Current issues and perspectives in hypoplasia of the left heart.
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.
Sedmera
David
D
Department of Cell Biology and Anatomy, Medical University of South Carolina, Charleston, South Carolina 29425, USA. sedmerad@musc.edu
Cook
Andrew C
AC
Shirali
Girish
G
McQuinn
Tim C
TC
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