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Health Information For Parents
Hypoplastic left heart syndrome (HLHS) is a rare birth defect of a baby’s heart. The left side of the heart doesn’t grow as it should, making it smaller and weaker than normal.
The left side of the heart is supposed to pump blood out to the body. But a baby’s heart with HLHS can’t do the job. This makes a baby very sick. Without medicines and a series of three surgeries to rebuild the heart, babies with HLHS won’t survive.
The left side of the heart can’t be fixed, so the goal of the surgeries is to rebuild parts of the heart and “redirect” the way blood flows to and from the body.
Soon after birth, a baby with HLHS needs around-the-clock care in a hospital for the first weeks or months of life.
The heart is a muscle that pumps blood. It acts like two pumps in one. The pumps are called ventricles and each has an important job:
In the lungs, blood fills up with oxygen and gets rid of carbon dioxide.
Because it pumps blood to the entire body, the left ventricle is a stronger pump than the right ventricle.
With hypoplastic left heart syndrome:
Within a few hours to a day or so after birth, a newborn with undiagnosed HLHS may have:
HLHS is a birth defect that happens when a baby is growing in the womb. No one knows exactly what causes it, but it could have a mix of causes, including a baby’s genes (DNA).
Hypoplastic left heart syndrome can be diagnosed with:
HLHS is first treated with medicines, and then a series of three surgeries. Without surgery, a baby won’t survive. Treatment for HLHS starts as soon as the baby is born.
Immediate treatment includes a medicine called prostaglandin. Prostaglandin keeps the PDA open so oxygen can get to the body.
Some babies need other medicines to help balance how much blood goes to the lungs and how much goes to the body.
Also if the ASD is too small, it might be made bigger. This is done with a cardiac catheterization procedure or by surgery.
Because the left ventricle and aorta are too small, the goals of the surgeries are to:
Surgeries are done in stages as a baby grows. They are (in order):
Being actively involved in your child’s care plan can help you feel more in control.
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The foramen ovale is a normal opening between the upper two chambers of an unborn babyâs heart. It usually closes soon after the babyâs birth â when it doesn’t, it’s called a patent foramen ovale.
Ebstein anomaly is a rare heart defect that affects the tricuspid valve. It can cause problems that range from very mild to very serious.
The ductus arteriosus is a blood vessel that connects two major arteries before birth and normally closes after a baby is born. If it stays open, the result is a condition called patent ductus arteriosus (PDA).
An interrupted aortic arch (IAA) is a rare heart condition in which the aorta doesnât form completely. Surgery must be done within the first few days of a babyâs life to close the gap in the aorta.
Note: All information is for educational purposes only. For specific medical advice, diagnoses, and treatment, consult your doctor.
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