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Hypoplastic Left Heart Syndrome and Mitral Atresia
Introduction
Hypoplastic left heart syndrome (HLHS) occurs in 0.1 : 1000 to 0.6 : 1000 live births and accounts for 2% to 3% of all congenital heart diseases (CHDs). HLHS is one of the most severe CHDs, with a high rate of perinatal death. Without treatment, it is responsible for 25% to 40% of all neonatal cardiac deaths.
Disease
Definition
HLHS encompasses a spectrum of congenital heart malformations characterized by underdevelopment of the left heart structures.
Prevalence and Epidemiology
HLHS represents 2% to 3% of all CHDs. Because it is usually easily diagnosed on a four-chamber view, widespread introduction of ultrasound (US) screening significantly improved the detection rate. HLHS is associated with a poor prognosis and an intrauterine mortality rate of about 5%. Males are more frequently affected than females. There is a 2% to 4% recurrence risk. Autosomal recessive inheritance has occasionally been described.
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