Treatments for anomalous pulmonary venous return - TAPVR or PAPVR in Children

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Contact the Heart Center

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.

Boston Children’s is so much more than a hospital—it’s a community of researchers, clinicians, administrators, support staff, innovators, teachers, patients and families, all working together to make the impossible possible. ”
- Sandra L. Fenwick, President and CEO

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