Hypoplastic Left Heart Syndrome

Hypoplastic Left Heart Syndrome is characterized by a severely underdeveloped left ventricle. The infant may seem perfectly healthy at birth. However, when the ductus arteriosus closes, as it normally does, a few days after birth, severe complications result ("Prenatal Detection" 2001). HLHS infants then develop metabolic acidosis, decreased systemic perfusion, circulatory collapse, and/or death.

Connor was born with Hypoplastic Left Heart Syndrome. After his Stage 1 operation, he was determined to have severe brain damage. His parents opted for him not to undergo any more operations. The doctors then predicted he would only survive eight to twelve months. Two years later, Connor is still doing well.
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Prenatal diagnosis is extremely important in infants who have HLHS. A study published in the June 2001 issue of Pediatrics reported that "infants diagnosed prenatally were half as likely to experience seizure or coma prior to or within six weeks of their [corrective] surgery." Treatment options which should be considered include medical termination of pregnancy, supportive care, Norwood surgical procedure, and cardiac transplantation. Medical termination of pregnancy is a viable alternative to uncertain and expensive surgeries when HLHS is detected early enough in pregnancy. Supportive, or pallative care, occurs when parents refuse surgical treatment on their newborn. Most infants receiving pallative care experience cardiovascular collapse and die within a few days. However, the more common treatment option is the Norwood procedure (Claxon-McKinney 2001). The early diagnosis of HLHS typically leads to the infant being delivered in a hospital with an "advanced, high-risk nursery" which can administer the drug prostaglandin immediately. This drug encourages blood flow maintenance until the Norwood reconstructive surgery occurs ("Prenatal Detection" 2001).

Although infants may be out of immediate danger after reconstructive surgery, children who had HLHS remain at a higher-than-normal risk for long-term cognitive and neurological problems. Prenatal diagnosis also helps lower this long-term risk ("Prenatal Detection" 2001).