Materiały źródłowe

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

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

  In total anomalous pulmonary venous return (TAPVR), the pulmonary veins incorrectly send blood to the heart’s upper right chamber. That chamber is called the right atrium. As a result, oxygen-rich blood mixes with oxygen-poor blood, as shown in purple. In a typical heart, shown on the left, oxygen-rich blood flows from the pulmonary veins to the upper left chamber, also called the left atrium. […] Total anomalous pulmonary venous return (TAPVR) is a rare heart condition that’s present at birth. That means it’s a congenital heart defect. […] In this heart condition, the lung blood vessels, called the pulmonary veins, attach to the wrong place in the heart. […] The type of TAPVR depends on where the veins connect. Most children born with TAPVR have no family history of congenital heart disease.

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

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

  Total anomalous pulmonary venous return (pronounced TOHT-l uh-NOM-uh-luh-s PUHL-muh-ner-ee VEE-nuh-s ri-TURN) or T-A-P-V-R is a congenital heart defect. […] TAPVR occurs when the pulmonary veins connect to the heart by way of an abnormal (anomalous) connection. […] The causes of TAPVR among most babies are unknown. Some babies have heart defects because of changes in their genes or chromosomes. A combination of genes and other risk factors may increase the risk for TAPVR. These factors can include things in a mother’s environment, what she eats or drinks, or the medicines she uses.

• #3 Total anomalous pulmonary venous return: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001115.htm

  Total anomalous pulmonary venous return (TAPVR) is a heart disease in which the 4 veins that take blood from the lungs to the heart do not attach normally to the left atrium (left upper chamber of the heart). Instead, they attach to another blood vessel or the wrong part of the heart. It is present at birth (congenital heart disease). […] The cause of total anomalous pulmonary venous return is unknown. […] For the infant to live, an atrial septal defect (ASD) or patent foramen ovale (passage between the left and right atria) must exist to allow oxygenated blood to flow to the left side of the heart and the rest of the body. […] How severe this condition is depends on whether the pulmonary veins are blocked or obstructed as they drain. Obstructed TAPVR causes symptoms early in life and can be deadly very quickly if it is not found and corrected with surgery. […] Some inherited factors may play a role in congenital heart disease. Many family members may be affected. If you are planning to get pregnant, talk to your provider about screening for genetic diseases.

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

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

  Total anomalous pulmonary venous return (TAPVR), also known as total anomalous pulmonary venous connection (TAPVC), is a rare heart defect in which the blood vessels that drain the lungs (pulmonary veins) are not connected normally to the heart. Instead, the pulmonary veins are redirected abnormally to other chambers of the heart. About 1 in every 20,000 babies is born with TAPVR. […] In many cases, we don’t know what causes TAPVR. It occurs because of abnormal development of the heart’s pulmonary veins during early fetal growth. Some congenital heart defects may have a genetic link, causing heart problems to occur more often in certain families. Most often, though, this heart defect occurs by chance, with no clear reason for its development.

• #5 Total Anomalous Pulmonary Venous Return (TAPVR) – Pediatrics – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/total-anomalous-pulmonary-venous-return-tapvr

  In total anomalous pulmonary venous return, the pulmonary veins drain to a confluence adjacent to the posterior wall of the left atrium but do not connect to the left atrium. […] Total anomalous pulmonary venous return (TAPVR) accounts for 1 to 2% of congenital heart anomalies. […] The infradiaphragmatic drainage type is invariably severely obstructed, leading to dramatic pulmonary edema and cyanosis unresponsive to supplemental oxygen that manifest shortly after birth. […] The other 2 types do not typically involve obstruction and lead to mild to moderate signs of heart failure and mild cyanosis in the first month of life. […] Diagnosis of total anomalous pulmonary venous return is suspected by chest x-ray and established by echocardiography. […] Neonates with total anomalous pulmonary venous return with obstruction require emergent surgical repair.

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

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

  Total and partial anomalous venous connection comprises a wide spectrum of congenital cardiovascular malformations where one or more pulmonary veins returns to the right atrium or systemic venous circulation instead of draining directly into the left atrium. […] Understanding the normal fetal heart and vasculature development is imperative to appreciate the diverse array of anatomic variations and abnormalities. […] Failure of the common pulmonary vein or atresia early in its development, while the persistent of the connections between systemic and pulmonary vasculature, leads to the development of TAPVC. […] TAPVC implies that all the pulmonary veins connect to the systemic veins or right atrium, either by a common vertical vein or individually. […] TAPVC usually occurs as an isolated cardiac lesion, but it can also be associated with other congenital heart defects. The incidence of TAPVC is higher in patients who have heterotaxy with polysplenia or asplenia. […] In untreated patients, TAPVC is almost always fatal within the first few weeks of life.

• #7 Total Anomalous Pulmonary Venous Return (TAPVR)

  https://livinghealthy.hawaiipacifichealth.org/Library/DiseasesConditions/Adult/Plastic/90,P01820

  Total anomalous pulmonary venous return (TAPVR) is a congenital heart defect. This means that your child is born with it. It happens as the babys heart develops during the first 8 weeks of pregnancy. […] Most of the time, the cause of TAPVR isnt known. It may occur with other heart or body system problems. […] In TAPVR, the 4 blood vessels that carry oxygen-rich blood to the heart from the lungs arent connected correctly. […] This condition prevents oxygen-rich blood from being pumped to the body. […] Your child can’t live with TAPVR long-term because people need oxygen-rich blood all over their body. […] All children with TAPVR will need surgery. This is done to restore normal blood flow through the heart.

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

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

  Most congenital heart defects are caused by changes that happen early as the unborn baby’s heart is developing before birth. An unborn baby is also called a fetus. The exact cause of most congenital heart defects, including total anomalous pulmonary venous return (TAPVR), is not known. […] Changes in the genes, some medicines or health conditions, and environmental or lifestyle factors, such as smoking, may play a role.

• #9 Total Anomalous Pulmonary Venous Return (TAPVR)

  https://livinghealthy.hawaiipacifichealth.org/Library/DiseasesConditions/Adult/Plastic/90,P01820

  Total anomalous pulmonary venous return (TAPVR) is a congenital heart defect. This means that your child is born with it. It happens as the babys heart develops during the first 8 weeks of pregnancy. […] Most of the time, the cause of TAPVR isnt known. It may occur with other heart or body system problems. […] In TAPVR, the 4 blood vessels that carry oxygen-rich blood to the heart from the lungs arent connected correctly. […] This condition prevents oxygen-rich blood from being pumped to the body. […] Your child can’t live with TAPVR long-term because people need oxygen-rich blood all over their body. […] All children with TAPVR will need surgery. This is done to restore normal blood flow through the heart.

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

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

  Total anomalous pulmonary venous return (TAPVR) develops during the first four to six weeks of pregnancy because the child’s heart doesnt develop normally. […] We dont know what causes TAPVR, but we do know the defect develops during the first four to six weeks of pregnancy. Its not caused by anything a mother or father did or did not do.

• #11 Total anomalous pulmonary venous connection – UpToDate

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

  Total anomalous pulmonary venous connection (TAPVC), also referred to as total anomalous pulmonary venous return (TAPVR), is a cyanotic congenital defect in which all four pulmonary veins fail to make their normal connection to the left atrium. This results in drainage of all pulmonary venous return into the systemic venous circulation. […] TAPVC arises from the failure of the left atrium to link with the pulmonary venous plexus, which results in the retention of connections through the primitive cardinal and umbilicovitelline drainage pathways. The anatomic variants of TAPVC are dependent upon which specific connections are retained. The cardinal venous system provides connections to the innominate vein, right atrium, superior vena cava, or azygous vein and the umbilicovitelline system to the portal or hepatic vein, or inferior vena cava.

• #12 Total anomalous pulmonary venous return | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/total-anomalous-pulmonary-venous-return?embed_domain=hackmd.io%2F%40yipuafecsl2jsu8smr5njq%2Fbnjhjgjghjghjgh&lang=us

  Total anomalous pulmonary venous return (TAPVR) is a cyanotic congenital heart anomaly with abnormal drainage anatomy of the entire pulmonary venous system. This contrasts with partial anomalous pulmonary venous return (PAPVR) where only part of the pulmonary venous anatomy is abnormal. […] The anomaly occurs secondary to an embryological failure of pulmonary venous development: […] Failure to completely transition between primitive and mature configurations according to this sequence results in persistent venous drainage from the lungs to one of several possible systemic veins. […] Approximately one-third of those with TAPVR also have other associated cardiac lesions; many have heterotaxy syndrome, particularly asplenia. Type III (infracardiac) is also associated with thoracic lymphangiectasia and pulmonary congestion.

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

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

  Total and partial anomalous venous connection comprises a wide spectrum of congenital cardiovascular malformations where one or more pulmonary veins returns to the right atrium or systemic venous circulation instead of draining directly into the left atrium. […] Understanding the normal fetal heart and vasculature development is imperative to appreciate the diverse array of anatomic variations and abnormalities. […] Failure of the common pulmonary vein or atresia early in its development, while the persistent of the connections between systemic and pulmonary vasculature, leads to the development of TAPVC. […] TAPVC implies that all the pulmonary veins connect to the systemic veins or right atrium, either by a common vertical vein or individually. […] TAPVC usually occurs as an isolated cardiac lesion, but it can also be associated with other congenital heart defects. The incidence of TAPVC is higher in patients who have heterotaxy with polysplenia or asplenia. […] In untreated patients, TAPVC is almost always fatal within the first few weeks of life.

• #14 Total Anomalous Pulmonary Venous Drainage – TeachMePaeds

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

  In a case of TAPVD, this pulmonary vein either does not form, or, if formed does not connect with pulmonary venous system. This leaves the pulmonary venous system connected to the systemic venous drainage. […] If the pulmonary venous system is neither connected to the primitive pulmonary vein nor the systemic venous system, then it results in either an intra-uterine death or an early neonatal death. […] TAPVD most frequently occurs as an isolated anomaly (with normal visceroatrial situs). In contrast, patients with visceral heterotaxy of right isomeric type, always have associated TAPVD as the pulmonary veins will have to drain into either one of the morphological right atria. […] The three factors that primarily influence the timing of presentation and the severity of symptoms in infants are as follows: Pulmonary venous obstruction, Inter-atrial communication, Left to Right shunting.

• #15 Total anomalous pulmonary venous connection – UpToDate

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

  Total anomalous pulmonary venous connection (TAPVC), also referred to as total anomalous pulmonary venous return (TAPVR), is a cyanotic congenital defect in which all four pulmonary veins fail to make their normal connection to the left atrium. This results in drainage of all pulmonary venous return into the systemic venous circulation. […] TAPVC arises from the failure of the left atrium to link with the pulmonary venous plexus, which results in the retention of connections through the primitive cardinal and umbilicovitelline drainage pathways. The anatomic variants of TAPVC are dependent upon which specific connections are retained. The cardinal venous system provides connections to the innominate vein, right atrium, superior vena cava, or azygous vein and the umbilicovitelline system to the portal or hepatic vein, or inferior vena cava.

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

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

  Total anomalous pulmonary venous connection is when all of the pulmonary veins returning from the lungs have an abnormal connection and drain anomalously to the right side of the heart instead of the left atrium. Supracardiac TAPVC is the most common type of anomalous pulmonary venous connection. […] Anomalous pulmonary venous connection results from aberrancies during embryologic development. TAPVC (no connection of pulmonary veins to the left atrium) occurs when the common pulmonary vein fails to develop or becomes atretic early in development and pulmonary venous blood drains via collaterals from primitive connections between the splanchnic plexus and the cardinal or umbilicovitelline system of veins. […] Genetic and syndromic associations include Cat eye syndrome, Heterotaxy syndrome (TAPVC most common with right atrial isomerism and PAPVC with left atrial isomerism) and Holt-Oram syndrome. A monogenic pattern of inheritance has been suggested. In a large Utah kindred, the gene for TAPVC was mapped to 4p13-q12.

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

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

  In total anomalous pulmonary venous return (TAPVR), the pulmonary veins incorrectly send blood to the heart’s upper right chamber. That chamber is called the right atrium. As a result, oxygen-rich blood mixes with oxygen-poor blood, as shown in purple. In a typical heart, shown on the left, oxygen-rich blood flows from the pulmonary veins to the upper left chamber, also called the left atrium. […] Total anomalous pulmonary venous return (TAPVR) is a rare heart condition that’s present at birth. That means it’s a congenital heart defect. […] In this heart condition, the lung blood vessels, called the pulmonary veins, attach to the wrong place in the heart. […] The type of TAPVR depends on where the veins connect. Most children born with TAPVR have no family history of congenital heart disease.

• #18 Total Anomalous Pulmonary Venous Connection (TAPVC) | American Heart Association

  https://www.heart.org/en/health-topics/congenital-heart-defects/about-congenital-heart-defects/total-anomalous-pulmonary-venous-connection-tapvc

  Total de conexión venosa pulmonar anómala […] In most children, the cause isn’t known. Some children can have other heart defects along with TAPVC. […] In most cases, the cause isn’t known. Some patients can have other heart defects along with TAPVC.

• #19 Total Anomalous Pulmonary Venous Return in Siblings

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4286644/

  Total anomalous pulmonary venous return (TAPVR) is a rare and critical congenital vascular anomaly that requires an early operation. […] This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. […] The mechanism of transmission of TAPVR has not been elucidated. Although it has no known definitive genetic transmission pattern to date, a monogenic pattern of inheritance has been suggested from the numbers of reported family cases of TAPVR, including siblings. […] Several chromosomal or gene abnormalities associated with TAPVR have been reported. […] Until now, 42 sibling patients have been reported with TAPVR including the two cases presented herein. […] However, the majority of cases have insufficient data from which to draw conclusions about hereditary predisposition. […] Although no other abnormality was associated with a congenital disorder in our case, we proposed a genetic study for this sibling. […] We suspected that a larger genetic study was needed to determine the genetic background of TAPVR recurrence in siblings in Korea.

• #20

  https://omim.org/entry/106700

  Total anomalous pulmonary venous return (TAPVR) is a cyanotic form of congenital heart defect in which the pulmonary veins fail to enter the left atrium and instead drain into the right atrium or one of the venous tributaries (summary by Bleyl et al., 1994). […] Bleyl et al. (1993) and Bleyl et al. (1994) reported a large Utah-Idaho family of Scottish origin in which nonsyndromic TAPVR appeared to be inherited as an autosomal dominant with incomplete penetrance and variable expression. The family contained 14 affected individuals. […] Zhou et al. (2022) performed whole-exome sequencing in 144 Chinese patients with anomalous pulmonary venous return and 1,636 healthy Chinese controls and observed enrichment of potentially deleterious variants in genes highly expressed in the human heart at the critical time point for development of pulmonary veins draining into the left atrium. The authors noted that PLXND1 (604282) represented a strong candidate gene, and validated the finding in an independent cohort of 82 case-control pairs.

• #21

  https://omim.org/entry/106700

  Total anomalous pulmonary venous return (TAPVR) is a cyanotic form of congenital heart defect in which the pulmonary veins fail to enter the left atrium and instead drain into the right atrium or one of the venous tributaries (summary by Bleyl et al., 1994). […] Bleyl et al. (1993) and Bleyl et al. (1994) reported a large Utah-Idaho family of Scottish origin in which nonsyndromic TAPVR appeared to be inherited as an autosomal dominant with incomplete penetrance and variable expression. The family contained 14 affected individuals. […] Zhou et al. (2022) performed whole-exome sequencing in 144 Chinese patients with anomalous pulmonary venous return and 1,636 healthy Chinese controls and observed enrichment of potentially deleterious variants in genes highly expressed in the human heart at the critical time point for development of pulmonary veins draining into the left atrium. The authors noted that PLXND1 (604282) represented a strong candidate gene, and validated the finding in an independent cohort of 82 case-control pairs.

• #22

  https://omim.org/entry/106700

  Total anomalous pulmonary venous return (TAPVR) is a cyanotic form of congenital heart defect in which the pulmonary veins fail to enter the left atrium and instead drain into the right atrium or one of the venous tributaries (summary by Bleyl et al., 1994). […] Bleyl et al. (1993) and Bleyl et al. (1994) reported a large Utah-Idaho family of Scottish origin in which nonsyndromic TAPVR appeared to be inherited as an autosomal dominant with incomplete penetrance and variable expression. The family contained 14 affected individuals. […] Zhou et al. (2022) performed whole-exome sequencing in 144 Chinese patients with anomalous pulmonary venous return and 1,636 healthy Chinese controls and observed enrichment of potentially deleterious variants in genes highly expressed in the human heart at the critical time point for development of pulmonary veins draining into the left atrium. The authors noted that PLXND1 (604282) represented a strong candidate gene, and validated the finding in an independent cohort of 82 case-control pairs.

• #23

  https://omim.org/entry/106700

  In gene expression studies in mouse and chick embryos for both the Pdgfra receptor (PDGFRA; 173490) and its ligand, Pdgfa, Bleyl et al. (2010) showed temporal and spatial patterns consistent with a role in pulmonary vein development. Loss of PDGFRA function in both chick and mouse embryos caused TAPVR with low penetrance (approximately 7%), reminiscent of that observed in human TAPVR kindreds.

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

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

  Total anomalous pulmonary venous connection is when all of the pulmonary veins returning from the lungs have an abnormal connection and drain anomalously to the right side of the heart instead of the left atrium. Supracardiac TAPVC is the most common type of anomalous pulmonary venous connection. […] Anomalous pulmonary venous connection results from aberrancies during embryologic development. TAPVC (no connection of pulmonary veins to the left atrium) occurs when the common pulmonary vein fails to develop or becomes atretic early in development and pulmonary venous blood drains via collaterals from primitive connections between the splanchnic plexus and the cardinal or umbilicovitelline system of veins. […] Genetic and syndromic associations include Cat eye syndrome, Heterotaxy syndrome (TAPVC most common with right atrial isomerism and PAPVC with left atrial isomerism) and Holt-Oram syndrome. A monogenic pattern of inheritance has been suggested. In a large Utah kindred, the gene for TAPVC was mapped to 4p13-q12.

• #25 Total Anomalous Pulmonary Venous Return – Causes, Symptoms, Diagnosis, and Treatment

  https://www.apollohospitals.com/diseases-and-conditions/total-anomalous-pulmonary-venous-return

  Total Anomalous Pulmonary Venous Return (TAPVR) is a rare but serious congenital heart defect that affects the way blood returns to the heart from the lungs. […] While the exact cause of TAPVR is not fully understood, certain environmental factors during pregnancy may contribute to its development. […] Genetic factors play a significant role in the development of TAPVR. […] Certain genetic syndromes, such as Down syndrome and Turner syndrome, are also associated with an increased incidence of TAPVR. […] Conditions such as diabetes, obesity, and autoimmune diseases in the mother can increase the risk of TAPVR.

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

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

  Total and partial anomalous venous connection comprises a wide spectrum of congenital cardiovascular malformations where one or more pulmonary veins returns to the right atrium or systemic venous circulation instead of draining directly into the left atrium. […] Understanding the normal fetal heart and vasculature development is imperative to appreciate the diverse array of anatomic variations and abnormalities. […] Failure of the common pulmonary vein or atresia early in its development, while the persistent of the connections between systemic and pulmonary vasculature, leads to the development of TAPVC. […] TAPVC implies that all the pulmonary veins connect to the systemic veins or right atrium, either by a common vertical vein or individually. […] TAPVC usually occurs as an isolated cardiac lesion, but it can also be associated with other congenital heart defects. The incidence of TAPVC is higher in patients who have heterotaxy with polysplenia or asplenia. […] In untreated patients, TAPVC is almost always fatal within the first few weeks of life.

• #27 Total anomalous pulmonary venous return | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/total-anomalous-pulmonary-venous-return?embed_domain=hackmd.io%2F%40yipuafecsl2jsu8smr5njq%2Fbnjhjgjghjghjgh&lang=us

  Total anomalous pulmonary venous return (TAPVR) is a cyanotic congenital heart anomaly with abnormal drainage anatomy of the entire pulmonary venous system. This contrasts with partial anomalous pulmonary venous return (PAPVR) where only part of the pulmonary venous anatomy is abnormal. […] The anomaly occurs secondary to an embryological failure of pulmonary venous development: […] Failure to completely transition between primitive and mature configurations according to this sequence results in persistent venous drainage from the lungs to one of several possible systemic veins. […] Approximately one-third of those with TAPVR also have other associated cardiac lesions; many have heterotaxy syndrome, particularly asplenia. Type III (infracardiac) is also associated with thoracic lymphangiectasia and pulmonary congestion.

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

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

  Most congenital heart defects are caused by changes that happen early as the unborn baby’s heart is developing before birth. An unborn baby is also called a fetus. The exact cause of most congenital heart defects, including total anomalous pulmonary venous return (TAPVR), is not known. […] Changes in the genes, some medicines or health conditions, and environmental or lifestyle factors, such as smoking, may play a role.

• #29 Total anomalous pulmonary venous return (TAPVR) // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/total-anomalous-pulmonary-venous-return-tapvr

  Total anomalous pulmonary venous return (TAPVR) is a rare heart condition that’s present at birth. That means it’s a congenital heart defect. […] The exact cause of most congenital heart defects, including total anomalous pulmonary venous return (TAPVR), is not known. […] Changes in the genes, some medicines or health conditions, and environmental or lifestyle factors, such as smoking, may play a role.

• #30 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  Total anomalous pulmonary venous return (TAPVR) happens when veins (blood vessels) that bring blood from your babys lungs connect to the wrong place in their heart. As a result, their heart cant put enough oxygen into the blood it sends to the rest of their body. TAPVR is a life-threatening heart condition. Its congenital, which means its present at birth. […] Healthcare providers arent sure what causes total anomalous pulmonary venous return. It happens when your babys heart and blood vessels are forming in the uterus during fetal development. […] This doesnt seem to be an inherited condition (passed down through families). But there have been similar cases among siblings. TAPVR has an association with certain syndromes and exposure to paint removers, pesticides and lead.

• #31 Total Anomalous Pulmonary Venous Drainage – TeachMePaeds

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

  Total anomalous pulmonary venous drainage is a cyanotic congenital heart defect in which the pulmonary veins fail to make their normal connection to left atrium, resulting in the drainage of the pulmonary venous return into the systemic venous circulation. […] The Baltimore- Washington Infant study (1981-87) has previously shown association with exposure to lead, paint, paint-stripping chemicals or pesticides. There is no known genetic pattern of inheritance associated with this condition to date. […] By definition, no direct communication exists between the pulmonary veins and left atrium. Instead these drain anomalously into the systemic venous tributaries or the right atrium. This results in a mixing of blood between the oxygenated and deoxygenated circuits which result in cyanosis.

• #32 Total anomalous pulmonary venous return (TAPVR) | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/total-anomalous-pulmonary-venous-return-tapvr

  Most congenital heart defects are caused by changes that happen early as the unborn baby’s heart is developing before birth. The exact cause of most congenital heart defects, including total anomalous pulmonary venous return (TAPVR), is not known. […] Changes in the genes, some medicines or health conditions, and environmental or lifestyle factors, such as smoking, may play a role. […] Possible risk factors for congenital heart defects, including total anomalous pulmonary venous return (TAPVR), may include: […] Genetics. Although TAPVR doesn’t usually run in families, changes in genes have been linked to heart conditions at birth. For example, people with Down syndrome are often born with heart conditions. […] Some medicines. Some medicines taken during pregnancy may increase the risk of congenital heart defects. These include lithium (Lithobid) for bipolar disorder and isotretinoin (Claravis, Myorisan, others), which is used to treat acne. Talk to your healthcare team about the medicines you take.

• #33 Total Anomalous Pulmonary Venous Return – Causes, Symptoms, Diagnosis, and Treatment

  https://www.apollohospitals.com/diseases-and-conditions/total-anomalous-pulmonary-venous-return

  Total Anomalous Pulmonary Venous Return (TAPVR) is a rare but serious congenital heart defect that affects the way blood returns to the heart from the lungs. […] While the exact cause of TAPVR is not fully understood, certain environmental factors during pregnancy may contribute to its development. […] Genetic factors play a significant role in the development of TAPVR. […] Certain genetic syndromes, such as Down syndrome and Turner syndrome, are also associated with an increased incidence of TAPVR. […] Conditions such as diabetes, obesity, and autoimmune diseases in the mother can increase the risk of TAPVR.

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

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

  Total anomalous pulmonary venous return is a congenital (meaning it’s present from birth) heart disease that affects the pulmonary veins. […] In a child born with total anomalous which means abnormal pulmonary venous return (TAPVR), the pulmonary veins connect to other veins and ultimately drain their blood into the right atrium. […] Children with TAPVR also have other heart defects. […] Total anomalous pulmonary venous return requires open heart surgery in all cases. […] Because of enormous strides in medicine and technology, today most children born with total anomalous pulmonary venous return go on to lead productive lives as adults. […] Rarely, the pulmonary veins become obstructed later in life and additional surgery or a catheterization procedure is required.

• #35 ~Total Anomalous Pulmonary Venous Return (TAPVR) | Abel Speaks

  https://abelspeaks.org/total-anomalous-pulmonary-venous-return-tapvr/

  Total anomalous pulmonary venous return is a congenital (meaning it’s present from birth) heart disease that affects the pulmonary veins. […] In a child born with total anomalous — which means abnormal — pulmonary venous return (TAPVR), the pulmonary veins connect to other veins and ultimately drain their blood into the right atrium. […] Frequently, there is blockage to the veins that receive blood from the pulmonary veins, which causes a backup of blood in the lungs. […] Additionally, the blood leaving the heart from the body has a lower level of oxygen. […] Children with TAPVR also have other heart defects. […] In fact, children without at least an atrial septal defect will not survive.

• #36 Total anomalous pulmonary venous return | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/total-anomalous-pulmonary-venous-return?embed_domain=hackmd.io%2F%40yipuafecsl2jsu8smr5njq%2Fbnjhjgjghjghjgh&lang=us

  Total anomalous pulmonary venous return (TAPVR) is a cyanotic congenital heart anomaly with abnormal drainage anatomy of the entire pulmonary venous system. This contrasts with partial anomalous pulmonary venous return (PAPVR) where only part of the pulmonary venous anatomy is abnormal. […] The anomaly occurs secondary to an embryological failure of pulmonary venous development: […] Failure to completely transition between primitive and mature configurations according to this sequence results in persistent venous drainage from the lungs to one of several possible systemic veins. […] Approximately one-third of those with TAPVR also have other associated cardiac lesions; many have heterotaxy syndrome, particularly asplenia. Type III (infracardiac) is also associated with thoracic lymphangiectasia and pulmonary congestion.

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

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

  Total anomalous pulmonary venous return (TAPVR) is a rare congenital (present at birth) defect. With TAPVR, all four pulmonary veins do not connect normally to the left atrium. Instead, the four pulmonary veins drain abnormally to the right atrium (right upper chamber) through an abnormal (anomalous) connection. […] All types of total anomalous pulmonary venous return must have an atrial septal defect (ASD). An ASD is a hole in the wall between the right and left upper chambers of the heart. […] Because of the abnormal pulmonary vein connection red (oxygenated) blood returning from the lungs mixes with the blue (lower oxygenated) blood returning from the body. […] In some cases of TAPVR, the route of blood from the pulmonary veins back to the heart may have areas of narrowing or obstruction. This obstruction may stop a normal amount of blood return from the pulmonary veins.

• #38 Total anomalous pulmonary venous return: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001115.htm

  Total anomalous pulmonary venous return (TAPVR) is a heart disease in which the 4 veins that take blood from the lungs to the heart do not attach normally to the left atrium (left upper chamber of the heart). Instead, they attach to another blood vessel or the wrong part of the heart. It is present at birth (congenital heart disease). […] The cause of total anomalous pulmonary venous return is unknown. […] For the infant to live, an atrial septal defect (ASD) or patent foramen ovale (passage between the left and right atria) must exist to allow oxygenated blood to flow to the left side of the heart and the rest of the body. […] How severe this condition is depends on whether the pulmonary veins are blocked or obstructed as they drain. Obstructed TAPVR causes symptoms early in life and can be deadly very quickly if it is not found and corrected with surgery. […] Some inherited factors may play a role in congenital heart disease. Many family members may be affected. If you are planning to get pregnant, talk to your provider about screening for genetic diseases.

• #39 Total anomalous pulmonary venous return Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/total-anomalous-pulmonary-venous-return

  Total anomalous pulmonary venous return (TAPVR) is a heart disease in which the 4 veins that take blood from the lungs to the heart do not attach normally to the left atrium (left upper chamber of the heart). Instead, they attach to another blood vessel or the wrong part of the heart. It is present at birth (congenital heart disease). […] The cause of total anomalous pulmonary venous return is unknown. […] For the infant to live, an atrial septal defect (ASD) or patent foramen ovale (passage between the left and right atria) must exist to allow oxygenated blood to flow to the left side of the heart and the rest of the body. […] How severe this condition is depends on whether the pulmonary veins are blocked or obstructed as they drain. Obstructed TAPVR causes symptoms early in life and can be deadly very quickly if it is not found and corrected with surgery.

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

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

  In total anomalous pulmonary venous return (TAPVR), the pulmonary veins incorrectly send blood to the heart’s upper right chamber. That chamber is called the right atrium. As a result, oxygen-rich blood mixes with oxygen-poor blood, as shown in purple. In a typical heart, shown on the left, oxygen-rich blood flows from the pulmonary veins to the upper left chamber, also called the left atrium. […] Total anomalous pulmonary venous return (TAPVR) is a rare heart condition that’s present at birth. That means it’s a congenital heart defect. […] In this heart condition, the lung blood vessels, called the pulmonary veins, attach to the wrong place in the heart. […] The type of TAPVR depends on where the veins connect. Most children born with TAPVR have no family history of congenital heart disease.

• #41 Anomalous pulmonary venous connection – Wikipedia

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

  Anomalous pulmonary venous connection (or anomalous pulmonary venous drainage or anomalous pulmonary venous return) is a congenital heart defect of the pulmonary veins. It can be a total anomalous pulmonary venous connection, wherein all four pulmonary veins are incorrectly positioned, or a partial anomalous pulmonary venous connection, wherein only some of the pulmonary veins are incorrectly positioned. […] Total anomalous pulmonary venous connection, also known as total anomalous pulmonary venous return, is a rare cyanotic congenital heart defect in which the pulmonary veins drain into the right side of the heart instead of the left, as is usually seen. This can happen within the heart (intracardiac) where it drains into the coronary sinus or right atrium, or below it (infracardiac) where it drains into the liver at the portal or hepatic vein. The anomalous connection causes low blood oxygenation and limitation of venous return to the heart. […] 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.

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

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

  Total anomalous pulmonary venous connection is when all of the pulmonary veins returning from the lungs have an abnormal connection and drain anomalously to the right side of the heart instead of the left atrium. Supracardiac TAPVC is the most common type of anomalous pulmonary venous connection. […] Anomalous pulmonary venous connection results from aberrancies during embryologic development. TAPVC (no connection of pulmonary veins to the left atrium) occurs when the common pulmonary vein fails to develop or becomes atretic early in development and pulmonary venous blood drains via collaterals from primitive connections between the splanchnic plexus and the cardinal or umbilicovitelline system of veins. […] Genetic and syndromic associations include Cat eye syndrome, Heterotaxy syndrome (TAPVC most common with right atrial isomerism and PAPVC with left atrial isomerism) and Holt-Oram syndrome. A monogenic pattern of inheritance has been suggested. In a large Utah kindred, the gene for TAPVC was mapped to 4p13-q12.

• #43 Total anomalous pulmonary venous connection – UpToDate

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

  Total anomalous pulmonary venous connection (TAPVC), also referred to as total anomalous pulmonary venous return (TAPVR), is a cyanotic congenital defect in which all four pulmonary veins fail to make their normal connection to the left atrium. This results in drainage of all pulmonary venous return into the systemic venous circulation. […] TAPVC arises from the failure of the left atrium to link with the pulmonary venous plexus, which results in the retention of connections through the primitive cardinal and umbilicovitelline drainage pathways. The anatomic variants of TAPVC are dependent upon which specific connections are retained. The cardinal venous system provides connections to the innominate vein, right atrium, superior vena cava, or azygous vein and the umbilicovitelline system to the portal or hepatic vein, or inferior vena cava.

• #44 Total Anomalous Pulmonary Venous Return (TAPVR) – Children’s Health Issues – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/children-s-health-issues/birth-defects-of-the-heart/total-anomalous-pulmonary-venous-return-tapvr

  In total anomalous pulmonary venous return, the pulmonary veins, blood vessels that normally carry oxygenated blood from the lungs to the left side of the heart, connect instead to the right side of the heart. […] Total anomalous pulmonary venous return (TAPVR) accounts for 1 to 2% of birth defects of the heart. […] In infants born with TAPVR, the pulmonary veins do not connect normally to the left atrium and connect instead by an abnormal pathway eventually leading to the right atrium. […] The pulmonary veins do not connect to the left atrium; instead, the entire pulmonary venous return enters systemic venous circulation through various connections (in this case the connecting vein is supracardiac or above the heart). […] Systemic blood flow depends on right-to-left atrial shunting.

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

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

  Obstructed pulmonary veins commonly occur in the infracardiac type of TAPVR. It can occur with the other anatomic types as well. […] Total anomalous pulmonary venous return is a defect that needs surgery to fix. […] Rarely, TAPVR is complicated by a restrictive atrial septal defect. This means the hole in the atrial septum is not big enough to let enough blood through to the left side. […] The surgical repair connects all of the veins to the back of the left atrium. This leads to a normal connection of pulmonary veins to left atrium. […] Rare complications of TAPVR can occur late after surgery. Regular follow up by a qualified cardiologist is needed to detect these problems early if they occur. […] Rarely, obstruction to one or more pulmonary veins can develop. This can occur at the site of surgical repair, or due to abnormalities of the pulmonary veins themselves.

• #46 Total Anomalous Pulmonary Venous Return (TAPVR) – Pediatrics – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/total-anomalous-pulmonary-venous-return-tapvr

  In total anomalous pulmonary venous return, the pulmonary veins drain to a confluence adjacent to the posterior wall of the left atrium but do not connect to the left atrium. […] Total anomalous pulmonary venous return (TAPVR) accounts for 1 to 2% of congenital heart anomalies. […] The infradiaphragmatic drainage type is invariably severely obstructed, leading to dramatic pulmonary edema and cyanosis unresponsive to supplemental oxygen that manifest shortly after birth. […] The other 2 types do not typically involve obstruction and lead to mild to moderate signs of heart failure and mild cyanosis in the first month of life. […] Diagnosis of total anomalous pulmonary venous return is suspected by chest x-ray and established by echocardiography. […] Neonates with total anomalous pulmonary venous return with obstruction require emergent surgical repair.

• #47 Total anomalous pulmonary venous return (TAPVR)

  https://www.mymlc.com/health-information/diseases-and-conditions/t/total-anomalous-pulmonary-venous-return/?section=Risk%20factors

  Total anomalous pulmonary venous return (TAPVR) is a rare heart defect that’s present at birth (congenital heart defect). It is sometimes called total anomalous pulmonary venous connection (TAPVC). […] In this heart defect, the lung blood vessels (pulmonary veins) are attached to the wrong place in the heart. […] The specific type of TAPVR depends on where the veins connect. Most children born with TAPVR have no family history of congenital heart disease. […] Total anomalous pulmonary venous connection is a defect that occurs when blood vessels from the lungs attach to wrong area of the heart.

• #48 Total anomalous pulmonary venous return: MedlinePlus Medical EncyclopediaLock

  https://medlineplus.gov/ency/article/001115.htm

  Total anomalous pulmonary venous return (TAPVR) is a heart disease in which the 4 veins that take blood from the lungs to the heart do not attach normally to the left atrium (left upper chamber of the heart). Instead, they attach to another blood vessel or the wrong part of the heart. It is present at birth (congenital heart disease). […] The cause of total anomalous pulmonary venous return is unknown. […] For the infant to live, an atrial septal defect (ASD) or patent foramen ovale (passage between the left and right atria) must exist to allow oxygenated blood to flow to the left side of the heart and the rest of the body. […] How severe this condition is depends on whether the pulmonary veins are blocked or obstructed as they drain. Obstructed TAPVR causes symptoms early in life and can be deadly very quickly if it is not found and corrected with surgery. […] Some inherited factors may play a role in congenital heart disease. Many family members may be affected. If you are planning to get pregnant, talk to your provider about screening for genetic diseases.

• #49 Total Anomalous Pulmonary Venous Drainage – TeachMePaeds

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

  In a case of TAPVD, this pulmonary vein either does not form, or, if formed does not connect with pulmonary venous system. This leaves the pulmonary venous system connected to the systemic venous drainage. […] If the pulmonary venous system is neither connected to the primitive pulmonary vein nor the systemic venous system, then it results in either an intra-uterine death or an early neonatal death. […] TAPVD most frequently occurs as an isolated anomaly (with normal visceroatrial situs). In contrast, patients with visceral heterotaxy of right isomeric type, always have associated TAPVD as the pulmonary veins will have to drain into either one of the morphological right atria. […] The three factors that primarily influence the timing of presentation and the severity of symptoms in infants are as follows: Pulmonary venous obstruction, Inter-atrial communication, Left to Right shunting.

• #50 Total anomalous pulmonary venous return (TAPVR) | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/total-anomalous-pulmonary-venous-return-tapvr

  Most congenital heart defects are caused by changes that happen early as the unborn baby’s heart is developing before birth. The exact cause of most congenital heart defects, including total anomalous pulmonary venous return (TAPVR), is not known. […] Changes in the genes, some medicines or health conditions, and environmental or lifestyle factors, such as smoking, may play a role. […] Possible risk factors for congenital heart defects, including total anomalous pulmonary venous return (TAPVR), may include: […] Genetics. Although TAPVR doesn’t usually run in families, changes in genes have been linked to heart conditions at birth. For example, people with Down syndrome are often born with heart conditions. […] Some medicines. Some medicines taken during pregnancy may increase the risk of congenital heart defects. These include lithium (Lithobid) for bipolar disorder and isotretinoin (Claravis, Myorisan, others), which is used to treat acne. Talk to your healthcare team about the medicines you take.

• #51 Total Anomalous Pulmonary Venous Return in Siblings

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4286644/

  Total anomalous pulmonary venous return (TAPVR) is a rare and critical congenital vascular anomaly that requires an early operation. […] This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. […] The mechanism of transmission of TAPVR has not been elucidated. Although it has no known definitive genetic transmission pattern to date, a monogenic pattern of inheritance has been suggested from the numbers of reported family cases of TAPVR, including siblings. […] Several chromosomal or gene abnormalities associated with TAPVR have been reported. […] Until now, 42 sibling patients have been reported with TAPVR including the two cases presented herein. […] However, the majority of cases have insufficient data from which to draw conclusions about hereditary predisposition. […] Although no other abnormality was associated with a congenital disorder in our case, we proposed a genetic study for this sibling. […] We suspected that a larger genetic study was needed to determine the genetic background of TAPVR recurrence in siblings in Korea.

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

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

  Most congenital heart defects are caused by changes that happen early as the unborn baby’s heart is developing before birth. An unborn baby is also called a fetus. The exact cause of most congenital heart defects, including total anomalous pulmonary venous return (TAPVR), is not known. […] Changes in the genes, some medicines or health conditions, and environmental or lifestyle factors, such as smoking, may play a role.

• #53 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  Total anomalous pulmonary venous return (TAPVR) happens when veins (blood vessels) that bring blood from your babys lungs connect to the wrong place in their heart. As a result, their heart cant put enough oxygen into the blood it sends to the rest of their body. TAPVR is a life-threatening heart condition. Its congenital, which means its present at birth. […] Healthcare providers arent sure what causes total anomalous pulmonary venous return. It happens when your babys heart and blood vessels are forming in the uterus during fetal development. […] This doesnt seem to be an inherited condition (passed down through families). But there have been similar cases among siblings. TAPVR has an association with certain syndromes and exposure to paint removers, pesticides and lead.

• #54 Total Anomalous Pulmonary Venous Drainage – TeachMePaeds

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

  Total anomalous pulmonary venous drainage is a cyanotic congenital heart defect in which the pulmonary veins fail to make their normal connection to left atrium, resulting in the drainage of the pulmonary venous return into the systemic venous circulation. […] The Baltimore- Washington Infant study (1981-87) has previously shown association with exposure to lead, paint, paint-stripping chemicals or pesticides. There is no known genetic pattern of inheritance associated with this condition to date. […] By definition, no direct communication exists between the pulmonary veins and left atrium. Instead these drain anomalously into the systemic venous tributaries or the right atrium. This results in a mixing of blood between the oxygenated and deoxygenated circuits which result in cyanosis.

• #55 Total Anomalous Pulmonary Venous Return (TAPVR) – Pediatrics – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/total-anomalous-pulmonary-venous-return-tapvr

  In total anomalous pulmonary venous return, the pulmonary veins drain to a confluence adjacent to the posterior wall of the left atrium but do not connect to the left atrium. […] Total anomalous pulmonary venous return (TAPVR) accounts for 1 to 2% of congenital heart anomalies. […] The infradiaphragmatic drainage type is invariably severely obstructed, leading to dramatic pulmonary edema and cyanosis unresponsive to supplemental oxygen that manifest shortly after birth. […] The other 2 types do not typically involve obstruction and lead to mild to moderate signs of heart failure and mild cyanosis in the first month of life. […] Diagnosis of total anomalous pulmonary venous return is suspected by chest x-ray and established by echocardiography. […] Neonates with total anomalous pulmonary venous return with obstruction require emergent surgical repair.

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

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

  Total and partial anomalous venous connection comprises a wide spectrum of congenital cardiovascular malformations where one or more pulmonary veins returns to the right atrium or systemic venous circulation instead of draining directly into the left atrium. […] Understanding the normal fetal heart and vasculature development is imperative to appreciate the diverse array of anatomic variations and abnormalities. […] Failure of the common pulmonary vein or atresia early in its development, while the persistent of the connections between systemic and pulmonary vasculature, leads to the development of TAPVC. […] TAPVC implies that all the pulmonary veins connect to the systemic veins or right atrium, either by a common vertical vein or individually. […] TAPVC usually occurs as an isolated cardiac lesion, but it can also be associated with other congenital heart defects. The incidence of TAPVC is higher in patients who have heterotaxy with polysplenia or asplenia. […] In untreated patients, TAPVC is almost always fatal within the first few weeks of life.

• #57 Total Anomalous Pulmonary Venous Drainage – TeachMePaeds

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

  Obstruction to the pulmonary venous return results in elevated pressures in the pulmonary venous channels, which is then transmitted to the pulmonary capillary bed. This results in progressive interstitial and alveolar oedema. These changes, in turn, lead to increased pulmonary vascular resistance and elevated pulmonary artery pressure (i.e. pulmonary hypertension). […] Surgical correction is the only definitive treatment and is required in all cases of TAPVD regardless of the degree of obstruction.

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