Materiały źródłowe

• #1 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. […] Total anomalous pulmonary venous connections (TAPVC) is a cyanotic congenital heart disease where all of the pulmonary veins drain directly or indirectly into the right atrium. […] 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. Anatomically TAPVC can be divided into four subtypes based on the level of the anomalous connection as supracardiac, cardiac, infracardiac, and mixed.

• #1 Total Anomalous Pulmonary Venous Return (TAPVR) – Stanford Medicine Children’s Health

  https://www.stanfordchildrens.org/en/topic/default?id=total-anomalous-pulmonary-venous-return-tapvr-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. […] In this condition, the 4 blood vessels (pulmonary veins) that carry oxygen-rich (red) blood to the heart from the lungs arent connected correctly. Normally these blood vessels should be connected to left upper chamber (atrium) of the heart. Instead theyre connected somewhere else. […] 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 right. 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.

• #1 Anomalous pulmonary venous connection – Wikipedia

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

  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. […] During embryonic development, the lungs start as outgrowths from the foregut and share its splanchnic vascular plexus. At around 25 to 27 days of gestation, there are no direct links to the heart. By 27 to 29 days of gestation, the primitive pulmonary vein begins to form as an outgrowth from the left atrium and eventually connects with the primitive pulmonary venous system.

• #1

  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. Intermediate inflow tract anomalies occurred in a higher percentage of embryos (approximately 30%), suggesting that TAPVR may occur at one end of a spectrum of defects.

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

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

  In TAPVC, the entire oxygenated blood from the lungs returns to the right atrium. Therefore, communication between the left and right atrium is crucial for survival in TAPVC. Mixing of the oxygenated and deoxygenated blood occurs in the right atrium, which is then shunted right to the left at the level of atria. Therefore, the left atrium and aorta get mixed blood, which leads to cyanosis in patients with TAPVC. […] Infracardiac type is the most common cause of obstructive TAPVC. Pulmonary venous obstruction can occur at the level of the diaphragm, vein entering the IVC, or in the hepatic sinusoids. Elevated pressures in the pulmonary venous system are transmitted to the lung bed leading to pulmonary edema. This, in turn, can lead to pulmonary vascular resistance, pulmonary hypertension, right ventricular hypertrophy, and right-sided heart failure. […] Corrective surgery is indicated in all patients with TAPVC as soon as possible, once the clinical condition is stabilized.

• #1 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. This blood then flows to the left heart through a hole in the wall separating the left and right heart chambers. […] Normally, the pulmonary veins return blood that has picked up oxygen in the lungs to the left atrium. This oxygenated blood then flows from the left atrium into the left ventricle, where it is pumped to the body. 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. So the right atrium, which usually receives only deoxygenated blood from the body to be pumped to the lungs, receives a mix of oxygenated and deoxygenated blood. The additional blood return causes an increased workload and enlargement of the right side of the heart. Even more importantly, blood flow from the pulmonary veins makes its way to the right atrium through various pathways, going up above the heart, down below the heart, or around the back wall of the heart. These pathways can be narrow or blocked, causing blood to back up in the lungs and pressure to build up in the lungs, preventing their normal function. Blood only flows to the left side of the heart through a hole between the right and left atria (a patent foramen ovale or atrial septal defect).

• #1 Total anomalous pulmonary venous return – UF Health

  https://ufhealth.org/conditions-and-treatments/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. […] In TAPVR, oxygen-rich blood returns from the lungs to the right atrium or to a vein flowing into the right atrium, instead of the left side of heart. In other words, blood simply circles to and from the lungs and never gets out to the body. […] 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.

• #1 Total Anomalous Pulmonary Venous Return | Thoracic Key

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

  When the obstruction is severe, significant pulmonary edema and pulmonary hypertension will occur immediately. This will result in an increase in the right-to-left intracardiac shunt and subsequently worsening systemic cyanosis. […] In children with no obstruction to the pulmonary venous connection the physiology is similar to that of other patients with significant left-to-right shunts and will likely go undetected in the neonate if not diagnosed on routine prenatal ultrasound examinations or postnatal screening. […] Children frequently present with a combination of excessive pulmonary blood flow and mild PVO. […] When PVO is severe, neonates are critically ill with pulmonary venous hypertension resulting in pulmonary edema, reflex pulmonary arterial vasoconstriction, and pulmonary arterial hypertension with associated decreased pulmonary blood flow.

• #1 :: JCVI :: Journal of Cardiovascular Imaging

  https://e-jcvi.org/DOIx.php?id=10.4250/jcu.2014.22.4.213

  Total anomalous pulmonary venous return (TAPVR) is a rare and critical congenital vascular anomaly that requires an early operation. […] 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. […] 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. […] Earlier detection and correction of TAPVR improves patient outcome.

• #1

  https://www.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). […] By linkage mapping with polymorphic microsatellite markers in a large Utah-Idaho family of Scottish origin segregating TAPVR, Bleyl et al. (1994, 1995) localized the TAPVR1 locus to a 30-cM interval on 4p13-q12; maximum lod = 6.51 at theta = 0.0. A vascular epithelial growth factor receptor, thought to have a role in vasculogenesis, also mapped near the centromere and therefore was considered a candidate for the TAPVR gene. […] 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. Intermediate inflow tract anomalies occurred in a higher percentage of embryos (approximately 30%), suggesting that TAPVR may occur at one end of a spectrum of defects.

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

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

  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.

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

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

  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. […] Total anomalous pulmonary venous return (TAPVR) is treated with surgery. The surgery usually is done when a child is a baby. The timing of surgery depends on whether there’s a blockage. To repair the heart, surgeons connect the pulmonary veins to the left upper heart chamber. They also close the hole between the upper heart chambers.

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

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

  The type of TAPVR depends on where the veins connect. Most children born with TAPVR have no family history of congenital heart disease. […] 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. […] To diagnose total anomalous pulmonary venous return (TAPVR), a healthcare professional listens to the heart and lungs. A whooshing sound, called a heart murmur, may be heard. […] An echocardiogram is the test used to diagnose total anomalous pulmonary venous return. It uses sound waves to create images of the heart as it beats. An echocardiogram can show the pulmonary veins, any holes in the heart and the size of the heart chambers. It also shows blood flow through the heart and heart valves.

• #1 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

  A defect in the veins leading from the lungs to the heart. […] In TAPVC, the blood does not take the normal route from the lungs to the heart and out to the body. Instead, the veins from the lungs attach to the heart in abnormal positions and this problem means that oxygenated blood enters or leaks into the wrong chamber. […] In most children, the cause isn’t known. Some children can have other heart defects along with TAPVC. […] Symptoms may develop soon after birth. In other children, symptoms may be delayed. This partly depends on whether the lung veins are blocked as they drain toward the right atrium. Severe obstruction of the pulmonary veins tends to make infants breathe harder and look bluer (have lower oxygen levels) than infants with little obstruction. […] This defect must be surgically repaired in early infancy. At the time of open-heart surgery, the pulmonary veins are reconnected to the left atrium and the atrial septal defect is closed.

• #1 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. Instead, the entire pulmonary venous return enters the systemic venous circulation through one or more persistent embryologic connections. […] Total anomalous pulmonary venous return (TAPVR) accounts for 1 to 2% of congenital heart anomalies. The clinical manifestation depends on the connection between the pulmonary venous confluence and the right side of the circulation. […] 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 pulmonary veins do not connect to the left atrium; instead, the entire pulmonary venous return enters systemic venous circulation through various connections. Systemic blood flow depends on right-to-left atrial shunting.

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

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

  Total anomalous pulmonary venous return (TAPVR) is a cyanotic congenital heart defect wherein the pulmonary veins drain into anatomical sites other than the left atrium. […] This condition arises due to the failure of the left atrium to merge with the pulmonary venous plexus during fetal development. […] Failure of common pulmonary vein to merge with venous plexus resulting in TAPVR: Connection with primitive cardinal venous system (draining into RA or SVC) and umbilicovitelline system. […] In non-obstructed TAPVR: Oxygenated blood from pulmonary venous plexus flows into deoxygenated blood found in RA, IVC, or SVC venous mixing. […] Blood mixing in total anomalous pulmonary venous return: In TAPVR, oxygenated blood from the lungs returns to the systemic venous circulation mixing with deoxygenated blood. This mixed blood then shunts to the left side of the heart through an atrial septum defect and flows to the rest of the body resulting in cyanosis. […] In moderate obstruction to pulmonary return: increased pressure in pulmonary vein increased pressure in pulmonary artery pulmonary hypertension.

• #1 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. This hole will allow some of the oxygenated blood that has entered the right atrium from the pulmonary vein to go across to the left atrium and out to the body. […] 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.

• #1 Total anomalous pulmonary venous return – UF Health

  https://ufhealth.org/conditions-and-treatments/total-anomalous-pulmonary-venous-return

  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. […] Surgery to repair the problem is needed as soon as possible. In surgery, the pulmonary veins are connected to the left atrium and the defect between the right and left atrium is closed. […] If this condition is not treated, the heart will get larger, leading to heart failure. Repairing the defect early provides excellent results if there is no blockage of the pulmonary veins at the new connection into the heart. Infants who have obstructed veins have worsened survival.

• #2 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.

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

  https://radiopaedia.org/articles/total-anomalous-pulmonary-venous-return?embed_domain=hackmd.io%2525252f%25252540yipuafecsl2jsu8smr5njq%2525252fbnjhjgjghjghjghradiopaedia-icon-144.pngfavicon.ico&lang=gb

  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. […] In TAPVR, all systemic and pulmonary venous blood enters the right atrium and nothing drains into the left atrium. A right-to-left shunt is required for survival and is usually via a large patent foramen ovale (PFO) or less commonly atrial septal defect (ASD). […] The anomaly occurs secondary to an embryological failure of pulmonary venous development: at the earliest stage of lung development, the primitive lung bud venous drainage occurs via the splanchnic plexus to primitive systemic (cardinal/umbilicovitelline) veins; at this point, the heart is still forming and there is no pulmonary connection. […] 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.

• #2 Clinical Presentation and Therapy of Total Anomalous Pulmonary Venous Return – PubMed

  https://pubmed.ncbi.nlm.nih.gov/38884734/

  Total anomalous pulmonary venous return (TAPVR) is rare (accounting for about 1% of all CHD) and can occur as a single lesion or in combination with other types of CHD (such as heterotaxy or HLHS). TAPVR is defined as an abnormal connection where all pulmonary veins do not drain into the left atrium but into the right atrium either directly or through a vein that is connected to the right atrium. TAPVR can be divided into four anatomic groups (Fig. 32.1): (1) supracardiac (about 55%), (2) cardiac (about 30%), (3) infracardiac (about 13%), and (4) mixed (very rare). […] Embryologically, all pulmonary veins usually connect to a pulmonary venous confluence that connects to the left atrium. If this connection does not occur, the pulmonary venous confluence connects to a systemic vein instead.

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

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

  The cause of TAPVR is not known. It occurs in one out of every 15,000 live births, and is seen in boys just as often as in girls. […] Babies with TAPVR cannot supply oxygen-rich (red) blood to the body after birth. Without an additional heart defect that allows mixing of oxygen-poor (blue) and oxygen-rich (red) blood, infants with TAPVR will have a mixture of oxygen-rich (red) and oxygen-poor (blue) blood circulating through the right heart and back to the lungs – a situation that is fatal. […] Another problem that occurs with TAPVR is that too much blood flows through the blood vessels in the lungs, since the pulmonary veins add extra blood back in to the circulation on the right side of the heart. The lungs become stressed by this extra blood flow, and can eventually become damaged from it.

• #2 LearningRadiology – Total Anomalous Pulmonary Venous Return, TAPVR

  http://learningradiology.com/notes/cardiacnotes/tapvrpage.htm

  Total Anomalous Pulmonary Venous Return TAPVR […] All must also have R to L shunt for survival […] Obligatory ASD to return blood to the systemic side […] All are cyanotic […] Identical oxygenation in all four chambers […] All have shunt through lungs to R side of heart.

• #2 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). This progressively causes right ventricular dilatation, hypertrophy and ultimately right heart failure. […] In unobstructed forms of TAPVD, there is a net left to right shunt due to the gradual decrease in pulmonary vascular resistance after birth. The leads to pulmonary over-circulation over a period of time. This persistently elevated pulmonary blood flow gradually leads to elevated pulmonary artery pressures. If left uncorrected, this eventually leads to right ventricular hypertrophy and right ventricular failure.

• #2

  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). […] Solymar et al. (1987) reported 3 pairs of sibs with total anomalous pulmonary venous connection. Four of the affected persons were male. Even in the same family, the connection was supracardial in one and infracardial in the other, indicating that genetic regulation deals with the left atrial connection to the intrapulmonary veins. Having failed to establish this connection, the intrapulmonary veins attach themselves to any adjacent venous structure; hence, the variety of connections found at birth. […] By linkage mapping with polymorphic microsatellite markers in a large Utah-Idaho family of Scottish origin segregating TAPVR, Bleyl et al. (1994, 1995) localized the TAPVR1 locus to a 30-cM interval on 4p13-q12; maximum lod = 6.51 at theta = 0.0. A vascular epithelial growth factor receptor, thought to have a role in vasculogenesis, also mapped near the centromere and therefore was considered a candidate for the TAPVR gene.

• #2 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. In this heart condition, the lung blood vessels, called the pulmonary veins, attach to the wrong place in the heart. […] In TAPVR, the connection of veins is changed. Blood flows through the upper right heart chamber, called the right atrium. This change in blood flow causes oxygen-poor blood to mix with oxygen-rich blood. As a result, blood flowing to the body doesn’t have enough oxygen. […] 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. […] 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.

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

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

  Total anomalous pulmonary venous return (TAPVR) is a rare congenital (present at birth) condition in which the pulmonary veins that carry oxygenated blood from the lungs into the heart are anomalous – abnormal – and do not connect properly to the left atrium. Instead, as the baby’s heart forms during the first eight weeks of the mother’s pregnancy, the baby’s blood vessels connect to other veins that empty into the right atrium. […] When all four pulmonary veins are connected improperly, the baby also develops a hole, known as an atrial septal defect or ASD, in the tissue between the two atria that allows the mixed blood – oxygenated and unoxygenated – to reach the left side of the heart and then be pumped out to the body. The ASD helps the newborn survive, but it also makes the baby’s heart have to work even harder to provide blood and oxygen to the rest of the body.

• #2 Total Anomalous Pulmonary Venous Return | Thoracic Key

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

  When the obstruction is severe, significant pulmonary edema and pulmonary hypertension will occur immediately. This will result in an increase in the right-to-left intracardiac shunt and subsequently worsening systemic cyanosis. […] In children with no obstruction to the pulmonary venous connection the physiology is similar to that of other patients with significant left-to-right shunts and will likely go undetected in the neonate if not diagnosed on routine prenatal ultrasound examinations or postnatal screening. […] Children frequently present with a combination of excessive pulmonary blood flow and mild PVO. […] When PVO is severe, neonates are critically ill with pulmonary venous hypertension resulting in pulmonary edema, reflex pulmonary arterial vasoconstriction, and pulmonary arterial hypertension with associated decreased pulmonary blood flow.

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

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

  Total anomalous pulmonary venous return (TAPVR) is a cyanotic congenital heart defect wherein the pulmonary veins drain into anatomical sites other than the left atrium. […] This condition arises due to the failure of the left atrium to merge with the pulmonary venous plexus during fetal development. […] Failure of common pulmonary vein to merge with venous plexus resulting in TAPVR: Connection with primitive cardinal venous system (draining into RA or SVC) and umbilicovitelline system. […] In non-obstructed TAPVR: Oxygenated blood from pulmonary venous plexus flows into deoxygenated blood found in RA, IVC, or SVC venous mixing. […] Blood mixing in total anomalous pulmonary venous return: In TAPVR, oxygenated blood from the lungs returns to the systemic venous circulation mixing with deoxygenated blood. This mixed blood then shunts to the left side of the heart through an atrial septum defect and flows to the rest of the body resulting in cyanosis. […] In moderate obstruction to pulmonary return: increased pressure in pulmonary vein increased pressure in pulmonary artery pulmonary hypertension.

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

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

  In TAPVC, the entire oxygenated blood from the lungs returns to the right atrium. Therefore, communication between the left and right atrium is crucial for survival in TAPVC. Mixing of the oxygenated and deoxygenated blood occurs in the right atrium, which is then shunted right to the left at the level of atria. Therefore, the left atrium and aorta get mixed blood, which leads to cyanosis in patients with TAPVC. […] Infracardiac type is the most common cause of obstructive TAPVC. Pulmonary venous obstruction can occur at the level of the diaphragm, vein entering the IVC, or in the hepatic sinusoids. Elevated pressures in the pulmonary venous system are transmitted to the lung bed leading to pulmonary edema. This, in turn, can lead to pulmonary vascular resistance, pulmonary hypertension, right ventricular hypertrophy, and right-sided heart failure. […] Corrective surgery is indicated in all patients with TAPVC as soon as possible, once the clinical condition is stabilized.

• #2 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. […] A baby with TAPVR can have heart failure and pulmonary hypertension. Even after the initial repair, TAPVR can sometimes lead to abnormal heart rhythms or blockages in your childs pulmonary veins. […] Nearly every baby with total anomalous pulmonary venous return needs surgery to survive. Healthcare providers treat TAPVR with open-heart surgery. Most often, providers perform this surgery as soon as they can after diagnosing the condition. […] Without surgery, some forms of total anomalous pulmonary venous return are typically fatal a few weeks after birth. With early diagnosis and surgical treatment, the outlook for babies with TAPVR is very good. The survival rate after surgery is around 97%.

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

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

  Diagnosis of total anomalous pulmonary venous return is suspected by chest x-ray and established by echocardiography. […] Surgical repair consists of creating a wide anastomosis between the pulmonary venous confluence and the posterior wall of the left atrium. Ligation of the vein decompressing the confluence into the systemic venous circulation is important to prevent a postoperative left-to-right shunt.

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