End-stage cardiac failure caused by isolated ventricular non-compaction: cardiac transplantation in a 5-year-old boy.
Isolated ventricular non-compaction (IVNC) is a rare congenital cardiomyopathy characterized by arrest in endomyocardial morphogenesis. Presenting features in children include systolic and diastolic ventricular dysfunction, cardiac arrhythmias, and thromboembolism. End-stage cardiac failure necessitating cardiac transplantation is described in adults. We report the unique case of a 5-year-old boy with IVNC and end-stage heart failure who underwent cardiac transplantation. Diagnostic echocardiographic features, gross cardiac morphology, and histopathologic findings are illustrated.
Vogel
Melanie
M
Department of Paediatric Cardiology Freeman Hospital, Newcastle upon Tyne, United Kingdom.
Chaudhari
Milind
M
Rassl
Doris
D
Mair
Lara
L
Hasan
Asif
A
D009202:Cardiomyopathies / Q000151:congenital* / Q000601:surgery*; D002675:Child, Preschool; D004699:Endocardium / Q000002:abnormalities / Q000196:embryology; D006321:Heart / Q000196:embryology; D006330:Heart Defects, Congenital / Q000601:surgery*; D006333:Heart Failure / Q000209:etiology / Q000601:surgery; D016027:Heart Transplantation; D006801:Humans; D008297:Male; D009206:Myocardium