Anomalous pulmonary venous return (TAPVR or PAPVR)

It’s natural for you to be concerned right now about your child’s health—a diagnosis of anomalous pulmonary venous return can be overwhelming. But you can rest assured that at Children’s Hospital Boston, your child is in expert hands.

Please note: Because TAPVR is a more serious condition than PAPVR and requires treatment in infancy, we’ve emphasized TAPVR in this discussion.

What is anomalous pulmonary venous return, and what are its forms?

Total or partial anomalous pulmonary venous return (TAPVR or PAPVR) is a rare heart defect in which a child’s pulmonary veins don’t connect normally to the left atrium. Instead they’re re-directed to the right atrium by way of an abnormal (anomalous) connection.

In the various forms of total anomalous pulmonary venous return (TAPVR), the abnormal development of the pulmonary veins prevents oxygen-rich (red) blood from passing from the left atrium to the left ventricle and on to the body as it should. Instead, oxygen-rich (red) blood returns to the right side of the heart rather than the left atrium, mixing with blue blood in the right atrium.

In total anomalous pulmonary venous return (TAPVR), surgery is always needed, since oxygen-poor (blue) blood cannot meet the body’s demands, either at rest or with activities.

The defect has many forms:

• total anomalous pulmonary venous return (TAPVR)
  • obstructed TAPVR—the most severe form, in which the anomalous veins are also obstructed; the baby is usually critically ill and may need emergency surgery
  • unobstructed TAPVR—a less severe form, in which the child may be only mildly cyanotic and may go weeks or months before being diagnosed; surgery will be performed after diagnosis
• partial anomalous pulmonary venous return (PAPVR)—the least severe form, in which the child may show only mild signs, such as tiring easily, or may not show signs or symptoms until later in life

Are there additional defects associated with TAPVR?

As with many congenital heart conditions, TAPVR has another defect associated with it—a hole called an atrial septal defect (ASD). The ASD is an abnormal opening between the right and left atria that allows some blood flow to the right atrium. It must be present with all forms of TAPVR in order for the baby to survive. The ASD can be easily corrected at the time of TAPVR repair surgery.

Sometimes, other congenital heart defects may also be present.

When is surgery for TAPVR usually performed?

Newborns with obstructed TAPVR need emergency surgery after birth. Babies or children with unobstructed TAPVR usually have surgery within days or weeks after they’re diagnosed.

What happens before surgery for TAPVR?

Obstructed TAPVR. Your child most likely will be admitted to Children’s cardiac intensive care unit (CICU) once symptoms are noted if there is obstructed TAPVR. Many babies with obstructed TAPVR are critically ill at the time of diagnosis and require intensive support with medications and a ventilator (breathing machine).

Before surgery, (and possibly afterwards, while his heart and lungs recover), some babies may be sick enough to require 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.

Care in Children’s cardiac ICU

Unobstructed TAPVR. Children who are diagnosed with unobstructed TAPVR are usually well enough to await surgery either at home or in the general inpatient cardiology unit. Some children may need medications to improve the function of their heart and lungs while awaiting surgery.      

What happens in surgery for TAPVR?

The goal of surgery for TAPVR is to restore normal red blood circulation to the left side of the heart. In the procedure, surgeons:

• re-direct the pulmonary veins to their proper connection at the left atrium
• tie-off the abnormal vessels that have developed as alternatives to normal flow
• close the ASD
                       

What are the signs and symptoms of TAPVR?

Most children with obstructed total anomalous pulmonary venous return are extremely ill soon after birth.

• Their skin and lips are cyanotic (blue).
• They may have trouble breathing.
• Breathing may be rapid, and may involve grunting and retractions of the rib cage muscles.
  • Sometimes, such infants may initially be thought to have pneumonia or another respiratory disease, until an accurate cardiac diagnosis is made.
• Non-obstructed TAPVR babies may not be highly symptomatic at birth, but after a few days, weeks, or months will usuallyshow poor feeding, rapid breathing, poor growth and/or cyanosis.

If your baby’s pediatrician notices any of these symptoms, the doctor may refer you to a pediatric cardiologist for immediate testing, diagnosis and a determination of treatment.

How do you diagnose TAPVR?

Most babies with TAPVR show symptoms on the day they’re born. Others show symptoms in the first few days or weeks of life. If your newborn baby was born with a bluish tint to his skin, if he’s having difficulty breathing, or if your pediatrician hears a heart murmur, you may be referred to a pediatric cardiologist, who will perform a physical exam.

The definitive diagnosis of total anomalous pulmonary venous return is usually made by cardiac ultrasound (echocardiogram).

A tiny patient gets an ultrasound at Children’s

Other tests may include:

• cardiac catheterization, to determine abnormal attachment of vessels
• cardiac MRI or CT angiogram to define pulmonary venous drainage
• chest x-ray, to see if there’s fluid in the lungs
• electrocardiogram (EKG), to evaluate heart rhythm

Will my child be OK after surgical repair for TAPVR?

The outlook varies from child to child, but open heart surgery at Children’s has among the highest success rates in the United States among large pediatric cardiac centers. And when surgical repair for TAPVR is done early in infancy, a child’s survival rate is very good.

What causes TAPVR or PAPVR?

The heart starts to form during the first eight weeks of fetal development. In TAPVR, during this development period the pulmonary venous return doesn’t develop properly.

Some congenital heart defects may have a genetic link, either due to a defect in a gene or a chromosome abnormality—causing heart problems to occur more often in certain families. Most often, though, this heart defect occurs sporadically (by chance), with no clear reason for its development.

It’s important for parents to understand that you’ve done nothing to cause your baby’s condition. Nothing you’ve ingested or have been exposed to environmentally has been definitively be linked to having a baby with TAPVR or PAPVR.

How common is TAPVR?

Total anomalous pulmonary venous return is a rare condition that accounts for 1 to 2 percent of congenital heart defects or about 1 in 20,000 births.

Will my baby be OK in the medium- and longer-term?

Surgical techniques for treating TAPVR and its associated defects are continually being refined, and surgical success rates at Children’s are very high. Nevertheless, your child will need lifelong monitoring (and possibly medication) to treat or prevent later problems that can occur, such as:

• endocarditis (infection of the heart’s walls or valves)
• irregularities in heart rhythm (arrhythmias)
• an obstruction in the pulmonary veins or at the surgical site at the left atrium

For the approximately 15 percent of babies who develop obstructed pulmonary veins after their initial surgery, additional surgeries or catheterizations may be needed to treat their continuing heart disease.

Where can my child find care and support when he grows up?

Our Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH) provides long-term inpatient and outpatient care and advanced therapeutic options for patients with congenital heart disease and pulmonary hypertension as they reach and progress through adulthood.

BACH is an international center for excellence, with physicians and services from Children’s, Brigham and Women’s Hospital and Beth Israel Deaconess Medical Center. The center promotes and supports clinical and scientific research for the advancement of care of these patients, and is a leader in the education of providers caring for this unique population.

The Adult Congenital Heart Association (ACHA) is a national not-for-profit organization dedicated to improving the quality of life—and extending the life—of adults with congenital heart defects. The organization serves and supports many of the adults with congenital heart defects (now thought to number as many as one million), their families and the medical community, as well as conducting research and providing advocacy, outreach and education.

FAQ
 

Q: What is anomalous pulmonary venous return?
A: The pulmonary veins are four blood vessels that bring oxygen-rich (red) blood from the lungs to the left atrium of the heart. Total or partial anomalous pulmonary venous return (TAPVR or PAPVR) is a rare heart defect in which all (total) or one or more (partial) of a child’s pulmonary veins do not connect normally to the left atrium. Instead, the veins are re-directed to the right atrium by way of an abnormal (anomalous) connection. TAPVR may require emergency surgery, while PAPVR can be repaired on a scheduled basis.

Q: Are there different types of TAPVR and PAPVR?
A: There are two main forms of anomalous pulmonary venous return, as well as several variations of each form. The main forms are:

•  total anomalous pulmonary venous return (TAPVR)
  • obstructed TAPVR—the most severe form, in which the anomalous veins are also obstructed; the baby is usually critically ill and may need emergency surgery
  • unobstructed TAPVR—a less severe form, in which the child may be only mildly cyanotic and may go a few weeks before being diagnosed; surgery will be performed after diagnosis
     
• partial anomalous pulmonary venous return (PAPVR)—the least severe form, in which the child may show only mild signs, such as tiring easily, or may not show signs or symptoms until later in life

Q: If my child has TAPVR, will he be OK?
A: Open heart surgery at Children’s Hospital Boston has among the highest success rates in the United States among large pediatric cardiac centers. Even so, your child will need lifelong monitoring (and possibly medication) to treat or prevent later problems that can occur, such as:

• endocarditis (infection of the heart’s walls or valves)
• irregularities in heart rhythm (arrhythmias)
• a blockage in the pulmonary veins or at the surgical site at the left atrium

For the approximately 20 percent of babies who develop obstructed pulmonary veins after their initial surgery, additional surgery or catheterization may be needed to treat their continuing heart disease.

Q: How does Children’s treat TAPVR and PAPVR?
A:

• Obstructed TAPVR is a cardiac surgery emergency and is always treated by surgical repair.
• Unobstructed TAPVR is a less severe form, but is surgically treated when symptoms appear, which can be a few weeks after birth.
• PAPVR is not a surgical emergency, and is diagnosed and treated when symptoms appear—often in childhood, but sometimes as late as adulthood.

Q: What are the signs and symptoms of TAPVR?
A: Most children with TAPVR are extremely ill soon after birth.

• Their skin and lips are cyanotic (blue).
• They may have trouble breathing.
• Breathing may be rapid, and may involve grunting and retractions of the rib cage muscles.
  • Sometimes, such infants may initially be thought to have pneumonia or another respiratory disease, until an accurate cardiac diagnosis is made.
• Non-obstructed TAPVR babies may not be highly symptomatic at birth, but after a few days or weeks will show poor feeding, rapid breathing, poor growth and/or cyanosis.

If your child has any of these symptoms, your pediatrician may refer you to a pediatric cardiologist for immediate testing, diagnosis and a determination of treatment.

 

Q: If my child has TAPVR or PAPVR, what should I ask my Children’s doctor?
A: Some of the questions you may want to ask include:

• What tests will you perform to further diagnose my child?
• What form of anomalous pulmonary venous return does he have?
• Does he need surgery? How long will the surgery take? How long for him to recover?
• Will my child be OK if he has congenital heart disease?
• Will there be restrictions on my child’s activities in the future?
• Will there be long-term health effects? Can this recur?
• What can we do at home?

Q: When is TAPVR usually diagnosed, and how?
A: Most babies with TAPVR show symptoms at birth. Others show symptoms in the first few days or weeks of life. If your newborn baby was born with a bluish tint to his skin (cyanosis) or rapid breathing, your pediatrician may refer you to a pediatric cardiologist, who will perform a physical exam.

The definitive diagnosis of TAPVR is usually made by cardiac ultrasound (echocardiogram). Other tests may include:

• cardiac catheterization, to determine abnormal attachment of vessels
• cardiac MRI or CT angiogram, to define pulmonary venous drainage
• chest x-ray, to see if there’s fluid in the lungs
• electrocardiogram (EKG), to look for signs of ventricular enlargement

Q: What should we do at home after TAPVR surgery?
A: Consult your child’s cardiologist for guidance on follow-up care—including:

• wound care while your baby is healing
• a nutritional program to encourage weight gain
• an oral hygiene program to prevent infection
• an appropriate exercise regimen to build body mass and achieve fitness

Your child may need additional surgery or catheterization sometime during his childhood, so it’s important that he be monitored by a pediatric cardiologist. As he recovers and grows, be sure to follow a program of regular well-baby/well-child checkups.

Q: What is the long-term outlook for TAPVR-affected children?
A: Surgical techniques for TAPVR and its associated defects are continually being refined, and the long-term outlook is continually improving. Nevertheless, your child will need lifelong monitoring and possibly medication, since he may be at some risk for arrhythmias, infections, or recurring obstruction in the pulmonary veins or at the surgical site at the left atrium.

Q: What causes anomalous pulmonary venous return?
A: TAPVR or PAPVR occurs due to the abnormal development of the heart’s pulmonary veins during  early fetal growth. Most often, this heart defect occurs sporadically (by chance), with no clear reason for its development.

It’s important for parents to understand that you’ve done nothing to cause your baby’s condition. Nothing you’ve ingested or have been exposed to environmentally has been definitively linked to having a baby with anomalous pulmonary venous return.



Q: What is Children’s experience treating congenital heart defects?
A: Children’s surgeons treat some of the most complex pediatric heart conditions in the world, with overall success rates approaching 98 percent—among the highest in the nation among large pediatric cardiac centers.

Our experts have pioneered some of medical science’s most advanced heart treatments, now in use around the globe. Children’s is among the only major pediatric heart centers in the world performing fetal heart interventions for certain congenital defects.

 

Q: What heart research and innovations are coming from Children’s?
A: A significant amount of Children’s groundbreaking cardiac research aims to refine and advance the open heart surgery and catheterization procedures that treat congenital heart defects in newborns and young children—including anomalous pulmonary venous return. Children’s Cardiac Surgery Research Laboratory is studying the mechanisms of heart disease and new treatments for children with congenital heart defects.
 

Learn more about Children’s cardiac research initiatives and Children’s current projects in cardiology research.
 

Causes
 

Anomalous pulmonary venous return occurs due to the abnormal development of the heart’s pulmonary veins during early fetal growth. It’s important for parents to understand that you’ve done nothing to cause your baby’s condition.

Some congenital heart defects may have a genetic link, causing heart problems to occur more often in certain families. Most often, though, this heart defect occurs sporadically (by chance), with no clear reason for its development.

Signs and symptoms
 

Most children with TAPVR are extremely ill soon after birth.

• Their skin and lips are cyanotic (blue).
• They may have trouble breathing.
• Breathing may be rapid, and may involve grunting and retractions of the rib cage muscles.
  • Sometimes, such infants may initially be thought to have pneumonia or another respiratory disease, until an accurate cardiac diagnosis is made.
• Non-obstructed TAPVR babies may not be highly symptomatic at birth, but after a few days or weeks will show poor feeding, rapid breathing, poor growth and/or cyanosis.

If your child has any of these symptoms, your pediatrician may refer you to a pediatric cardiologist for immediate testing, diagnosis and a determination of treatment.

When to seek medical advice
 

Call your health care provider immediately if your baby or child is having difficulty breathing or is breathing rapidly, has a bluish color or seems to tire too easily.

Questions to ask your doctor
 

After your child is diagnosed with TAPVR or PAPVR, you may feel overwhelmed with information. It’s easy to lose track of the questions that occur to you. Lots of parents find it helpful to jot down questions as they arise—that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.

Some of the questions you may want to ask include:

• What tests will you perform to further diagnose my child?
• What form of anomalous pulmonary venous return does he have?
• Does he need surgery? How long will the surgery take? How long for him to recover?
• Will my child be OK if he has congenital heart disease?
• Will there be restrictions on my child’s activities in the future?
• Will there be long-term health effects? Can this recur?
• What can we do at home?

Who’s at risk
 

Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development. So, it’s difficult to predict who’s at risk. Familial cases have been reported, but no genetic link has been confirmed. In general, if you have a child with a congenital cardiac defect, the chance of other children you may have being born with a defect is about 2 to 3 percent.

Complications
 

Most children who have surgical repairs for TAPVR do very well after surgery. But complications from your child's condition can occur later on, during childhood or later in life—including arrhythmias, recurring obstructions in the pulmonary veins or at the surgical site, or infections within the heart (endocarditis). So, as your child grows, and on into adulthood, it’s important for him to be closely monitored by his cardiologist.

Long-term outlook
 

Surgical techniques for TAPVR and its associated defects are continually being refined, and the long-term outlook is continually improving. Even so, your child will need lifelong monitoring and possibly medication, since he may be at some risk for arrhythmias, recurring obstructions in the pulmonary veins or at the surgical site, or infections within the heart (endocarditis).

For teens
 

If you’re a teen with a congenital heart defect, you have a lot to cope with. Besides the typical issues any teenager faces—from social acceptance to body changes and more—you’ll also have to deal with medical appointments and procedures, some delay of your natural wish for independence, feeling different and assuming a lot of personal responsibility for maintaining your own good health.

If you feel overwhelmed, depressed or anxious through this important time in your transition to adulthood, speak to your doctor or counselor to get help.

For adults
 

If you were treated for congenital heart disease as a child, you’re probably being followed by your cardiologist, since complications from early heart disease can arise in adulthood.

You may need lifelong monitoring and medication, since you could be at some risk for arrhythmias, blood clots, infections, leaky valves or obstructed vessels. Going forward, your cardiologist will advise you on activity levels, pregnancy issues and certain lifestyle choices.

The good news is that Children’s can help adults with congenital heart defects. Many adults who were heart patients at Children’s as babies or children continue to be monitored by the clinicians who’ve followed them since childhood.

In addition, our Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH) provides long-term inpatient and outpatient care and advanced therapeutic options for patients with congenital heart disease and pulmonary hypertension as they reach, and progress through, adulthood.

BACH is an international center for excellence, with physicians and services from Children’s, Brigham and Women’s Hospital and Beth Israel Deaconess Medical Center. The center promotes and supports clinical and scientific research for the advancement of care of these patients, and is a leader in the education of providers caring for this unique population.

What you can do at home
 

After surgery, your child’s cardiologist will offer recommendations for follow-up care, including:

• wound care while your baby is healing
• a nutritional program to encourage weight gain
• an oral hygiene program to prevent infection
• an appropriate exercise regimen to build body mass and achieve fitness

Prevention
 

It’s important to understand that as parents, you’ve done nothing to cause your baby’s condition, and there are no precautions you could have taken to prevent it. Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development.

TAPVR/PAPVR glossary
 

• acidosis: excessive acidity in the blood

• anomalous: abnormal

• anomalous pulmonary venous return (connection): a condition in which the abnormal development of the pulmonary veins prevents oxygen-rich (red) blood from returning from the lungs to the left side of the heart and on to the body as it should. Surgery is needed to correct this defect.

• aorta: one of the heart’s two great arteries. In a normal heart, the aorta arises from the left ventricle and carries oxygen-rich blood out to the body.

• atrial septal defect (ASD): a congenital (present at birth) heart defect in which there is an opening in the tissue wall (septum) that divides the two upper chambers of the heart (right and left atria). This hole is necessary in TAPVR to sustain the baby’s life, because it allows at least some oxygen-rich (red) blood to circulate until surgical repair takes place.

• atrium: one of the two upper chambers of the heart, which are the left atrium and the right atrium.

• cardiac catheterization: an invasive diagnostic procedure performed under sedation to obtain detailed visual information and measurements about the structures inside the baby’s heart. For anomalous pulmonary venous return, cardiac catheterization may be used to determine abnormal attachment of vessels.

• cardiac/cardio-: pertaining to the heart

• cardiac magnetic resonance imaging (MRI): a non-invasive diagnostic tool using 3-D imaging technology produced by magnets to accurately determine the blood flow and functioning of your child's heart. For anomalous pulmonary venous return, cardiac MRI and fast CT are used to define pulmonary venous drainage.

• cardiac surgery: a surgical procedure performed on the heart or one of the blood vessels connected to the heart

• cardiac surgeon: a doctor who performs surgery on the heart. A pediatric cardiac surgeon performs surgery on the hearts of infants and children.

• cardiologist: a doctor who diagnoses and treats heart problems non-surgically. A pediatric cardiologist treats infants, children and some adults with heart problems.

• chest x-ray: a diagnostic tool to evaluate the size and spatial relationships of the heart within the child’s chest. For anomalous pulmonary venous return, chest x-ray is used to detect fluid in the lungs.

• cyanosis (cyanotic): blue color of skin, lips and nailbeds caused by a reduction in the amount of oxygen-rich (red) blood circulating in baby’s bloodstream

• congenital heart defect: a heart defect that’s present at birth. The heart usually starts to form in the first eight weeks of fetal development. It’s thought that most congenital heart defects develop during this period.

• diagnosis: medical determination of illness or disease based on history, physical examinations and advanced technology diagnostic testing tools

• echocardiogram (echo, cardiac ultrasound): a diagnostic tool that evaluates the structure and function of the heart using sound waves that produce a moving picture of your child’s heart and heart valves. Usually, cardiac ultrasound is the definitive test for anomalous pulmonary venous return.

• ECMO (ExtraCorporeal Membrane Oxygenation): an advanced life support technology that functions as a replacement for a critically ill child’s heart and lungs. ECMO is similar to the heart lung bypass machine used during open heart surgery.

• electrocardiogram (ECG, EKG): a diagnostic tool that evaluates the electrical activity of your child’s heart. For anomalous pulmonary venous return, EKG is used to detect signs of ventricular enlargement.

• CT angiogram: high-speed, multi-slice CT scanner that dramatically reduces the time a child needs to remain still for an exam

• infracardiac (infradiaphragmatic) TAPVR: one of the forms of TAPVR, in which the veins first drain downwards and then come into the right atrium from below the diaphragm via the hepatic veins and inferior vena cava. This form is more likely to be obstructed than other forms of TAPVR.

• innominate vein: one of two veins in the neck that join to form the superior vena cava; involved in supracardiac abnormal venous pathway in TAPVR

• intracardiac TAPVR: one of the forms of TAPVR, in which the veins come directly into the right atrium via a vessel called the coronary sinus. This form of TAPVR is less likely to be obstructed than the infracardiac form.

• IV: intravenous, into the vein; one method of delivering medication

• neonatologist: a doctor who specializes in illnesses affecting newborns, both premature and full-term

• obstructed TAPVR: a form of TAPVR in which the pulmonary veins are blocked in addition to being abnormally attached to the heart. Obstructed TAPVR is a cardiac surgical emergency.

• PAPVR: acronym for partial anomalous pulmonary venous return. In PAPVR, all of the pulmonary veins return to the right side of the heart abnormally.

• pulmonary vein: one of four blood vessels that normally carry oxygen-rich (red) blood from the lungs to the left side of the heart, to be circulated to the body. In anomalous pulmonary venous return, all (total, TAPVR) or one or some (partial, PAPVR) of these veins returns to the right side of the heart abnormally.

• sporadic: occurring by chance, occasionally, not inherited
   
• signs and symptoms: the presenting reasons why a child needs medical attention. Characteristics such as onset, quality, triggers and severity help diagnosticians to determine a disease, or to decide which testing is needed to determine the disease.
   
• supracardiac TAPVR: most common form of TAPVR, in which the pulmonary veins come in to the right side of the heart above the diaphragm via a circuitous pathway to the superior vena cava. This form of TAPVR is less likely to be obstructed than the infracardiac form.
   
• TAPVR: acronym for total anomalous pulmonary venous return. In TAPVR, all of the pulmonary veins return to the right side of the heart abnormally.
   
• unobstructed TAPVR: a form of TAPVR in which the pulmonary veins are abnormally attached to the heart, but are not blocked in addition. Severe, but somewhat less so than obstructed TAPVR.

For a more complete list of cardiovascular terms, visit our Cardiovascular Glossary.

For in-depth visual information on several of the conditions, diagnostic tools and procedures described above, visit our cardiovascular Multimedia Library.