Hypoplastic left heart syndrome

Hypoplastic left heart syndrome

Hypoplastic left heart syndrome is one of the serious congenital cardiac malformations. They have varying degrees of hypoplasia of the left heart at multiple levels, which could include a hypoplastic left ventricle, aortic atresia and a hypoplastic ascending aorta. They go in for cardiogenic shock when the ductus closes as it is a ductus dependent systemic circulation. Immediate measure for salvaging those babies is to start an intravenous infusion of prostaglandin E1 (PGE1). Surgical treatment could be in the form of an initial Norwood procedure followed by a bidirectional Glenn shunt (end to side anastomosis of superior vena cava to the right pulmonary artery) at six months and a Fontan procedure at about two years of age.