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 |
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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 |
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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 < 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. Therefore, early listing for transplant may lead to better outcomes in this population.</description><identifier>ISSN: 0172-0643</identifier><identifier>EISSN: 1432-1971</identifier><identifier>DOI: 10.1007/s00246-010-9868-5</identifier><identifier>PMID: 21188370</identifier><language>eng</language><publisher>New York: Springer-Verlag</publisher><subject>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</subject><ispartof>Pediatric cardiology, 2011-04, Vol.32 (4), p.406-412</ispartof><rights>Springer Science+Business Media, LLC 2010</rights><rights>COPYRIGHT 2011 Springer</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c476t-2277875d414d09ee6e974fccade173ec7cb37889d648be076573b64b1c01f5f73</citedby><cites>FETCH-LOGICAL-c476t-2277875d414d09ee6e974fccade173ec7cb37889d648be076573b64b1c01f5f73</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s00246-010-9868-5$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s00246-010-9868-5$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,776,780,27901,27902,41464,42533,51294</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/21188370$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Zuckerman, Warren A.</creatorcontrib><creatorcontrib>Richmond, Marc E.</creatorcontrib><creatorcontrib>Singh, Rakesh K.</creatorcontrib><creatorcontrib>Carroll, Sheila J.</creatorcontrib><creatorcontrib>Starc, Thomas J.</creatorcontrib><creatorcontrib>Addonizio, Linda J.</creatorcontrib><title>Left-Ventricular Noncompaction in a Pediatric Population: Predictors of Survival</title><title>Pediatric cardiology</title><addtitle>Pediatr Cardiol</addtitle><addtitle>Pediatr Cardiol</addtitle><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 < 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. 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 & 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. ; Richmond, Marc E. ; Singh, Rakesh K. ; Carroll, Sheila J. ; Starc, Thomas J. ; Addonizio, Linda J.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c476t-2277875d414d09ee6e974fccade173ec7cb37889d648be076573b64b1c01f5f73</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2011</creationdate><topic>Cardiac Surgery</topic><topic>Cardiology</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Children</topic><topic>Echocardiography - methods</topic><topic>Female</topic><topic>Follow-Up Studies</topic><topic>Health aspects</topic><topic>Heart</topic><topic>Humans</topic><topic>Infant</topic><topic>Infant, Newborn</topic><topic>Isolated Noncompaction of the Ventricular Myocardium - diagnostic imaging</topic><topic>Isolated Noncompaction of the Ventricular Myocardium - mortality</topic><topic>Isolated Noncompaction of the Ventricular Myocardium - physiopathology</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>New York - epidemiology</topic><topic>Original Article</topic><topic>Pediatrics</topic><topic>Predictive Value of Tests</topic><topic>Prognosis</topic><topic>Retrospective Studies</topic><topic>Risk Assessment - methods</topic><topic>Survival Rate - trends</topic><topic>Transplantation</topic><topic>Vascular Surgery</topic><topic>Ventricular Function, Left - physiology</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Zuckerman, Warren A.</creatorcontrib><creatorcontrib>Richmond, Marc E.</creatorcontrib><creatorcontrib>Singh, Rakesh K.</creatorcontrib><creatorcontrib>Carroll, Sheila J.</creatorcontrib><creatorcontrib>Starc, Thomas J.</creatorcontrib><creatorcontrib>Addonizio, Linda J.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatric cardiology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Zuckerman, Warren A.</au><au>Richmond, Marc E.</au><au>Singh, Rakesh K.</au><au>Carroll, Sheila J.</au><au>Starc, Thomas J.</au><au>Addonizio, Linda J.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Left-Ventricular Noncompaction in a Pediatric Population: Predictors of Survival</atitle><jtitle>Pediatric cardiology</jtitle><stitle>Pediatr Cardiol</stitle><addtitle>Pediatr Cardiol</addtitle><date>2011-04-01</date><risdate>2011</risdate><volume>32</volume><issue>4</issue><spage>406</spage><epage>412</epage><pages>406-412</pages><issn>0172-0643</issn><eissn>1432-1971</eissn><abstract>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 < 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. Therefore, early listing for transplant may lead to better outcomes in this population.</abstract><cop>New York</cop><pub>Springer-Verlag</pub><pmid>21188370</pmid><doi>10.1007/s00246-010-9868-5</doi><tpages>7</tpages></addata></record> |
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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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