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.

Biventricular Assist Device Support for Intractable Arrhythmias From Histiocytoid Cardiomyopathy

“…There have been three case reports with a total of four patients who received heart transplants for HICMP, and all but one of these patients had severely depressed LV systolic function and advanced heart failure. [1][2][3] One patient, with an left ventricular ejection fraction (LVEF) of 27%, demonstrated multiorgan failure necessitating extracorporeal membrane oxygenation and was ultimately transitioned to Berlin EXCOR (Berlin Heart Inc) LVAD as BTT. However, there have been no prior reports of intractable arrhythmias as an indication for biventricular mechanical support as BTT in this population.…”

Left ventricular non-compaction cardiomyopathy and left ventricular assist device: a word of caution

“…LVNC was found to lead to severe heart failure in a considerable share of those diagnosed with the condition, with 47 % [ 1 ] and 60 % [ 8 ] reported to have either died or undergone transplantation within a mean follow-up period of 44 ± 40 months and within 6 years of diagnosis, respectively. Nevertheless, reports on VAD implantation in patients with LVNC are infrequent, especially when those featuring pediatric patients supported with pulsatile systems [ 9 – 11 ] are left aside (Table 1 ).…”