title: Current issues and perspectives in hypoplasia of the left heart.
abstract: Hypoplastic left heart syndrome is a rare but serious form of congenital cardiac disease, characterized by underdevelopment of the components of the left heart, rendering the left ventricle non-functional. Its aetiology is largely unknown, but there is certainly a genetic component. Prenatal diagnosis nowadays uncovers about half of cases. Postnatal options for treatment include comfort care, 3-stage palliative surgery, or cardiac transplantation. In this review, we discuss the morphology, possible pathogenetic mechanisms, clinical management, and perspectives of prenatal intervention based on work in animal models.
authors.affiliation: Department of Cell Biology and Anatomy, Medical University of South Carolina, Charleston, South Carolina 29425, USA. sedmerad@musc.edu
authors.forename: David
authors.initials: D
authors.lastname: Sedmera
authors.forename: Andrew C
authors.initials: AC
authors.lastname: Cook
authors.forename: Girish
authors.initials: G
authors.lastname: Shirali
authors.forename: Tim C
authors.initials: TC
authors.lastname: McQuinn
mesh_terms: D000818:Animals; D001021:Aortic Valve / Q000473:pathology; D001024:Aortic Valve Stenosis / Q000473:pathology; D003326:Coronary Circulation; D004195:Disease Models, Animal; D015150:Echocardiography, Doppler; D005333:Fetus / Q000601:surgery; D006345:Heart Septal Defects, Ventricular / Q000473:pathology; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D007223:Infant; D008943:Mitral Valve / Q000473:pathology; D011296:Prenatal Diagnosis; D014655:Vascular Resistance