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There are many ways you can help children and their families get the care they need.
This comprehensive guide was prepared to help families for the short and long-term journey ahead. Learn more about the Fetal Cardiology Program and request your free copy.
Hypoplastic left heart syndrome (HLHS) is one of the most challenging congenital heart defects to care for. But Boston Children's Hospital is a world pioneer in innovative HLHS care—including fetal cardiac intervention—and our team is well-qualified to care for your child.
We are known for our science-driven approach. We’re home to the world’s most extensive pediatric hospital research enterprise and we partner with elite health care and biotech organizations around the globe. But as specialists in innovative, family-centered care, our physicians never forget that your baby is precious, and not just a patient.
Hypoplastic left heart syndrome is one of several congenital (present at birth) heart defects in which the heart has only one fully functioning ventricle. Learn about single ventricle defect.
In HLHS, most of the structures on the left side of the heart are too small and underdeveloped (hypoplastic) to provide enough red blood flow for the body’s needs. The most critical defect in HLHS is the small left ventricle, which needs to be large enough and strong enough to pump blood out to the body. If it’s too small, it simply can’t function effectively.
Other left heart structures can also be underdeveloped in varying degrees, including:
In HLHS, despite the severity of the heart defect, the fetus or newborn is able to survive because of two naturally occurring “holes in the heart,” which allow for the communication of blood between the left and right sides of the heart. These are the:
In HLHS, blood returning to the right atrium from the lungs must pass through the PFO to reach the right side of the heart. The right ventricle must then do “double duty”—pumping blood to the lungs through the pulmonary artery and to the body through the PDA.
Once the PDA begins to close (a natural occurrence), the baby will become extremely sick (go into “shock”) due to lack of blood supply to the body. At this point, treatment is necessary for the baby to survive.
HLHS can be diagnosed on fetal ultrasound when the person performing the ultrasound notices that the left heart structures are small. If HLHS is suspected, you should be referred to a fetal pediatric cardiologist for a fetal echocardiogram (a specialized fetal cardiac ultrasound).
A diagnosis of HLHS through prenatal cardiac ultrasound enables us to plan to treat your newborn immediately after delivery. The family and medical team should plan for delivery at, or next to, a cardiac center that’s equipped to care for the newborn, such as:
At first, a newborn with HLHS may appear normal. But symptoms usually develop in the first few hours or days of life. Lack of vital blood flow causes the baby to go into profound shock, endangering the brain, liver, kidneys and other vital organs. Symptoms may include:
An intravenous medication called prostaglandin (PGE), to keep the ductus arteriosus from closing, will be started and administered until the surgery.
The second surgery is called the bidirectional-Glenn shunt, and the third is called the Fontan. Together, they make up a series of surgeries called the Fontan Sequence. The goals of these surgeries are to:
Open heart surgery at Boston Children’s has the one of the highest success rates in the United States among large pediatric cardiac centers. However, your child will need at least three staged surgeries in his first few years of life.
Note: Infants who’ve had initial surgery for a single ventricle defect are typically enrolled in Boston Children’s Home Monitoring Program between the baby’s Stage I and Stage II operations in the Fontan sequence. For more, see “Boston Children’s Home Monitoring Program for your child” in Treatment & care.
Children with hypoplastic left heart syndrome will need follow-up care throughout their lives to ensure that their hearts continue to function adequately. Most children will also need heart medication(s). Complications going forward can include arrhythmias (abnormal heart rhythms), heart failure, blood clots, and a few other rare problems.
Your cardiologist will help you create a long-term care program as your baby grows into childhood and his teen years. Families with a child with HLHS will have an ongoing relationship with their cardiologist. We will treat your child if complications arise, and we’ll advise on daily-life issues such as exercise, activity levels, nutrition and pregnancy precautions.
It’s important to understand that as parents, you’ve done nothing to cause HLHS and its associated defects. Nothing you have ingested or have been exposed to environmentally has been linked to having a child with HLHS. Familial cases have been reported, but the genetic link has yet to be clarified.
HLHS is the result of underdevelopment of sections of the fetal heart during the first eight weeks of pregnancy. But it’s not understood why some babies’ hearts don’t develop normally. Congenital heart defects usually occur sporadically (by chance), with no clear reason for their development.
Call your health care provider immediately if your baby or child is having difficulty breathing or is breathing rapidly, has a bluish color, tires easily, has cold hands and feet, is drowsy or unresponsive and is uninterested in eating.
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 the genetic link has yet to be clarified.
Children who have had HLHS reconstruction surgeries can be susceptible to arrhythmias, heart failure and blood clots as they go forward. And the potential does exist for needing a heart transplant in early or mid-childhood.
In many cases, the Fontan reconstructive surgeries now can palliate (not cure) HLHS, so the outlook for your child is positive. Children who have undergone HLHS reconstruction can be susceptible to arrhythmias, heart failure, blood clots and a few other complications. And the potential does exist for needing a heart transplant in early or mid-childhood.
Your cardiologist will help create a long-term care plan as your baby grows into childhood and his teen years. Most people who have had congenital heart disease repair have an ongoing relationship with their cardiologist; we will consult and treat your child if complications arise, and we’ll advise on daily-life issues such as exercise, activity levels, nutrition and pregnancy precautions.
As 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 also have to deal with medical appointments and procedures, some delay of your natural wish for independence, the consciousness of feeling different and personal responsibility for maintaining your good health.
If you as a teen (or as the parent of a teen) feel overwhelmed, depressed or anxious through this time, speak to your doctor to get help.
Consult your child’s cardiologist for guidance on specific steps for follow-up care at home. And as your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups.
It’s important to understand that as parents, you’ve done nothing to cause HLHS and its accompanying defects, 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.
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