Ross-Konno and endocardial fibroelastosis resection after hybrid stage I palliation in infancy: successful staged left-ventricular rehabilitation and conversion to biventricular circulation after fetal diagnosis of aortic stenosis.
We report a patient who presented during fetal life with severe aortic stenosis, left-ventricular dysfunction, and endocardial fibroelastosis (evolving hypoplastic left heart syndrome). Management involved in utero and postnatal balloon aortic valvuloplasty for partial relief of obstruction and early postnatal hybrid stage I palliation until recovery of left-ventricular systolic function had occurred. The infant subsequently had successful conversion to a biventricular circulation by combining resection of endocardial fibroelastosis with single-stage Ross-Konno, aortic arch reconstruction, hybrid takedown, and pulmonary artery reconstruction.
Pediatric Heart Center, UCSF Benioff Children's Hospital, University of California San Francisco, San Francisco, CA 94143-0117, USA.
Anita J
AJ
Moon-Grady
Phillip
P
Moore
Anthony
A
Azakie
D001021:Aortic Valve / Q000473:pathology; D001024:Aortic Valve Stenosis / Q000175:diagnosis / Q000628:therapy*; D006348:Cardiac Surgical Procedures; D002404:Catheterization; D018450:Disease Progression; D004695:Endocardial Fibroelastosis / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D006352:Heart Ventricles / Q000473:pathology*; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000175:diagnosis / Q000628:therapy; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D007231:Infant, Newborn; D008297:Male; D011651:Pulmonary Artery / Q000473:pathology; D012720:Severity of Illness Index