Anomalous pulmonary venous return - TAPVR or PAPVR symptoms & causes in children

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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 Boston Children's Hospital, 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

TAPVR: different types of total anomalous pulmonary venous return

There are four major types of TAPVR, characterized by whether blood coming from the pulmonary veins drains to the right atrium by a vein entering from above, below, at the level of the heart or in some combination of these. For each type, there is an atrial communication that enables blood to reach the left side of the heart.

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.

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 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.

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.

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.
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- Sandra L. Fenwick, President and CEO

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