Materiały źródłowe

• #1 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  Possible complications after surgery include: Arrhythmias. Pulmonary hypertension. Pulmonary venous obstruction. Inability of the heart to beat on its own after the use of a bypass machine. […] Without surgery, some forms of total anomalous pulmonary venous return are typically fatal a few weeks after birth. With early diagnosis and surgical treatment, the outlook for babies with TAPVR is very good. The survival rate after surgery is around 97%.

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

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

  Total anomalous pulmonary venous return is a heart problem where veins connect to the wrong place. It needs surgery to fix. […] Nationwide Children’s Hospital offers a team of experts focused on the treatment of total anomalous pulmonary venous return. […] All children born with TAPVR will need surgery to connect the pulmonary veins to the left atrium and close the atrial septal defect. This lets blood flow through the heart and lungs the right way. Depending on how sick your baby is after birth, surgery may be done a few days after they are born. However, they may have to wait a few months until they are stable enough to handle surgery. […] When your baby gets to the CTICU at Nationwide Childrens Hospital, they will have many tests. These tests include an echocardiogram and an EKG. These tests will help the team make a plan for your baby at the Heart Center Case Management Conference. This plan includes choices about surgeries, procedures, and timing.

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

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

  Total anomalous pulmonary venous return is a defect that needs surgery to fix. The timing of the surgical repair varies depending on the type of TAPVR present. The condition of the child is considered as well […] Surgery is done right away for newborns with obstructed TAPVR. Some of these children will need extracorporeal life support (ECMO) prior to surgery because of their blood flow instability. […] Children with TAPVR without obstruction have surgery days to weeks after the diagnosis is made. […] The surgical repair connects all of the veins to the back of the left atrium. This leads to a normal connection of pulmonary veins to left atrium. All other routes for pulmonary venous drainage are tied off. […] Finally, the atrial septal defect (ASD) is also closed. […] The outcome of surgical repair for total anomalous pulmonary venous return is excellent. The surgical mortality is less than 5% when repair is performed electively. This is in healthy children without obstructed pulmonary veins.

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

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

  Total anomalous pulmonary venous return (TAPVR) is a congenital (present at birth) heart defect. […] TAPVR is a serious and complicated condition that requires the attention of a medical team skilled in its diagnosis and treatment. […] The Herma Heart Institute’s pediatric cardiologists and heart surgeons offer babies and families experienced, nationally recognized care and TAPVR treatment. […] Specific TAPVR treatment will be determined by your child’s physician based on: Your child’s age, overall health, and medical history; Extent of the disease; Your child’s tolerance for specific medications, procedures, or therapies; Expectations for the course of the disease; Your opinion or preference. […] Your child

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

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

  Total anomalous pulmonary venous return requires open heart surgery in all cases. Critically ill newborns will have surgery immediately. If the child is not critically ill, doctors may wait up to two months to perform surgery, depending on the strength of the child and the heart anatomy. […] To understand the surgery, one important thing to know about TAPVR is that the pulmonary veins, despite their abnormal connections to other veins, all end in a collection (called a confluence) at the back of the left atrium. The surgeon opens the confluence so that the veins can drain into the left atrium. Then he or she ties off all the abnormal connections between the pulmonary veins and other veins, so that blood can follow only the path to the left atrium. The surgeon also closes septal defects (the abnormal holes) with tiny patches or stitches and closes the patent ductus arteriosus. As the child ages, the lining of the heart will grow over the stitches.

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

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

  Total anomalous pulmonary venous return is a defect that needs surgery to fix. The timing of the surgical repair varies depending on the type of TAPVR present. The condition of the child is considered as well […] Surgery is done right away for newborns with obstructed TAPVR. Some of these children will need extracorporeal life support (ECMO) prior to surgery because of their blood flow instability. […] Children with TAPVR without obstruction have surgery days to weeks after the diagnosis is made. […] The surgical repair connects all of the veins to the back of the left atrium. This leads to a normal connection of pulmonary veins to left atrium. All other routes for pulmonary venous drainage are tied off. […] Finally, the atrial septal defect (ASD) is also closed. […] The outcome of surgical repair for total anomalous pulmonary venous return is excellent. The surgical mortality is less than 5% when repair is performed electively. This is in healthy children without obstructed pulmonary veins.

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

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

  Babies born with TAPVR will need surgery to restore normal blood flow through the heart. Surgery options include placing the child on a heart-lung machine (cardiopulmonary bypass) and making the connection between the pulmonary veins and the left atrium. […] In the most common type of TAPVR, all four pulmonary veins come to a central spot — called a confluence — that is behind the left atrium. In this case, a full repair is done by connecting the confluence into the back of the left atrium. In other forms, the surgeons find other ways to connect the pulmonary veins back to the left atrium. […] After surgery, it is possible that scar tissue can form around the connection between the pulmonary veins and the left atrium. If the scar tissue growth is severe enough, additional surgery may be needed. Between 15% and 20% of children with TAPVR may need cardiac catheterization or surgery later in life to repair narrowed veins. […] A baby who has an obstructive form of TAPVR (meaning the blood cannot get to the heart from the lungs) may need emergency surgery. This surgery often is performed when the baby is just hours old. If the pulmonary veins are not obstructed, surgery can wait until a few weeks after birth.

• #8 Repair of Total Anomalous Pulmonary Venous Return

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2607107/

  We present herein the case of a 48-year-old patient who had undergone repair of total anomalous pulmonary venous return at the age of 14 months, in March 1960, at Texas Children’s Hospital. […] The surgical repair of total anomalous pulmonary venous return (TAPVR) has evolved since the earliest corrective procedures were performed in the late 1950s. Here, we present the case of a patient who underwent repair of TAPVR as a child in 1960 at Texas Children’s Hospital, and who was re-examined in 2007. We discuss the types of TAPVR and the various treatment approaches therefor. […] The diagnosis of TAPVR is an indication for surgery. If the condition remains untreated, it is usually fatal before 1 year of age. […] Thanks to better diagnostic capabilities and to improvements in surgical techniques, operative death has decreased from 15.8% in 1984 to as low as 0 in a later case series. The repair of TAPVR has become a surgical procedure with very low risks of morbidity and death.

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

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

  Surgery is the only way to fix a total anomalous pulmonary venous return, a rare congenital birth defect of the heart. […] Children with TAPVR need surgery to restore normal blood flow to and through the heart. Your child’s surgeon will discuss which surgery is right for your child. […] Exactly when your child has surgery depends on how serious their condition is: If your child is very sick (critically ill) they will have surgery right away. Children who are not critically ill may wait two to six weeks for surgery, depending on the structure of the abnormal connections, as well as other symptoms. […] After surgery, your child will need regular follow-up care to check for complications and other heart conditions. […] Yes. The only effective treatment for TAPVR is surgery. The good news is that after your child recovers, he or she can expect to live a healthy, active life.

• #10 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). They may be on a breathing machine and have many tubes, wires, and equipment to keep them stable and make sure they are okay. Members of their health care team may make changes to your babys medicines and breathing machine often. This will happen the most in the first 24 to 48 hours after surgery. […] Other treatments your baby will probably get after surgery are: Antibiotic medicine to prevent infection, Fluids and nutrition through an IV or PICC line, Heart medicines by IV or by mouth, Chest drainage tube, Oxygen. […] Your medical team will help you decide on the safest feeding approach. Lactation nurses and occupational and speech therapists can work with your baby on oral feeding skills. They can also help you with pumping or direct chest/breastfeeding, when needed. Your baby may go home eating by mouth, but some babies may need help getting the calories needed to grow.

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

  https://www.stanfordchildrens.org/en/topic/default?id=total-anomalous-pulmonary-venous-return-tapvr-90-P01820

  All children with a TAPVR will need to have surgery to fix it. […] Treatment will depend on your child’s symptoms, age, and overall health. It will also depend on how severe the condition is. […] At first, your baby may get the following care: Supplemental oxygen or a machine that helps your baby breathe (ventilator). Prostaglandin therapy. This medicine keeps the ductus arteriosus open and lets blood flow through the heart. This medicine isn’t used in all types of TAPVR. Different medicine to support the function of the heart. ECMO (extracorporeal membrane oxygenation). This method gives both cardiac and respiratory support for babies who can’t maintain oxygen levels on a ventilator. […] This procedure can be used to diagnose and treat some heart defects. To treat TAPVR, your child’s healthcare provider may do a test called a balloon atrial septostomy. This test makes it easier for oxygen-rich blood to get to the left side of the heart and then the rest of the body.

• #12 Total Anomalous Pulmonary Venous Return (TAPVR) | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions—pediatrics/t/total-anomalous-pulmonary-venous-return.html

  Treatment will depend on your childs symptoms, age, and overall health. It will also depend on how severe the condition is. […] All children with a TAPVR will need to have surgery to fix it. Your baby may need to go into the intensive care unit (ICU) for treatment. […] At first, your baby may get the following care: Supplemental oxygen or a machine that helps your baby breathe (ventilator). Prostaglandin therapy. This medicine keeps the ductus arteriosus open and lets blood flow through the heart. This medicine isnt used in all types of TAPVR. Different medicine to support the function of the heart. ECMO (extracorporeal membrane oxygenation). This method gives both cardiac and respiratory support for babies who can’t maintain oxygen levels on a ventilator. […] This procedure can be used to diagnose and treat some heart defects. To treat TAPVR, your childs healthcare provider may do a test called a balloon atrial septostomy. This test makes it easier for oxygen-rich blood to get to the left side of the heart and then the rest of the body.

• #13 Total Anomalous Pulmonary Venous Return (TAPVR)

  http://www.texaschildrenshealthresources.org/Library/Wellness/Safety/90,P01820

  Treatment will depend on your childs symptoms, age, and overall health. It will also depend on how severe the condition is. […] All children with a TAPVR will need to have surgery to fix it. Your baby may need to go into the intensive care unit (ICU) for treatment. […] At first, your baby may get the following care: Supplemental oxygen or a machine that helps your baby breathe (ventilator). […] Prostaglandin therapy. This medicine keeps the ductus arteriosus open and lets blood flow through the heart. This medicine isnt used in all types of TAPVR. […] Different medicine to support the function of the heart. […] ECMO (extracorporeal membrane oxygenation). This method gives both cardiac and respiratory support for babies who can’t maintain oxygen levels on a ventilator. […] This procedure can be used to diagnose and treat some heart defects. To treat TAPVR, your childs healthcare provider may do a test called a balloon atrial septostomy. This test makes it easier for oxygen-rich blood to get to the left side of the heart and then the rest of the body.

• #14 Total Anomalous Pulmonary Venous Return (TAPVR) – Congenital Heart Disorders for Medicine

  https://www.picmonic.com/pathways/medicine/courses/standard/pathology-196/congenital-heart-disorders-39277/total-anomalous-pulmonary-venous-return-tapvr_2475

  Total anomalous pulmonary venous return (TAPVR) occurs when the left atrium fails to form a connection with the pulmonary venous plexus in the first month of life, resulting in persistence of the primitive drainage pattern which bypasses the left atrium and drains oxygenated blood into the systemic venous system. This causes cyanosis, fluid overload, and right heart strain. […] Regardless of degree of obstruction, surgery is the definitive treatment for all levels of TAPVR. […] Initial treatment of infants with severe TAPVR is aimed at stabilization for surgery. Patients are given oxygen, and mechanically ventilated if necessary. Inotropic medications are used to increase the contractility of the heart, and prostaglandins may be beneficial in keeping the ductus arteriosus open to increase the systemic cardiac output. […] Surgical management of TAPVR is recommended regardless of the presence or absence of obstruction, and should be performed as soon as the patient is stable. The goal of surgery is to create a direct pathway between the pulmonary veins and the left atrium.

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

  https://www.stanfordchildrens.org/en/topic/default?id=total-anomalous-pulmonary-venous-return-tapvr-90-P01820

  All children with a TAPVR will need to have surgery to fix it. […] Treatment will depend on your child’s symptoms, age, and overall health. It will also depend on how severe the condition is. […] At first, your baby may get the following care: Supplemental oxygen or a machine that helps your baby breathe (ventilator). Prostaglandin therapy. This medicine keeps the ductus arteriosus open and lets blood flow through the heart. This medicine isn’t used in all types of TAPVR. Different medicine to support the function of the heart. ECMO (extracorporeal membrane oxygenation). This method gives both cardiac and respiratory support for babies who can’t maintain oxygen levels on a ventilator. […] This procedure can be used to diagnose and treat some heart defects. To treat TAPVR, your child’s healthcare provider may do a test called a balloon atrial septostomy. This test makes it easier for oxygen-rich blood to get to the left side of the heart and then the rest of the body.

• #16 Total Anomalous Pulmonary Venous Return (TAPVR) | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions—pediatrics/t/total-anomalous-pulmonary-venous-return.html

  Treatment will depend on your childs symptoms, age, and overall health. It will also depend on how severe the condition is. […] All children with a TAPVR will need to have surgery to fix it. Your baby may need to go into the intensive care unit (ICU) for treatment. […] At first, your baby may get the following care: Supplemental oxygen or a machine that helps your baby breathe (ventilator). Prostaglandin therapy. This medicine keeps the ductus arteriosus open and lets blood flow through the heart. This medicine isnt used in all types of TAPVR. Different medicine to support the function of the heart. ECMO (extracorporeal membrane oxygenation). This method gives both cardiac and respiratory support for babies who can’t maintain oxygen levels on a ventilator. […] This procedure can be used to diagnose and treat some heart defects. To treat TAPVR, your childs healthcare provider may do a test called a balloon atrial septostomy. This test makes it easier for oxygen-rich blood to get to the left side of the heart and then the rest of the body.

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

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

  Total anomalous pulmonary venous return is a heart problem where veins connect to the wrong place. It needs surgery to fix. […] Nationwide Children’s Hospital offers a team of experts focused on the treatment of total anomalous pulmonary venous return. […] All children born with TAPVR will need surgery to connect the pulmonary veins to the left atrium and close the atrial septal defect. This lets blood flow through the heart and lungs the right way. Depending on how sick your baby is after birth, surgery may be done a few days after they are born. However, they may have to wait a few months until they are stable enough to handle surgery. […] When your baby gets to the CTICU at Nationwide Childrens Hospital, they will have many tests. These tests include an echocardiogram and an EKG. These tests will help the team make a plan for your baby at the Heart Center Case Management Conference. This plan includes choices about surgeries, procedures, and timing.

• #18 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

  Neonates with total anomalous pulmonary venous return with obstruction require emergent surgical repair. […] In older infants, heart failure should be treated, followed by surgical repair as soon as the infant is stabilized. […] Surgical repair consists of creating a wide anastomosis between the pulmonary venous confluence and the posterior wall of the left atrium. […] Ligation of the vein decompressing the confluence into the systemic venous circulation is important to prevent a postoperative left-to-right shunt. […] Endocarditis prophylaxis is recommended preoperatively but is required only for the first 6 months after repair unless there is a residual defect adjacent to a surgical patch or prosthetic material.

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

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

  Total anomalous pulmonary venous return is a defect that needs surgery to fix. The timing of the surgical repair varies depending on the type of TAPVR present. The condition of the child is considered as well […] Surgery is done right away for newborns with obstructed TAPVR. Some of these children will need extracorporeal life support (ECMO) prior to surgery because of their blood flow instability. […] Children with TAPVR without obstruction have surgery days to weeks after the diagnosis is made. […] The surgical repair connects all of the veins to the back of the left atrium. This leads to a normal connection of pulmonary veins to left atrium. All other routes for pulmonary venous drainage are tied off. […] Finally, the atrial septal defect (ASD) is also closed. […] The outcome of surgical repair for total anomalous pulmonary venous return is excellent. The surgical mortality is less than 5% when repair is performed electively. This is in healthy children without obstructed pulmonary veins.

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

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

  Babies with TAPVR will need surgery to repair the defect. The age at which the surgery is done depends on: […] The goal of surgical repair of TAPVR is to restore normal blood flow through the heart. To repair this defect, doctors usually: […] Surgical repairs for TAPVR are not a cure. People living with TAPVR may have lifelong complications. Routine checkups with a heart doctor are needed to monitor progress, avoid complications, and check for other health conditions that might develop over time.

• #21 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. […] 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. […] While waiting for surgery, your child may receive extra oxygen or a ventilator to help them breathe. They may receive an inotrope, which is a medicine that helps their heart beat more forcefully. […] While your baby is asleep under general anesthesia, a surgeon: Makes cuts (incisions) in your babys chest and heart. Connects the pulmonary veins to the correct place (the left atrium) in your babys heart. Closes abnormal connections. Removes any blockage in your childs pulmonary vein. Most often, closes the hole between their left and right atria (atrial septal defect).

• #22 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 requires open heart surgery in all cases. Critically ill newborns will have surgery immediately. If the child is not critically ill, doctors may wait up to two months to perform surgery, depending on the strength of the child and the heart anatomy. […] To understand the surgery, one important thing to know about TAPVR is that the pulmonary veins, despite their abnormal connections to other veins, all end in a collection (called a confluence) at the back of the left atrium. The surgeon opens the confluence so that the veins can drain into the left atrium. Then he or she ties off all the abnormal connections between the pulmonary veins and other veins, so that blood can follow only the path to the left atrium. The surgeon also closes septal defects (the abnormal holes) with tiny patches or stitches and closes the patent ductus arteriosus. As the child ages, the lining of the heart will grow over the stitches.

• #23 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

  Neonates with total anomalous pulmonary venous return with obstruction require emergent surgical repair. […] In older infants, heart failure should be treated, followed by surgical repair as soon as the infant is stabilized. […] Surgical repair consists of creating a wide anastomosis between the pulmonary venous confluence and the posterior wall of the left atrium. […] Ligation of the vein decompressing the confluence into the systemic venous circulation is important to prevent a postoperative left-to-right shunt. […] Endocarditis prophylaxis is recommended preoperatively but is required only for the first 6 months after repair unless there is a residual defect adjacent to a surgical patch or prosthetic material.

• #24 Total Anomalous Pulmonary Venous Return (TAPVR) | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions—pediatrics/t/total-anomalous-pulmonary-venous-return.html

  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. The details of the surgery depend on the type of TAPVR your baby has. Your childs healthcare provider will explain the procedure to you. Some babies with severe forms of TAPVR may need surgery shortly after birth.

• #25 Mayo Clinic Health Library – Total anomalous pulmonary venous return (TAPVR) | Swiss Medical Network

  https://www.swissmedical.net/fr/healtcare-library/con-20359713

  Total anomalous pulmonary venous return (TAPVR) is treated with surgery. The surgery usually is done when a child is a baby. The timing of surgery depends on whether there’s a blockage. To repair the heart, surgeons connect the pulmonary veins to the left upper heart chamber. They also close the hole between the upper heart chambers. […] A person with TAPVR needs regular health checkups for life to check for infection, blockages or irregular heartbeats. A doctor trained in congenital heart diseases should provide care. This type of healthcare professional is called a congenital cardiologist.

• #26 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

  Specific treatment for TAPVR will be determined by your child’s doctor based on: […] Your child may be admitted to the intensive care unit (ICU) or special care nursery once symptoms are noted. Initially, your child may be placed on oxygen and possibly even on a ventilator to assist his or her breathing. IV medications may be given to help the heart and lungs function more efficiently. […] Initial medical management may include oxygen, medications to increase blood pressure and treatments with prostaglandins, a medication used to keep the ductus arteriosus open (although this medication may be contraindicated in certain forms of the disease). […] A cardiac catheterization procedure can be used as a diagnostic procedure, as well as initial treatment procedure for some heart defects. […] The operation is performed under general anesthesia. The four pulmonary veins are reconnected to the left atrium and any associated heart defects such as atrial septal defect, ventricular septal defect, patent foramen ovale and/or patent ductus arteriosus are surgically closed.

• #27 Total Anomalous Pulmonary Venous Return Repair

  https://www.rwjbh.org/treatment-care/heart-and-vascular-care/tests-procedures/total-anomalous-pulmonary-venous-return-repair/

  Total Anomalous Pulmonary Venous Return (TAVPR) is a type of congenital heart defect (present at birth) that affects the pulmonary veins. […] Regardless of form, your doctor will typically advise for your baby to get surgery immediately. Surgery is performed via open-heart surgery (involves dividing the breast bone / general anesthesia). […] In some cases, the surgeon will create a connection between the vessel coming from the lung and the left atrium. The surgeon may close off the vessel where the vessel from the lung was previously joined. […] In other cases, the surgeon will rebuild the wall between the left and right atrium. That way, the blood from the lungs flows directly into the left atrium. […] The surgeon will make other repairs to the heart as needed. […] After the procedure is completed, the doctor will closely check to make sure everything is working properly. Once checked, the doctor will let the blood circulating through the bypass machine back into your baby’s heart. […] Once the procedure is complete, the machine will be turned off. The tubes will be removed and the sternum will be sewn together with the use or sutures or surgical staples. […] Total Anomalous Pulmonary Venous Return Repair.

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

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

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

• #29 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. […] In cases without obstruction there is a large left-to-right shunt and patients present with heart failure months after birth up to early childhood. Patients with non obstructed TAPVR are repaired electively when diagnosed, usually when symptoms develop around 6 months of age. […] When there is pulmonary venous obstruction, infants present in extremis within hours to days of birth; they are profoundly cyanotic and in severe congestive heart failure. Obstructed TAPVR is a surgical emergency. Patients require intubation with 100% oxygen, hyperventilation, and correction of acidosis. ECMO has been used as a temporizing measure.

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

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

  Total anomalous pulmonary venous return (TAPVR), also known as total anomalous pulmonary venous connection (TAPVC), is a rare heart defect in which the blood vessels that drain the lungs (pulmonary veins) are not connected normally to the heart. Instead, the pulmonary veins are redirected abnormally to other chambers of the heart. About 1 in every 20,000 babies is born with TAPVR. […] TAPVR can occur with obstruction, meaning that some of the draining blood vessels are obstructed. This can cause high blood pressure in the lungs (pulmonary hypertension) and can be a surgical emergency. […] Children with TAPVR will need surgery in infancy to repair the problem. […] 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).

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

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

  After your child has open-heart surgery, theyll spend one or two days in the pediatric intensive care unit (ICU). Theyll get medications and fluids through an IV (in their vein) and stay in the hospital for two to four weeks as they recover. Full recovery can take at least six to 12 weeks for open-heart surgery. […] Follow-up care for TAPVR means lifelong checkups. These regular follow up visits help your childs care team keep an eye out for other conditions and complications like narrowing of their pulmonary veins (pulmonary artery stenosis), irregular heartbeats (arrhythmia), abnormal heart function or infections. […] When your baby is born with a serious heart condition like total anomalous pulmonary venous return, getting a diagnosis and treatment right away can be lifesaving. Our pediatric cardiology specialists are here to diagnose this condition and repair your childs heart right away.

• #32 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

  After surgery, infants will return to the intensive care unit (ICU) for a few days to be closely monitored during recovery. […] Pain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable at home. Your child’s doctor will discuss pain control before your child is discharged from the hospital. […] Many infants who have had TAPVR surgical repair will grow and develop normally. However, after TAPVR repair, your infant will need to be followed periodically by a pediatric cardiologist who will make assessments to check for any heart-related problems. […] Regular follow-up care at a center offering pediatric or adult congenital cardiac care should continue throughout the individuals lifespan.

• #33 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). They may be on a breathing machine and have many tubes, wires, and equipment to keep them stable and make sure they are okay. Members of their health care team may make changes to your babys medicines and breathing machine often. This will happen the most in the first 24 to 48 hours after surgery. […] Other treatments your baby will probably get after surgery are: Antibiotic medicine to prevent infection, Fluids and nutrition through an IV or PICC line, Heart medicines by IV or by mouth, Chest drainage tube, Oxygen. […] Your medical team will help you decide on the safest feeding approach. Lactation nurses and occupational and speech therapists can work with your baby on oral feeding skills. They can also help you with pumping or direct chest/breastfeeding, when needed. Your baby may go home eating by mouth, but some babies may need help getting the calories needed to grow.

• #34

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

  In the early post operative period, pulmonary hypertension due to a small and poorly compliant left heart leading to cardiac failure and pulmonary edema is common and may require prolonged respiratory and medication support postoperatively. […] Following the repair of TAPVD, medical therapy is directed towards augmenting cardiac output and minimizing pulmonary vascular resistance (PVR) using adrenergic agonists and pulmonary vasodilators such as dobutamine, isoproterenol, and milrinone. […] The following measures are to be followed for prevention and management of pulmonary hypertension.

• #35

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

  In the early post operative period, pulmonary hypertension due to a small and poorly compliant left heart leading to cardiac failure and pulmonary edema is common and may require prolonged respiratory and medication support postoperatively. […] Following the repair of TAPVD, medical therapy is directed towards augmenting cardiac output and minimizing pulmonary vascular resistance (PVR) using adrenergic agonists and pulmonary vasodilators such as dobutamine, isoproterenol, and milrinone. […] The following measures are to be followed for prevention and management of pulmonary hypertension.

• #36 Outcomes of total anomalous pulmonary venous drainage repair in neonates and the impact of pulmonary hypertension on survival – JTCVS Open

  https://www.jtcvsopen.org/article/S2666-2736(22)00360-6/fulltext

  Mortality after repair of total anomalous pulmonary venous drainage (TAPVD) in neonates has remained high. […] The aim of this study was to determine the outcomes of simple TAPVD repair in neonates over a time span of almost 50 years and to identify risk factors for mortality. In particular, we investigated whether pulmonary hypertension (PHT) could affect survival of these patients. […] Mortality after TAPVD repair occurred mainly within the first year of life. Urgency of surgery and persistent PHT appears to be risk factors for mortality. Lung biopsy might be useful for identifying patients at risk and guiding newer treatment modalities. […] Identification of patients with pulmonary artery disease could help guide treatment strategies and potentially improve outcomes. […] The fact that operative and perioperative refinements have not significantly improved neonatal survival may indicate that intrinsic patient characteristics influence outcomes.

• #37 Total Anomalous Pulmonary Venous Return (TAPVR)

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

  Possible complications after surgery include: Arrhythmias. Pulmonary hypertension. Pulmonary venous obstruction. Inability of the heart to beat on its own after the use of a bypass machine. […] 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%.

• #38

  https://www.pted.org/?id=tapvr4

  As repair of TAPVR requires extensive atrial surgery, arrhythmias may develop with time (e.g. supraventricular tachycardia), requiring treatment. […] Significant pulmonary vein stenosis may require repeat operations to lower pulmonary artery pressures. […] Persistent anomalous pulmonary veins may not require repair if the resultant left to right shunt is small.

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

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

  The long-term outcome after surgical repair of TAPV is also excellent. Because the surgical repair results in a normal circulation, these children are expected to grow and develop normally. […] Rare complications of TAPVR can occur late after surgery. Regular follow up by a qualified cardiologist is needed to detect these problems early if they occur. […] When these impulses occur in single beats, they are typically benign. They do not need any treatment. On rare occasion, patients can have either continuous episodes of rapid heartbeat, or slow heart rates. Either sustained fast heart rates or slow heart rates may need treatment. The results of treatment (either medical or with a pacemaker) are excellent.

• #40 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.

• #41 Outcomes of total anomalous pulmonary venous drainage repair in neonates and the impact of pulmonary hypertension on survival – JTCVS Open

  https://www.jtcvsopen.org/article/S2666-2736(22)00360-6/fulltext

  Therefore, an analysis of risk factors could help identify potential treatment targets to improve survival in these patients. […] Patients with PHT crisis had the lowest survival, at 57.1% after 1 year. […] Lung biopsy could help identify patients with early pulmonary artery disease and trigger timely medical treatment. Further investigations are needed to understand how many patients are affected by early changes and whether preemptive treatment might be warranted.

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

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

  Some babies with severe TAPVR may need a specialized life support system called ECMO (extracorporeal membrane oxygenation), an advanced technology that functions as a replacement for a critically ill child’s heart and lungs. […] 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.

• #43 Repair of Total Anomalous Pulmonary Venous Return

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2607107/

  Regarding the long-term postoperative outlook, it is known that repair of supradiaphragmatic TAPVR in infants, children, and even adolescents is followed by a clinical course similar to that after the closure of a nonrestrictive ostium secundum atrial septal defect. […] Despite favorable outcomes overall, there has been no systematic, comprehensive, long-term evaluation of patients who underwent repair of TAPVR in early childhood.

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

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

  Total anomalous pulmonary venous return is a defect that needs surgery to fix. The timing of the surgical repair varies depending on the type of TAPVR present. The condition of the child is considered as well […] Surgery is done right away for newborns with obstructed TAPVR. Some of these children will need extracorporeal life support (ECMO) prior to surgery because of their blood flow instability. […] Children with TAPVR without obstruction have surgery days to weeks after the diagnosis is made. […] The surgical repair connects all of the veins to the back of the left atrium. This leads to a normal connection of pulmonary veins to left atrium. All other routes for pulmonary venous drainage are tied off. […] Finally, the atrial septal defect (ASD) is also closed. […] The outcome of surgical repair for total anomalous pulmonary venous return is excellent. The surgical mortality is less than 5% when repair is performed electively. This is in healthy children without obstructed pulmonary veins.

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

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

  The long-term outcome after surgical repair of TAPV is also excellent. Because the surgical repair results in a normal circulation, these children are expected to grow and develop normally. […] Rare complications of TAPVR can occur late after surgery. Regular follow up by a qualified cardiologist is needed to detect these problems early if they occur. […] When these impulses occur in single beats, they are typically benign. They do not need any treatment. On rare occasion, patients can have either continuous episodes of rapid heartbeat, or slow heart rates. Either sustained fast heart rates or slow heart rates may need treatment. The results of treatment (either medical or with a pacemaker) are excellent.

• #46 Total anomalous pulmonary venous return – UF Health

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

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

• #47 Repair of Total Anomalous Pulmonary Venous Return

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2607107/

  Regarding the long-term postoperative outlook, it is known that repair of supradiaphragmatic TAPVR in infants, children, and even adolescents is followed by a clinical course similar to that after the closure of a nonrestrictive ostium secundum atrial septal defect. […] Despite favorable outcomes overall, there has been no systematic, comprehensive, long-term evaluation of patients who underwent repair of TAPVR in early childhood.

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

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

  Babies with TAPVR will need surgery to repair the defect. The age at which the surgery is done depends on: […] The goal of surgical repair of TAPVR is to restore normal blood flow through the heart. To repair this defect, doctors usually: […] Surgical repairs for TAPVR are not a cure. People living with TAPVR may have lifelong complications. Routine checkups with a heart doctor are needed to monitor progress, avoid complications, and check for other health conditions that might develop over time.

• #49 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

  After surgery, infants will return to the intensive care unit (ICU) for a few days to be closely monitored during recovery. […] Pain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable at home. Your child’s doctor will discuss pain control before your child is discharged from the hospital. […] Many infants who have had TAPVR surgical repair will grow and develop normally. However, after TAPVR repair, your infant will need to be followed periodically by a pediatric cardiologist who will make assessments to check for any heart-related problems. […] Regular follow-up care at a center offering pediatric or adult congenital cardiac care should continue throughout the individuals lifespan.

• #50 Mayo Clinic Health Library – Total anomalous pulmonary venous return (TAPVR) | Swiss Medical Network

  https://www.swissmedical.net/fr/healtcare-library/con-20359713

  Total anomalous pulmonary venous return (TAPVR) is treated with surgery. The surgery usually is done when a child is a baby. The timing of surgery depends on whether there’s a blockage. To repair the heart, surgeons connect the pulmonary veins to the left upper heart chamber. They also close the hole between the upper heart chambers. […] A person with TAPVR needs regular health checkups for life to check for infection, blockages or irregular heartbeats. A doctor trained in congenital heart diseases should provide care. This type of healthcare professional is called a congenital cardiologist.

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

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

  Some babies with severe TAPVR may need a specialized life support system called ECMO (extracorporeal membrane oxygenation), an advanced technology that functions as a replacement for a critically ill child’s heart and lungs. […] 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.

• #52 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

  Neonates with total anomalous pulmonary venous return with obstruction require emergent surgical repair. […] In older infants, heart failure should be treated, followed by surgical repair as soon as the infant is stabilized. […] Surgical repair consists of creating a wide anastomosis between the pulmonary venous confluence and the posterior wall of the left atrium. […] Ligation of the vein decompressing the confluence into the systemic venous circulation is important to prevent a postoperative left-to-right shunt. […] Endocarditis prophylaxis is recommended preoperatively but is required only for the first 6 months after repair unless there is a residual defect adjacent to a surgical patch or prosthetic material.

• #53 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

  This defect must be surgically repaired in early infancy. At the time of open-heart surgery, the pulmonary veins are reconnected to the left atrium and the atrial septal defect is closed. […] This defect is almost always surgically repaired in early infancy or childhood. At the time of open-heart surgery, the pulmonary veins are reconnected to the left atrium and the atrial septal defect is closed. […] Once the TAPVC has been repaired, it’s unlikely that more surgery will be needed. Rarely, an adult patient may have obstruction that may need to be corrected depending on the severity.

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

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

  After your child has open-heart surgery, theyll spend one or two days in the pediatric intensive care unit (ICU). Theyll get medications and fluids through an IV (in their vein) and stay in the hospital for two to four weeks as they recover. Full recovery can take at least six to 12 weeks for open-heart surgery. […] Follow-up care for TAPVR means lifelong checkups. These regular follow up visits help your childs care team keep an eye out for other conditions and complications like narrowing of their pulmonary veins (pulmonary artery stenosis), irregular heartbeats (arrhythmia), abnormal heart function or infections. […] When your baby is born with a serious heart condition like total anomalous pulmonary venous return, getting a diagnosis and treatment right away can be lifesaving. Our pediatric cardiology specialists are here to diagnose this condition and repair your childs heart right away.

• #55 Expert Care: TAPVR Surgery | UVA Health Children’s

  https://childrens.uvahealth.com/services/heart/chd/tapvr-treatment

  Your child will need TAPVR treatment. […] Your baby will need open-heart surgery. […] During a single surgical procedure, we will restore normal blood flow through your child’s heart. We’ll: Connect the pulmonary veins to the left side of the heart, Close off any abnormal connections between blood vessels, Close the hole (atrial septal defect) in the heart. […] Your child will recover in a private room in a special area of our Pediatric Intensive Care Unit (PICU). […] Once we repair your child’s heart defect, you can look forward to your child having a normal active childhood, even playing sports. […] When they become an adult, they can easily transition to our Adult Congenital Heart Disease Clinic.

• #56 Outcomes of total anomalous pulmonary venous drainage repair in neonates and the impact of pulmonary hypertension on survival – JTCVS Open

  https://www.jtcvsopen.org/article/S2666-2736(22)00360-6/fulltext

  Mortality after repair of total anomalous pulmonary venous drainage (TAPVD) in neonates has remained high. […] The aim of this study was to determine the outcomes of simple TAPVD repair in neonates over a time span of almost 50 years and to identify risk factors for mortality. In particular, we investigated whether pulmonary hypertension (PHT) could affect survival of these patients. […] Mortality after TAPVD repair occurred mainly within the first year of life. Urgency of surgery and persistent PHT appears to be risk factors for mortality. Lung biopsy might be useful for identifying patients at risk and guiding newer treatment modalities. […] Identification of patients with pulmonary artery disease could help guide treatment strategies and potentially improve outcomes. […] The fact that operative and perioperative refinements have not significantly improved neonatal survival may indicate that intrinsic patient characteristics influence outcomes.

• #57 Outcomes of total anomalous pulmonary venous drainage repair in neonates and the impact of pulmonary hypertension on survival – JTCVS Open

  https://www.jtcvsopen.org/article/S2666-2736(22)00360-6/fulltext

  Mortality after repair of total anomalous pulmonary venous drainage (TAPVD) in neonates has remained high. […] The aim of this study was to determine the outcomes of simple TAPVD repair in neonates over a time span of almost 50 years and to identify risk factors for mortality. In particular, we investigated whether pulmonary hypertension (PHT) could affect survival of these patients. […] Mortality after TAPVD repair occurred mainly within the first year of life. Urgency of surgery and persistent PHT appears to be risk factors for mortality. Lung biopsy might be useful for identifying patients at risk and guiding newer treatment modalities. […] Identification of patients with pulmonary artery disease could help guide treatment strategies and potentially improve outcomes. […] The fact that operative and perioperative refinements have not significantly improved neonatal survival may indicate that intrinsic patient characteristics influence outcomes.

• #58 Outcomes of total anomalous pulmonary venous drainage repair in neonates and the impact of pulmonary hypertension on survival – JTCVS Open

  https://www.jtcvsopen.org/article/S2666-2736(22)00360-6/fulltext

  Therefore, an analysis of risk factors could help identify potential treatment targets to improve survival in these patients. […] Patients with PHT crisis had the lowest survival, at 57.1% after 1 year. […] Lung biopsy could help identify patients with early pulmonary artery disease and trigger timely medical treatment. Further investigations are needed to understand how many patients are affected by early changes and whether preemptive treatment might be warranted.

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

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

  This condition can only be repaired by surgery. […] In the operation, the surgeon opens the back of the left atrium and connects the common vein to the left atrium. This creates proper flow of oxygen-rich blood from the lungs to the heart. The surgeon also closes the abnormal connection that went to the right side of the heart, as well as the atrial septal defect. […] The timing of the surgery depends on how severe your child’s condition is. Some children need surgery soon after birth because they have severe symptoms. Sometimes doctors can stabilize babies who have a blocked common vein by using cardiac catheterization to place a stent in this vein. […] If your child’s symptoms are not severe, the doctor may suggest waiting so your child can grow. This makes it easier to do the surgery. Most children have surgery by the age of 6 months.

• #60 Outcomes of total anomalous pulmonary venous drainage repair in neonates and the impact of pulmonary hypertension on survival – JTCVS Open

  https://www.jtcvsopen.org/article/S2666-2736(22)00360-6/fulltext

  Therefore, an analysis of risk factors could help identify potential treatment targets to improve survival in these patients. […] Patients with PHT crisis had the lowest survival, at 57.1% after 1 year. […] Lung biopsy could help identify patients with early pulmonary artery disease and trigger timely medical treatment. Further investigations are needed to understand how many patients are affected by early changes and whether preemptive treatment might be warranted.

• #61 Repair of Total Anomalous Pulmonary Venous Return

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2607107/

  We present herein the case of a 48-year-old patient who had undergone repair of total anomalous pulmonary venous return at the age of 14 months, in March 1960, at Texas Children’s Hospital. […] The surgical repair of total anomalous pulmonary venous return (TAPVR) has evolved since the earliest corrective procedures were performed in the late 1950s. Here, we present the case of a patient who underwent repair of TAPVR as a child in 1960 at Texas Children’s Hospital, and who was re-examined in 2007. We discuss the types of TAPVR and the various treatment approaches therefor. […] The diagnosis of TAPVR is an indication for surgery. If the condition remains untreated, it is usually fatal before 1 year of age. […] Thanks to better diagnostic capabilities and to improvements in surgical techniques, operative death has decreased from 15.8% in 1984 to as low as 0 in a later case series. The repair of TAPVR has become a surgical procedure with very low risks of morbidity and death.

• #62 Repair of Total Anomalous Pulmonary Venous Return

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2607107/

  We present herein the case of a 48-year-old patient who had undergone repair of total anomalous pulmonary venous return at the age of 14 months, in March 1960, at Texas Children’s Hospital. […] The surgical repair of total anomalous pulmonary venous return (TAPVR) has evolved since the earliest corrective procedures were performed in the late 1950s. Here, we present the case of a patient who underwent repair of TAPVR as a child in 1960 at Texas Children’s Hospital, and who was re-examined in 2007. We discuss the types of TAPVR and the various treatment approaches therefor. […] The diagnosis of TAPVR is an indication for surgery. If the condition remains untreated, it is usually fatal before 1 year of age. […] Thanks to better diagnostic capabilities and to improvements in surgical techniques, operative death has decreased from 15.8% in 1984 to as low as 0 in a later case series. The repair of TAPVR has become a surgical procedure with very low risks of morbidity and death.

• #63 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. […] 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. […] While waiting for surgery, your child may receive extra oxygen or a ventilator to help them breathe. They may receive an inotrope, which is a medicine that helps their heart beat more forcefully. […] While your baby is asleep under general anesthesia, a surgeon: Makes cuts (incisions) in your babys chest and heart. Connects the pulmonary veins to the correct place (the left atrium) in your babys heart. Closes abnormal connections. Removes any blockage in your childs pulmonary vein. Most often, closes the hole between their left and right atria (atrial septal defect).

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

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

  Total anomalous pulmonary venous return is a defect that needs surgery to fix. The timing of the surgical repair varies depending on the type of TAPVR present. The condition of the child is considered as well […] Surgery is done right away for newborns with obstructed TAPVR. Some of these children will need extracorporeal life support (ECMO) prior to surgery because of their blood flow instability. […] Children with TAPVR without obstruction have surgery days to weeks after the diagnosis is made. […] The surgical repair connects all of the veins to the back of the left atrium. This leads to a normal connection of pulmonary veins to left atrium. All other routes for pulmonary venous drainage are tied off. […] Finally, the atrial septal defect (ASD) is also closed. […] The outcome of surgical repair for total anomalous pulmonary venous return is excellent. The surgical mortality is less than 5% when repair is performed electively. This is in healthy children without obstructed pulmonary veins.

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

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

  Babies with TAPVR will need surgery to repair the defect. The age at which the surgery is done depends on: […] The goal of surgical repair of TAPVR is to restore normal blood flow through the heart. To repair this defect, doctors usually: […] Surgical repairs for TAPVR are not a cure. People living with TAPVR may have lifelong complications. Routine checkups with a heart doctor are needed to monitor progress, avoid complications, and check for other health conditions that might develop over time.

• #66 Expert Care: TAPVR Surgery | UVA Health Children’s

  https://childrens.uvahealth.com/services/heart/chd/tapvr-treatment

  Your child will need TAPVR treatment. […] Your baby will need open-heart surgery. […] During a single surgical procedure, we will restore normal blood flow through your child’s heart. We’ll: Connect the pulmonary veins to the left side of the heart, Close off any abnormal connections between blood vessels, Close the hole (atrial septal defect) in the heart. […] Your child will recover in a private room in a special area of our Pediatric Intensive Care Unit (PICU). […] Once we repair your child’s heart defect, you can look forward to your child having a normal active childhood, even playing sports. […] When they become an adult, they can easily transition to our Adult Congenital Heart Disease Clinic.

• #67 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

  This defect must be surgically repaired in early infancy. At the time of open-heart surgery, the pulmonary veins are reconnected to the left atrium and the atrial septal defect is closed. […] This defect is almost always surgically repaired in early infancy or childhood. At the time of open-heart surgery, the pulmonary veins are reconnected to the left atrium and the atrial septal defect is closed. […] Once the TAPVC has been repaired, it’s unlikely that more surgery will be needed. Rarely, an adult patient may have obstruction that may need to be corrected depending on the severity.

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