Left-Ventricular Noncompaction in a Pediatric Population: Predictors of Survival

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 o...

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Veröffentlicht in:Pediatric cardiology 2011-04, Vol.32 (4), p.406-412
Hauptverfasser: Zuckerman, Warren A., Richmond, Marc E., Singh, Rakesh K., Carroll, Sheila J., Starc, Thomas J., Addonizio, Linda J.
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container_end_page 412
container_issue 4
container_start_page 406
container_title Pediatric cardiology
container_volume 32
creator Zuckerman, Warren A.
Richmond, Marc E.
Singh, Rakesh K.
Carroll, Sheila J.
Starc, Thomas J.
Addonizio, Linda J.
description 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 
doi_str_mv 10.1007/s00246-010-9868-5
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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 &lt; 1 year of age. Death or transplantation occurred in 26 patients, with a median survival of 1.17 years after presentation. Patients surviving 1 year after presentation had 75% conditional survival, and patients surviving 2 years 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 0.08 years 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. 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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.</description><subject>Cardiac Surgery</subject><subject>Cardiology</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Children</subject><subject>Echocardiography - methods</subject><subject>Female</subject><subject>Follow-Up Studies</subject><subject>Health aspects</subject><subject>Heart</subject><subject>Humans</subject><subject>Infant</subject><subject>Infant, Newborn</subject><subject>Isolated Noncompaction of the Ventricular Myocardium - diagnostic imaging</subject><subject>Isolated Noncompaction of the Ventricular Myocardium - mortality</subject><subject>Isolated Noncompaction of the Ventricular Myocardium - physiopathology</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine &amp; Public Health</subject><subject>New York - epidemiology</subject><subject>Original Article</subject><subject>Pediatrics</subject><subject>Predictive Value of Tests</subject><subject>Prognosis</subject><subject>Retrospective Studies</subject><subject>Risk Assessment - methods</subject><subject>Survival Rate - trends</subject><subject>Transplantation</subject><subject>Vascular Surgery</subject><subject>Ventricular Function, Left - physiology</subject><subject>Young Adult</subject><issn>0172-0643</issn><issn>1432-1971</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2011</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kc1q3TAQRkVpaG7SPkA3xdBFV0pHsmTJ3YXQJIVLe6E_WyHLo6BgS7eSHejbx8ZpoVC6Gpg53zDMIeQ1gwsGoN4XAC4aCgxoqxtN5TOyY6LmlLWKPSc7YIpTaER9Ss5KuQcADVq-IKecMa1rBTty2KOf6A-MUw5uHmyuPqfo0ni0bgopViFWtjpgH-wKVId0XKB18qE65KXtppRLlXz1dc4P4cEOL8mJt0PBV0_1nHy__vjt6pbuv9x8urrcUydUM1HOldJK9oKJHlrEBlslvHO2R6ZqdMp1tdK67RuhOwTVSFV3jeiYA-alV_U5ebftPeb0c8YymTEUh8NgI6a5GC01120rVvLtRt7ZAU2IPk3ZupU2l4pJyeuWyYW6-Adl14PG4FJEH5b-XwG2BVxOpWT05pjDaPMvw8Csesymxyx6zKrHrJk3T0fP3Yj9n8RvHwvAN6Aso3iH2dynOcflkf_Z-gijYJkU</recordid><startdate>20110401</startdate><enddate>20110401</enddate><creator>Zuckerman, Warren A.</creator><creator>Richmond, Marc E.</creator><creator>Singh, Rakesh K.</creator><creator>Carroll, Sheila J.</creator><creator>Starc, Thomas J.</creator><creator>Addonizio, Linda J.</creator><general>Springer-Verlag</general><general>Springer</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20110401</creationdate><title>Left-Ventricular Noncompaction in a Pediatric Population: Predictors of Survival</title><author>Zuckerman, Warren A. ; 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subjects Cardiac Surgery
Cardiology
Child
Child, Preschool
Children
Echocardiography - methods
Female
Follow-Up Studies
Health aspects
Heart
Humans
Infant
Infant, Newborn
Isolated Noncompaction of the Ventricular Myocardium - diagnostic imaging
Isolated Noncompaction of the Ventricular Myocardium - mortality
Isolated Noncompaction of the Ventricular Myocardium - physiopathology
Male
Medicine
Medicine & Public Health
New York - epidemiology
Original Article
Pediatrics
Predictive Value of Tests
Prognosis
Retrospective Studies
Risk Assessment - methods
Survival Rate - trends
Transplantation
Vascular Surgery
Ventricular Function, Left - physiology
Young Adult
title Left-Ventricular Noncompaction in a Pediatric Population: Predictors of Survival
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