User:Mr. Ibrahem/Hypoplastic left heart syndrome

Hypoplastic left heart syndrome (HLHS) is a congenital heart defect in which the left side of the heart is severely underdeveloped. It may affect the left ventricle, aorta, aortic valve, and mitral valve. While the baby may appear normal in the first few days of life, they than rapidly develop trouble breathing. Other symptoms may include bluish skin with oxygen saturations between 75 and 85%.

The cause is generally unknown. It is associated with a number of genetic syndromes including Turner, DiGeorge, and Down. Diagnosis is generally by prenatal ultrasound.

Initial treatment is with prostaglandin E1 or comfort care. A procedure to make a hole in the atrial septum may also be required. This may be followed by a set of three surgeries. ECMO may be used to bridge the baby until surgery. Without treatment children die within the first week of life. With treatment about 66% live to at least 5 years of age.

HLHS is rare, affecting about 1 in 5,000 newborns. It makes up about 3% of cases of congenital heart disease. It was first described in 1952 by Lev. Among cases diagnosed before birth 12% to 48% of parents elect to abort the pregnancy.