Treatments for single ventricle defects in children

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Contact the Complex Biventricular Repair Program

Having identified your child's heart condition, we're able to begin the process of treating him, so that we may ultimately return him to good health. Specific treatments for your child's single ventricle defect depend on the extent and location of the defect, as well as other variables.

Your child most likely will be admitted to Boston Children's cardiac intensive care unit (CICU) once symptoms are noted. Before surgery, (and possibly afterwards, while his heart and lungs recover), he may be placed on a 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.

Treatment options

Once he's stabilized, your baby's treatments may include: 

medication: Doctors may administer an IV (intravenous) medication to keep the infant's ductus arteriosus (the prenatal connection between the aorta and the pulmonary artery, which usually closes shortly after birth, but which is now important as a temporary alternative opening for blood flow) from closing.
cardiac catheterization: Prior to your child's initial surgery, or between staged operations (see next bullet below), doctors may perform a cardiac catheterization procedure to improve the mixing of oxygen-rich (red) blood and oxygen-poor (blue) blood. A special catheter with a balloon in the tip is used to create or enlarge an opening in the atrial septum (wall between the left and right atria).
surgery: Some single ventricle defects are treated using the Fontan Sequence—a staged series of three operations performed between the first few days or months and the first few years of life.
The first stage is to optimize the blood flow to the lungs if it is too much or too little. If the blood flow is too little, a Blalock-Taussig shunt is usually performed. If it is too much, the pulmonary artery may be banded to control blood flow. If blood flow is neither too little nor too much, the first stage may be skipped, and the second stage—the bi-directional Glenn—may be performed at 4 to 8 months of age.
Blalock-Taussig shunt: This first operation, done when the blood flow to the lungs is inadequate, is usually performed soon after birth to create a pathway for blood to reach the lungs. A connection is made between the first artery off the aorta (right subclavian artery) and the right pulmonary artery.

Some of the blood traveling through the aorta towards the body will “shunt” through this connection and flow into the pulmonary artery to receive oxygen.

Following the procedure, your child will still have some degree of cyanosis, since oxygen-poor (blue) blood from the right atrium and oxygen-rich (red) blood from the left side of the heart will mix and flow through the aorta to the body.
pulmonary artery banding (PAB): This alternative first operation, done when the blood flow to the lungs is excessive, is performed to reduce and limit pulmonary artery blood flow and to protect the pulmonary vessels from hypertrophy and pulmonary hypertension.
For HLHS, the first stage surgery is called the Norwood procedure. Developed at Children's, the procedure is usually performed in the first week of the baby's life. Itconnects the right ventricle to the aorta so that its flow will be delivered to the body through branches of the aorta.

Two alternatives to the Norwood procedure are sometimes performed, based on surgical preference, the child's size and the anatomy of the defects. These are:
  • the Sano modification: places a conduit between the pulmonary artery and the right ventricle instead of the modified Blalock-Taussig Shunt
  • the hybrid procedure: combines cath lab techniques, such as stenting, with surgical techniques. In this procedure, the interventional cardiologist and the cardiovascular surgeon work together.
bi-directional Glenn: The second operation, often performed when a child is between 4 and 12 months old, reduces the single ventricle's workload, and thus the risk of damage, and sets the stage for the Fontan procedure to come.

This procedure replaces the Blalock-Taussig shunt (which the baby's heart will outgrow) with another connection to the pulmonary artery. The superior vena cava (the large vein that returns oxygen-poor blood from the head and arms back to the heart) is surgically connected to the pulmonary artery so that blood can proceed to the lungs to receive oxygen.
Fontan procedure: This final operation in the sequence, done in the first few years of life, is performed as the baby achieves further lung maturity, enabling the vessels of the lungs to now accommodate all the body's blue blood.

The principle of the procedure is that it's not necessary to have a ventricle that pumps to the lungs so long as the lung arteries have a low resistance, are well developed and are of good size, and as long as the single ventricle fills at a low pressure.

Surgery involves directly connecting the returning blue blood into the pulmonary arteries. This can be achieved in a number of different ways, sometimes with, and sometimes without, the use of synthetic tubes (conduits).

At home: caring for your child after surgery for a single ventricle defect

Babies usually remain cyanotic after the first two operations and until the final (Fontan) operation is performed. After the Fontan procedure, you can expect your child's oxygen levels to improve. In fact, many children experience major improvements in growth and development after Fontan, eventually catching up to other children.

After each operation, your child will need to be followed by a pediatric cardiologist who will adjust your child's medications, measure his oxygen levels and determine when it is time for the next operation.

Your child's cardiologist will also offer recommendations for post-operative 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

As your baby 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.
 

Boston Children's Home Monitoring Program for your child

An infant with single ventricle anatomy needs support with shunt-dependent blood flow between his Stage I and Stage II surgical repairs (see surgery descriptions above on this page).

The results of the Stage I surgery have improved—with nearly 90% of infants who are cared for in experienced centers discharged home after the first stage. So we can now focus new attention on reducing the known mortality of 10 to15 percent for these infants between their Stage I and Stage II surgical repairs.

Research shows the vital importance of a Home Monitoring Program, including daily at-home assessments of oxygen saturations and weight between the Stage I and Stage II surgeries.

Checking your baby's daily weight:

  • alerts you and your child's providers to dehydration
  • enables appropriate immediate intervention
  • decreases the risk of sudden death at this very fragile stage in your infant's multiple-surgery sequence

During this period between the Stage I and Stage II surgeries, your child's pediatric cardiologist and pediatrician will be in close contact with you as your child's primary home caregiver. (After your child's Stage II repair, this intensive level of home monitoring will no longer be necessary.)

In Boston Children's Home Monitoring Program:

  • You'll be given detailed guidelines and goals for your child's:
    • growth
    • weight gain
    • oxygen saturations       
  • You'll be instructed to call your child's cardiologist if his goals for growth, weight gain and oxygen saturation aren't met, or if:
    • your child is breathing harder, faster or is fussy beyond his baseline
    • you have any questions or concerns
  • You'll be supplied with a pulse oximeter and a baby scale and shown how to use both instruments. You'll measure your child's saturations twice a day and weigh the baby daily—checking against given guidelines and goals.

  • You'll record your baby's weights, oxygen saturations and general observations in a daily log, and will share this log with the pediatrician/cardiologist during check ups.
      
  • You'll consult your pediatrician if your child:
    • is experiencing nausea/vomiting/diarrhea
    • is not having sufficient wet diapers
    • is feverish
     
  • You'll have weekly calls with your designated pediatric nurse practitioner, who will consult with you, and who will notify your child's cardiologist of any concerns that arise.

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

Surgical techniques for single ventricle defects are continually being refined, with long-term outcomes constantly improving. Still, patients will need lifelong monitoring and medication, since they'll always be at some risk for arrhythmias, infections, heart failure or stroke.

Your cardiologist will help you create a long-term care program as your baby matures into childhood, the teen years and even adulthood. Most people who've had congenital heart disease repair will have an ongoing relationship with their cardiologist. 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 The 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 a single ventricle defect. How will it affect my child long term? What do we do next? 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? What will the operating room be like? They will also reach out to you by phone, continuing the care and support you received while at Boston Children's.
  • parent-to-parent: Want to talk with someone whose child has been treated for a single ventricle defect? 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 the Boston Adult Congenital Heart (BACH) and Pulmonary Hypertension Service. 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.


You'll be comforted to know …

… that Boston Children's Hospital pioneered interventional catheterization for many congenital heart defects.

Boston Children's Heart Care Center 

The Heart Care Center at Boston Children's is the largest pediatric heart program in the United States. Our staff of more than 80 pediatric cardiac specialists cares for thousands of children and adults with congenital and acquired heart defects each year, from simple to complex cases. We have experience treating rare heart problems—with results that are among the best in the world


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- Sandra L. Fenwick, President and CEO

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