Key result
A 29-year-old man with primary pleomorphic rhabdomyosarcoma of the heart died 10 days after mitral valve repair, pacemaker insertion, and CABG due to renal and liver failure.
Why the study?
Primary cardiac tumours are rare and difficult to diagnose due to asymptomatic or varied non-specific presentations.
Case Report (n=1)
Primary pleomorphic rhabdomyosarcoma of the heart is a rare, highly fatal tumor that can present unusually with complete heart block and severe mitral regurgitation.
May warrant extreme caution with surgery for cardiac rhabdomyosarcoma; leaves open optimal management strategies.
Primary cardiac tumours are rare and difcult to diagnose because most are asymptomatic or have varied non-specic presentations. This report describes a 29-year-old man presenting with complete heart block, primary cardiac tumour in the left atrium, and severe mitral regurgitation. In view of the primary severe mitral regurgitation and complete heart block, mitral valve repair and pacemaker insertion were planned. Mitral valve repair was done with 29mm St Jude tailor annuloplasty ring, and the biopsy was taken from the nodules noted in the left atrium; temporary right ventricular epicardial pacemaker implantation and CABG with SVG to PDA graft were done to look for the recovery of complete heart block. Histopathological examination revealed pleomorphic rhabdomyosarcoma. The patient developed renal failure and liver failure during the postoperative period and expired after 10 days
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Pranoy et al. (2021) conducted a case report in Primary pleomorphic rhabdomyosarcoma of the heart (n=1). Mitral valve repair, pacemaker insertion, and CABG was evaluated on Patient survival. A 29-year-old man with primary pleomorphic rhabdomyosarcoma of the heart died 10 days after mitral valve repair, pacemaker insertion, and CABG due to renal and liver failure.
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