Miocardio no compactado y esferocitosis hereditaria: Un caso clínico / Noncompaction of the Ventricular Myocardium and Hereditary Spherocytosis: A Case Report

RESUMEN

El miocardio no compactado es una entidad de origen congénito que pertenece al grupo de las miocardiopatías no clasificables. Su asociación con la esferocitosis hereditaria se ha publicado en un único caso en el mundo. Se presenta el caso de una paciente con diagnóstico de esferocitosis hereditaria admitida en nuestra unidad coronaria con diagnóstico clínico de insuficiencia cardíaca por miocardiopatía dilatada. El hallazgo de una imagen hiperecogénica y móvil en el ecocardiograma motivó la realización de una resonancia magnética cardiovascular que determinó el diagnóstico de miocardio no compactado. Se derivó a la paciente a un centro de referencia para trasplante cardíaco, que no llegó a concretarse.

ABSTRACT

Noncompaction of the ventricular myocardium is a congenital disorder that has been categorized as an unclassified cardiomyopathy. The association of this congenital cardiomyopathy with hereditary spherocytosis has been published only once. In this case report, a woman with a history of hereditary spherocytosis was admitted to our Coronary Care Unit with heart failure due to dilated cardiomyopathy. The echocardiogram showed a mobile and hyperechogenic image and, subsequently, a cardiovascular magnetic resonance imaging was performed, resulting in a diagnosis of noncompaction of the ventricular myocardium. The patient was transferred to a Tertiary Medical Center for cardiac transplantation, but she died before it could be carried out.