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.
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.