caa a22 4500 388116323 CHVBK 20180307125407.0 cr unu---uuuuu 161130e199911 xx s 000 0 eng 10.1017/S1047951100005722 doi S1047951100005722 pii (NATIONALLICENCE)cambridge-10.1017/S1047951100005722 Grossfeld Paul D. Division of Pediatric Cardiology and Department of Medicine, University of California, San Diego, USA The genetics of hypoplastic left heart syndrome [Elektronische Daten] [Paul D. Grossfeld] Hypoplastic left heart syndrome is one of the most therapeutically challenging congenital cardiac defects. It accounts for as many as 1.5% of all congenital heart defects, but is responsible for up to one quarter of deaths in neonates with heart disease.1 The management of hypoplastic left heart syndrome is controversial. Two surgical options exist:2,3 the Norwood procedure, is a three stage repair in which the morphologically right ventricle is converted to function as the systemic ventricle. Alternatively, orthotopic transplantation can be performed. Although both surgical options have had improved outcomes, the prognosis for long-term survival is guarded, with a five year survival for either approach reported to be in the region of 50-60%. In this review, I explore the evidence for a genetic etiology for the "classic” hypoplastic left heart syndrome, defined as mitral and/or aortic atresia with hypoplasia of the left ventricular cavity and the other left-sided structures. Copyright © Cambridge University Press 1999 Cardiology in the Young Cambridge University Press 9/6(1999-11), 627-632 1047-9511 9:6<627 1999 9 CTY https://doi.org/10.1017/S1047951100005722 text/html Onlinezugriff via DOI 1 review-article jats NATIONALLICENCE 856 40 https://doi.org/10.1017/S1047951100005722 text/html Onlinezugriff via DOI NATIONALLICENCE 100 1- Grossfeld Paul D. Division of Pediatric Cardiology and Department of Medicine, University of California, San Diego, USA NATIONALLICENCE 773 0- Cardiology in the Young Cambridge University Press 9/6(1999-11), 627-632 1047-9511 9:6<627 1999 9 CTY CC0 http://creativecommons.org/publicdomain/zero/1.0 nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-cambridge