Miocardiopatía no compactada: una serie de 15 casos

Background: Non compaction cardiomyopathy is a rare disorder caused by the arrest of myocardial compaction during embryogenesis, leading to a non compacted endocardial layer with marked hypertrabeculation and deep recesses. Aim: To report the clinical and echocardiographic characteristics of a serie...

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Autores principales:	Enríquez R,Andrés, Baeza V,Ricardo, Gabrielli N,Luigi, Córdova A,Samuel, Castro G,Pablo
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2011
Materias:	Cardiomyopathies Heart failure Isolated non compaction of the ventricular myocardium
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872011000700006
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spelling	oai:scielo:S0034-988720110007000062011-09-16Miocardiopatía no compactada: una serie de 15 casosEnríquez R,AndrésBaeza V,RicardoGabrielli N,LuigiCórdova A,SamuelCastro G,Pablo Cardiomyopathies Heart failure Isolated non compaction of the ventricular myocardium Background: Non compaction cardiomyopathy is a rare disorder caused by the arrest of myocardial compaction during embryogenesis, leading to a non compacted endocardial layer with marked hypertrabeculation and deep recesses. Aim: To report the clinical and echocardiographic characteristics of a series of 15 adult patients with non-compaction cardiomyopathy. Patients and Methods: We included a total of 15 patients aged 52 ± 17 years (40% males) diagnosed at our echocardiography laboratory between January 2001 and July 2010. Results: Theform of presentation was heart failure in 53% of subjects, syncope in 20%o, ventricular arrhythmias in 13%o and stroke in 7%>. Left ventricular end-diastolic diameter was 66 ±11 mm and estimated ejection fraction was 27 ± 10%>. Apical and/or mid-ventricular segments of the left ventricle were involved in all the cases. Pulmonary hypertension was present in 40%o. The average follow-up was 19 months and no patient died during this period. Sixty seven percent ofthe patients had manifestations of heart failure, 27%o presented sustained ventricular arrhythmias and 20%> had atrial fibrillation orflutter, whereas 13%o had cerebral embolic events. An automated internal cardioverter defibrillator was implanted in 47%o of patients. Conclusions: Non-compaction cardiomyopathy is associated with high cardiovascular morbidity. The diagnosis is made in advanced stages of the disease, with significant dilation and ventricular dysfunction.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.139 n.7 20112011-07-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872011000700006es10.4067/S0034-98872011000700006
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Cardiomyopathies Heart failure Isolated non compaction of the ventricular myocardium
spellingShingle	Cardiomyopathies Heart failure Isolated non compaction of the ventricular myocardium Enríquez R,Andrés Baeza V,Ricardo Gabrielli N,Luigi Córdova A,Samuel Castro G,Pablo Miocardiopatía no compactada: una serie de 15 casos
description	Background: Non compaction cardiomyopathy is a rare disorder caused by the arrest of myocardial compaction during embryogenesis, leading to a non compacted endocardial layer with marked hypertrabeculation and deep recesses. Aim: To report the clinical and echocardiographic characteristics of a series of 15 adult patients with non-compaction cardiomyopathy. Patients and Methods: We included a total of 15 patients aged 52 ± 17 years (40% males) diagnosed at our echocardiography laboratory between January 2001 and July 2010. Results: Theform of presentation was heart failure in 53% of subjects, syncope in 20%o, ventricular arrhythmias in 13%o and stroke in 7%>. Left ventricular end-diastolic diameter was 66 ±11 mm and estimated ejection fraction was 27 ± 10%>. Apical and/or mid-ventricular segments of the left ventricle were involved in all the cases. Pulmonary hypertension was present in 40%o. The average follow-up was 19 months and no patient died during this period. Sixty seven percent ofthe patients had manifestations of heart failure, 27%o presented sustained ventricular arrhythmias and 20%> had atrial fibrillation orflutter, whereas 13%o had cerebral embolic events. An automated internal cardioverter defibrillator was implanted in 47%o of patients. Conclusions: Non-compaction cardiomyopathy is associated with high cardiovascular morbidity. The diagnosis is made in advanced stages of the disease, with significant dilation and ventricular dysfunction.
author	Enríquez R,Andrés Baeza V,Ricardo Gabrielli N,Luigi Córdova A,Samuel Castro G,Pablo
author_facet	Enríquez R,Andrés Baeza V,Ricardo Gabrielli N,Luigi Córdova A,Samuel Castro G,Pablo
author_sort	Enríquez R,Andrés
title	Miocardiopatía no compactada: una serie de 15 casos
title_short	Miocardiopatía no compactada: una serie de 15 casos
title_full	Miocardiopatía no compactada: una serie de 15 casos
title_fullStr	Miocardiopatía no compactada: una serie de 15 casos
title_full_unstemmed	Miocardiopatía no compactada: una serie de 15 casos
title_sort	miocardiopatía no compactada: una serie de 15 casos
publisher	Sociedad Médica de Santiago
publishDate	2011
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872011000700006
work_keys_str_mv	AT enriquezrandres miocardiopatianocompactadaunaseriede15casos AT baezavricardo miocardiopatianocompactadaunaseriede15casos AT gabriellinluigi miocardiopatianocompactadaunaseriede15casos AT cordovaasamuel miocardiopatianocompactadaunaseriede15casos AT castrogpablo miocardiopatianocompactadaunaseriede15casos
_version_	1718436575222693888

Miocardiopatía no compactada: una serie de 15 casos

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