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        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">El miocardio ventricular no compacto aislado es una entidad muy rara caracterizada por una alteraci&#243;n en la morfog&#233;nesis endomioc&#225;rdica con presencia de numerosas trabeculaciones mioc&#225;rdicas probablemente debida a una detenci&#243;n de la embriog&#233;nesis endomioc&#225;rdica normal&#46; Se presentan 2 casos de miocardio ventricular no compacto aislado&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El caso 1 tuvo un hermano fallecido en el primer a&#241;o de vida tras un trasplante card&#237;aco por insuficiencia card&#237;aca refractaria debida a una miocardiopat&#237;a restrictiva biventricular asociada a divert&#237;culo ventricular derecho&#46; Pudo tratarse de un caso de miocardio no compacto biventricular no diagnosticado&#46; Su hermano&#44; de 8 a&#241;os de edad&#44; est&#225; asintom&#225;tico y presenta un ecocardiograma caracter&#237;stico con numerosas trabeculaciones en &#225;pex de ventr&#237;culo izquierdo&#44; ligera depresi&#243;n de la funci&#243;n ventricular y patr&#243;n de llenado mitral restrictivo&#46;</p><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">El caso 2 es un ni&#241;o de 7 a&#241;os de edad remitido por episodios sincopales de repetici&#243;n&#46; Presenta un ecocardiograma con im&#225;genes endomioc&#225;rdicas caracter&#237;sticas junto con patr&#243;n de llenado mitral restrictivo y funci&#243;n ventricular conservada&#46; Present&#243; en el estudio Holter extrasistolia ventricular frecuente y rachas de taquicardia ventricular no sostenida&#44; por lo que se aplic&#243; un Holter implantable que mostr&#243; asociado a un episodio de muerte s&#250;bita abortada taquicardia ventricular polimorfa seguida de asistolia&#46; Se le ha implantado un desfibrilador&#46;</p>"
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        "resumen" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Isolated non-compaction of the ventricular myocardium is a rare disorder of endomyocardial morphogenesis characterized by numerous&#44; excessively prominent ventricular trabeculations&#44; probably due to arrest of normal endomyocardial embryogenesis&#46; We report two cases of isolated non-compaction of the ventricular myocardium&#46;</p><p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">The first patient had a brother who died in the first year of life after heart transplantation due to refractory heart failure caused by restrictive biventricular cardiomyopathy associated with a right ventricular diverticulum&#46; This could have been a case of isolated non-compaction of the ventricular myocardium that was not diagnosed&#46; The patient&#44; who is 8 years old&#44; is asymptomatic and echocardiogram &#40;ECG&#41; showed multiple prominent ventricular trabeculations in the apical portion of the left ventricle&#44; slightly depressed systolic and diastolic function and restrictive ventricular filling pattern&#46;</p><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">The second patient is a 7-year-old boy who presents recurrent syncopes&#46; ECG showed multiple prominent ventricular trabeculations&#44; restrictive ventricular filling pattern and normal systolic function&#46; The Holter ECG recording showed multiple premature ventricular complexes and nonsustained ventricular tachycardia&#46; An implantable subcutaneous Holter recorder was inserted&#44; which was associated with an episode of aborted sudden death and polymorphic ventricular tachycardia followed by asystole&#46; The patient was then treated with an implantable defibrillator&#46;</p>"
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Vol. 57. Núm. 6.
Páginas 570-573 (diciembre 2002)
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Vol. 57. Núm. 6.
Páginas 570-573 (diciembre 2002)
Acceso a texto completo
Miocardio ventricular no compacto aislado
Isolated non-compaction of the ventricular myocardium
Visitas
11045
J.J. Salazar González
Autor para correspondencia
jjsalazar@comz.org

Correspondencia: Francisco Vitoria, 24, esc. dcha., 3.° A. 50008 Zaragoza. España.
, S. Rite Montañés, A. Asso Abadía, E. Pueo Crespo, E. Salazar González, L.J. Placer Peralta
Servicios de Cardiología y Pediatría. Hospital Universitario Miguel Servet. Zaragoza. España
Este artículo ha recibido
Información del artículo

El miocardio ventricular no compacto aislado es una entidad muy rara caracterizada por una alteración en la morfogénesis endomiocárdica con presencia de numerosas trabeculaciones miocárdicas probablemente debida a una detención de la embriogénesis endomiocárdica normal. Se presentan 2 casos de miocardio ventricular no compacto aislado.

El caso 1 tuvo un hermano fallecido en el primer año de vida tras un trasplante cardíaco por insuficiencia cardíaca refractaria debida a una miocardiopatía restrictiva biventricular asociada a divertículo ventricular derecho. Pudo tratarse de un caso de miocardio no compacto biventricular no diagnosticado. Su hermano, de 8 años de edad, está asintomático y presenta un ecocardiograma característico con numerosas trabeculaciones en ápex de ventrículo izquierdo, ligera depresión de la función ventricular y patrón de llenado mitral restrictivo.

El caso 2 es un niño de 7 años de edad remitido por episodios sincopales de repetición. Presenta un ecocardiograma con imágenes endomiocárdicas características junto con patrón de llenado mitral restrictivo y función ventricular conservada. Presentó en el estudio Holter extrasistolia ventricular frecuente y rachas de taquicardia ventricular no sostenida, por lo que se aplicó un Holter implantable que mostró asociado a un episodio de muerte súbita abortada taquicardia ventricular polimorfa seguida de asistolia. Se le ha implantado un desfibrilador.

Palabras clave:
Miocardio ventricular no compacto aislado
Taquicardia ventricular
Miocardiopatías
Síncope

Isolated non-compaction of the ventricular myocardium is a rare disorder of endomyocardial morphogenesis characterized by numerous, excessively prominent ventricular trabeculations, probably due to arrest of normal endomyocardial embryogenesis. We report two cases of isolated non-compaction of the ventricular myocardium.

The first patient had a brother who died in the first year of life after heart transplantation due to refractory heart failure caused by restrictive biventricular cardiomyopathy associated with a right ventricular diverticulum. This could have been a case of isolated non-compaction of the ventricular myocardium that was not diagnosed. The patient, who is 8 years old, is asymptomatic and echocardiogram (ECG) showed multiple prominent ventricular trabeculations in the apical portion of the left ventricle, slightly depressed systolic and diastolic function and restrictive ventricular filling pattern.

The second patient is a 7-year-old boy who presents recurrent syncopes. ECG showed multiple prominent ventricular trabeculations, restrictive ventricular filling pattern and normal systolic function. The Holter ECG recording showed multiple premature ventricular complexes and nonsustained ventricular tachycardia. An implantable subcutaneous Holter recorder was inserted, which was associated with an episode of aborted sudden death and polymorphic ventricular tachycardia followed by asystole. The patient was then treated with an implantable defibrillator.

Key words:
Isolated non-compaction of the ventricular myocardium
Ventricular tachycardia
Myocardial diseases
Syncope
El Texto completo está disponible en PDF
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Copyright © 2002. Asociación Española de Pediatría
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