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 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.

• #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. […] TAPVR and PAPVR might be diagnosed during pregnancy, but more often these defects are diagnosed soon after a baby is born. […] During pregnancy, an ultrasound, a tool that creates pictures of the baby, may detect TAPVR. […] If the health care provider suspects TAPVR from the ultrasound findings, they can request a fetal echocardiogram to confirm the diagnosis. […] However, TAPVR is not commonly detected during pregnancy. […] It is easier to detect TAPVR after birth when blood is flowing to the lungs and returning to the heart.

• #3 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  Total anomalous pulmonary venous return (TAPVR) is an issue where veins from your babys lungs connect to the right side of their heart instead of the left. Blood with and without oxygen mixes, which keeps their body from getting enough oxygen. This is a rare, life-threatening congenital heart issue that affects newborns. […] 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. […] All children with TAPVR also have an atrial septal defect (ASD). An ASD is a hole between your hearts right and left atria.

• #4 Total anomalous pulmonary venous connection – UpToDate

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

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

• #5

  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. […] Bleyl et al. (2010) stated that TAPVR affects 1 in 15,000 live births. […] 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 suggested that PLXND1 represented a potentially important APVR-associated gene.

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

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

  Total anomalous pulmonary venous return (TAPVR) is a congenital (present at birth) heart defect. Due to abnormal development of the prenatal heart during the first eight weeks of pregnancy, the vessels that bring oxygen-rich (red) blood back to the heart from the lungs are improperly connected. […] In TAPVR, the four pulmonary veins are connected somewhere besides the left atrium. There are several possible places where the pulmonary veins can connect. The most common connection is to a blood vessel that brings oxygen-poor (blue) blood back to the right atrium, usually the superior vena cava. […] In TAPVR, oxygen-rich (red) blood that should return to the left atrium, the left ventricle, the aorta and then the body, instead mixes with the oxygen-poor (blue) blood flowing into the right side of the heart. This situation by itself will not support life, because there is no way for oxygen-rich (red) blood to be delivered to the body.

• #7 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 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.

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

  https://radiopaedia.org/articles/total-anomalous-pulmonary-venous-return?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. […] 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). […] TAPVR can be classified into four types (in decreasing order of frequency) depending on the site of anomalous venous union. […] The right heart is prominent in TAPVR because of the increased flow volume, but the left atrium remains normal in size. […] Transthoracic echocardiography may be used to define the anatomical connections present, as well as assess the hemodynamic consequences. […] Direct visualization of anomalous venous return.

• #9 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  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%. […] Your child will need regular visits with their cardiologist as they grow into adulthood. Lifelong follow-up visits can help cardiologists find problems like an irregular heartbeat or blockages (obstructions) in their blood vessels. An obstruction requires another surgery and may be hard to treat. […] After surgery, your baby will have checkups every six to 12 months. Theyll need regular follow-up appointments through adulthood. Your childs provider may want to order tests like an electrocardiogram, exercise stress test or echocardiogram.

• #10 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 (TAPVR) may be diagnosed before birth, with fetal echocardiogram (ultrasound). […] In other cases, TAPVR is diagnosed in the first few months of life, after a child demonstrates milder symptoms such as a heart murmur or cyanosis (blue tint to skin). […] Diagnosis of TAPVR may require some or all of these tests: Echocardiogram: sound waves create an image of the heart. This is generally the definitive test for diagnosis. […] Electrocardiogram (ECG): a record of the electrical activity of the heart. This usually shows evidence of right heart hypertrophy (increased size of the right ventricle). […] Chest X-ray: this will show right heart enlargement and increased blood flow through the pulmonary artery. If the veins are obstructed, there will be pulmonary edema (buildup of fluid in the lungs).

• #11 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 some cases, total anomalous pulmonary venous return is found before birth on a fetal echocardiogram. Most babies with total anomalous pulmonary venous return (TAPVR) have symptoms on the day they’re born. Severe TAPVR can usually be diagnosed promptly based on symptoms and tests, including chest X-ray and cardiac ultrasound. These babies are generally admitted immediately to the hospital. […] Your baby’s doctor may order one or more additional tests to diagnose or confirm a diagnosis of TAPVR: Cardiac MRI, Electrocardiogram (ECG or EKG), Cardiac catheterization.

• #12 Can Prenatal Diagnosis of Total Anomalous Pulmonary Venous Return (TAPVR) Using Routine Fetal Ultrasound be Improved? A Case-Control Study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9825205/

  To determine the most common fetal ultrasound markers of total anomalous pulmonary venous return (TAPVR) during mid-trimester ultrasound using standardly obtained images and evaluate the performance of diagnostic algorithms for improving prenatal diagnosis. […] The most common ultrasound marker of TAPVR was absence of pulmonary vein entry into the LA (42.9%), followed by abnormal Coumadin ridge (38.1%). […] Using standardly obtained images from routine fetal ultrasound, improved prenatal detection of isolated TAPVR is possible. A standardized diagnostic approach can be highly specific for fetal TAPVR, however, algorithms that are sufficiently sensitive for screening in the general population are still needed. […] Prenatal diagnosis of TAPVR is particularly difficult when it is isolated and in the absence of other cardiac anomalies. […] Accurate identification of fetuses affected by TAPVR is essential for improving postnatal outcomes. […] Prenatal diagnosis of TAPVR is feasible during routine obstetrical ultrasound. […] Future studies are still needed to improve prenatal screening of TAPVR in the general population.

• #13 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. […] TAPVR and PAPVR might be diagnosed during pregnancy, but more often these defects are diagnosed soon after a baby is born. […] During pregnancy, an ultrasound, a tool that creates pictures of the baby, may detect TAPVR. […] If the health care provider suspects TAPVR from the ultrasound findings, they can request a fetal echocardiogram to confirm the diagnosis. […] However, TAPVR is not commonly detected during pregnancy. […] It is easier to detect TAPVR after birth when blood is flowing to the lungs and returning to the heart.

• #14 Can Prenatal Diagnosis of Total Anomalous Pulmonary Venous Return (TAPVR) Using Routine Fetal Ultrasound be Improved? A Case-Control Study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9825205/

  To determine the most common fetal ultrasound markers of total anomalous pulmonary venous return (TAPVR) during mid-trimester ultrasound using standardly obtained images and evaluate the performance of diagnostic algorithms for improving prenatal diagnosis. […] The most common ultrasound marker of TAPVR was absence of pulmonary vein entry into the LA (42.9%), followed by abnormal Coumadin ridge (38.1%). […] Using standardly obtained images from routine fetal ultrasound, improved prenatal detection of isolated TAPVR is possible. A standardized diagnostic approach can be highly specific for fetal TAPVR, however, algorithms that are sufficiently sensitive for screening in the general population are still needed. […] Prenatal diagnosis of TAPVR is particularly difficult when it is isolated and in the absence of other cardiac anomalies. […] Accurate identification of fetuses affected by TAPVR is essential for improving postnatal outcomes. […] Prenatal diagnosis of TAPVR is feasible during routine obstetrical ultrasound. […] Future studies are still needed to improve prenatal screening of TAPVR in the general population.

• #15 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. […] TAPVR and PAPVR might be diagnosed during pregnancy, but more often these defects are diagnosed soon after a baby is born. […] During pregnancy, an ultrasound, a tool that creates pictures of the baby, may detect TAPVR. […] If the health care provider suspects TAPVR from the ultrasound findings, they can request a fetal echocardiogram to confirm the diagnosis. […] However, TAPVR is not commonly detected during pregnancy. […] It is easier to detect TAPVR after birth when blood is flowing to the lungs and returning to the heart.

• #16 Total Anomalous Pulmonary Venous Return – Seattle Children’s Hospital

  https://www.seattlechildrens.org/conditions/total-anomalous/

  Total anomalous pulmonary venous return (TAPVR) is a birth defect in which the veins from the lungs do not connect to the left atrium. Instead, they connect to the right atrium or to the blood vessels leading into the right atrium. […] Often, doctors can diagnose TAPVR when a baby is in the womb using a fetal echocardiogram (fetal echo). This is a special ultrasound that uses sound waves to view and make pictures of a developing babys heart during pregnancy. The results are interpreted by a pediatric heart doctor (cardiologist) who specializes in fetal congenital heart disease. […] To diagnose this condition, your childs doctor will examine your child and use a stethoscope to listen to their heart. The doctor will ask for details about your childs symptoms, their health history and your family health history. Your child will also need tests that provide information about how their heart looks and works. These may include: Chest X-ray, Echocardiography, ECG or EKG (electrocardiogram), Cardiac catheterization.

• #17 Total Anomalous Pulmonary Venous Return – Seattle Children’s Hospital

  https://www.seattlechildrens.org/conditions/total-anomalous/

  Total anomalous pulmonary venous return (TAPVR) is a birth defect in which the veins from the lungs do not connect to the left atrium. Instead, they connect to the right atrium or to the blood vessels leading into the right atrium. […] Often, doctors can diagnose TAPVR when a baby is in the womb using a fetal echocardiogram (fetal echo). This is a special ultrasound that uses sound waves to view and make pictures of a developing babys heart during pregnancy. The results are interpreted by a pediatric heart doctor (cardiologist) who specializes in fetal congenital heart disease. […] To diagnose this condition, your childs doctor will examine your child and use a stethoscope to listen to their heart. The doctor will ask for details about your childs symptoms, their health history and your family health history. Your child will also need tests that provide information about how their heart looks and works. These may include: Chest X-ray, Echocardiography, ECG or EKG (electrocardiogram), Cardiac catheterization.

• #18 Get TAPVR Treatment | Cleveland Clinic Children’s

  https://my.clevelandclinic.org/pediatrics/services/tapvr-treatment

  When your baby is born with veins connecting to the wrong place in their heart, surgery is a must and that can be a lot to take in. […] The good news is that total anomalous pulmonary venous return (TAPVR) also called total anomalous pulmonary venous connection (TAPVC) can be repaired when its diagnosed early. […] If your child has total anomalous pulmonary venous return, it means the veins from their lungs connect to their heart in the wrong place. And it can affect how blood flows through their body to deliver oxygen to their organs. […] In most cases, we can diagnose TAPVR before birth during a prenatal ultrasound. If we suspect TAPVR, we typically do a fetal echocardiogram, which can show the structure of your babys heart and confirm that they have the condition. […] After birth, if your childs healthcare provider suspects TAPVR, theyll give your child a transthoracic echocardiogram (TTE) to see the structure of their heart and veins.

• #19 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. […] TAPVR and PAPVR might be diagnosed during pregnancy, but more often these defects are diagnosed soon after a baby is born. […] During pregnancy, an ultrasound, a tool that creates pictures of the baby, may detect TAPVR. […] If the health care provider suspects TAPVR from the ultrasound findings, they can request a fetal echocardiogram to confirm the diagnosis. […] However, TAPVR is not commonly detected during pregnancy. […] It is easier to detect TAPVR after birth when blood is flowing to the lungs and returning to the heart.

• #20

  https://journals.lww.com/jmut/fulltext/2024/32020/prenatal_ultrasound_markers_of_isolated_total.2.aspx

  In fetal cardiac screening, the left and right pulmonary veins entering the LA are commonly observed in the four-chamber view. Their presence effectively rules out the possibility of TAPVR. However, when the connection between the pulmonary veins and the LA is not clear, the sonographer should raise suspicions of TAPVR and conduct a systematic search for other possible pulmonary venous drainage routes. […] In the absence of a single sonographic sign for the diagnosis of fetal TAPVR, a systematic and sequential approach is crucial to improve the prenatal detection and diagnosis of TAPVR. The key to enhancing prenatal diagnosis is adherence to the fetal cardiac screening guidelines and evaluation of pulmonary venous entry into the LA in the four-chamber view. Other sonographic signs including direct and indirect markers, when used in conjunction with color and spectral Doppler, serve as adjuncts to precise prenatal diagnosis. Further follow-up at later gestation can be suggested in cases with equivocal findings.

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

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

  Symptoms of total anomalous pulmonary venous return (TAPVR) in babies may include trouble breathing, poor feeding and a weak pulse. The baby’s skin may look gray or blue due to low oxygen levels. This is called cyanosis. Depending on skin color, these changes may be easier or harder to see. […] Serious congenital heart defects are often diagnosed before or soon after a child is born. If you think that your baby has symptoms of total anomalous pulmonary venous return (TAPVR), call your child’s healthcare professional. […] To diagnose total anomalous pulmonary venus return (TAPVR), a healthcare professional listens to the heart and lungs. A whooshing sound, called a heart murmur, may be heard. […] 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. […] Other tests such as an electrocardiogram, a chest X-ray or a CT scan may be done if more information is needed.

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

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

  Total anomalous pulmonary venous return occurs equally in boys and in girls. […] The obvious indication of TAPVR is a newborn who becomes cyanotic (blue coloring of the skin, lips and nail beds) in the first days of life after the maternal source of oxygen (from the placenta) is removed. The degree of cyanosis is related to the degree of obstruction of the anomalous pulmonary veins and the size of the foramen ovale that allows the red and blue blood to mix. […] The symptoms of total anomalous pulmonary venous may resemble other medical conditions or heart problems. Always consult your child’s doctor for a diagnosis. […] Cyanosis is the major indication that there is a problem with your newborn. Your child’s doctor may have also heard a heart murmur during a physical examination. […] Diagnostic testing for congenital heart disease varies by the child’s age, clinical condition, and institutional preferences.

• #23 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  Babies who have TAPVR need an operation. With early surgery, most children with TAPVR survive into adulthood. But some will need repeat surgery or procedures to treat narrowing in their veins later in life. Because of this, people with TAPVR need to see a cardiologist (a heart expert) regularly to monitor their health following surgery. […] Symptoms of TAPVR usually appear very soon after birth. Babies have symptoms at birth if they have any narrowing where their lung veins connect to their hearts. But some babies dont have symptoms for several weeks. […] 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. […] Cardiologists can sometimes diagnose this condition with an echocardiogram before birth. Some babies dont get a diagnosis until theyre several weeks or months old.

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

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

  Symptoms of total anomalous pulmonary venous return (TAPVR) in babies may include trouble breathing, poor feeding and a weak pulse. The baby’s skin may look gray or blue due to low oxygen levels. This is called cyanosis. Depending on skin color, these changes may be easier or harder to see. […] Serious congenital heart defects are often diagnosed before or soon after a child is born. If you think that your baby has symptoms of total anomalous pulmonary venous return (TAPVR), call your child’s healthcare professional. […] To diagnose total anomalous pulmonary venus return (TAPVR), a healthcare professional listens to the heart and lungs. A whooshing sound, called a heart murmur, may be heard. […] 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. […] Other tests such as an electrocardiogram, a chest X-ray or a CT scan may be done if more information is needed.

• #25 Understanding Total Anomalous Pulmonary Venous Return

  https://lagans.org.uk/total-anomalous-pulmonary-venus-return/

  Total Anomalous Pulmonary Venous Return is a rare heart defect that is present at birth and it occurs when pulmonary veins connect to the heart in an abnormal way. […] Total Anomalous Pulmonary Venous Return (TAPVR) is a congenital heart defect that affects the pulmonary veins. […] Symptoms of Total Anomalous Pulmonary Venous Return will usually occur immediately after birth or soon after. Infants with TAPVR will often have bluish looking skin, called cyanosis, because their blood doesnt carry enough oxygen. […] All types of Total Anomalous Pulmonary Venous Return will need to be treated with surgery, but the timing of the surgery will depend on the type of TAPVR present. […] Newborns who have an obstructed TAPVR will need surgery immediately after birth. […] Infants with an unobstructed TAPVR will typically have surgery days to weeks after the diagnosis is made.

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

  http://memorialhermann.org/ar-sa/services/conditions/pediatric-total-anomalous-pulmonary-venous-return

  Treatment of TAPVR is surgery, which consists of placing the patient on the heart-lung machine (cardiopulmonary bypass) and making the connection between the pulmonary veins and the left atrium. Fortunately, in most cases of TAPVR, all four pulmonary veins come to a central place (called a confluence) just behind the left atrium and a full anatomical repair can be achieved. However, after surgery, scar tissue may form around the connection between the pulmonary veins and the left atrium. If the scar tissue formation is severe enough, additional surgery may be required in the future. […] If your child has a form of TAPVR that is obstructed (where the blood cannot get to the heart from the lungs), this may require an emergent surgical intervention. This is often performed when the child is only hours old. If the pulmonary veins are unobstructed, surgery can take place at an agreed upon elective date, usually at a few weeks of life.

• #27 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  Babies who have TAPVR need an operation. With early surgery, most children with TAPVR survive into adulthood. But some will need repeat surgery or procedures to treat narrowing in their veins later in life. Because of this, people with TAPVR need to see a cardiologist (a heart expert) regularly to monitor their health following surgery. […] Symptoms of TAPVR usually appear very soon after birth. Babies have symptoms at birth if they have any narrowing where their lung veins connect to their hearts. But some babies dont have symptoms for several weeks. […] 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. […] Cardiologists can sometimes diagnose this condition with an echocardiogram before birth. Some babies dont get a diagnosis until theyre several weeks or months old.

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

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

  During a physical exam, a doctor may observe symptoms or hear a heart murmur. […] If a doctor suspects a problem, the health care provider might request additional tests to confirm the diagnosis. […] The most common test is an echocardiogram, which is an ultrasound of the heart. […] TAPVR can also be detected with newborn pulse oximetry screening. […] Newborn screening using pulse oximetry can identify some infants with TAPVR before they show any symptoms. […] Babies with TAPVR will need surgery to repair the defect. […] The goal of surgical repair of TAPVR is to restore normal blood flow through the heart. […] To repair this defect, doctors usually connect the pulmonary veins to the left atrium. […] Surgical repairs for TAPVR are not a cure. […] Routine checkups with a heart doctor are needed to monitor progress.

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

  http://memorialhermann.org/ar-sa/services/conditions/pediatric-total-anomalous-pulmonary-venous-return

  If TAPVR is not diagnosed in utero, and suspicion of a heart defect occurs after the baby is born, a pediatrician will refer the patient to a pediatric cardiologist to determine the diagnosis. […] Signs of TAPVR include a bluish tint to lips, nails and skin, rapid breathing and sometimes a heart murmur – or whooshing sound – heard via a stethoscope. The worst cases are obvious at birth: Newborns may have difficulty breathing, an extreme bluish tint (from a lack of oxygen in the blood), a pounding heart, weak pulse, clammy skin, blood pressure issues or high acidity in the blood (acidosis). […] The following tests may be utilized to find more details: Pulse oximetry, a small and painless gadget placed on a finger, which may reveal low oxygen rates from an inefficient heart. Chest X-rays, which create images of the heart and lungs, making major flaws visible, such as fluid buildup in the lungs. A cardiac echocardiogram (echo), using sound waves (ultrasound) to produce images of the heart and blood vessels’ structures on a screen. This reveals whether the heart is pumping properly. An electrocardiogram (EKG or ECG), a painless exam, which checks the heart’s electrical action to reveal damage or irregular rhythms, suggesting problems with the heart. A cardiac MRI (magnetic resonance imaging), using radio waves, magnets and a computer to form three-dimensional images of the heart, which can reveal structural abnormalities (such as an enlarged right ventricle). Cardiac catheterization, which involves a thin, long tube that is inserted into a blood vessel and guided into the heart. The doctor can see more details of a mixed TAPVR and widen the ASD or narrowed vessels at that time, to boost blood oxygen levels short-term.

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

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

  During a physical exam, a doctor may observe symptoms or hear a heart murmur. […] If a doctor suspects a problem, the health care provider might request additional tests to confirm the diagnosis. […] The most common test is an echocardiogram, which is an ultrasound of the heart. […] TAPVR can also be detected with newborn pulse oximetry screening. […] Newborn screening using pulse oximetry can identify some infants with TAPVR before they show any symptoms. […] Babies with TAPVR will need surgery to repair the defect. […] The goal of surgical repair of TAPVR is to restore normal blood flow through the heart. […] To repair this defect, doctors usually connect the pulmonary veins to the left atrium. […] Surgical repairs for TAPVR are not a cure. […] Routine checkups with a heart doctor are needed to monitor progress.

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

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

  Symptoms of total anomalous pulmonary venous return (TAPVR) in babies may include trouble breathing, poor feeding and a weak pulse. The baby’s skin may look gray or blue due to low oxygen levels. This is called cyanosis. Depending on skin color, these changes may be easier or harder to see. […] Serious congenital heart defects are often diagnosed before or soon after a child is born. If you think that your baby has symptoms of total anomalous pulmonary venous return (TAPVR), call your child’s healthcare professional. […] To diagnose total anomalous pulmonary venus return (TAPVR), a healthcare professional listens to the heart and lungs. A whooshing sound, called a heart murmur, may be heard. […] 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. […] Other tests such as an electrocardiogram, a chest X-ray or a CT scan may be done if more information is needed.

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

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

  During a physical exam, a doctor may observe symptoms or hear a heart murmur. […] If a doctor suspects a problem, the health care provider might request additional tests to confirm the diagnosis. […] The most common test is an echocardiogram, which is an ultrasound of the heart. […] TAPVR can also be detected with newborn pulse oximetry screening. […] Newborn screening using pulse oximetry can identify some infants with TAPVR before they show any symptoms. […] Babies with TAPVR will need surgery to repair the defect. […] The goal of surgical repair of TAPVR is to restore normal blood flow through the heart. […] To repair this defect, doctors usually connect the pulmonary veins to the left atrium. […] Surgical repairs for TAPVR are not a cure. […] Routine checkups with a heart doctor are needed to monitor progress.

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

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

  Symptoms of total anomalous pulmonary venous return (TAPVR) in babies may include trouble breathing, poor feeding and a weak pulse. The baby’s skin may look gray or blue due to low oxygen levels. This is called cyanosis. Depending on skin color, these changes may be easier or harder to see. […] Serious congenital heart defects are often diagnosed before or soon after a child is born. If you think that your baby has symptoms of total anomalous pulmonary venous return (TAPVR), call your child’s healthcare professional. […] To diagnose total anomalous pulmonary venus return (TAPVR), a healthcare professional listens to the heart and lungs. A whooshing sound, called a heart murmur, may be heard. […] 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. […] Other tests such as an electrocardiogram, a chest X-ray or a CT scan may be done if more information is needed.

• #34 Total anomalous pulmonary venous return (TAPVR) – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/total-anomalous-pulmonary-venous-return-tapvr/

  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). […] Your child’s doctor will perform a physical exam and listen to your child’s heart with a stethoscope to check for a heart murmur. […] An echocardiogram is generally used to diagnose total anomalous pulmonary venous return. This test uses sound waves to create images of your child’s heart in motion. An echocardiogram can show pulmonary veins, holes in the heart and the size of the heart chambers. It can also measure the rate of blood flow. […] Other tests such as an electrocardiogram (ECG or EKG), a chest X-ray or computerized tomography (CT) scan may be done if more information is needed.

• #35 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 (TAPVR) may be diagnosed before birth, with fetal echocardiogram (ultrasound). […] In other cases, TAPVR is diagnosed in the first few months of life, after a child demonstrates milder symptoms such as a heart murmur or cyanosis (blue tint to skin). […] Diagnosis of TAPVR may require some or all of these tests: Echocardiogram: sound waves create an image of the heart. This is generally the definitive test for diagnosis. […] Electrocardiogram (ECG): a record of the electrical activity of the heart. This usually shows evidence of right heart hypertrophy (increased size of the right ventricle). […] Chest X-ray: this will show right heart enlargement and increased blood flow through the pulmonary artery. If the veins are obstructed, there will be pulmonary edema (buildup of fluid in the lungs).

• #36 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. […] Diagnosis is by echocardiography. Surgical repair is required. […] Diagnosis of total anomalous pulmonary venous return is suspected by chest x-ray and established by echocardiography. Cardiac catheterization is rarely necessary; occasionally, cardiac MRI or CT angiography may need to be done to better delineate the anatomy of pulmonary venous return.

• #37 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 (TAPVR) may be diagnosed before birth, with fetal echocardiogram (ultrasound). […] In other cases, TAPVR is diagnosed in the first few months of life, after a child demonstrates milder symptoms such as a heart murmur or cyanosis (blue tint to skin). […] Diagnosis of TAPVR may require some or all of these tests: Echocardiogram: sound waves create an image of the heart. This is generally the definitive test for diagnosis. […] Electrocardiogram (ECG): a record of the electrical activity of the heart. This usually shows evidence of right heart hypertrophy (increased size of the right ventricle). […] Chest X-ray: this will show right heart enlargement and increased blood flow through the pulmonary artery. If the veins are obstructed, there will be pulmonary edema (buildup of fluid in the lungs).

• #38 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  Next, your childs healthcare provider may do tests to check for heart problems. The tests your child needs depend on their age and condition. They also depend on the providers preferences. […] A chest X-ray may show changes in the heart and lungs caused by TAPVR. […] An ECG records the electrical activity of the heart. It also shows abnormal rhythms (arrhythmias) and finds heart muscle stress. These problems may be caused by TAPVR. […] This test uses sound waves to make a moving picture of the heart and heart valves. An echo can show the structural changes of TAPVR and abnormal connections of the pulmonary veins. […] A cardiac catheterization gives detailed information about the structures in the heart. […] A CT and MRA can show detailed information about the pathways and connections of the pulmonary veins.

• #39 Anomalous pulmonary venous connection – Wikipedia

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

  The diagnosis of TAPVC is dependent upon whether or not there is an obstruction. TAPVC with obstruction will clinically present with cyanosis, tachypnea, dyspnea, and hypoxemia. Chest x-ray may show a ground-glass appearance due to pulmonary edema resulting from increased pressure in the obstructed pulmonary veins.

• #40 Total anomalous pulmonary venous return – WikEM

  https://wikem.org/wiki/Total_anomalous_pulmonary_venous_return

  All four pulmonary veins drain into the systemic venous circulation rather than into the left atrium. […] In obstructed forms of TAPVR, the pulmonary venous system is compressed by surrounding structures. […] Differentiation by pulmonary vascularity on CXR. […] Total anomalous pulmonary venous return. […] Echocardiography. […] Angiography: gold standard but generally no longer required given other less invasive diagnostic modalities. […] Chest x-ray: Nonspecific findings, cannot be used to make diagnosis. […] Prostaglandin E1 may be required to prevent closure of ductus arteriosus to maintain systemic cardiac output.

• #41 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 (TAPVR) may be diagnosed before birth, with fetal echocardiogram (ultrasound). […] In other cases, TAPVR is diagnosed in the first few months of life, after a child demonstrates milder symptoms such as a heart murmur or cyanosis (blue tint to skin). […] Diagnosis of TAPVR may require some or all of these tests: Echocardiogram: sound waves create an image of the heart. This is generally the definitive test for diagnosis. […] Electrocardiogram (ECG): a record of the electrical activity of the heart. This usually shows evidence of right heart hypertrophy (increased size of the right ventricle). […] Chest X-ray: this will show right heart enlargement and increased blood flow through the pulmonary artery. If the veins are obstructed, there will be pulmonary edema (buildup of fluid in the lungs).

• #42 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  Next, your childs healthcare provider may do tests to check for heart problems. The tests your child needs depend on their age and condition. They also depend on the providers preferences. […] A chest X-ray may show changes in the heart and lungs caused by TAPVR. […] An ECG records the electrical activity of the heart. It also shows abnormal rhythms (arrhythmias) and finds heart muscle stress. These problems may be caused by TAPVR. […] This test uses sound waves to make a moving picture of the heart and heart valves. An echo can show the structural changes of TAPVR and abnormal connections of the pulmonary veins. […] A cardiac catheterization gives detailed information about the structures in the heart. […] A CT and MRA can show detailed information about the pathways and connections of the pulmonary veins.

• #43 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. […] Echocardiography is needed for diagnosis. […] Doctors suspect the diagnosis based on the findings on a chest x-ray, a heart murmur heard with a stethoscope, or low oxygen level in the blood detected by pulse oximetry. The diagnosis is confirmed by echocardiography (ultrasonography of the heart). Occasionally, magnetic resonance imaging (MRI) of the heart or computed tomography (CT) angiography may be needed so doctors can more clearly view the defect.

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

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

  Pulse oximetry: a noninvasive way to monitor the oxygen content of the blood. This number will be low, generally in the mid to high 80s (normal is 95 to 100 percent). […] Cardiac catheterization: a thin tube is inserted into the heart through a vein and/or artery in the leg. This is occasionally needed to aid in diagnosis. […] Cardiac MRI: a three-dimensional image that shows the hearts abnormalities.

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

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

  During a physical exam, a doctor may observe symptoms or hear a heart murmur. […] If a doctor suspects a problem, the health care provider might request additional tests to confirm the diagnosis. […] The most common test is an echocardiogram, which is an ultrasound of the heart. […] TAPVR can also be detected with newborn pulse oximetry screening. […] Newborn screening using pulse oximetry can identify some infants with TAPVR before they show any symptoms. […] Babies with TAPVR will need surgery to repair the defect. […] The goal of surgical repair of TAPVR is to restore normal blood flow through the heart. […] To repair this defect, doctors usually connect the pulmonary veins to the left atrium. […] Surgical repairs for TAPVR are not a cure. […] Routine checkups with a heart doctor are needed to monitor progress.

• #46 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). […] Tests may include: […] Cardiac catheterization can confirm the diagnosis by showing that the blood vessels are abnormally attached […] 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. […] Contact your health care provider if you notice symptoms of TAPVR. Prompt attention is required.

• #47 Total Anomalous Pulmonary Venous Return (TAPVR) – Cardiothoracic Surgery

  https://lsom.uthscsa.edu/ct-surgery/patient-care/congenital-heart/conditions-we-treat/total-anomalous-pulmonary-venous-return-tapvr/

  In TAPVR the entire pulmonary venous circulation drains to the RA, either directly or by a systemic vein or sinus connecting with the RA via the SVC, IVC, or coronary sinus. An ASD is necessary to deliver oxygenated blood to the left heart. TAPVR is classified as supracardiac, cardiac, or infracardiac. In the most common variant of supracardiac TAPVR, all four pulmonary veins drain into a common, left vertical vein, which then drains into the innominate vein, azygous vein, SVC, or RA. The classic CXR finding is that of a “snowman” or “figure-of-eight” due to the large vertical vein on the left and SVC on the right. In the cardiac subtype, pulmonary venous drainage is into the coronary sinus. In the infracardiac variant, a vertical vein descends below the diaphragm, where it usually connects with the portal vein and then into the IVC. […] Diagnosis and mapping of the anomalous connections is possible by echocardiography. Obstructed TAPVR is a surgical emergency.

• #48 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. […] The diagnosis of total anomalous pulmonary venous return may be suspected when a doctor hears a typical heart murmur. They may see evidence of overload on the right side of the heart. […] The diagnosis of TAPVR is made by echocardiogram (echo). This study will show the abnormal connection of the pulmonary veins. […] Sometimes cardiac catheterization is needed to make a diagnosis of TAPVR. […] Cardiac catheterization will define the abnormal connection of all pulmonary veins. This is helpful finding unusual patterns of mixed TAPVR.

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

  http://memorialhermann.org/ar-sa/services/conditions/pediatric-total-anomalous-pulmonary-venous-return

  If TAPVR is not diagnosed in utero, and suspicion of a heart defect occurs after the baby is born, a pediatrician will refer the patient to a pediatric cardiologist to determine the diagnosis. […] Signs of TAPVR include a bluish tint to lips, nails and skin, rapid breathing and sometimes a heart murmur – or whooshing sound – heard via a stethoscope. The worst cases are obvious at birth: Newborns may have difficulty breathing, an extreme bluish tint (from a lack of oxygen in the blood), a pounding heart, weak pulse, clammy skin, blood pressure issues or high acidity in the blood (acidosis). […] The following tests may be utilized to find more details: Pulse oximetry, a small and painless gadget placed on a finger, which may reveal low oxygen rates from an inefficient heart. Chest X-rays, which create images of the heart and lungs, making major flaws visible, such as fluid buildup in the lungs. A cardiac echocardiogram (echo), using sound waves (ultrasound) to produce images of the heart and blood vessels’ structures on a screen. This reveals whether the heart is pumping properly. An electrocardiogram (EKG or ECG), a painless exam, which checks the heart’s electrical action to reveal damage or irregular rhythms, suggesting problems with the heart. A cardiac MRI (magnetic resonance imaging), using radio waves, magnets and a computer to form three-dimensional images of the heart, which can reveal structural abnormalities (such as an enlarged right ventricle). Cardiac catheterization, which involves a thin, long tube that is inserted into a blood vessel and guided into the heart. The doctor can see more details of a mixed TAPVR and widen the ASD or narrowed vessels at that time, to boost blood oxygen levels short-term.

• #50 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. […] Diagnosis is by echocardiography. Surgical repair is required. […] Diagnosis of total anomalous pulmonary venous return is suspected by chest x-ray and established by echocardiography. Cardiac catheterization is rarely necessary; occasionally, cardiac MRI or CT angiography may need to be done to better delineate the anatomy of pulmonary venous return.

• #51 Total Anomalous Pulmonary Venous Return | Thoracic Key

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

  With the advent of multidetector computed tomography (CT) scanning, detailed images of the pulmonary venous drainage, including inflow and runoff vessels from the confluence, can be achieved. […] Currently available magnetic resonance imaging (MRI) scanners and cardiovascular imaging software from all manufacturers provide high-resolution angiographic images without patient exposure to radiation. […] Diagnostic cardiac catheterization is reserved for patients in whom echocardiography or CT imaging is not satisfactory or when associated lesions need to be further defined.

• #52 Use of CT angiography in the diagnosis of total anomalous venous return – PubMed

  https://pubmed.ncbi.nlm.nih.gov/19474817/

  Total anomalous pulmonary venous return (TAPVR) is an uncommon cause of cyanotic congenital heart disease in the neonatal period. This diagnosis is often made by echocardiography. […] We present two cases where echocardiography was not confirmatory. Computerized tomography (CT) scan of the chest and abdomen with contrast was performed instead of cardiac catheterization to establish the diagnosis. […] We suggest that CT with contrast is a noninvasive modality to obtain anatomic details of pulmonary venous drainage in TAPVR when echocardiography is inconclusive.

• #53 Supracardiac Total Anomalous Pulmonary Venous Return (TAPVR)

  https://www.diagnosticimaging.com/view/supracardiac-total-anomalous-pulmonary-venous-return-tapvr

  Supracardiac total anomalous pulmonary venous return (TAPVR) case study: A term newborn infant developed profound respiratory distress shortly after birth. […] After a negative echocardiogram (images not shown), venovenous ECMO (arrowheads) was performed. A cardiac CTA was requested, but complicated by the fact that the patient was on ECMO. […] The CTA revealed all four pulmonary veins formed a common vein that tapered to a short segment obstructing stenosis before draining into the right superior vena cava (video, arrows) consistent with supracardiac total anomalous pulmonary venous return (TAPVR). […] Diagnosis: Supracardiac total anomalous pulmonary venous return (TAPVR). […] This illustrates an unusual case of obstructing Type 1 TAPVR, whose imaging was complicated by ECMO therapy. […] TAPVR is the embryologic failure of connection between the pulmonary veins and left atrium that results in abnormal connection of the pulmonary veins to the right atrium resulting in a left to right shunt.

• #54 Total Anomalous Pulmonary Venous Return | Thoracic Key

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

  With the advent of multidetector computed tomography (CT) scanning, detailed images of the pulmonary venous drainage, including inflow and runoff vessels from the confluence, can be achieved. […] Currently available magnetic resonance imaging (MRI) scanners and cardiovascular imaging software from all manufacturers provide high-resolution angiographic images without patient exposure to radiation. […] Diagnostic cardiac catheterization is reserved for patients in whom echocardiography or CT imaging is not satisfactory or when associated lesions need to be further defined.

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

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

  Symptoms of total anomalous pulmonary venous return (TAPVR) in babies may include trouble breathing, poor feeding and a weak pulse. The baby’s skin may look gray or blue due to low oxygen levels. This is called cyanosis. Depending on skin color, these changes may be easier or harder to see. […] Serious congenital heart defects are often diagnosed before or soon after a child is born. If you think that your baby has symptoms of total anomalous pulmonary venous return (TAPVR), call your child’s healthcare professional. […] To diagnose total anomalous pulmonary venus return (TAPVR), a healthcare professional listens to the heart and lungs. A whooshing sound, called a heart murmur, may be heard. […] 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. […] Other tests such as an electrocardiogram, a chest X-ray or a CT scan may be done if more information is needed.

• #56 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  Next, your childs healthcare provider may do tests to check for heart problems. The tests your child needs depend on their age and condition. They also depend on the providers preferences. […] A chest X-ray may show changes in the heart and lungs caused by TAPVR. […] An ECG records the electrical activity of the heart. It also shows abnormal rhythms (arrhythmias) and finds heart muscle stress. These problems may be caused by TAPVR. […] This test uses sound waves to make a moving picture of the heart and heart valves. An echo can show the structural changes of TAPVR and abnormal connections of the pulmonary veins. […] A cardiac catheterization gives detailed information about the structures in the heart. […] A CT and MRA can show detailed information about the pathways and connections of the pulmonary veins.

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

  http://memorialhermann.org/ar-sa/services/conditions/pediatric-total-anomalous-pulmonary-venous-return

  If TAPVR is not diagnosed in utero, and suspicion of a heart defect occurs after the baby is born, a pediatrician will refer the patient to a pediatric cardiologist to determine the diagnosis. […] Signs of TAPVR include a bluish tint to lips, nails and skin, rapid breathing and sometimes a heart murmur – or whooshing sound – heard via a stethoscope. The worst cases are obvious at birth: Newborns may have difficulty breathing, an extreme bluish tint (from a lack of oxygen in the blood), a pounding heart, weak pulse, clammy skin, blood pressure issues or high acidity in the blood (acidosis). […] The following tests may be utilized to find more details: Pulse oximetry, a small and painless gadget placed on a finger, which may reveal low oxygen rates from an inefficient heart. Chest X-rays, which create images of the heart and lungs, making major flaws visible, such as fluid buildup in the lungs. A cardiac echocardiogram (echo), using sound waves (ultrasound) to produce images of the heart and blood vessels’ structures on a screen. This reveals whether the heart is pumping properly. An electrocardiogram (EKG or ECG), a painless exam, which checks the heart’s electrical action to reveal damage or irregular rhythms, suggesting problems with the heart. A cardiac MRI (magnetic resonance imaging), using radio waves, magnets and a computer to form three-dimensional images of the heart, which can reveal structural abnormalities (such as an enlarged right ventricle). Cardiac catheterization, which involves a thin, long tube that is inserted into a blood vessel and guided into the heart. The doctor can see more details of a mixed TAPVR and widen the ASD or narrowed vessels at that time, to boost blood oxygen levels short-term.

• #58 Total Anomalous Pulmonary Venous Return | Thoracic Key

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

  With the advent of multidetector computed tomography (CT) scanning, detailed images of the pulmonary venous drainage, including inflow and runoff vessels from the confluence, can be achieved. […] Currently available magnetic resonance imaging (MRI) scanners and cardiovascular imaging software from all manufacturers provide high-resolution angiographic images without patient exposure to radiation. […] Diagnostic cardiac catheterization is reserved for patients in whom echocardiography or CT imaging is not satisfactory or when associated lesions need to be further defined.

• #59 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  Next, your childs healthcare provider may do tests to check for heart problems. The tests your child needs depend on their age and condition. They also depend on the providers preferences. […] A chest X-ray may show changes in the heart and lungs caused by TAPVR. […] An ECG records the electrical activity of the heart. It also shows abnormal rhythms (arrhythmias) and finds heart muscle stress. These problems may be caused by TAPVR. […] This test uses sound waves to make a moving picture of the heart and heart valves. An echo can show the structural changes of TAPVR and abnormal connections of the pulmonary veins. […] A cardiac catheterization gives detailed information about the structures in the heart. […] A CT and MRA can show detailed information about the pathways and connections of the pulmonary veins.

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

  http://memorialhermann.org/ar-sa/services/conditions/pediatric-total-anomalous-pulmonary-venous-return

  If TAPVR is not diagnosed in utero, and suspicion of a heart defect occurs after the baby is born, a pediatrician will refer the patient to a pediatric cardiologist to determine the diagnosis. […] Signs of TAPVR include a bluish tint to lips, nails and skin, rapid breathing and sometimes a heart murmur – or whooshing sound – heard via a stethoscope. The worst cases are obvious at birth: Newborns may have difficulty breathing, an extreme bluish tint (from a lack of oxygen in the blood), a pounding heart, weak pulse, clammy skin, blood pressure issues or high acidity in the blood (acidosis). […] The following tests may be utilized to find more details: Pulse oximetry, a small and painless gadget placed on a finger, which may reveal low oxygen rates from an inefficient heart. Chest X-rays, which create images of the heart and lungs, making major flaws visible, such as fluid buildup in the lungs. A cardiac echocardiogram (echo), using sound waves (ultrasound) to produce images of the heart and blood vessels’ structures on a screen. This reveals whether the heart is pumping properly. An electrocardiogram (EKG or ECG), a painless exam, which checks the heart’s electrical action to reveal damage or irregular rhythms, suggesting problems with the heart. A cardiac MRI (magnetic resonance imaging), using radio waves, magnets and a computer to form three-dimensional images of the heart, which can reveal structural abnormalities (such as an enlarged right ventricle). Cardiac catheterization, which involves a thin, long tube that is inserted into a blood vessel and guided into the heart. The doctor can see more details of a mixed TAPVR and widen the ASD or narrowed vessels at that time, to boost blood oxygen levels short-term.

• #61 Total Anomalous Pulmonary Venous Return | Thoracic Key

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

  With the advent of multidetector computed tomography (CT) scanning, detailed images of the pulmonary venous drainage, including inflow and runoff vessels from the confluence, can be achieved. […] Currently available magnetic resonance imaging (MRI) scanners and cardiovascular imaging software from all manufacturers provide high-resolution angiographic images without patient exposure to radiation. […] Diagnostic cardiac catheterization is reserved for patients in whom echocardiography or CT imaging is not satisfactory or when associated lesions need to be further defined.

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

  https://radiopaedia.org/articles/total-anomalous-pulmonary-venous-return?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. […] 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). […] TAPVR can be classified into four types (in decreasing order of frequency) depending on the site of anomalous venous union. […] The right heart is prominent in TAPVR because of the increased flow volume, but the left atrium remains normal in size. […] Transthoracic echocardiography may be used to define the anatomical connections present, as well as assess the hemodynamic consequences. […] Direct visualization of anomalous venous return.

• #63 ~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. […] As a result, the right side of the heart is overworked from pumping the extra blood it receives. […] 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. […] Generally, there are four types of TAPVR.

• #64 Total Anomalous Pulmonary Venous Return (TAPVR) – Cardiothoracic Surgery

  https://lsom.uthscsa.edu/ct-surgery/patient-care/congenital-heart/conditions-we-treat/total-anomalous-pulmonary-venous-return-tapvr/

  In TAPVR the entire pulmonary venous circulation drains to the RA, either directly or by a systemic vein or sinus connecting with the RA via the SVC, IVC, or coronary sinus. An ASD is necessary to deliver oxygenated blood to the left heart. TAPVR is classified as supracardiac, cardiac, or infracardiac. In the most common variant of supracardiac TAPVR, all four pulmonary veins drain into a common, left vertical vein, which then drains into the innominate vein, azygous vein, SVC, or RA. The classic CXR finding is that of a “snowman” or “figure-of-eight” due to the large vertical vein on the left and SVC on the right. In the cardiac subtype, pulmonary venous drainage is into the coronary sinus. In the infracardiac variant, a vertical vein descends below the diaphragm, where it usually connects with the portal vein and then into the IVC. […] Diagnosis and mapping of the anomalous connections is possible by echocardiography. Obstructed TAPVR is a surgical emergency.

• #65 Total Anomalous Pulmonary Venous Return (TAPVR) – Cardiothoracic Surgery

  https://lsom.uthscsa.edu/ct-surgery/patient-care/congenital-heart/conditions-we-treat/total-anomalous-pulmonary-venous-return-tapvr/

  In TAPVR the entire pulmonary venous circulation drains to the RA, either directly or by a systemic vein or sinus connecting with the RA via the SVC, IVC, or coronary sinus. An ASD is necessary to deliver oxygenated blood to the left heart. TAPVR is classified as supracardiac, cardiac, or infracardiac. In the most common variant of supracardiac TAPVR, all four pulmonary veins drain into a common, left vertical vein, which then drains into the innominate vein, azygous vein, SVC, or RA. The classic CXR finding is that of a “snowman” or “figure-of-eight” due to the large vertical vein on the left and SVC on the right. In the cardiac subtype, pulmonary venous drainage is into the coronary sinus. In the infracardiac variant, a vertical vein descends below the diaphragm, where it usually connects with the portal vein and then into the IVC. […] Diagnosis and mapping of the anomalous connections is possible by echocardiography. Obstructed TAPVR is a surgical emergency.

• #66 Total Anomalous Pulmonary Venous Return (TAPVR) – Cardiothoracic Surgery

  https://lsom.uthscsa.edu/ct-surgery/patient-care/congenital-heart/conditions-we-treat/total-anomalous-pulmonary-venous-return-tapvr/

  In TAPVR the entire pulmonary venous circulation drains to the RA, either directly or by a systemic vein or sinus connecting with the RA via the SVC, IVC, or coronary sinus. An ASD is necessary to deliver oxygenated blood to the left heart. TAPVR is classified as supracardiac, cardiac, or infracardiac. In the most common variant of supracardiac TAPVR, all four pulmonary veins drain into a common, left vertical vein, which then drains into the innominate vein, azygous vein, SVC, or RA. The classic CXR finding is that of a “snowman” or “figure-of-eight” due to the large vertical vein on the left and SVC on the right. In the cardiac subtype, pulmonary venous drainage is into the coronary sinus. In the infracardiac variant, a vertical vein descends below the diaphragm, where it usually connects with the portal vein and then into the IVC. […] Diagnosis and mapping of the anomalous connections is possible by echocardiography. Obstructed TAPVR is a surgical emergency.

• #67 Total Anomalous Pulmonary Venous Return (TAPVR) – Cardiothoracic Surgery

  https://lsom.uthscsa.edu/ct-surgery/patient-care/congenital-heart/conditions-we-treat/total-anomalous-pulmonary-venous-return-tapvr/

  In TAPVR the entire pulmonary venous circulation drains to the RA, either directly or by a systemic vein or sinus connecting with the RA via the SVC, IVC, or coronary sinus. An ASD is necessary to deliver oxygenated blood to the left heart. TAPVR is classified as supracardiac, cardiac, or infracardiac. In the most common variant of supracardiac TAPVR, all four pulmonary veins drain into a common, left vertical vein, which then drains into the innominate vein, azygous vein, SVC, or RA. The classic CXR finding is that of a “snowman” or “figure-of-eight” due to the large vertical vein on the left and SVC on the right. In the cardiac subtype, pulmonary venous drainage is into the coronary sinus. In the infracardiac variant, a vertical vein descends below the diaphragm, where it usually connects with the portal vein and then into the IVC. […] Diagnosis and mapping of the anomalous connections is possible by echocardiography. Obstructed TAPVR is a surgical emergency.

• #68 2025 ICD-10-CM Diagnosis Code Q26.2: Total anomalous pulmonary venous connection

  https://www.icd10data.com/ICD10CM/Codes/Q00-Q99/Q20-Q28/Q26-/Q26.2

  Q26.2 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. […] Total anomalous pulmonary venous return [TAPVR], subdiaphragmatic […] Total anomalous pulmonary venous return [TAPVR], supradiaphragmatic […] A rare congenital cardiopulmonary defect characterized by abnormal right-sided pulmonary venous drainage and right lung malformations. […] A rare congenital heart disorder in which all four pulmonary veins are not connected to the left atrium and drain into the systemic veins or the right atrium instead. Infants present with cyanosis, lethargy, tachypnea, respiratory infections, and poor growth. […] An anomalous pulmonary venous return in which the right pulmonary vein is not connected to the left atrium but to the inferior vena cava. Scimitar syndrome is named for the crescent- or turkish sword-like shadow in the chest radiography and is often associated with hypoplasia of the right lung and right pulmonary artery, and dextroposition of the heart.

• #69 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. […] The diagnosis of total anomalous pulmonary venous return may be suspected when a doctor hears a typical heart murmur. They may see evidence of overload on the right side of the heart. […] The diagnosis of TAPVR is made by echocardiogram (echo). This study will show the abnormal connection of the pulmonary veins. […] Sometimes cardiac catheterization is needed to make a diagnosis of TAPVR. […] Cardiac catheterization will define the abnormal connection of all pulmonary veins. This is helpful finding unusual patterns of mixed TAPVR.

• #70

  https://journals.lww.com/jmut/fulltext/2024/32020/prenatal_ultrasound_markers_of_isolated_total.2.aspx

  In fetal cardiac screening, the left and right pulmonary veins entering the LA are commonly observed in the four-chamber view. Their presence effectively rules out the possibility of TAPVR. However, when the connection between the pulmonary veins and the LA is not clear, the sonographer should raise suspicions of TAPVR and conduct a systematic search for other possible pulmonary venous drainage routes. […] In the absence of a single sonographic sign for the diagnosis of fetal TAPVR, a systematic and sequential approach is crucial to improve the prenatal detection and diagnosis of TAPVR. The key to enhancing prenatal diagnosis is adherence to the fetal cardiac screening guidelines and evaluation of pulmonary venous entry into the LA in the four-chamber view. Other sonographic signs including direct and indirect markers, when used in conjunction with color and spectral Doppler, serve as adjuncts to precise prenatal diagnosis. Further follow-up at later gestation can be suggested in cases with equivocal findings.

• #71 :: 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. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those of respiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. […] This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. […] Therefore, misdiagnosis and a delayed operation can increase mortality and morbidity in infants with TAPVR. […] 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.

• #72 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  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%. […] Your child will need regular visits with their cardiologist as they grow into adulthood. Lifelong follow-up visits can help cardiologists find problems like an irregular heartbeat or blockages (obstructions) in their blood vessels. An obstruction requires another surgery and may be hard to treat. […] After surgery, your baby will have checkups every six to 12 months. Theyll need regular follow-up appointments through adulthood. Your childs provider may want to order tests like an electrocardiogram, exercise stress test or echocardiogram.

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

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

  During a physical exam, a doctor may observe symptoms or hear a heart murmur. […] If a doctor suspects a problem, the health care provider might request additional tests to confirm the diagnosis. […] The most common test is an echocardiogram, which is an ultrasound of the heart. […] TAPVR can also be detected with newborn pulse oximetry screening. […] Newborn screening using pulse oximetry can identify some infants with TAPVR before they show any symptoms. […] Babies with TAPVR will need surgery to repair the defect. […] The goal of surgical repair of TAPVR is to restore normal blood flow through the heart. […] To repair this defect, doctors usually connect the pulmonary veins to the left atrium. […] Surgical repairs for TAPVR are not a cure. […] Routine checkups with a heart doctor are needed to monitor progress.

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

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

  Newborns with severe TAPVR will need emergency surgery shortly after birth. They often need to be admitted to the cardiac intensive care unit (CICU) and require intensive support with medications and a ventilator (breathing machine). […] Babies with less severe TAPVR usually have surgery in the days or weeks after they’re diagnosed. The goal of surgery for TAPVR is to restore normal connections of the pulmonary veins to the heart, alleviate any obstructions or narrowing of the pulmonary veins, to tie up any vessels that have developed and to close the atrial septal defect (ASD). […] Thanks to updates in surgical techniques for repairing TAPVR, the long-term outlook is continually improving. Children who have had a repair for TAPVR will require lifelong checkups with a cardiologist to make sure their veins remain open. If the veins become narrowed, they may need catheterizations or surgeries to repair these veins.

• #75 Understanding Total Anomalous Pulmonary Venous Return

  https://lagans.org.uk/total-anomalous-pulmonary-venus-return/

  Total Anomalous Pulmonary Venous Return is a rare heart defect that is present at birth and it occurs when pulmonary veins connect to the heart in an abnormal way. […] Total Anomalous Pulmonary Venous Return (TAPVR) is a congenital heart defect that affects the pulmonary veins. […] Symptoms of Total Anomalous Pulmonary Venous Return will usually occur immediately after birth or soon after. Infants with TAPVR will often have bluish looking skin, called cyanosis, because their blood doesnt carry enough oxygen. […] All types of Total Anomalous Pulmonary Venous Return will need to be treated with surgery, but the timing of the surgery will depend on the type of TAPVR present. […] Newborns who have an obstructed TAPVR will need surgery immediately after birth. […] Infants with an unobstructed TAPVR will typically have surgery days to weeks after the diagnosis is made.

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

  http://memorialhermann.org/ar-sa/services/conditions/pediatric-total-anomalous-pulmonary-venous-return

  Treatment of TAPVR is surgery, which consists of placing the patient on the heart-lung machine (cardiopulmonary bypass) and making the connection between the pulmonary veins and the left atrium. Fortunately, in most cases of TAPVR, all four pulmonary veins come to a central place (called a confluence) just behind the left atrium and a full anatomical repair can be achieved. However, after surgery, scar tissue may form around the connection between the pulmonary veins and the left atrium. If the scar tissue formation is severe enough, additional surgery may be required in the future. […] If your child has a form of TAPVR that is obstructed (where the blood cannot get to the heart from the lungs), this may require an emergent surgical intervention. This is often performed when the child is only hours old. If the pulmonary veins are unobstructed, surgery can take place at an agreed upon elective date, usually at a few weeks of life.

• #77 Total Anomalous Pulmonary Venous Return (TAPVR)

  https://healthlibrary.brighamandwomens.org/library/diseasesconditions/adult/CompAltMed/90,P01820

  An ECG records the electrical activity of the heart. It also shows abnormal rhythms (arrhythmias) and finds heart muscle stress. These problems may be caused by TAPVR. […] This test uses sound waves to make a moving picture of the heart and heart valves. An echo can show the structural changes of TAPVR and abnormal connections of the pulmonary veins. […] A cardiac catheterization gives detailed information about the structures in the heart. […] A CT and MRA can show detailed information about the pathways and connections of the pulmonary veins. […] All children with a TAPVR will need to have surgery to fix it. […] The surgery is done through a cut (incision) through the breastbone (sternum) and into the chest. Your baby will be connected to a heart-lung machine during surgery. This device does the work of your baby’s heart and lungs during surgery.

• #78 Total Anomalous Pulmonary Venous Return (TAPVR)

  https://healthlibrary.brighamandwomens.org/library/diseasesconditions/adult/CompAltMed/90,P01820

  The complications of TAPVR include: Enlarged heart, Lung problems, including respiratory failure and high blood pressure in the lungs, Abnormal heart rhythms, Shock, Heart failure, Slowed growth and development, Enlarged liver, Need for another surgery, Infection, Death. […] Many children with TAPVR will grow and develop normally after they have surgery. […] Your child will need to see a heart doctor for regular checkups for the rest of their life.

• #79 :: 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. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those of respiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. […] This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. […] Therefore, misdiagnosis and a delayed operation can increase mortality and morbidity in infants with TAPVR. […] 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.

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

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

  Newborns with severe TAPVR will need emergency surgery shortly after birth. They often need to be admitted to the cardiac intensive care unit (CICU) and require intensive support with medications and a ventilator (breathing machine). […] Babies with less severe TAPVR usually have surgery in the days or weeks after they’re diagnosed. The goal of surgery for TAPVR is to restore normal connections of the pulmonary veins to the heart, alleviate any obstructions or narrowing of the pulmonary veins, to tie up any vessels that have developed and to close the atrial septal defect (ASD). […] Thanks to updates in surgical techniques for repairing TAPVR, the long-term outlook is continually improving. Children who have had a repair for TAPVR will require lifelong checkups with a cardiologist to make sure their veins remain open. If the veins become narrowed, they may need catheterizations or surgeries to repair these veins.

• #81 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  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%. […] Your child will need regular visits with their cardiologist as they grow into adulthood. Lifelong follow-up visits can help cardiologists find problems like an irregular heartbeat or blockages (obstructions) in their blood vessels. An obstruction requires another surgery and may be hard to treat. […] After surgery, your baby will have checkups every six to 12 months. Theyll need regular follow-up appointments through adulthood. Your childs provider may want to order tests like an electrocardiogram, exercise stress test or echocardiogram.

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

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

  Newborns with severe TAPVR will need emergency surgery shortly after birth. They often need to be admitted to the cardiac intensive care unit (CICU) and require intensive support with medications and a ventilator (breathing machine). […] Babies with less severe TAPVR usually have surgery in the days or weeks after they’re diagnosed. The goal of surgery for TAPVR is to restore normal connections of the pulmonary veins to the heart, alleviate any obstructions or narrowing of the pulmonary veins, to tie up any vessels that have developed and to close the atrial septal defect (ASD). […] Thanks to updates in surgical techniques for repairing TAPVR, the long-term outlook is continually improving. Children who have had a repair for TAPVR will require lifelong checkups with a cardiologist to make sure their veins remain open. If the veins become narrowed, they may need catheterizations or surgeries to repair these veins.

• #83 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

  Patients with a history of TAPVC should be seen periodically by a cardiologist with expertise in adult congenital heart disease to look for uncommon problems. The long-term outlook is excellent, and usually no medicines and no more surgery or catheterization are needed. […] Most adults with repaired TAPVC will not need to limit their physical activities to their own endurance. Some competitive sports may have greater risk if there is leftover obstruction in the pulmonary veins, or if the patient has heart rhythm problems. Your cardiologist will help determine the proper level of activity for you.

• #84 :: 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. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those of respiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. […] This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. […] Therefore, misdiagnosis and a delayed operation can increase mortality and morbidity in infants with TAPVR. […] 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.

• #85

  https://journals.lww.com/jmut/fulltext/2024/32020/prenatal_ultrasound_markers_of_isolated_total.2.aspx

  This article comprehensively reviews the literature concerning prenatal ultrasound findings of isolated total anomalous pulmonary venous return (TAPVR) and the application of specific sonographic markers to differentiate among the TAPVR types. These markers can be categorized as direct and indirect, based on either morphological features or hemodynamic changes in TAPVR. Diagnosing isolated TAPVR can pose challenges, but following a sequential approach can improve detection rates and outcomes. Early neonatal or prenatal diagnosis, followed by timely postnatal intervention, is crucial for improving outcomes. However, despite recent advances in ultrasound evaluation of the fetal venous system, the prenatal diagnosis of isolated TAPVR remains challenging. This article aims to review the current understanding of sonographic features of fetal TAPVR and provides a sequential approach starting from obstetric screening views to identify possible cases and categorize the TAPVR types step by step.

• #86 Can Prenatal Diagnosis of Total Anomalous Pulmonary Venous Return (TAPVR) Using Routine Fetal Ultrasound be Improved? A Case-Control Study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9825205/

  To determine the most common fetal ultrasound markers of total anomalous pulmonary venous return (TAPVR) during mid-trimester ultrasound using standardly obtained images and evaluate the performance of diagnostic algorithms for improving prenatal diagnosis. […] The most common ultrasound marker of TAPVR was absence of pulmonary vein entry into the LA (42.9%), followed by abnormal Coumadin ridge (38.1%). […] Using standardly obtained images from routine fetal ultrasound, improved prenatal detection of isolated TAPVR is possible. A standardized diagnostic approach can be highly specific for fetal TAPVR, however, algorithms that are sufficiently sensitive for screening in the general population are still needed. […] Prenatal diagnosis of TAPVR is particularly difficult when it is isolated and in the absence of other cardiac anomalies. […] Accurate identification of fetuses affected by TAPVR is essential for improving postnatal outcomes. […] Prenatal diagnosis of TAPVR is feasible during routine obstetrical ultrasound. […] Future studies are still needed to improve prenatal screening of TAPVR in the general population.

• #87

  https://journals.lww.com/jmut/fulltext/2024/32020/prenatal_ultrasound_markers_of_isolated_total.2.aspx

  This article comprehensively reviews the literature concerning prenatal ultrasound findings of isolated total anomalous pulmonary venous return (TAPVR) and the application of specific sonographic markers to differentiate among the TAPVR types. These markers can be categorized as direct and indirect, based on either morphological features or hemodynamic changes in TAPVR. Diagnosing isolated TAPVR can pose challenges, but following a sequential approach can improve detection rates and outcomes. Early neonatal or prenatal diagnosis, followed by timely postnatal intervention, is crucial for improving outcomes. However, despite recent advances in ultrasound evaluation of the fetal venous system, the prenatal diagnosis of isolated TAPVR remains challenging. This article aims to review the current understanding of sonographic features of fetal TAPVR and provides a sequential approach starting from obstetric screening views to identify possible cases and categorize the TAPVR types step by step.

• #88

  https://journals.lww.com/jmut/fulltext/2024/32020/prenatal_ultrasound_markers_of_isolated_total.2.aspx

  In fetal cardiac screening, the left and right pulmonary veins entering the LA are commonly observed in the four-chamber view. Their presence effectively rules out the possibility of TAPVR. However, when the connection between the pulmonary veins and the LA is not clear, the sonographer should raise suspicions of TAPVR and conduct a systematic search for other possible pulmonary venous drainage routes. […] In the absence of a single sonographic sign for the diagnosis of fetal TAPVR, a systematic and sequential approach is crucial to improve the prenatal detection and diagnosis of TAPVR. The key to enhancing prenatal diagnosis is adherence to the fetal cardiac screening guidelines and evaluation of pulmonary venous entry into the LA in the four-chamber view. Other sonographic signs including direct and indirect markers, when used in conjunction with color and spectral Doppler, serve as adjuncts to precise prenatal diagnosis. Further follow-up at later gestation can be suggested in cases with equivocal findings.

• #89 :: 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. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those of respiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. […] This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. […] Therefore, misdiagnosis and a delayed operation can increase mortality and morbidity in infants with TAPVR. […] 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.

• #90 :: 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. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those of respiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. […] This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. […] Therefore, misdiagnosis and a delayed operation can increase mortality and morbidity in infants with TAPVR. […] 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.

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

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

  Earlier detection and correction of TAPVR improves patient outcome. However, it is difficult to make a differential diagnosis between TAPVR and other neonatal respiratory diseases, such as respiratory distress syndrome, in newborns because initial symptoms or signs may be non-specific and cardiac murmur can be inaudible in TAPVR. Therefore, a high degree of suspicion is essential for an early diagnosis of TAPVR. […] Routine echocardiographic screening of siblings or first relatives is recommended in those with an LV outflow obstruction or a bicuspid aortic valve (BAV). […] Screening of all siblings with TAPVR is a burden to patients due to psychological stress or financial problems on the parents; thus, not all siblings will present for screening. However, these consequences are outweighed by the benefit of diagnosing this critical disease so the patient can be taken for surgery before severe congestive heart failure develops. Therefore, if a genetic background is detected in a case of TAPVR, routine fetal echocardiography or cardiography immediately after birth is reasonable to screen for TAPVR.

• #92 A NEW DIAGNOSIS OF TOTAL ANOMALOUS PULMONARY VENOUS RETURN IN A 56 YEAR OLD MAN – SHM Abstracts | Society of Hospital Medicine

  https://shmabstracts.org/abstract/a-new-diagnosis-of-total-anomalous-pulmonary-venous-return-in-a-56-year-old-man/

  A 56 year old male with no prior medical or surgical history presented to the hospital with fatigue, palpitations, shortness of breath, and bilateral ankle swelling for 2 months. […] A transthoracic echocardiogram revealed anomalous pulmonary venous return and a large secundum atrial septal defect (ASD) later confirmed by cardiac catheterization. […] He was diagnosed with Type 1 total anomalous pulmonary venous return (TAPVR) associated with a large secundum type ASD which had led to a dilated right atrium, right ventricle, and right pulmonary artery. […] Total anomalous pulmonary venous return (TAPVR) is a rare cyanotic congenital heart defect seen in neonates which carries up to an 80% mortality rate at 1 year of age if untreated. […] The case presented was a rare instance of TAPVR diagnosed in an adult patient.

• #93 A NEW DIAGNOSIS OF TOTAL ANOMALOUS PULMONARY VENOUS RETURN IN A 56 YEAR OLD MAN – SHM Abstracts | Society of Hospital Medicine

  https://shmabstracts.org/abstract/a-new-diagnosis-of-total-anomalous-pulmonary-venous-return-in-a-56-year-old-man/

  A 56 year old male with no prior medical or surgical history presented to the hospital with fatigue, palpitations, shortness of breath, and bilateral ankle swelling for 2 months. […] A transthoracic echocardiogram revealed anomalous pulmonary venous return and a large secundum atrial septal defect (ASD) later confirmed by cardiac catheterization. […] He was diagnosed with Type 1 total anomalous pulmonary venous return (TAPVR) associated with a large secundum type ASD which had led to a dilated right atrium, right ventricle, and right pulmonary artery. […] Total anomalous pulmonary venous return (TAPVR) is a rare cyanotic congenital heart defect seen in neonates which carries up to an 80% mortality rate at 1 year of age if untreated. […] The case presented was a rare instance of TAPVR diagnosed in an adult patient.

• #94 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  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%. […] Your child will need regular visits with their cardiologist as they grow into adulthood. Lifelong follow-up visits can help cardiologists find problems like an irregular heartbeat or blockages (obstructions) in their blood vessels. An obstruction requires another surgery and may be hard to treat. […] After surgery, your baby will have checkups every six to 12 months. Theyll need regular follow-up appointments through adulthood. Your childs provider may want to order tests like an electrocardiogram, exercise stress test or echocardiogram.

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

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

  Newborns with severe TAPVR will need emergency surgery shortly after birth. They often need to be admitted to the cardiac intensive care unit (CICU) and require intensive support with medications and a ventilator (breathing machine). […] Babies with less severe TAPVR usually have surgery in the days or weeks after they’re diagnosed. The goal of surgery for TAPVR is to restore normal connections of the pulmonary veins to the heart, alleviate any obstructions or narrowing of the pulmonary veins, to tie up any vessels that have developed and to close the atrial septal defect (ASD). […] Thanks to updates in surgical techniques for repairing TAPVR, the long-term outlook is continually improving. Children who have had a repair for TAPVR will require lifelong checkups with a cardiologist to make sure their veins remain open. If the veins become narrowed, they may need catheterizations or surgeries to repair these veins.

• #96 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  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%. […] Your child will need regular visits with their cardiologist as they grow into adulthood. Lifelong follow-up visits can help cardiologists find problems like an irregular heartbeat or blockages (obstructions) in their blood vessels. An obstruction requires another surgery and may be hard to treat. […] After surgery, your baby will have checkups every six to 12 months. Theyll need regular follow-up appointments through adulthood. Your childs provider may want to order tests like an electrocardiogram, exercise stress test or echocardiogram.

• #97 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  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%. […] Your child will need regular visits with their cardiologist as they grow into adulthood. Lifelong follow-up visits can help cardiologists find problems like an irregular heartbeat or blockages (obstructions) in their blood vessels. An obstruction requires another surgery and may be hard to treat. […] After surgery, your baby will have checkups every six to 12 months. Theyll need regular follow-up appointments through adulthood. Your childs provider may want to order tests like an electrocardiogram, exercise stress test or echocardiogram.

• #98 :: 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. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those of respiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. […] This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. […] Therefore, misdiagnosis and a delayed operation can increase mortality and morbidity in infants with TAPVR. […] 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.

• #99 Total Anomalous Pulmonary Venous Return (TAPVR)

  https://www.isuog.org/clinical-resources/patient-information-series/patient-information-pregnancy-conditions/heart/total-anomalous-pulmonary-venous-return-tapvr.html

  After birth, your baby will be watched carefully for any signs of severe disease. An echocardiogram will be performed to verify the diagnosis and establish the type of TAPVR and rule out other structural changes in the heart such as the presence and degree of obstruction in the venous system. […] Surgical intervention is required to correct this condition, the timing of which will be decided by the pediatric cardiologist and cardiovascular surgeon, after newborn examination and investigations are completed. TAPVR is a serious heart anomaly. Without corrective surgery, many babies will die in the first year of life. […] Recurrence of TAPVR in siblings and other family members has been reported. Because of this recurrence risk, future pregnancies should be evaluated by careful ultrasound examination of the heart with fetal echocardiography.

• #100 Total Anomalous Pulmonary Venous Return (TAPVR)

  https://www.isuog.org/clinical-resources/patient-information-series/patient-information-pregnancy-conditions/heart/total-anomalous-pulmonary-venous-return-tapvr.html

  After birth, your baby will be watched carefully for any signs of severe disease. An echocardiogram will be performed to verify the diagnosis and establish the type of TAPVR and rule out other structural changes in the heart such as the presence and degree of obstruction in the venous system. […] Surgical intervention is required to correct this condition, the timing of which will be decided by the pediatric cardiologist and cardiovascular surgeon, after newborn examination and investigations are completed. TAPVR is a serious heart anomaly. Without corrective surgery, many babies will die in the first year of life. […] Recurrence of TAPVR in siblings and other family members has been reported. Because of this recurrence risk, future pregnancies should be evaluated by careful ultrasound examination of the heart with fetal echocardiography.

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

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

  Earlier detection and correction of TAPVR improves patient outcome. However, it is difficult to make a differential diagnosis between TAPVR and other neonatal respiratory diseases, such as respiratory distress syndrome, in newborns because initial symptoms or signs may be non-specific and cardiac murmur can be inaudible in TAPVR. Therefore, a high degree of suspicion is essential for an early diagnosis of TAPVR. […] Routine echocardiographic screening of siblings or first relatives is recommended in those with an LV outflow obstruction or a bicuspid aortic valve (BAV). […] Screening of all siblings with TAPVR is a burden to patients due to psychological stress or financial problems on the parents; thus, not all siblings will present for screening. However, these consequences are outweighed by the benefit of diagnosing this critical disease so the patient can be taken for surgery before severe congestive heart failure develops. Therefore, if a genetic background is detected in a case of TAPVR, routine fetal echocardiography or cardiography immediately after birth is reasonable to screen for TAPVR.

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

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

  Newborns with severe TAPVR will need emergency surgery shortly after birth. They often need to be admitted to the cardiac intensive care unit (CICU) and require intensive support with medications and a ventilator (breathing machine). […] Babies with less severe TAPVR usually have surgery in the days or weeks after they’re diagnosed. The goal of surgery for TAPVR is to restore normal connections of the pulmonary veins to the heart, alleviate any obstructions or narrowing of the pulmonary veins, to tie up any vessels that have developed and to close the atrial septal defect (ASD). […] Thanks to updates in surgical techniques for repairing TAPVR, the long-term outlook is continually improving. Children who have had a repair for TAPVR will require lifelong checkups with a cardiologist to make sure their veins remain open. If the veins become narrowed, they may need catheterizations or surgeries to repair these veins.

• #103 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

  Patients with a history of TAPVC should be seen periodically by a cardiologist with expertise in adult congenital heart disease to look for uncommon problems. The long-term outlook is excellent, and usually no medicines and no more surgery or catheterization are needed. […] Most adults with repaired TAPVC will not need to limit their physical activities to their own endurance. Some competitive sports may have greater risk if there is leftover obstruction in the pulmonary veins, or if the patient has heart rhythm problems. Your cardiologist will help determine the proper level of activity for you.

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

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

  After surgery, your baby will go to the Cardiothoracic Intensive Care Unit (CTICU). […] Your baby will, likely, have pediatrician appointments every 2 months for well-baby checks and immunizations. […] Your cardiologist will follow up with your babys heart needs even after they are discharged from the hospital. […] Most of the time, congenital heart defects dont happen again with future pregnancies.

• #105 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. […] Diagnosis is by echocardiography. Surgical repair is required. […] Diagnosis of total anomalous pulmonary venous return is suspected by chest x-ray and established by echocardiography. Cardiac catheterization is rarely necessary; occasionally, cardiac MRI or CT angiography may need to be done to better delineate the anatomy of pulmonary venous return.

• #106 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. […] Echocardiography is needed for diagnosis. […] Doctors suspect the diagnosis based on the findings on a chest x-ray, a heart murmur heard with a stethoscope, or low oxygen level in the blood detected by pulse oximetry. The diagnosis is confirmed by echocardiography (ultrasonography of the heart). Occasionally, magnetic resonance imaging (MRI) of the heart or computed tomography (CT) angiography may be needed so doctors can more clearly view the defect.

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

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

  Newborns with severe TAPVR will need emergency surgery shortly after birth. They often need to be admitted to the cardiac intensive care unit (CICU) and require intensive support with medications and a ventilator (breathing machine). […] Babies with less severe TAPVR usually have surgery in the days or weeks after they’re diagnosed. The goal of surgery for TAPVR is to restore normal connections of the pulmonary veins to the heart, alleviate any obstructions or narrowing of the pulmonary veins, to tie up any vessels that have developed and to close the atrial septal defect (ASD). […] Thanks to updates in surgical techniques for repairing TAPVR, the long-term outlook is continually improving. Children who have had a repair for TAPVR will require lifelong checkups with a cardiologist to make sure their veins remain open. If the veins become narrowed, they may need catheterizations or surgeries to repair these veins.

• #108 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

  Patients with a history of TAPVC should be seen periodically by a cardiologist with expertise in adult congenital heart disease to look for uncommon problems. The long-term outlook is excellent, and usually no medicines and no more surgery or catheterization are needed. […] Most adults with repaired TAPVC will not need to limit their physical activities to their own endurance. Some competitive sports may have greater risk if there is leftover obstruction in the pulmonary veins, or if the patient has heart rhythm problems. Your cardiologist will help determine the proper level of activity for you.

• #109

  https://journals.lww.com/jmut/fulltext/2024/32020/prenatal_ultrasound_markers_of_isolated_total.2.aspx

  In fetal cardiac screening, the left and right pulmonary veins entering the LA are commonly observed in the four-chamber view. Their presence effectively rules out the possibility of TAPVR. However, when the connection between the pulmonary veins and the LA is not clear, the sonographer should raise suspicions of TAPVR and conduct a systematic search for other possible pulmonary venous drainage routes. […] In the absence of a single sonographic sign for the diagnosis of fetal TAPVR, a systematic and sequential approach is crucial to improve the prenatal detection and diagnosis of TAPVR. The key to enhancing prenatal diagnosis is adherence to the fetal cardiac screening guidelines and evaluation of pulmonary venous entry into the LA in the four-chamber view. Other sonographic signs including direct and indirect markers, when used in conjunction with color and spectral Doppler, serve as adjuncts to precise prenatal diagnosis. Further follow-up at later gestation can be suggested in cases with equivocal findings.

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