein. […] The estimated prevalence of PAPVR is 0.2 to 0.7 percent. The true prevalence may be higher since mild cases may go unrecognized. PAPVR is more common in patients with Turner syndrome. […] For congenital anomalies of the pulmonary vein, the terms „anomalous venous return,” „anomalous venous connection,” and „anomalous venous drainage” are often used synonymously and reflect the same physiology.

• #2

  https://www.polradiol.com/Partial-anomalous-pulmonary-venous-connection-state-of-the-art-review-with-assessment,146849,0,2.html

  Pulmonary venous anomalies occur as a result of failure in normal embryological development. […] Though not rare, PAPVC is an uncommon anomaly in which some of the pulmonary veins abnormally connect and drain into the vascular compartments other than the left atrium (LA); however, the others drain normally into the LA. […] The prevalence of PAPVC has been reported between 0.4 and 0.7%. […] The incidence of occurrence is more common on the right side, and it may be detected incidentally on CT or MR imaging. […] Partial connections behave like a left to right shunt similar to ASD, VSD, or PDA. […] Most of the PAPVC cases are detected incidentally because patients are usually asymptomatic or present with mild clinical symptoms. […] PAPVC is considered as a treatable cause of pulmonary artery hypertension. […] Partial anomalous pulmonary venous connections are uncommon congenital anomalies. Depending on the severity of anomalous drainage, the site of connection, and associated defects, this may be detected incidentally or present with variable symptoms like the other CHDs.

• #3 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #4 Partial anomalous pulmonary venous connection epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Partial_anomalous_pulmonary_venous_connection_epidemiology_and_demographics

  Partial anomalous pulmonary venous connection is a rare congenital heart disease. There is a very low incidence rate. Prevalence is difficult to determine due to the asymptomatic nature of the disease. […] Autopsy data suggests that partial anomalous pulmonary venous connection (PAPVC) has an incidence of 0.4-0.7%. Many of these autopsy cases were asymptomatic and as a result the clinical incidence of PAPVC is much lower.

• #5 Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/1749-8090-6-126

  Partial anomalous pulmonary venous return (PAPVR) is defined as a left-to-right shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. […] All PAPVRs are left-to-right shunts, but unless more than 50% of the pulmonary flow drains to the right side of the heart clinical manifestations are rare. Dyspnea, fatigue, exercise intolerance, palpitations, syncope, atrial arrhythmias, right heart failure, and pulmonary hypertension may occur. […] PAPVR is usually diagnosed by transthoracic echocardiography (TTE) or transesophageal echocardiography (TEE) and catheter based angiography. […] Modern 128-slice multidetector computed tomography (MDCT) scans are accurate in defining ASDs and PAPVR. ECG-gated MDCT enables a non-invasive and rapid image acquisition with high spatial and temporal resolution, optimized contrast bolus timing, and wide anatomic coverage. The presence, course, number of anomalous veins, and associated cardiovascular defects can be reliably observed by MDCT angiography.

• #6 Partial anomalous pulmonary venous return | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/partial-anomalous-pulmonary-venous-return?embed_domain=hackmd.io%25252F%252540yipuafecsl2jsu8smr5njq%25252Fbnjhjgjghjghjghfavicon.ico&lang=us

  The overall prevalence of PAPVR is 0.4-0.7% […] Left-sided PAPVR has been reported to be found more often in adults, whereas right-sided PAPVR is reported more commonly in children. It is unclear if this is because of a higher proportion of symptomatic manifestations of the latter. The left upper lobe vein anomaly is thought to be the most common. […] In ~40% of patients with right-sided PAPVR, an atrial septal defect is seen.

• #7 Partial Anomalous Pulmonary Venous Connection: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/897686-overview

  Most data regarding the prevalence of partial anomalous pulmonary venous connection (PAPVC) have been garnered from autopsy series that estimate an incidence of 0.4-0.7%. However, autopsy series may overestimate the clinical significance of this condition because many of these cases were asymptomatic; thus, the true incidence of patients who present antemortem with this condition is lower. Clinical diagnosis of isolated PAPVC is quite rare. PAPVC occurs in approximately 10% of patients with a proven ASD. […] The incidence is higher in the female population. […] PAPVC is a congenital defect. Clinical evidence of this congenital defect may not be apparent until the patient reaches middle age.

• #8 Partial Anomalous Pulmonary Venous Return Presenting in Adults: A Case Series With Review of Literature

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7331899/

  Partial anomalous pulmonary venous return (PAPVR) is a congenital anomaly in which some of the pulmonary veins drain erroneously into the superior vena cava (SVC) or directly into the right atrium (RA). […] The overall incidence of PAPVR is 0.7% based on an autopsy series. However, a more recent retrospective study examining chest CT showed a disease prevalence of 0.1%. […] The management of PAPVR is individualized based on clinical presentation and degree of the left-to-right shunt. Asymptomatic patients with small left-to-right shunt due to PAPVR generally do not require surgery. Surgical repair is indicated for symptomatic patients with a hemodynamically significant left-to-right shunt (Qp/Qs 2) on cardiac MRI. […] In PAPVR patients with or without associated ASD, the decision for surgical repair depends on symptoms, shunt fraction, recurrent pulmonary infections, and concurrent indication for cardiac surgery.

• #9 Isolated Partial Anomalous Pulmonary Venous Connection: Development of Volume Overload and Elevated Estimated Pulmonary Pressure in Adults – Journal of Clinical Imaging Science

  https://clinicalimagingscience.org/isolated-partial-anomalous-pulmonary-venous-connection-development-of-volume-overload-and-elevated-estimated-pulmonary-pressure-in-adults/

  Partial anomalous pulmonary venous connection (PAPVC) is one of the rare congenital cardiac diseases with a prevalence of 0.40.7% of autopsies. The prevalence of a partial anomalous pulmonary venous connection is 0.2% in computed tomography (CT) among adults. […] Among the 110 subjects, 54 (49%) had isolated PAPVC. Of 54, 26 patients had volume overload of RA/RV or elevated estimated systolic pulmonary artery pressure. […] We found statistically significant association (P = 0.02) between isolated PAPVC in adults and pulmonary hypertension. […] Isolated PAPVC has association with the development of pulmonary hypertension in adults, approaching statistically significant p value. […] The prevalence of PAPVC in adults was 0.2% on computed tomography (CT) scans. […] The right-sided cardiac volume overload due to PAPVC can manifest from early childhood to late adulthood with a varying spectrum of severity.

• #10 Partial anomalous pulmonary venous connection epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Partial_anomalous_pulmonary_venous_connection_epidemiology_and_demographics

  Partial anomalous pulmonary venous connection is a rare congenital heart disease. There is a very low incidence rate. Prevalence is difficult to determine due to the asymptomatic nature of the disease. […] Autopsy data suggests that partial anomalous pulmonary venous connection (PAPVC) has an incidence of 0.4-0.7%. Many of these autopsy cases were asymptomatic and as a result the clinical incidence of PAPVC is much lower.

• #11 Partial anomalous pulmonary venous return – UpToDate

  https://www.uptodate.com/contents/partial-anomalous-pulmonary-venous-return

  Partial anomalous pulmonary venous return (PAPVR; also known as partial anomalous pulmonary venous connection [PAPVC]), encompasses a spectrum of congenital cardiovascular anomalies. Blood from one or more pulmonary veins returns abnormally to the right atrium, either directly or indirectly through a variety of systemic venous pathways that connect with the anomalous pulmonary vein. […] The estimated prevalence of PAPVR is 0.2 to 0.7 percent. The true prevalence may be higher since mild cases may go unrecognized. PAPVR is more common in patients with Turner syndrome. […] For congenital anomalies of the pulmonary vein, the terms „anomalous venous return,” „anomalous venous connection,” and „anomalous venous drainage” are often used synonymously and reflect the same physiology.

• #12 Anomalous pulmonary venous connection – Wikipedia

  https://en.wikipedia.org/wiki/Anomalous_pulmonary_venous_connection

  Partial anomalous pulmonary venous connection (or Partial anomalous pulmonary venous drainage or Partial anomalous pulmonary venous return) is a congenital defect where the left atrium is the point of return for the blood from some (but not all) of the pulmonary veins. […] It is less severe than total anomalous pulmonary venous connection which is a life-threatening anomaly requiring emergent surgical correction, usually diagnosed in the first few days of life. Partial anomalous venous connection may be diagnosed at any time from birth to old age. The severity of symptoms, and thus the likelihood of diagnosis, varies significantly depending on the amount of blood flow through the anomalous connections. In less severe cases, with smaller amounts of blood flow, diagnosis may be delayed until adulthood, when it can be confused with other causes of pulmonary hypertension.

• #13 Partial Anomalous Pulmonary Venous Connection: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/897686-overview

  Most data regarding the prevalence of partial anomalous pulmonary venous connection (PAPVC) have been garnered from autopsy series that estimate an incidence of 0.4-0.7%. However, autopsy series may overestimate the clinical significance of this condition because many of these cases were asymptomatic; thus, the true incidence of patients who present antemortem with this condition is lower. Clinical diagnosis of isolated PAPVC is quite rare. PAPVC occurs in approximately 10% of patients with a proven ASD. […] The incidence is higher in the female population. […] PAPVC is a congenital defect. Clinical evidence of this congenital defect may not be apparent until the patient reaches middle age.

• #14 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #15 Anomalous pulmonary venous connection – Wikipedia

  https://en.wikipedia.org/wiki/Anomalous_pulmonary_venous_connection

  There is also evidence that a significant number of mild cases are never diagnosed, or diagnosed incidentally. […] It is associated with other vascular anomalies, and some genetic syndromes such as Turner syndrome. […] It can be diagnosed with CT scan, angiography, transesophageal echocardiography, or cardiac MRI. […] It is sometimes treated with surgery, which involves rerouting blood from the right atrium into the left atrium with a patch or use of the Warden procedure. However, interest is increasing in catheter-based interventional approaches, as well as medical therapy for less severe cases.

• #16 Partial anomalous pulmonary venous return

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20359714

  Partial anomalous pulmonary venous return is a rare heart problem that’s present at birth. That means it is a congenital heart defect. […] A child born with Turner syndrome has an increased risk of PAPVR. […] If PAPVR occurs with other heart problems, it may be diagnosed soon after birth. If the condition is mild, it may not be diagnosed until adulthood. […] A person with partial anomalous pulmonary venous return needs regular health checkups for life to check for complications. It’s best to see a health care provider who is trained in congenital heart diseases. This type of provider is called a congenital cardiologist.

• #17 Management of Total Left Partial Anomalous Pulmonary Venous Connection

  https://www.fortunejournals.com/articles/management-of-total-left-partial-anomalous-pulmonary-venous-connection.html

  Given the myriad of anatomic variations of PAPVR lesions and depending on the severity of the lesion, the vast majority of patients are asymptomatic through childhood. […] Patients with symptomatic PAPVR usually present in early adulthood. […] The embryologic origin of left sided PAPVR typically starts around postnatal day 32, due to malregression of the primordial pulmonary trunk and the primitive posterior left atrium, along with concomitant maldevelopment of the sinus venosus, and the left common cardinal and right vitelline veins. […] Left sided PAPVR leads to physiologic left-to-right shunting. […] Atrial septal defects (ASD) are traditionally associated with partial anomalous pulmonary venous return, and up to 80% of patients with right sided PAPVR have concomitant ASD. […] Of these, approximately 10–15% of patients have secundum ASD and 85% of patients have sinus venosus ASDs.

• #18 Partial Anomalous Pulmonary Venous Return: To Fix or Not to Fix?

  https://scholarlycommons.hcahealthcare.com/northtexas2024/18/

  Patient was identified to have 90-95% stenosis of his left circumflex but was unable to be stented due to tortuosity of the vessel and tight lesion. […] Chest CT scan was identified to have left upper lobe pulmonary vein that drains into the left lateral brachiocephalic vein which is consistent with a partial anomalous pulmonary venous return (PAPVR). […] The prevalence of PAPVR is about 0.4-0.7%. […] PAPVR more commonly affects the right upper lobe vein and is associated with an atrial septal defect in 80-90% of cases. […] Left-sided PAPVR is only present in about 10% cases, of which the vast majority go undiagnosed until the development of symptoms or the need for pulmonary mapping in instances of percutaneous ablation for atrial fibrillation or consideration of lobectomy in lung neoplasm.

• #19 Partial anomalous pulmonary venous return | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/partial-anomalous-pulmonary-venous-return?embed_domain=hackmd.io%25252F%252540yipuafecsl2jsu8smr5njq%25252Fbnjhjgjghjghjghfavicon.ico&lang=us

  The overall prevalence of PAPVR is 0.4-0.7% […] Left-sided PAPVR has been reported to be found more often in adults, whereas right-sided PAPVR is reported more commonly in children. It is unclear if this is because of a higher proportion of symptomatic manifestations of the latter. The left upper lobe vein anomaly is thought to be the most common. […] In ~40% of patients with right-sided PAPVR, an atrial septal defect is seen.

• #20 Management of Total Left Partial Anomalous Pulmonary Venous Connection

  https://www.fortunejournals.com/articles/management-of-total-left-partial-anomalous-pulmonary-venous-connection.html

  Partially anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more of the pulmonary veins are confluent with the systemic venous system. […] In an autopsy series of patients with congenital heart disease (CHD), right sided PAPVR was found in 0.4–0.7% of patients. […] There are several anatomic variations of PAPVR that have been described. […] Left sided PAPVR occurs an order of magnitude less than right sided PAPVR. […] Drainage directly to the coronary sinus accounts for 3% of patients with PAPVR and very few, include left sided PAPVR with all three left pulmonary veins draining to the coronary sinus. […] PAPVR historically does not have an associated untreated mortality rate, however, predisposes an individual to developing several complications such as fatigue, early exertional dyspnea, right ventricular dilation, tricuspid and pulmonary valve insufficiency, atrial arrhythmia, reduced ejection fraction, and pulmonary artery hypertension (PAH) which can be lethal.

• #21 Partial anomalous pulmonary venous return | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/partial-anomalous-pulmonary-venous-return?embed_domain=hackmd.io%25252F%252540yipuafecsl2jsu8smr5njq%25252Fbnjhjgjghjghjghfavicon.ico&lang=us

  The overall prevalence of PAPVR is 0.4-0.7% […] Left-sided PAPVR has been reported to be found more often in adults, whereas right-sided PAPVR is reported more commonly in children. It is unclear if this is because of a higher proportion of symptomatic manifestations of the latter. The left upper lobe vein anomaly is thought to be the most common. […] In ~40% of patients with right-sided PAPVR, an atrial septal defect is seen.

• #22 Partial anomalous pulmonary venous return | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/partial-anomalous-pulmonary-venous-return?embed_domain=hackmd.io%25252F%252540yipuafecsl2jsu8smr5njq%25252Fbnjhjgjghjghjghfavicon.ico&lang=us

  The overall prevalence of PAPVR is 0.4-0.7% […] Left-sided PAPVR has been reported to be found more often in adults, whereas right-sided PAPVR is reported more commonly in children. It is unclear if this is because of a higher proportion of symptomatic manifestations of the latter. The left upper lobe vein anomaly is thought to be the most common. […] In ~40% of patients with right-sided PAPVR, an atrial septal defect is seen.

• #23 Management of Total Left Partial Anomalous Pulmonary Venous Connection

  https://www.fortunejournals.com/articles/management-of-total-left-partial-anomalous-pulmonary-venous-connection.html

  Partially anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more of the pulmonary veins are confluent with the systemic venous system. […] In an autopsy series of patients with congenital heart disease (CHD), right sided PAPVR was found in 0.4–0.7% of patients. […] There are several anatomic variations of PAPVR that have been described. […] Left sided PAPVR occurs an order of magnitude less than right sided PAPVR. […] Drainage directly to the coronary sinus accounts for 3% of patients with PAPVR and very few, include left sided PAPVR with all three left pulmonary veins draining to the coronary sinus. […] PAPVR historically does not have an associated untreated mortality rate, however, predisposes an individual to developing several complications such as fatigue, early exertional dyspnea, right ventricular dilation, tricuspid and pulmonary valve insufficiency, atrial arrhythmia, reduced ejection fraction, and pulmonary artery hypertension (PAH) which can be lethal.

• #24 Unusual presentation of a patient with partial anomalous pulmonary venous connections without a septal defect: a case report and literature review | International Journal of Emergency Medicine | Full Text

  https://intjem.biomedcentral.com/articles/10.1186/s12245-025-00809-3

  Partial anomalous pulmonary vein connections (PAPVC) are rare congenital abnormalities in which one or more pulmonary veins drain into the right atrium. This pathological condition may present in various ways, such as chest pain and dyspnea, or it may be diagnosed incidentally. Consequently, missed or late diagnoses are common, highlighting the importance of optimal diagnostic modalities. […] Detection of PAPVC is not straightforward in many cases and often requires evaluation with various imaging techniques for accurate diagnosis. Therefore, physicians encountering undefined causes of chest pain or dyspnea should consider multiple imaging modalities. Follow-up is also important, as certain groups of patients may require surgical treatment to prevent complications. […] PAPVC has been diagnosed in approximately 0.4-0.7% of the normal population during autopsy evaluations. The prevalence of symptomatic patients is nearly equal to that of the lower range of this spectrum (0.41%). Therefore, it is considered a rare anomaly that cardiologists should keep in mind.

• #25 Anomalous pulmonary venous connection: An underestimated entity | Revista Portuguesa de Cardiologia

  https://www.revportcardiol.org/pt-anomalous-pulmonary-venous-connection-an-articulo-S0870255116302700

  Patients with PAPVC are typically acyanotic and most commonly only mildly symptomatic or asymptomatic. Some authors have suggested that PAPVC becomes clinically significant only when 50% or more of the pulmonary blood flow returns anomalously. […] Imaging techniques, especially CT and MRI, have a pivotal role in the accurate characterization of these abnormalities, predicting outcomes and establishing appropriate preoperative planning. […] Unlike PAPVC, no catheter-corrective treatment is possible for TAPVC, although atrial septostomy is used in some patients when corrective surgery is delayed. […] In pediatric patients, PAPVC is usually treated if they have Qp:Qs of 1:1.5 or more as they are more likely to develop pulmonary hypertension and right ventricular failure, although this cutoff has not been subject to rigorous study. […] Thus, for patients with PAPVC who have already developed mild to moderate pulmonary hypertension, surgical repair is usually safe and effective, although catheter-guided and medical therapies may play an increasing role.

• #26 Anomalous pulmonary venous connection: An underestimated entity | Revista Portuguesa de Cardiologia

  https://www.revportcardiol.org/pt-anomalous-pulmonary-venous-connection-an-articulo-S0870255116302700

  Patients with PAPVC are typically acyanotic and most commonly only mildly symptomatic or asymptomatic. Some authors have suggested that PAPVC becomes clinically significant only when 50% or more of the pulmonary blood flow returns anomalously. […] Imaging techniques, especially CT and MRI, have a pivotal role in the accurate characterization of these abnormalities, predicting outcomes and establishing appropriate preoperative planning. […] Unlike PAPVC, no catheter-corrective treatment is possible for TAPVC, although atrial septostomy is used in some patients when corrective surgery is delayed. […] In pediatric patients, PAPVC is usually treated if they have Qp:Qs of 1:1.5 or more as they are more likely to develop pulmonary hypertension and right ventricular failure, although this cutoff has not been subject to rigorous study. […] Thus, for patients with PAPVC who have already developed mild to moderate pulmonary hypertension, surgical repair is usually safe and effective, although catheter-guided and medical therapies may play an increasing role.

• #27 Abnormal chest X-ray leading to diagnosis of partial anomalous pulmonary venous connection

  https://atm.amegroups.org/article/view/18960/html

  Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiovascular condition that often goes undiagnosed due to lack of symptoms early in life. […] PAPVC has a reported incidence of 0.7%. However, as this congenital defect is commonly asymptomatic and only found during autopsy, the incidence is likely higher. […] Generally, no hemodynamic abnormality or clinical symptoms are noted unless 50% or more of the pulmonary blood flow is re-directed to the pulmonary vasculature. […] Diagnosis is confirmed with echocardiogram and cardiac CT. Cardiac MRI can further elucidate anatomy. […] With the growing use of imaging to both aid in diagnosis and treatment in patients with multiple comorbidities, it is important for clinicians and radiologists to become familiar with finding and evaluating congenital anomalies in adults. This case demonstrates the importance of accurately diagnosing anomalous venous connections on imaging, as they are primarily incidental findings but can have significant clinical impact. It is, therefore, crucial that once diagnosed, patients maintain follow-up with a cardiologist to monitor for signs of cardiac dysfunction.

• #28 Partial anomalous pulmonary venous return | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/partial-anomalous-pulmonary-venous-return?content_id=CON-20359714

  In partial anomalous pulmonary venous return, some of the pulmonary veins incorrectly send blood to the heart’s upper right chamber. That chamber is called the right atrium. Partial anomalous pulmonary venous return is a rare heart condition that’s present at birth. […] Most people with PAPVR have a hole between the upper heart chambers called sinus venosus atrial septal defect. The hole lets blood flow between the upper heart chambers. Other heart problems also may occur. […] Symptoms of partial anomalous pulmonary venous return (PAPVR) can include trouble breathing or fatigue. […] The exact cause of partial anomalous pulmonary venous return (PAPVR) is not known. […] What increases the risk of PAPVR is not well known. […] PAPVR may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] Most patients with partial anomalous pulmonary venous return (PAPVR) need surgery. Surgery to repair the heart may be needed if a lot of oxygen-rich and oxygen-poor blood mixes in the heart.

• #29 Management of Total Left Partial Anomalous Pulmonary Venous Connection

  https://www.fortunejournals.com/articles/management-of-total-left-partial-anomalous-pulmonary-venous-connection.html

  Partially anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more of the pulmonary veins are confluent with the systemic venous system. […] In an autopsy series of patients with congenital heart disease (CHD), right sided PAPVR was found in 0.4–0.7% of patients. […] There are several anatomic variations of PAPVR that have been described. […] Left sided PAPVR occurs an order of magnitude less than right sided PAPVR. […] Drainage directly to the coronary sinus accounts for 3% of patients with PAPVR and very few, include left sided PAPVR with all three left pulmonary veins draining to the coronary sinus. […] PAPVR historically does not have an associated untreated mortality rate, however, predisposes an individual to developing several complications such as fatigue, early exertional dyspnea, right ventricular dilation, tricuspid and pulmonary valve insufficiency, atrial arrhythmia, reduced ejection fraction, and pulmonary artery hypertension (PAH) which can be lethal.

• #30 Partial anomalous pulmonary venous return | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/partial-anomalous-pulmonary-venous-return?content_id=CON-20359714

  In partial anomalous pulmonary venous return, some of the pulmonary veins incorrectly send blood to the heart’s upper right chamber. That chamber is called the right atrium. Partial anomalous pulmonary venous return is a rare heart condition that’s present at birth. […] Most people with PAPVR have a hole between the upper heart chambers called sinus venosus atrial septal defect. The hole lets blood flow between the upper heart chambers. Other heart problems also may occur. […] Symptoms of partial anomalous pulmonary venous return (PAPVR) can include trouble breathing or fatigue. […] The exact cause of partial anomalous pulmonary venous return (PAPVR) is not known. […] What increases the risk of PAPVR is not well known. […] PAPVR may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] Most patients with partial anomalous pulmonary venous return (PAPVR) need surgery. Surgery to repair the heart may be needed if a lot of oxygen-rich and oxygen-poor blood mixes in the heart.

• #31 Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/1749-8090-6-126

  Partial anomalous pulmonary venous return (PAPVR) is defined as a left-to-right shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. […] All PAPVRs are left-to-right shunts, but unless more than 50% of the pulmonary flow drains to the right side of the heart clinical manifestations are rare. Dyspnea, fatigue, exercise intolerance, palpitations, syncope, atrial arrhythmias, right heart failure, and pulmonary hypertension may occur. […] PAPVR is usually diagnosed by transthoracic echocardiography (TTE) or transesophageal echocardiography (TEE) and catheter based angiography. […] Modern 128-slice multidetector computed tomography (MDCT) scans are accurate in defining ASDs and PAPVR. ECG-gated MDCT enables a non-invasive and rapid image acquisition with high spatial and temporal resolution, optimized contrast bolus timing, and wide anatomic coverage. The presence, course, number of anomalous veins, and associated cardiovascular defects can be reliably observed by MDCT angiography.

• #32 Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/1749-8090-6-126

  Partial anomalous pulmonary venous return (PAPVR) is defined as a left-to-right shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. […] All PAPVRs are left-to-right shunts, but unless more than 50% of the pulmonary flow drains to the right side of the heart clinical manifestations are rare. Dyspnea, fatigue, exercise intolerance, palpitations, syncope, atrial arrhythmias, right heart failure, and pulmonary hypertension may occur. […] PAPVR is usually diagnosed by transthoracic echocardiography (TTE) or transesophageal echocardiography (TEE) and catheter based angiography. […] Modern 128-slice multidetector computed tomography (MDCT) scans are accurate in defining ASDs and PAPVR. ECG-gated MDCT enables a non-invasive and rapid image acquisition with high spatial and temporal resolution, optimized contrast bolus timing, and wide anatomic coverage. The presence, course, number of anomalous veins, and associated cardiovascular defects can be reliably observed by MDCT angiography.

• #33 Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/1749-8090-6-126

  Partial anomalous pulmonary venous return (PAPVR) is defined as a left-to-right shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. […] All PAPVRs are left-to-right shunts, but unless more than 50% of the pulmonary flow drains to the right side of the heart clinical manifestations are rare. Dyspnea, fatigue, exercise intolerance, palpitations, syncope, atrial arrhythmias, right heart failure, and pulmonary hypertension may occur. […] PAPVR is usually diagnosed by transthoracic echocardiography (TTE) or transesophageal echocardiography (TEE) and catheter based angiography. […] Modern 128-slice multidetector computed tomography (MDCT) scans are accurate in defining ASDs and PAPVR. ECG-gated MDCT enables a non-invasive and rapid image acquisition with high spatial and temporal resolution, optimized contrast bolus timing, and wide anatomic coverage. The presence, course, number of anomalous veins, and associated cardiovascular defects can be reliably observed by MDCT angiography.

• #34 Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/1749-8090-6-126

  Partial anomalous pulmonary venous return (PAPVR) is defined as a left-to-right shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. […] All PAPVRs are left-to-right shunts, but unless more than 50% of the pulmonary flow drains to the right side of the heart clinical manifestations are rare. Dyspnea, fatigue, exercise intolerance, palpitations, syncope, atrial arrhythmias, right heart failure, and pulmonary hypertension may occur. […] PAPVR is usually diagnosed by transthoracic echocardiography (TTE) or transesophageal echocardiography (TEE) and catheter based angiography. […] Modern 128-slice multidetector computed tomography (MDCT) scans are accurate in defining ASDs and PAPVR. ECG-gated MDCT enables a non-invasive and rapid image acquisition with high spatial and temporal resolution, optimized contrast bolus timing, and wide anatomic coverage. The presence, course, number of anomalous veins, and associated cardiovascular defects can be reliably observed by MDCT angiography.

• #35 Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/1749-8090-6-126

  Partial anomalous pulmonary venous return (PAPVR) is defined as a left-to-right shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. […] All PAPVRs are left-to-right shunts, but unless more than 50% of the pulmonary flow drains to the right side of the heart clinical manifestations are rare. Dyspnea, fatigue, exercise intolerance, palpitations, syncope, atrial arrhythmias, right heart failure, and pulmonary hypertension may occur. […] PAPVR is usually diagnosed by transthoracic echocardiography (TTE) or transesophageal echocardiography (TEE) and catheter based angiography. […] Modern 128-slice multidetector computed tomography (MDCT) scans are accurate in defining ASDs and PAPVR. ECG-gated MDCT enables a non-invasive and rapid image acquisition with high spatial and temporal resolution, optimized contrast bolus timing, and wide anatomic coverage. The presence, course, number of anomalous veins, and associated cardiovascular defects can be reliably observed by MDCT angiography.

• #36 Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/1749-8090-6-126

  Partial anomalous pulmonary venous return (PAPVR) is defined as a left-to-right shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. […] All PAPVRs are left-to-right shunts, but unless more than 50% of the pulmonary flow drains to the right side of the heart clinical manifestations are rare. Dyspnea, fatigue, exercise intolerance, palpitations, syncope, atrial arrhythmias, right heart failure, and pulmonary hypertension may occur. […] PAPVR is usually diagnosed by transthoracic echocardiography (TTE) or transesophageal echocardiography (TEE) and catheter based angiography. […] Modern 128-slice multidetector computed tomography (MDCT) scans are accurate in defining ASDs and PAPVR. ECG-gated MDCT enables a non-invasive and rapid image acquisition with high spatial and temporal resolution, optimized contrast bolus timing, and wide anatomic coverage. The presence, course, number of anomalous veins, and associated cardiovascular defects can be reliably observed by MDCT angiography.

• #37 250. ACHD: Partial Anomalous Pulmonary Venous Return (PAPVR) with Dr. Ian Harris

  https://www.cardionerds.com/250-achd-partial-anomalous-pulmonary-venous-return-papvr-with-dr-ian-harris/

  Partial anomalous pulmonary venous return refers to anomalies in which one or more (but not all) of the pulmonary veins connects to a location other than the left atrium. This causes left to right shunting which may have hemodynamic and therefore clinical significance, warranting repair in some patients. […] Diagnosis is usually incidental on a cross sectional imaging such as CTA or CMR. […] A surgical correction is recommended for patients with PAPVR when functional capacity is impaired and RV enlargement is present, there is a net left-to-right shunt sufficiently large to cause physiological sequelae (aka: ratio of pulmonary flow (Qp) to systemic flow (Qs) is 1.5:1), PA systolic pressure is less than 50% systemic pressure and pulmonary venous resistance is less than one third of systemic venous resistance.

• #38 Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/1749-8090-6-126

  Partial anomalous pulmonary venous return (PAPVR) is defined as a left-to-right shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. […] All PAPVRs are left-to-right shunts, but unless more than 50% of the pulmonary flow drains to the right side of the heart clinical manifestations are rare. Dyspnea, fatigue, exercise intolerance, palpitations, syncope, atrial arrhythmias, right heart failure, and pulmonary hypertension may occur. […] PAPVR is usually diagnosed by transthoracic echocardiography (TTE) or transesophageal echocardiography (TEE) and catheter based angiography. […] Modern 128-slice multidetector computed tomography (MDCT) scans are accurate in defining ASDs and PAPVR. ECG-gated MDCT enables a non-invasive and rapid image acquisition with high spatial and temporal resolution, optimized contrast bolus timing, and wide anatomic coverage. The presence, course, number of anomalous veins, and associated cardiovascular defects can be reliably observed by MDCT angiography.

• #39 Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/1749-8090-6-126

  In recent years modern MDCT and MRI techniques have gained increasing importance in the non-invasive assessment of vascular pathologies of the chest. […] In our experience ECG-gated MDCT with fast data acquisition and multidimensional reconstructions offers excellent spatial resolution and the possibility to reliably depict intracardiac and pulmonary shunts.

• #40 Surgical repair of partial anomalous pulmonary venous connection in adulthood: A 4-dimensional flow magnetic resonance imaging postoperative evaluation – JTCVS Techniques

  https://www.jtcvstechniques.org/article/S2666-2507(23)00363-2/fulltext

  Partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital anomaly encountered in 0.4% to 0.7% of autopsies. […] PAPVC correction with the single-patch technique allows the deviation of the blood flow from the PVC to the LA. […] Five years after surgical repair, 4-D flow CMR allows the anatomic and blood flow evaluation. […] 4-D flow CMR is emerging as a promising, noninvasive technology in presurgical and postsurgical repair of congenital heart disease, as PAPVC with ASD of sinus venous type, and with IAS, or in association with other cardiac diseases. […] However, further studies are needed to validate the application of measurement to assess advanced hemodynamic parameters and detect overall clinical outcomes in congenital heart disease.

• #41 Surgical repair of partial anomalous pulmonary venous connection in adulthood: A 4-dimensional flow magnetic resonance imaging postoperative evaluation – JTCVS Techniques

  https://www.jtcvstechniques.org/article/S2666-2507(23)00363-2/fulltext

  Partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital anomaly encountered in 0.4% to 0.7% of autopsies. […] PAPVC correction with the single-patch technique allows the deviation of the blood flow from the PVC to the LA. […] Five years after surgical repair, 4-D flow CMR allows the anatomic and blood flow evaluation. […] 4-D flow CMR is emerging as a promising, noninvasive technology in presurgical and postsurgical repair of congenital heart disease, as PAPVC with ASD of sinus venous type, and with IAS, or in association with other cardiac diseases. […] However, further studies are needed to validate the application of measurement to assess advanced hemodynamic parameters and detect overall clinical outcomes in congenital heart disease.

• #42 Partial anomalous pulmonary venous return and atrial septal defect in adult patients detected with 128-slice multidetector computed tomography | Journal of Cardiothoracic Surgery | Full Text

  https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/1749-8090-6-126

  Partial anomalous pulmonary venous return (PAPVR) is defined as a left-to-right shunt where one or more, but not all, pulmonary veins drain into a systemic vein or the right atrium. […] All PAPVRs are left-to-right shunts, but unless more than 50% of the pulmonary flow drains to the right side of the heart clinical manifestations are rare. Dyspnea, fatigue, exercise intolerance, palpitations, syncope, atrial arrhythmias, right heart failure, and pulmonary hypertension may occur. […] PAPVR is usually diagnosed by transthoracic echocardiography (TTE) or transesophageal echocardiography (TEE) and catheter based angiography. […] Modern 128-slice multidetector computed tomography (MDCT) scans are accurate in defining ASDs and PAPVR. ECG-gated MDCT enables a non-invasive and rapid image acquisition with high spatial and temporal resolution, optimized contrast bolus timing, and wide anatomic coverage. The presence, course, number of anomalous veins, and associated cardiovascular defects can be reliably observed by MDCT angiography.

• #43 Partial anomalous pulmonary venous return – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/partial-anomalous-pulmonary-venous-return/cdc-20385691

  Partial anomalous pulmonary venous return is a rare heart condition that’s present at birth. That means it is a congenital heart defect. […] Partial anomalous pulmonary venous return (PAPVR) may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] A person with partial anomalous pulmonary venous return needs regular health checkups for life to check for complications. It’s best to see a doctor who is trained in congenital heart diseases. This type of doctor is called a congenital cardiologist.

• #44 Partial anomalous pulmonary venous return

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20359714

  Partial anomalous pulmonary venous return is a rare heart problem that’s present at birth. That means it is a congenital heart defect. […] A child born with Turner syndrome has an increased risk of PAPVR. […] If PAPVR occurs with other heart problems, it may be diagnosed soon after birth. If the condition is mild, it may not be diagnosed until adulthood. […] A person with partial anomalous pulmonary venous return needs regular health checkups for life to check for complications. It’s best to see a health care provider who is trained in congenital heart diseases. This type of provider is called a congenital cardiologist.

• #45 Partial anomalous pulmonary venous return – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/partial-anomalous-pulmonary-venous-return/cdc-20385691

  Partial anomalous pulmonary venous return is a rare heart condition that’s present at birth. That means it is a congenital heart defect. […] Partial anomalous pulmonary venous return (PAPVR) may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] A person with partial anomalous pulmonary venous return needs regular health checkups for life to check for complications. It’s best to see a doctor who is trained in congenital heart diseases. This type of doctor is called a congenital cardiologist.

• #46 Lower Extremity Edema: Long Term Complication of Partial Anomalous Pulmonary Venous Return Repair | Society for Cardiovascular Magnetic Resonance

  https://scmr.org/cases-of-scmr/number-18-03/

  Partial anomalous pulmonary venous return (PAPVR) occurs in 0.1 – 0.7% of the general population and usually affects the right sided pulmonary veins. PAPVR is typically associated with a sinus venous atrial septal defect. […] If there are multiple anomalous pulmonary veins draining into the right atrium, the veins can cause right sided volume overload and is an indication for repair. […] Diagnosis can occur in either childhood or adulthood. Adults may present with right ventricular enlargement or may be found incidentally on advanced cardiac imaging including cardiac MRI. […] The goal of partial anomalous venous return repair is to divert the pulmonary veins back to the left atrium and systemic veins to the right atrium. […] Long term complications after anomalous pulmonary venous return repair include SVC stenosis, pulmonary vein obstruction, sinus node dysfunction or atrial arrhythmias. […] Our patient has a SVC obstruction at the azygous arch, with upper body venous return via the hemiazygous vein to the inferior vena cava.

• #47 Lower Extremity Edema: Long Term Complication of Partial Anomalous Pulmonary Venous Return Repair | Society for Cardiovascular Magnetic Resonance

  https://scmr.org/cases-of-scmr/number-18-03/

  Partial anomalous pulmonary venous return (PAPVR) occurs in 0.1 – 0.7% of the general population and usually affects the right sided pulmonary veins. PAPVR is typically associated with a sinus venous atrial septal defect. […] If there are multiple anomalous pulmonary veins draining into the right atrium, the veins can cause right sided volume overload and is an indication for repair. […] Diagnosis can occur in either childhood or adulthood. Adults may present with right ventricular enlargement or may be found incidentally on advanced cardiac imaging including cardiac MRI. […] The goal of partial anomalous venous return repair is to divert the pulmonary veins back to the left atrium and systemic veins to the right atrium. […] Long term complications after anomalous pulmonary venous return repair include SVC stenosis, pulmonary vein obstruction, sinus node dysfunction or atrial arrhythmias. […] Our patient has a SVC obstruction at the azygous arch, with upper body venous return via the hemiazygous vein to the inferior vena cava.

• #48 Lower Extremity Edema: Long Term Complication of Partial Anomalous Pulmonary Venous Return Repair | Society for Cardiovascular Magnetic Resonance

  https://scmr.org/cases-of-scmr/number-18-03/

  Partial anomalous pulmonary venous return (PAPVR) occurs in 0.1 – 0.7% of the general population and usually affects the right sided pulmonary veins. PAPVR is typically associated with a sinus venous atrial septal defect. […] If there are multiple anomalous pulmonary veins draining into the right atrium, the veins can cause right sided volume overload and is an indication for repair. […] Diagnosis can occur in either childhood or adulthood. Adults may present with right ventricular enlargement or may be found incidentally on advanced cardiac imaging including cardiac MRI. […] The goal of partial anomalous venous return repair is to divert the pulmonary veins back to the left atrium and systemic veins to the right atrium. […] Long term complications after anomalous pulmonary venous return repair include SVC stenosis, pulmonary vein obstruction, sinus node dysfunction or atrial arrhythmias. […] Our patient has a SVC obstruction at the azygous arch, with upper body venous return via the hemiazygous vein to the inferior vena cava.

• #49 Management of Total Left Partial Anomalous Pulmonary Venous Connection

  https://www.fortunejournals.com/articles/management-of-total-left-partial-anomalous-pulmonary-venous-connection.html

  Partially anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more of the pulmonary veins are confluent with the systemic venous system. […] In an autopsy series of patients with congenital heart disease (CHD), right sided PAPVR was found in 0.4–0.7% of patients. […] There are several anatomic variations of PAPVR that have been described. […] Left sided PAPVR occurs an order of magnitude less than right sided PAPVR. […] Drainage directly to the coronary sinus accounts for 3% of patients with PAPVR and very few, include left sided PAPVR with all three left pulmonary veins draining to the coronary sinus. […] PAPVR historically does not have an associated untreated mortality rate, however, predisposes an individual to developing several complications such as fatigue, early exertional dyspnea, right ventricular dilation, tricuspid and pulmonary valve insufficiency, atrial arrhythmia, reduced ejection fraction, and pulmonary artery hypertension (PAH) which can be lethal.

• #50 Management of Total Left Partial Anomalous Pulmonary Venous Connection

  https://www.fortunejournals.com/articles/management-of-total-left-partial-anomalous-pulmonary-venous-connection.html

  Partially anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more of the pulmonary veins are confluent with the systemic venous system. […] In an autopsy series of patients with congenital heart disease (CHD), right sided PAPVR was found in 0.4–0.7% of patients. […] There are several anatomic variations of PAPVR that have been described. […] Left sided PAPVR occurs an order of magnitude less than right sided PAPVR. […] Drainage directly to the coronary sinus accounts for 3% of patients with PAPVR and very few, include left sided PAPVR with all three left pulmonary veins draining to the coronary sinus. […] PAPVR historically does not have an associated untreated mortality rate, however, predisposes an individual to developing several complications such as fatigue, early exertional dyspnea, right ventricular dilation, tricuspid and pulmonary valve insufficiency, atrial arrhythmia, reduced ejection fraction, and pulmonary artery hypertension (PAH) which can be lethal.

• #51 Management of Total Left Partial Anomalous Pulmonary Venous Connection

  https://www.fortunejournals.com/articles/management-of-total-left-partial-anomalous-pulmonary-venous-connection.html

  Partially anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more of the pulmonary veins are confluent with the systemic venous system. […] In an autopsy series of patients with congenital heart disease (CHD), right sided PAPVR was found in 0.4–0.7% of patients. […] There are several anatomic variations of PAPVR that have been described. […] Left sided PAPVR occurs an order of magnitude less than right sided PAPVR. […] Drainage directly to the coronary sinus accounts for 3% of patients with PAPVR and very few, include left sided PAPVR with all three left pulmonary veins draining to the coronary sinus. […] PAPVR historically does not have an associated untreated mortality rate, however, predisposes an individual to developing several complications such as fatigue, early exertional dyspnea, right ventricular dilation, tricuspid and pulmonary valve insufficiency, atrial arrhythmia, reduced ejection fraction, and pulmonary artery hypertension (PAH) which can be lethal.

• #52 Management of Total Left Partial Anomalous Pulmonary Venous Connection

  https://www.fortunejournals.com/articles/management-of-total-left-partial-anomalous-pulmonary-venous-connection.html

  Partially anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more of the pulmonary veins are confluent with the systemic venous system. […] In an autopsy series of patients with congenital heart disease (CHD), right sided PAPVR was found in 0.4–0.7% of patients. […] There are several anatomic variations of PAPVR that have been described. […] Left sided PAPVR occurs an order of magnitude less than right sided PAPVR. […] Drainage directly to the coronary sinus accounts for 3% of patients with PAPVR and very few, include left sided PAPVR with all three left pulmonary veins draining to the coronary sinus. […] PAPVR historically does not have an associated untreated mortality rate, however, predisposes an individual to developing several complications such as fatigue, early exertional dyspnea, right ventricular dilation, tricuspid and pulmonary valve insufficiency, atrial arrhythmia, reduced ejection fraction, and pulmonary artery hypertension (PAH) which can be lethal.

• #53 Management of Total Left Partial Anomalous Pulmonary Venous Connection

  https://www.fortunejournals.com/articles/management-of-total-left-partial-anomalous-pulmonary-venous-connection.html

  Partially anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more of the pulmonary veins are confluent with the systemic venous system. […] In an autopsy series of patients with congenital heart disease (CHD), right sided PAPVR was found in 0.4–0.7% of patients. […] There are several anatomic variations of PAPVR that have been described. […] Left sided PAPVR occurs an order of magnitude less than right sided PAPVR. […] Drainage directly to the coronary sinus accounts for 3% of patients with PAPVR and very few, include left sided PAPVR with all three left pulmonary veins draining to the coronary sinus. […] PAPVR historically does not have an associated untreated mortality rate, however, predisposes an individual to developing several complications such as fatigue, early exertional dyspnea, right ventricular dilation, tricuspid and pulmonary valve insufficiency, atrial arrhythmia, reduced ejection fraction, and pulmonary artery hypertension (PAH) which can be lethal.

• #54 Management of Total Left Partial Anomalous Pulmonary Venous Connection

  https://www.fortunejournals.com/articles/management-of-total-left-partial-anomalous-pulmonary-venous-connection.html

  Partially anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more of the pulmonary veins are confluent with the systemic venous system. […] In an autopsy series of patients with congenital heart disease (CHD), right sided PAPVR was found in 0.4–0.7% of patients. […] There are several anatomic variations of PAPVR that have been described. […] Left sided PAPVR occurs an order of magnitude less than right sided PAPVR. […] Drainage directly to the coronary sinus accounts for 3% of patients with PAPVR and very few, include left sided PAPVR with all three left pulmonary veins draining to the coronary sinus. […] PAPVR historically does not have an associated untreated mortality rate, however, predisposes an individual to developing several complications such as fatigue, early exertional dyspnea, right ventricular dilation, tricuspid and pulmonary valve insufficiency, atrial arrhythmia, reduced ejection fraction, and pulmonary artery hypertension (PAH) which can be lethal.

• #55 Management of Total Left Partial Anomalous Pulmonary Venous Connection

  https://www.fortunejournals.com/articles/management-of-total-left-partial-anomalous-pulmonary-venous-connection.html

  Partially anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more of the pulmonary veins are confluent with the systemic venous system. […] In an autopsy series of patients with congenital heart disease (CHD), right sided PAPVR was found in 0.4–0.7% of patients. […] There are several anatomic variations of PAPVR that have been described. […] Left sided PAPVR occurs an order of magnitude less than right sided PAPVR. […] Drainage directly to the coronary sinus accounts for 3% of patients with PAPVR and very few, include left sided PAPVR with all three left pulmonary veins draining to the coronary sinus. […] PAPVR historically does not have an associated untreated mortality rate, however, predisposes an individual to developing several complications such as fatigue, early exertional dyspnea, right ventricular dilation, tricuspid and pulmonary valve insufficiency, atrial arrhythmia, reduced ejection fraction, and pulmonary artery hypertension (PAH) which can be lethal.

• #56 Management of Total Left Partial Anomalous Pulmonary Venous Connection

  https://www.fortunejournals.com/articles/management-of-total-left-partial-anomalous-pulmonary-venous-connection.html

  Partially anomalous pulmonary venous return (PAPVR) is a rare congenital anomaly in which one or more of the pulmonary veins are confluent with the systemic venous system. […] In an autopsy series of patients with congenital heart disease (CHD), right sided PAPVR was found in 0.4–0.7% of patients. […] There are several anatomic variations of PAPVR that have been described. […] Left sided PAPVR occurs an order of magnitude less than right sided PAPVR. […] Drainage directly to the coronary sinus accounts for 3% of patients with PAPVR and very few, include left sided PAPVR with all three left pulmonary veins draining to the coronary sinus. […] PAPVR historically does not have an associated untreated mortality rate, however, predisposes an individual to developing several complications such as fatigue, early exertional dyspnea, right ventricular dilation, tricuspid and pulmonary valve insufficiency, atrial arrhythmia, reduced ejection fraction, and pulmonary artery hypertension (PAH) which can be lethal.

• #57 Pulmonary Arterial Hypertension Secondary to Partial Anomalous Pulmonary Venous Return in an Elderly Patient | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-pulmonary-arterial-hypertension-secondary-partial-articulo-S1579212916302816

  Partial anomalous pulmonary venous return (PAPVR) is an uncommon congenital abnormality that can be diagnosed in adult life, although it is more often detected during childhood. It consists of abnormal, incomplete pulmonary venous return to the systemic venous circulation (superior vena cava, azygos vein, coronary sinus, brachiocephalic vein, inferior vena cava, etc.) causing left-to-right shunt. PAPVR is more common in the right side, and is often associated with other congenital abnormalities, such as heart defects (particularly atrial septal defect), or an abnormally developed airway. […] PAPVR is a rare congenital abnormality that is usually diagnosed in children, although it may also be detected in adults, particularly if they develop PAH. Less than 10% of PAPVRs are left-sided, and up to 80% are associated with atrial septal defect (ASD). This case reminds us that PAPVR, if untreated, leads to volume overload of the right heart, tricuspid valve insufficiency, PAH, and finally right ventricular failure. PAPVR should always be suspected, even in elderly patients, in cases with primary or unexplained PAH.

• #58 Partial anomalous pulmonary venous return, the neglected congeni

  https://www.openaccessjournals.com/articles/partial-anomalous-pulmonary-venous-return-the–neglected-congenital-malformation-catheterization–assisted-echocardiography-16598.html

  Partial Anomalous Pulmonary Venous Return (PAPVR) is a rare congenital heart disease that can lead to major complications in adulthood, such as Pulmonary Arterial Hypertension (PAH). […] There is a lack of follow-up for patients newly diagnosed with PAPVR and the inadequate management of these patients in current clinical practice, both in terms of screening for PAH and identifying at-risk profiles, as well as the lack of recourse to Right Heart Catheterization (RHC) when this is recommended. […] PAPVR is therefore a disease that seems to be poorly known by the medical professionals even though its prevalence is similar to the atrial septal defects (0.2%) and the consequences can be dramatic, particularly if patients develop PAH, or even its extreme form: Eisenmenger syndrome. […] There is therefore a real need to raise awareness of this congenital malformation and its consequences.

• #59 Lower Extremity Edema: Long Term Complication of Partial Anomalous Pulmonary Venous Return Repair | Society for Cardiovascular Magnetic Resonance

  https://scmr.org/cases-of-scmr/number-18-03/

  Partial anomalous pulmonary venous return (PAPVR) occurs in 0.1 – 0.7% of the general population and usually affects the right sided pulmonary veins. PAPVR is typically associated with a sinus venous atrial septal defect. […] If there are multiple anomalous pulmonary veins draining into the right atrium, the veins can cause right sided volume overload and is an indication for repair. […] Diagnosis can occur in either childhood or adulthood. Adults may present with right ventricular enlargement or may be found incidentally on advanced cardiac imaging including cardiac MRI. […] The goal of partial anomalous venous return repair is to divert the pulmonary veins back to the left atrium and systemic veins to the right atrium. […] Long term complications after anomalous pulmonary venous return repair include SVC stenosis, pulmonary vein obstruction, sinus node dysfunction or atrial arrhythmias. […] Our patient has a SVC obstruction at the azygous arch, with upper body venous return via the hemiazygous vein to the inferior vena cava.

• #60 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #61 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #62 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #63 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #64 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #65 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #66 Partial Anomalous Pulmonary Venous Return Presenting in Adults: A Case Series With Review of Literature

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7331899/

  Partial anomalous pulmonary venous return (PAPVR) is a congenital anomaly in which some of the pulmonary veins drain erroneously into the superior vena cava (SVC) or directly into the right atrium (RA). […] The overall incidence of PAPVR is 0.7% based on an autopsy series. However, a more recent retrospective study examining chest CT showed a disease prevalence of 0.1%. […] The management of PAPVR is individualized based on clinical presentation and degree of the left-to-right shunt. Asymptomatic patients with small left-to-right shunt due to PAPVR generally do not require surgery. Surgical repair is indicated for symptomatic patients with a hemodynamically significant left-to-right shunt (Qp/Qs 2) on cardiac MRI. […] In PAPVR patients with or without associated ASD, the decision for surgical repair depends on symptoms, shunt fraction, recurrent pulmonary infections, and concurrent indication for cardiac surgery.

• #67 Partial Anomalous Pulmonary Venous Return Presenting in Adults: A Case Series With Review of Literature

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7331899/

  Partial anomalous pulmonary venous return (PAPVR) is a congenital anomaly in which some of the pulmonary veins drain erroneously into the superior vena cava (SVC) or directly into the right atrium (RA). […] The overall incidence of PAPVR is 0.7% based on an autopsy series. However, a more recent retrospective study examining chest CT showed a disease prevalence of 0.1%. […] The management of PAPVR is individualized based on clinical presentation and degree of the left-to-right shunt. Asymptomatic patients with small left-to-right shunt due to PAPVR generally do not require surgery. Surgical repair is indicated for symptomatic patients with a hemodynamically significant left-to-right shunt (Qp/Qs 2) on cardiac MRI. […] In PAPVR patients with or without associated ASD, the decision for surgical repair depends on symptoms, shunt fraction, recurrent pulmonary infections, and concurrent indication for cardiac surgery.

• #68 Partial Anomalous Pulmonary Venous Return Presenting in Adults: A Case Series With Review of Literature

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7331899/

  Partial anomalous pulmonary venous return (PAPVR) is a congenital anomaly in which some of the pulmonary veins drain erroneously into the superior vena cava (SVC) or directly into the right atrium (RA). […] The overall incidence of PAPVR is 0.7% based on an autopsy series. However, a more recent retrospective study examining chest CT showed a disease prevalence of 0.1%. […] The management of PAPVR is individualized based on clinical presentation and degree of the left-to-right shunt. Asymptomatic patients with small left-to-right shunt due to PAPVR generally do not require surgery. Surgical repair is indicated for symptomatic patients with a hemodynamically significant left-to-right shunt (Qp/Qs 2) on cardiac MRI. […] In PAPVR patients with or without associated ASD, the decision for surgical repair depends on symptoms, shunt fraction, recurrent pulmonary infections, and concurrent indication for cardiac surgery.

• #69 Management of Total Left Partial Anomalous Pulmonary Venous Connection

  https://www.fortunejournals.com/articles/management-of-total-left-partial-anomalous-pulmonary-venous-connection.html

  PAPVR is often associated with congenital syndromes such as Turner’s. […] Patients with or without symptoms and PAPVR with a Qp: Qs >1.5, moderate to severe tricuspid regurgitation (TR), pulmonary insufficiency, and dysfunction of the right ventricle are all indicated for surgical treatment to mitigate progressive right ventricular failure and irreversible pulmonary vascular disease. […] Reports of total left sided PAPVR are very scant, and we describe a simple functional novel repair strategy in contrast to prior reports. […] Left sided partial anomalous pulmonary venous return of all three left pulmonary veins to the coronary sinus is a rare anatomic anomaly thoughtful surgical correction is required when treating these unique PAPVR lesions. […] Partial anomalous pulmonary venous return (PAPVR) of all left pulmonary veins draining into the coronary sinus is a very rare congenital lesion discovered in adulthood, and can be treated with simple repair by coronary sinus unroofing and patch closure of atrial septal closure.

• #70 Anomalous pulmonary venous connection: An underestimated entity | Revista Portuguesa de Cardiologia

  https://www.revportcardiol.org/pt-anomalous-pulmonary-venous-connection-an-articulo-S0870255116302700

  Patients with PAPVC are typically acyanotic and most commonly only mildly symptomatic or asymptomatic. Some authors have suggested that PAPVC becomes clinically significant only when 50% or more of the pulmonary blood flow returns anomalously. […] Imaging techniques, especially CT and MRI, have a pivotal role in the accurate characterization of these abnormalities, predicting outcomes and establishing appropriate preoperative planning. […] Unlike PAPVC, no catheter-corrective treatment is possible for TAPVC, although atrial septostomy is used in some patients when corrective surgery is delayed. […] In pediatric patients, PAPVC is usually treated if they have Qp:Qs of 1:1.5 or more as they are more likely to develop pulmonary hypertension and right ventricular failure, although this cutoff has not been subject to rigorous study. […] Thus, for patients with PAPVC who have already developed mild to moderate pulmonary hypertension, surgical repair is usually safe and effective, although catheter-guided and medical therapies may play an increasing role.

• #71 Partial anomalous pulmonary venous return – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/partial-anomalous-pulmonary-venous-return/cdc-20385691

  Partial anomalous pulmonary venous return is a rare heart condition that’s present at birth. That means it is a congenital heart defect. […] Partial anomalous pulmonary venous return (PAPVR) may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] A person with partial anomalous pulmonary venous return needs regular health checkups for life to check for complications. It’s best to see a doctor who is trained in congenital heart diseases. This type of doctor is called a congenital cardiologist.

• #72 Partial anomalous pulmonary venous return – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/partial-anomalous-pulmonary-venous-return/cdc-20385691

  Partial anomalous pulmonary venous return is a rare heart condition that’s present at birth. That means it is a congenital heart defect. […] Partial anomalous pulmonary venous return (PAPVR) may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] A person with partial anomalous pulmonary venous return needs regular health checkups for life to check for complications. It’s best to see a doctor who is trained in congenital heart diseases. This type of doctor is called a congenital cardiologist.

• #73 Partial anomalous pulmonary venous return

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20359714

  Partial anomalous pulmonary venous return is a rare heart problem that’s present at birth. That means it is a congenital heart defect. […] A child born with Turner syndrome has an increased risk of PAPVR. […] If PAPVR occurs with other heart problems, it may be diagnosed soon after birth. If the condition is mild, it may not be diagnosed until adulthood. […] A person with partial anomalous pulmonary venous return needs regular health checkups for life to check for complications. It’s best to see a health care provider who is trained in congenital heart diseases. This type of provider is called a congenital cardiologist.

• #74 Partial anomalous pulmonary venous return – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/partial-anomalous-pulmonary-venous-return/cdc-20385691

  Partial anomalous pulmonary venous return is a rare heart condition that’s present at birth. That means it is a congenital heart defect. […] Partial anomalous pulmonary venous return (PAPVR) may be diagnosed soon after birth. Other times, the condition is not discovered until later in life. […] A person with partial anomalous pulmonary venous return needs regular health checkups for life to check for complications. It’s best to see a doctor who is trained in congenital heart diseases. This type of doctor is called a congenital cardiologist.

• #75 Partial anomalous pulmonary venous return

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20359714

  Partial anomalous pulmonary venous return is a rare heart problem that’s present at birth. That means it is a congenital heart defect. […] A child born with Turner syndrome has an increased risk of PAPVR. […] If PAPVR occurs with other heart problems, it may be diagnosed soon after birth. If the condition is mild, it may not be diagnosed until adulthood. […] A person with partial anomalous pulmonary venous return needs regular health checkups for life to check for complications. It’s best to see a health care provider who is trained in congenital heart diseases. This type of provider is called a congenital cardiologist.

• #76 Isolated Partial Anomalous Pulmonary Venous Connection: Development of Volume Overload and Elevated Estimated Pulmonary Pressure in Adults – Journal of Clinical Imaging Science

  https://clinicalimagingscience.org/isolated-partial-anomalous-pulmonary-venous-connection-development-of-volume-overload-and-elevated-estimated-pulmonary-pressure-in-adults/

  Partial anomalous pulmonary venous connection (PAPVC) is one of the rare congenital cardiac diseases with a prevalence of 0.40.7% of autopsies. The prevalence of a partial anomalous pulmonary venous connection is 0.2% in computed tomography (CT) among adults. […] Among the 110 subjects, 54 (49%) had isolated PAPVC. Of 54, 26 patients had volume overload of RA/RV or elevated estimated systolic pulmonary artery pressure. […] We found statistically significant association (P = 0.02) between isolated PAPVC in adults and pulmonary hypertension. […] Isolated PAPVC has association with the development of pulmonary hypertension in adults, approaching statistically significant p value. […] The prevalence of PAPVC in adults was 0.2% on computed tomography (CT) scans. […] The right-sided cardiac volume overload due to PAPVC can manifest from early childhood to late adulthood with a varying spectrum of severity.

• #77 Isolated Partial Anomalous Pulmonary Venous Connection: Development of Volume Overload and Elevated Estimated Pulmonary Pressure in Adults – Journal of Clinical Imaging Science

  https://clinicalimagingscience.org/isolated-partial-anomalous-pulmonary-venous-connection-development-of-volume-overload-and-elevated-estimated-pulmonary-pressure-in-adults/

  Partial anomalous pulmonary venous connection (PAPVC) is one of the rare congenital cardiac diseases with a prevalence of 0.40.7% of autopsies. The prevalence of a partial anomalous pulmonary venous connection is 0.2% in computed tomography (CT) among adults. […] Among the 110 subjects, 54 (49%) had isolated PAPVC. Of 54, 26 patients had volume overload of RA/RV or elevated estimated systolic pulmonary artery pressure. […] We found statistically significant association (P = 0.02) between isolated PAPVC in adults and pulmonary hypertension. […] Isolated PAPVC has association with the development of pulmonary hypertension in adults, approaching statistically significant p value. […] The prevalence of PAPVC in adults was 0.2% on computed tomography (CT) scans. […] The right-sided cardiac volume overload due to PAPVC can manifest from early childhood to late adulthood with a varying spectrum of severity.

• #78 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #79 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #80 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #81 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #82 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #83 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #84 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #85 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. […] An understanding of the various anatomical subtypes, physiological assessment, clinical and diagnostic evaluation is important for medical and surgical management. […] The incidence of PAPVC is higher in patients with Turners syndrome. […] The risk factors for increased mortality include younger age at surgery, associated cardiac lesions, hypoplastic pulmonary veins, postoperative pulmonary hypertension, and postoperative pulmonary venous obstruction. […] An interprofessional team of specialists including a primary pediatrician, cardiologist, cardiovascular surgeon, radiologist, nurse, and pharmacist is vital for the delivery of appropriate care and follow up of these patients.

• #86 Partial anomalous pulmonary venous return, the neglected congeni

  https://www.openaccessjournals.com/articles/partial-anomalous-pulmonary-venous-return-the–neglected-congenital-malformation-catheterization–assisted-echocardiography-16598.html

  Partial Anomalous Pulmonary Venous Return (PAPVR) is a rare congenital heart disease that can lead to major complications in adulthood, such as Pulmonary Arterial Hypertension (PAH). […] There is a lack of follow-up for patients newly diagnosed with PAPVR and the inadequate management of these patients in current clinical practice, both in terms of screening for PAH and identifying at-risk profiles, as well as the lack of recourse to Right Heart Catheterization (RHC) when this is recommended. […] PAPVR is therefore a disease that seems to be poorly known by the medical professionals even though its prevalence is similar to the atrial septal defects (0.2%) and the consequences can be dramatic, particularly if patients develop PAH, or even its extreme form: Eisenmenger syndrome. […] There is therefore a real need to raise awareness of this congenital malformation and its consequences.

• #87 Partial anomalous pulmonary venous return, the neglected congeni

  https://www.openaccessjournals.com/articles/partial-anomalous-pulmonary-venous-return-the–neglected-congenital-malformation-catheterization–assisted-echocardiography-16598.html

  Partial Anomalous Pulmonary Venous Return (PAPVR) is a rare congenital heart disease that can lead to major complications in adulthood, such as Pulmonary Arterial Hypertension (PAH). […] There is a lack of follow-up for patients newly diagnosed with PAPVR and the inadequate management of these patients in current clinical practice, both in terms of screening for PAH and identifying at-risk profiles, as well as the lack of recourse to Right Heart Catheterization (RHC) when this is recommended. […] PAPVR is therefore a disease that seems to be poorly known by the medical professionals even though its prevalence is similar to the atrial septal defects (0.2%) and the consequences can be dramatic, particularly if patients develop PAH, or even its extreme form: Eisenmenger syndrome. […] There is therefore a real need to raise awareness of this congenital malformation and its consequences.

• #88 Partial anomalous pulmonary venous return, the neglected congeni

  https://www.openaccessjournals.com/articles/partial-anomalous-pulmonary-venous-return-the–neglected-congenital-malformation-catheterization–assisted-echocardiography-16598.html

  Partial Anomalous Pulmonary Venous Return (PAPVR) is a rare congenital heart disease that can lead to major complications in adulthood, such as Pulmonary Arterial Hypertension (PAH). […] There is a lack of follow-up for patients newly diagnosed with PAPVR and the inadequate management of these patients in current clinical practice, both in terms of screening for PAH and identifying at-risk profiles, as well as the lack of recourse to Right Heart Catheterization (RHC) when this is recommended. […] PAPVR is therefore a disease that seems to be poorly known by the medical professionals even though its prevalence is similar to the atrial septal defects (0.2%) and the consequences can be dramatic, particularly if patients develop PAH, or even its extreme form: Eisenmenger syndrome. […] There is therefore a real need to raise awareness of this congenital malformation and its consequences.

• #89 Partial anomalous pulmonary venous return, the neglected congeni

  https://www.openaccessjournals.com/articles/partial-anomalous-pulmonary-venous-return-the–neglected-congenital-malformation-catheterization–assisted-echocardiography-16598.html

  Partial Anomalous Pulmonary Venous Return (PAPVR) is a rare congenital heart disease that can lead to major complications in adulthood, such as Pulmonary Arterial Hypertension (PAH). […] There is a lack of follow-up for patients newly diagnosed with PAPVR and the inadequate management of these patients in current clinical practice, both in terms of screening for PAH and identifying at-risk profiles, as well as the lack of recourse to Right Heart Catheterization (RHC) when this is recommended. […] PAPVR is therefore a disease that seems to be poorly known by the medical professionals even though its prevalence is similar to the atrial septal defects (0.2%) and the consequences can be dramatic, particularly if patients develop PAH, or even its extreme form: Eisenmenger syndrome. […] There is therefore a real need to raise awareness of this congenital malformation and its consequences.

• #90 Partial anomalous pulmonary venous return, the neglected congeni

  https://www.openaccessjournals.com/articles/partial-anomalous-pulmonary-venous-return-the–neglected-congenital-malformation-catheterization–assisted-echocardiography-16598.html

  Partial Anomalous Pulmonary Venous Return (PAPVR) is a rare congenital heart disease that can lead to major complications in adulthood, such as Pulmonary Arterial Hypertension (PAH). […] There is a lack of follow-up for patients newly diagnosed with PAPVR and the inadequate management of these patients in current clinical practice, both in terms of screening for PAH and identifying at-risk profiles, as well as the lack of recourse to Right Heart Catheterization (RHC) when this is recommended. […] PAPVR is therefore a disease that seems to be poorly known by the medical professionals even though its prevalence is similar to the atrial septal defects (0.2%) and the consequences can be dramatic, particularly if patients develop PAH, or even its extreme form: Eisenmenger syndrome. […] There is therefore a real need to raise awareness of this congenital malformation and its consequences.

• #91 Partial anomalous pulmonary venous return, the neglected congeni

  https://www.openaccessjournals.com/articles/partial-anomalous-pulmonary-venous-return-the–neglected-congenital-malformation-catheterization–assisted-echocardiography-16598.html

  PAPVR is a rare congenital heart disease that unfortunately remains neglected and poorly managed to date, both in terms of medical follow-up after diagnosis and screening for complications Pulmonary Arterial Hypertension (PAH) and risk factors for developing PAH. More extensive use of current guidelines is essential in the assessment of these patients and referral to RHC should be systematic.

• #92 Partial anomalous pulmonary venous return, the neglected congeni

  https://www.openaccessjournals.com/articles/partial-anomalous-pulmonary-venous-return-the–neglected-congenital-malformation-catheterization–assisted-echocardiography-16598.html

  PAPVR is a rare congenital heart disease that unfortunately remains neglected and poorly managed to date, both in terms of medical follow-up after diagnosis and screening for complications Pulmonary Arterial Hypertension (PAH) and risk factors for developing PAH. More extensive use of current guidelines is essential in the assessment of these patients and referral to RHC should be systematic.

• #93 Partial Anomalous Pulmonary Venous Return: To Fix or Not to Fix?

  https://scholarlycommons.hcahealthcare.com/northtexas2024/18/

  Recent advancements in sophisticated diagnostic imaging and greater knowledge of this anatomical variation have increased the frequency with which this condition is diagnosed. […] With the suspected increase in incidence, it may be time for the creation of formal guidelines on a global scale instead of relying on recommendations on consensus of symptomatology.

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