caa a22 4500 445824972 CHVBK 20180317145256.0 cr unu---uuuuu 170323e20110401xx s 000 0 eng 10.1007/s00246-010-9868-5 doi (NATIONALLICENCE)springer-10.1007/s00246-010-9868-5 Left-Ventricular Noncompaction in a Pediatric Population: Predictors of Survival [Elektronische Daten] [Warren Zuckerman, Marc Richmond, Rakesh Singh, Sheila Carroll, Thomas Starc, Linda Addonizio] Left-ventricular noncompaction (LVNC) is an echocardiographic finding of increasing frequency in pediatrics; however, predictors of outcomes have been difficult to identify. We conducted a retrospective review of pediatric patients at the Morgan Stanley Children's Hospital of New York from January of 1993 to September of 2009 to identify predictors of the primary outcome of death or heart transplantation. LVNC was identified in 50 patients, 34 of them<1year of age. Death or transplantation occurred in 26 patients, with a median survival of 1.17years after presentation. Patients surviving 1year after presentation had 75% conditional survival, and patients surviving 2years after presentation had 92% conditional survival. Hemodynamic instability, poor ventricular function, and LV dilatation were each independent predictors of poor outcome. Of the 21 patients who presented with hemodynamic instability, 17 died or underwent transplantation at a median of 0.08years after presentation. In conclusion, LVNC is recognized more in younger patients; however, age is not a predictor of outcome. Patients who present with hemodynamic instability and poor ventricular function have decreased transplant-free survival, and most poor outcomes occur within the first year after presentation. Therefore, early listing for transplant may lead to better outcomes in this population. Springer Science+Business Media, LLC, 2010 Cardiomyopathy nationallicence Cardiac function nationallicence Heart failure nationallicence Echocardiography nationallicence Pediatric nationallicence ASD : Atrial septal defect nationallicence BSA : Body surface area nationallicence CHD : Congenital heart disease nationallicence CI : Confidence interval nationallicence FS : Fractional shortening nationallicence HR : Hazard ratio nationallicence LV : Left ventricle nationallicence LVEDd : Left ventricular end-diastolic diameter nationallicence LVNC : Left ventricular noncompaction nationallicence Met/Gen : Associated metabolic and/or genetic syndrome nationallicence MS-CHONY : Morgan Stanley Children's Hospital of New York nationallicence NC:Cmax : Maximum ratio of noncompacted to compacted myocardium nationallicence OHT : Orthotopic heart transplantation nationallicence VSD : Ventricular septal defect nationallicence Zuckerman Warren Division of Pediatric Cardiology, Columbia University Medical Center, 10032, New York, NY, USA aut Richmond Marc Division of Pediatric Cardiology, Columbia University Medical Center, 10032, New York, NY, USA aut Singh Rakesh Division of Pediatric Cardiology, Columbia University Medical Center, 10032, New York, NY, USA aut Carroll Sheila Division of Pediatric Cardiology, Cornell University Medical Center, 10065, New York, NY, USA aut Starc Thomas Division of Pediatric Cardiology, Columbia University Medical Center, 10032, New York, NY, USA aut Addonizio Linda Division of Pediatric Cardiology, Columbia University Medical Center, 10032, New York, NY, USA aut Pediatric Cardiology Springer-Verlag 32/4(2011-04-01), 406-412 0172-0643 32:4<406 2011 32 246 https://doi.org/10.1007/s00246-010-9868-5 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00246-010-9868-5 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Zuckerman Warren Division of Pediatric Cardiology, Columbia University Medical Center, 10032, New York, NY, USA aut NATIONALLICENCE 700 1- Richmond Marc Division of Pediatric Cardiology, Columbia University Medical Center, 10032, New York, NY, USA aut NATIONALLICENCE 700 1- Singh Rakesh Division of Pediatric Cardiology, Columbia University Medical Center, 10032, New York, NY, USA aut NATIONALLICENCE 700 1- Carroll Sheila Division of Pediatric Cardiology, Cornell University Medical Center, 10065, New York, NY, USA aut NATIONALLICENCE 700 1- Starc Thomas Division of Pediatric Cardiology, Columbia University Medical Center, 10032, New York, NY, USA aut NATIONALLICENCE 700 1- Addonizio Linda Division of Pediatric Cardiology, Columbia University Medical Center, 10032, New York, NY, USA aut NATIONALLICENCE 773 0- Pediatric Cardiology Springer-Verlag 32/4(2011-04-01), 406-412 0172-0643 32:4<406 2011 32 246 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer