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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.
AuthorsNurcan Arat, Murat Akyıldız, Gürkan Tellioğlu, Yaman Tokat
JournalTurk Kardiyoloji Dernegi arsivi : Turk Kardiyoloji Derneginin yayin organidir (Turk Kardiyol Dern Ars) Vol. 43 Issue 3 Pg. 288-91 (Apr 2015) ISSN: 1016-5169 [Print] Turkey
PMID25906003 (Publication Type: Case Reports, Journal Article)
Topics
  • Adult
  • Cardiomyopathies (diagnostic imaging, physiopathology)
  • Ductus Arteriosus, Patent (diagnostic imaging, physiopathology)
  • Echocardiography
  • Humans
  • Hyperoxaluria, Primary (diagnostic imaging, physiopathology)
  • Male
  • Young Adult

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