Hypoplastic Left Heart Syndrome, HLHS

What is Hypoplastic Left Heart Syndrome, HLHS?

Hypoplastic left heart syndrome is a rare congenital heart defect where the left side of the heart is severely underdeveloped. Coarctation or narrowing of the aorta is usually present with HLHS. The left side of the heart can’t properly supply blood to the body because the lower left ventricle is too small or doesn't exist. In addition, the valves on the left side of the heart don’t work properly, and the aorta is smaller than normal.

Hypoplastic left heart syndrome is a uniformly lethal cardiac abnormality if not surgically addressed. Infants with HLHS have a good chance of survival if treated in a timely fashion. However, they may suffer chronic health problems for the rest of their lives.

What causes HLHS?

Hypoplastic left heart syndrome occurs during the first trimester when the baby’s heart is developing. The cause is unknown but more studies are under way to determine if anti seizure drugs and some antidepressants like Wellbutrin have been known to cause HLHS and congenital heart defects.

There may be genetic causes also. If your family has one child with HLHS, the risk of having another with the same condition increases.

According to the Centers for Disease Control, women who are pregnant, or thinking about becoming pregnant, should know there are risks associated with using prescription painkillers. One of the risks is hypoplastic left heart syndrome. Women taking opioid painkiller drugs had double the risk for HLHS than women who took no opioid drugs.

What is the treatment for HLHS?

HLHS operations are complex and must be individualized for each patient. Your cardiologist will assess medical and surgical options on a case-by-case basis.

Successful preoperative management depends on providing adequate systemic blood flow while limiting pulmonary over-circulation.

The goal of surgical reconstruction of HLHS is to eventually separate the pulmonary and systemic circulations. The right ventricle remains the systemic ventricle while blood passively flows to the lungs. This ultimate reconstruction is accomplished in three stages.

Heart transplantation is another surgical option. Approximately 20 percent of infants listed for heart transplantation die while waiting for a suitable donor organ. After successful cardiac transplantation, infants require multiple medications to benefit the immune system and to prevent of graft rejection. Frequent outpatient surveillance is required to identify and monitor transplant rejection and to prevent lasting damage to the transplanted heart.

If you were prescribed Wellbutrin or an anti-seizure or migraine headache preventative drug or painkiller during your first trimester and your baby suffers from HLHS - you may qualify for a lawsuit to compensate for the pain and suffering of your child. Contact Anapol Schwartz for more info.

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