Our Health Topics

Anomalous Pulmonary Venous Return (TAPVR or PAPVR)

  • The care of critically ill infants with obstructed anomalous pulmonary venous return requires a multidisciplinary team of cardiac ICU doctors and nurses, non-invasive cardiologists and surgeons to ensure the best possible recovery.

    --Catherine Allan, MD, assistant in Cardiology, Boston Children's Hospital

    If your infant or child has been diagnosed with one of several forms of anomalous pulmonary venous return (or “connection”), an understanding of the condition will help you to cope with this rare and complex congenital (present at birth) heart defect. While very serious, anomalous pulmonary venous return is treatable surgically at Boston Children's Hospital, and recent surgical advances have greatly improved your child’s outlook for success.

    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 causes varying degrees of symptoms, including cyanosis (blue-ish discoloration of skin and lips) and difficulty breathing. Severe cases will develop severe cyanosis, difficulty breathing, low blood pressure and excessive acidity in the blood (acidosis).

    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 (narrowed) prior to entering the right atrium, making it difficult for blood to return from the lungs to the heart; the baby is usually critically ill and will need emergency surgery
      • unobstructed TAPVR—a less severe form, in which the child may be only mildly cyanotic and may go a few weeks or even longer before being diagnosed, at which time surgery is performed
    • 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

    After surgery, most children lead healthy lives, but they will need lifelong monitoring and follow-up care.

    Comparison with normal heart

    normal heart

    In a normal heart, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs, where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle, then is pumped through the aorta out to the body.

    Total anomalous pulmonary venous return

    When a fetus is developing, the pulmonary veins are a single channel that must establish a connection to the developing left atrium. If it doesn’t connect, other pathways for pulmonary venous drainage develop.

    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. In all forms of TAPVR oxygen-rich blood returns to the right atrium and mixes with oxygen-poor blood coming back from the body.

    In order to get blood to the left atrium and left ventricle and out to the body, some of this “mixed” blood, which has lower than normal oxygen levels, must pass through a hole (atrial septal defect or ASD) from the right atrium to the left atrium. Surgery is needed, since oxygen-poor (blue) blood cannot meet the body’s demands, either at rest or with activities.

    Some babies with TAPVR also have obstruction (narrowing) of the vessels bringing blood from the pulmonary veins back to the right atrium, with two major consequences:

    • Return of blood to heart is impaired, compromising the heart’s ability to pump enough blood to the body—a condition known as low cardiac output.
    • The obstruction causes a “back-up” of blood flow through the veins, which leads to fluid leakage out of the pulmonary vessels (pulmonary edema) into the lung tissue, making it hard for babies to breathe and for the lungs to do their job of adding oxygen to blood and removing carbon dioxide.
       

    As a result, babies with obstructed TAPVR may have one or more of the following signs and symptoms:

    • low blood pressure and acid build-up in blood due to an inability to pump adequate amounts of oxygen and nutrient-rich blood to the body
    • cyanosis resulting from both the mixing of oxygen-rich and oxygen-poor blood in the heart and the lungs’ decreased ability to oxygenate blood
    • increasing difficulty breathing resulting from fluid build-up in the lungs (pulmonary edema)

    Infants with obstructed TAPVR are usually critically ill immediately after birth and need emergency surgery to restore the normal blood flow from the pulmonary veins to the left atrium.

    Infants who have unobstructed TAPVR are much less symptomatic. Many of these babies aren’t diagnosed until weeks or even months after birth. Although these babies usually have lower than normal oxygen levels in their blood, the degree of cyanosis is much less significant than for babies with obstructed TAPVR, and may not be noticeable.

    These babies are often diagnosed when a murmur is detected by the pediatrician at a well-child visit, or because they are noted to be breathing faster or harder than normal or are growing poorly. Although surgery is also necessary for babies with unobstructed TAPVR, it can usually be scheduled as an elective procedure.

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

    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. 

    For more visual and audio information on TAPVR/PAPVR, visit our cardiovascular Multimedia Library.

    How Boston Children’s Hospital approaches TAPVR or PAPVR

    Our team in Boston Children’s Department of Cardiac Surgery understands how distressing a diagnosis of an anomalous pulmonary venous return can be for parents. You can have peace of mind knowing that our 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.

    We use the following elements to provide the best possible outcomes:

    • accurate diagnosis and assessment: Subtle variations in heart anatomy—such as the arrangement of the arteries that feed the heart—can negatively impact surgical outcomes if not identified ahead of time. We utilize the most advanced techniques available for precisely determining your child’s heart anatomy, with interpretation by highly experienced cardiologists.
    • sophisticated and effective therapies: Babies with severe cases of TAPVR need emergency therapy. Our cardiac intensive care unit (CICU), cardiac cath labs and operating rooms deal with the urgent needs of our smallest patients 24/7. Children’s CICU was one of the first such units developed anywhere. Professionals from many countries visit our CICU to learn advanced techniques of post-operative care.
    • experienced and skilled team to perform surgery and other procedures: Boston Children’s cardiac surgeons have vast experience in the surgical procedures used to repair this defect, and they work with nurses and doctors who are focused on providing expert care after surgery.
    • close, expert medical follow-up: If you live in the Boston area, a Boston Children’s cardiologist will follow your child after surgical repair; if you live in another part of the country or the world, a Boston Children’s cardiologist will work closely with your local cardiologist. Adult patients with TAPVR are followed by Boston Children’s cardiologists with special training for adults with congenital heart problems.
       

    TAPVR/PAPVR: Reviewed by Catherine Allan, MD
    © Boston Children's Hospital, 2011

  • 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.
  • At Boston Children's Hospital, we know that the first step in treating your child is forming an accurate, complete and timely diagnosis.

    Most babies with TAPVR show symptoms on the day they’re born. Severe cases are obvious, are considered medical emergencies, and are admitted immediately to the cardiac ICU.

    Less severe cases show symptoms in the first few days 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. Your child’s doctor will listen to your baby’s heart and lungs, measure the oxygen level in his blood (non-invasively) and make other observations that help to determine the diagnosis.

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

    Some (not necessarily all) of the following medical tests may also used to diagnose, or confirm a diagnosis of, TAPVR or PAPVR:

    • cardiac catheterization, to determine abnormal attachment of vessels. Cardiac catheterization is an invasive diagnostic procedure performed under sedation to obtain detailed visual information and measurements about the structures inside the baby’s heart.
    • cardiac MRI or CT angiogram, to define pulmonary venous drainage. Cardiac MRI is 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. Children’s multi-slice CT scanners dramatically reduce the time a child needs to remain still for an exam.
    • chest x-ray, to see if there’s fluid in the lungs. A chest x-ray is a diagnostic tool usually used to evaluate the size and spatial relationships of the heart within the child’s chest.
    • electrocardiogram (EKG), to detect signs of ventricular enlargement. An EKG is a diagnostic tool that evaluates the electrical activity of your child’s heart.

    Boston Children’s an interventional catheterization pioneer

    You’ll be comforted to know that Boston Children’s pioneered interventional catheterization for many congenital heart defects and is a leader in the use of this procedure.

  • Having identified your child's heart condition at Boston Children's, we'll begin treating him, so that we may ultimately return him to good health. Specific treatments for TAPVR or PAPVR depend on the extent, location and variation of the defect.

    • Newborns with obstructed TAPVR need emergency surgery after birth.
    • Babies or children with unobstructed TAPVR usually have scheduled surgery within days or weeks after they're diagnosed.
    • Children with PAPVR may go undiagnosed for years, or even into adulthood. Upon being diagnosed, they too need surgery because of the cardiac risks of leaving PAPVR untreated.

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

    Babies with severe obstructed TAPVR may 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.

    We may also give him IV (intravenous) medications to help his heart and lungs function more efficiently.

    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.

    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           

    At home: caring for your child after surgery

    After any procedure, your child will need to be followed by a pediatric cardiologist who will:

    • advise you on wound care while your baby is healing
    • adjust your child's medications
    • help you with feeding problems
    • measure his oxygen levels
    • Your child's cardiologist will also offer recommendations for follow-up care, including:
    • 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 during his childhood, so it's important that his cardiologist follow him periodically. And as he recovers and grows, be sure to follow a program of regular well-baby/well-child checkups. And to the greatest extent possible, encourage your child to live normally. Even if some physical activities are limited, your child and your family can enjoy a full life together.

    As he grows: your child's long-term outlook

    Surgical techniques for anomalous pulmonary venous return are continually being refined, with the long-term outlook continually improving. Still, your child will need lifelong monitoring by his cardiologist (and possibly medication), since he may be at some risk for arrhythmias, infections, or a recurrence of obstruction of the pulmonary veins or at the surgical site.

    For the approximately 20 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.

    Your cardiologist will help you create a long-term care program as your baby matures into childhood, the teen years and even adulthood. The doctor may recommend that certain strenuous activities be limited, especially if your child has any arrhythmias or residual obstruction. We'll prevent and treat complications, and will advise on daily-life issues, such as activity levels, nutrition and precautions related to pregnancy.

    Coping and support

    At Boston Children's, we understand that a hospital visit can be difficult, and sometimes overwhelming. So, we offer many amenities to make your child's—and your own—hospital experience as pleasant as possible. Visit our Center for Families for all you need to know about:

    • getting to Boston Children's
    • accommodations
    • navigating the hospital experience
    • resources that are available for your family

    In particular, we understand that you may have a lot of questions if your child is diagnosed with TAPVR. How will it affect my child long term? What other problems could arise? We can connect you with a number of resources to help you and your family through this difficult time, including:

    • patient education: From the office visit to pre-op to the recovery room, our nurses will be on hand to walk you through your child's treatment and help answer any questions you may have—How long will I be separated from my child during surgery? How long will his recuperation take?
    • We'll also reach out to you by phone, continuing the care and support you received while your child was at Boston Children's.
    • parent-to-parent: Want to talk with someone whose child has been treated for TAPVR or PAPVR? We can often put you in touch with other families who've been through the same procedure that you and your child are facing, and who will share their experiences.
    • faith-based support: If you're in need of spiritual support, we'll connect you with the Boston Children's chaplaincy. Our program includes nearly a dozen clergy— representing Protestant, Jewish, Muslim, Roman Catholic and other faith traditions—who will listen to you, pray with you and help you observe your own faith practices during your hospital experience.
    • social work: Our social workers and mental health clinicians have helped many families in your situation. We can offer counseling and assistance with issues such as coping with your child's diagnosis, stresses relating to coping with illness and dealing with financial difficulties.
    • As your child reaches adulthood, you'll want him to know about our Boston Adult Congenital Heart and Pulmonary Hypertension Service (BACH). Boston Children's is a founding institution of BACH, an international center for excellence providing long-term inpatient and outpatient care and advanced therapeutic options as needed for congenital heart disease patients as they reach and progress through adulthood.

    To find out more, visit the Family resources page of Boston Children's For Patients and Families website.

    Screening Neurodevelopment Problems

    Children who’ve had surgery for heart disease as infants are at greater risk of neurodevelopmental problems. By school age, they tend to have more academic, behavioral and coordination difficulties than other children. Boston Children’s Cardiac Neurodevelopment Program—one of just a handful in the United States—provides expert screening, evaluation and care for infants, children and teens with congenital heart disease who are at risk for neurodevelopmental problems. Screening begins soon after your child’s first cardiac surgery and continues as your child grows to make sure she’s hitting her developmental milestones.

  • Pediatric cardiologists and pediatric cardiovascular surgeons at Boston Children's Hospital have pioneered the interventional catheterization techniques now used widely for many congenital heart defects.

    And a significant amount of the groundbreaking cardiac research currently being conducted at Boston Children’s aims to refine and advance the open heart surgery and catheterization procedures that correct congenital heart defects in newborns and young children—including anomalous pulmonary venous return.

    Cardiac surgery research

    Members of Boston Children’s Cardiac Surgery Research Laboratory—a multidisciplinary team of basic and applied research investigators who hold faculty appointments at Harvard Medical School—are studying the mechanisms of heart disease and new treatments for children with congenital heart defects.

    Some principal areas of active research are:

    • surgical robotics and ultrasound-guided intracardiac surgery: The department is pioneering the use of 3-D ultrasound and laparoscopic techniques to operate on the beating heart.
    • myocardial metabolism and myocardial hypertrophy and heart failure: Researchers are exploring new methods of myocardial preservation during heart surgery and the role of angiogenic growth factors in heart failure.
    • tissue engineering to stimulate the growth of new tissue to repair congenital defects, including valve abnormalities, right ventricular defects and arrhythmias
       

    Cardiology research

    Boston Children’s is a world leader in opening new avenues of “translational research,” bringing laboratory advances to the bedside and doctor’s office as soon as possible. Senior medical staff members of the Department of Cardiology—who hold faculty appointments at Harvard Medical School—participate in clinical research activities, and many do laboratory research, as well.

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

    Innovations

    New Congenital Heart Valve Center

    Clinical heart researchers at Boston Children’s are creating a new heart valve center with a focus on valve repair rather than replacement. The new center has formed in response to the greater emphasis currently being placed on identifying and treating valve abnormalities in children and young adults with congenital heart disease.

    Part of our approach to repairing AV canal is finding new ways to get more accurate imaging information ahead of time with techniques such as 3-dimensional (3D) echo and cardiac magnetic resonance imaging. Through use of such techniques, our surgeons can better understand the mechanism of valve dysfunction, and the appropriate repair to address that mechanism. Boston Children’s studies in the new center will be ongoing.

    Creating new ways to perform surgery

    Problem: When surgeons perform heart surgery on a baby, they need to open the infant’s chest and stop her heart—an inva­sive, lengthy procedure that can cause life-threatening complications. Pedro del Nido, MD, chief of Cardiac Surgery at Children’s, had to perform surgery on his tiny patients using this method, or come up with a way to improve it.

    Innovative solution: Del Nido decided to develop a way to perform surgery on a still-beating heart. But he needed two things that didn’t exist: superior imaging tools that could show the structures inside the heart while it’s beating, and tiny instruments to perform the intricate surgery.

    So, he bor­rowed technology from the videogame industry and developed stereo-rendered 3-D ultrasound imaging that allows surgeons to see inside the beating heart as a hologram.

    Del Nido also designed new instruments. One is a millimeter-sized tool that extends into the heart through needle-sized incisions. Using a joystick controller and real-time imaging, a surgeon can now navigate through the beating heart’s chambers in animals to remove blockages, repair faulty valves and close leaks.

    The other new instrument is a cardioportdevice that allows instruments to be safely introduced into the cardiac chambers with­out the usual risks of blood loss or an air embolism.

    Results: Del Nido’s 3-D tool appears not only to provide superior imaging, but also to yield faster surgery times. Researchers using it to operate on pigs with congenital heart disease performed the procedure 44 per­cent faster than before. Dr. Del Nido’s cardioport will soon be tested in clinical trials and will facilitate further development of similarly novel instruments for heart repair.

    Del Nido’s newly-developed cardioport
    will someday make possible faster, less invasive heart surgery.

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The future of pediatrics will be forged by thinking differently, breaking paradigms and joining together in a shared vision of tackling the toughest challenges before us.”
- Sandra L. Fenwick, President and CEO
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