Article: Left ventricular noncompaction in children

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TitleLeft ventricular noncompaction in children
AuthorsKoh, C1
Lee, PW1
Yung, TC1
Lun, KS1
Cheung, YF1
Issue Date2009
PublisherBlackwell Publishing, Inc. The Journal's web site is located at http://www.blackwellpublishing.com/chd
CitationCongenital Heart Disease, 2009, v. 4 n. 4, p. 288-294 [How to Cite?]
DOI: http://dx.doi.org/10.1111/j.1747-0803.2009.00269.x
AbstractObjective. Left ventricular noncompaction (LVNC) is an uncommon type of cardiomyopathy in children. We sought to determine the clinical presentations and outcomes of children diagnosed to have LVNC. Design. The case records of children diagnosed to have LVNC between 1999 and 2007 were reviewed. The diagnosis was based on echocardiographic finding of a thick noncompacted myocardium layer characterized by a trabecular meshwork with deep endomyocardial spaces. Results. Ten patients (seven males) were diagnosed to have LVNC at a median age of 2 years (range, 7 days to 12 years). Seven patients had isolated LVNC while three had associated structural congenital heart diseases. The right ventricle was also involved in two patients. Clinical presentations included congestive heart failure in eight patients, asymptomatic heart murmur in one, and syncope with exercise intolerance in one. At presentation, cardiomegaly was found in nine patients, electrocardiographic abnormalities in nine, and impaired LV contraction in six. Eight patients received anti-heart failure medications. Three patients died at a median of 1.5 years after diagnosis, two died suddenly of unknown causes, and one of heart failure. The seven surviving patients were followed up for a median of 2 years (range, 2 months to 3 years). Three patients developed cardiac arrhythmias. The LV function improved in three patients and worsened in one on follow-up. None of the patients developed thromboembolic complications. Conclusions. Left ventricular noncompaction in children is a heterogeneous condition. Long-term follow-up for development of progressive LV dysfunction and cardiac arrhythmias is indicated. © 2009 Copyright the Authors Journal compilation © 2009 Wiley Periodicals, Inc.
ISSN1747-079X
2011 Impact Factor: 0.901
2011 SCImago Journal Rankings: 0.100
DOIhttp://dx.doi.org/10.1111/j.1747-0803.2009.00269.x
ReferencesReferences in Scopus
DC Field
Value
dc.contributor.authorKoh, C
dc.contributor.authorLee, PW
dc.contributor.authorYung, TC
dc.contributor.authorLun, KS
dc.contributor.authorCheung, YF
dc.date.accessioned2012-10-30T06:08:24Z
dc.date.available2012-10-30T06:08:24Z
dc.date.issued2009
dc.description.abstractObjective. Left ventricular noncompaction (LVNC) is an uncommon type of cardiomyopathy in children. We sought to determine the clinical presentations and outcomes of children diagnosed to have LVNC. Design. The case records of children diagnosed to have LVNC between 1999 and 2007 were reviewed. The diagnosis was based on echocardiographic finding of a thick noncompacted myocardium layer characterized by a trabecular meshwork with deep endomyocardial spaces. Results. Ten patients (seven males) were diagnosed to have LVNC at a median age of 2 years (range, 7 days to 12 years). Seven patients had isolated LVNC while three had associated structural congenital heart diseases. The right ventricle was also involved in two patients. Clinical presentations included congestive heart failure in eight patients, asymptomatic heart murmur in one, and syncope with exercise intolerance in one. At presentation, cardiomegaly was found in nine patients, electrocardiographic abnormalities in nine, and impaired LV contraction in six. Eight patients received anti-heart failure medications. Three patients died at a median of 1.5 years after diagnosis, two died suddenly of unknown causes, and one of heart failure. The seven surviving patients were followed up for a median of 2 years (range, 2 months to 3 years). Three patients developed cardiac arrhythmias. The LV function improved in three patients and worsened in one on follow-up. None of the patients developed thromboembolic complications. Conclusions. Left ventricular noncompaction in children is a heterogeneous condition. Long-term follow-up for development of progressive LV dysfunction and cardiac arrhythmias is indicated. © 2009 Copyright the Authors Journal compilation © 2009 Wiley Periodicals, Inc.
dc.description.natureLink_to_subscribed_fulltext
dc.identifier.citationCongenital Heart Disease, 2009, v. 4 n. 4, p. 288-294 [How to Cite?]
DOI: http://dx.doi.org/10.1111/j.1747-0803.2009.00269.x
dc.identifier.citeulike5490354
dc.identifier.doihttp://dx.doi.org/10.1111/j.1747-0803.2009.00269.x
dc.identifier.epage294
dc.identifier.issn1747-079X
2011 Impact Factor: 0.901
2011 SCImago Journal Rankings: 0.100
dc.identifier.issue4
dc.identifier.pmid19664035
dc.identifier.scopuseid_2-s2.0-68349090331
dc.identifier.spage288
dc.identifier.urihttp://hdl.handle.net/10722/170419
dc.identifier.volume4
dc.languageeng
dc.publisherBlackwell Publishing, Inc. The Journal's web site is located at http://www.blackwellpublishing.com/chd
dc.publisher.placeUnited States
dc.relation.ispartofCongenital Heart Disease
dc.relation.referencesReferences in Scopus
dc.subject.meshCardiomyopathies - Therapy - Ultrasonography
dc.subject.meshChild
dc.subject.meshChild, Preschool
dc.subject.meshFatal Outcome
dc.subject.meshFemale
dc.subject.meshHeart Defects, Congenital - Therapy - Ultrasonography
dc.subject.meshHeart Failure - Therapy - Ultrasonography
dc.subject.meshHumans
dc.subject.meshInfant
dc.subject.meshInfant, Newborn
dc.subject.meshMale
dc.subject.meshRetrospective Studies
dc.subject.meshVentricular Dysfunction, Left - Therapy - Ultrasonography
dc.titleLeft ventricular noncompaction in children
dc.typeArticle
Author Affiliations
  1. The University of Hong Kong