Key result
Heart transplantation successfully rescues infants with histiocytoid cardiomyopathy and non-compaction presenting in cardiogenic shock.
Why the study?
Histiocytoid cardiomyopathy is a rare cause of infant heart failure often presenting with malignant arrhythmias and requiring urgent intervention, with limited reports of successful heart transplantation and rare descriptions of concurrent left ventricular non-compaction.
Case Report (n=2)
Successful OHT in rare infant cases; hypothesis-generating and leaves open long-term outcomes and applicability.
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.
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Siehr et al. (2013) conducted a case report in Histiocytoid cardiomyopathy (HC) and left ventricular non-compaction (LVNC) (n=2). Orthotopic heart transplantation was evaluated. Orthotopic heart transplantation was successfully performed in two infants with histiocytoid cardiomyopathy and left ventricular non-compaction presenting with cardiogenic shock.
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