hypoplastic left heart syndrome


hypoplastic left heart syndrome

 congenital hypoplasia or atresia of the ventricle" >left ventricle, the aortic or valve" >mitral valve, and the ascending aorta" >aorta, with respiratory distress, cardiac failure, and death in infancy.

hy·po·plas·tic left heart syn·drome

[MIM*241550] association of underdevelopment of the left heart chambers with atresia or stenosis of the aortic and/or mitral valve and hypoplasia of the ascending aorta.

hypoplastic left heart syndrome

Pediatric cardiology A group of congenital often AR cardiac defects characterized by hypo- or agenesis of the left ventricle, aortic and mitral valves, an atrial right-to-left shunt; right-sided hypoplasia of tricuspid or pulmonary valves, pulmonary stenosis, or right ventricular hypoplasia; the ascending and transverse aorta is narrowed with a diaphragm-like aortic coarctation at the preductal aortic isthmus; postnatal life hinges on adequate blood supply–ie, is ductus dependent, unrestricted atrial shunting, and a balance between the pulmonary and systemic vascular resistances; surgery is difficult as there are extensive malformations Mortality High. See Baby Fae heart.