Hypoplastic left heart syndrome natural history, complications and prognosis

Failure to treat hypoplastic left heart syndrome can be fatal for the infant. However, with treatment, infants have a good chance at survival well into late adulthood. Potential complications may develop such as heart failure, arrhythmias, and even neurological complications.

• Irregular, fast heart rhythms (arrhythmias)
• Chronic diarrhea (due to protein loosing enteropathy)
• Heart failure
• Ascites and pleural effusion
• Blockage of the artificial shunt
• Strokes and other neurological complications
• Sudden death

While infants successfully treated for HLHS have a good chance of survival, they may experience chronic health problems for the rest of their lives. The 3-stage surgeries were developed in the early 1980’s with no survivors prior to that time. Therefore, the earliest survivors are in their early 20’s and the long term prognosis is unknown. However, the advances in surgical and medical techniques have helped increase the survival rate dramatically since the surgeries were first developed.

As is true for patients with other types of heart defects involving malformed valves, HLHS patients run a high risk of endocarditis, and must be monitored by a cardiologist for the rest of their lives to check on their heart function.

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