title: Cardiac involvement of primary hyperoxaluria accompanied by non-compaction cardiomyopathy and patent ductus arteriosus.
abstract: Primary hyperoxaluria is a rare hereditary metabolic disorder resulting in accumulation of calcium oxalate in visceral organs, including the heart. We report a 19-year-old male with non- compaction cardiomyopathy combined with patent ductus arteriosus awaiting combined liver-kidney transplantation for primary hyperoxaluria. After surgical closure of the patent ductus arteriosus, the patient underwent a successful renal and subsequent liver transplantation. The presence of hypertrophic cardiomyopathy in hyperoxaluria patients has been reported before, but this is the first report of non-compaction myocardium with patent ductus arteriosus in a patient with primary hyperoxaluria. At the third month after combined liver and renal transplantation, improvement in cardiac functions were observed. Primary hyperoxaluria is a clinical entity to be taken into consideration in differential diagnosis of hypertrophied myocardium with high myocardial echocardiographic intensity. In cases of hyperoxaluria, additional congenital abnormalities may complicate the clinical picture.
authors.affiliation: Department of Cardiology, İstanbul Bilim University Faculty of Medicine, İstanbul, Turkey. aratnurcan@gmail.com.
authors.forename: Nurcan
authors.initials: N
authors.lastname: Arat
authors.affiliation: Department of Gastroenterology, İstanbul Bilim University Faculty of Medicine, İstanbul, Turkey.
authors.forename: Murat
authors.initials: M
authors.lastname: Akyıldız
authors.affiliation: Department of Renal Transplantation, Şişli Florence Nightingale Hospital, İstanbul, Turkey.
authors.forename: Gürkan
authors.initials: G
authors.lastname: Tellioğlu
authors.affiliation: Department of Liver Transplantation, Şişli Florence Nightingale Hospital, İstanbul, Turkey.
authors.forename: Yaman
authors.initials: Y
authors.lastname: Tokat
mesh_terms: D000328:Adult; D009202:Cardiomyopathies / Q000000981:diagnostic imaging / Q000503:physiopathology*; D004374:Ductus Arteriosus, Patent / Q000000981:diagnostic imaging / Q000503:physiopathology*; D004452:Echocardiography; D006801:Humans; D006960:Hyperoxaluria, Primary / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008297:Male; D055815:Young Adult