Orthotopic heart transplantation in two infants with histiocytoid cardiomyopathy and left ventricular non-compaction.

ABSTRACT

HC is a rare cause of congestive heart failure that typically presents with malignant ventricular arrhythmias in infants, often requiring urgent intervention. Successful heart transplantation in a patient with HC has only been reported once (J Heart Lung Transplant 2004 23 902). The combination of HC with concurrent LVNC has only been described three times (Int J Legal Med 2009 123 47; Hum Pathol 2005 36 403; Pediatr Dev Pathol 2012 15 397). We report two rare cases of HC with LVNC in two infants presenting with cardiogenic shock, one requiring ECMO support who was successfully bridged to orthotopic heart transplantation with a Berlin Heart LVAD.