RESUMEN
Left ventricle non-compaction cardiomyopathy is currently considered as a well-defined individual entity. However, it includes a broad spectrum of clinical, radiological and pathophysiological findings. In this review we describe 3 different scenarios of this entity: an isolated case with severe left ventricle dysfunction, an "associated" case in a patient with previous atrial septum defect and pulmonary stenosis and finally, as a finding in a patient with a transient cerebrovascular ischemic attack. In the 2 last cases, both asymptomatic, morphological criteria of left ventricle non-compaction were found but, ventricular function was normal and cardiac-MRI showed no late gadolinium hyperenhancement. Periodical follow-up and familial screening were recommended. Natural history and prognosis factors of this disease are still not well known. Further and longer series of patients with this diagnosis are needed to completely define radiological criteria, clinical presentation and evolution.
La miocardiopatía no compactada está considerada actualmente como una entidad independiente y bien definida. Sin embargo, presenta un espectro amplio de hallazgos clínicos, radiológicos y fisiopatológicos. En la presente revisión describimos 3 escenarios clínicos diferentes de dicha entidad: un caso con disfunción ventricular severa, un caso como entidad «asociada¼ a una cardiopatía congènita en un pacientes con un defecto del septo interauricular previo y estenosis pulmonar, y finalmente, como un hallazgo casual en un paciente con un accidente cerebrovascular transitorio. En estos 2 últimos casos se encontraron criterios morfológicos de miocardiopatía no compactada con función ventricular normal y sin presencia de realce tardío de gadolinio en el estudio de cardio-RM. En todos ellos se recomendó estudio familiar. La historia natural y el pronóstico de esta anatomía patológica no son todavía del todo conocidos. Series mayores y seguimiento más largos son necesarios para definir completamente los criterios radiológicos, la presentación clínica y la evolución de esta fascinante entidad.
Asunto(s)
Adulto , Femenino , Humanos , Masculino , Adulto Joven , Técnicas de Imagen Cardíaca , No Compactación Aislada del Miocardio Ventricular/diagnóstico , Imagen por Resonancia MagnéticaRESUMEN
Left ventricle non-compaction cardiomyopathy is currently considered as a well-defined individual entity. However, it includes a broad spectrum of clinical, radiological and pathophysiological findings. In this review we describe 3 different scenarios of this entity: an isolated case with severe left ventricle dysfunction, an "associated" case in a patient with previous atrial septum defect and pulmonary stenosis and finally, as a finding in a patient with a transient cerebrovascular ischemic attack. In the 2 last cases, both asymptomatic, morphological criteria of left ventricle non-compaction were found but, ventricular function was normal and cardiac-MRI showed no late gadolinium hyperenhancement. Periodical follow-up and familial screening were recommended. Natural history and prognosis factors of this disease are still not well known. Further and longer series of patients with this diagnosis are needed to completely define radiological criteria, clinical presentation and evolution.