Resection of Multiple Recurrent Cardiac Myxomas in an Adult Man With Carney's Complex

Abstract

Cardiac myxomas have many manifestations, including an association with endocrine overreactivity, unusual testicular tumors, and manifestations in Carney's complex [1McCarthy P.M. Piehler J.M. Schaff H.V. et al.The significance of multiple, recurrent, and “complex” cardiac myxomas.J Thorac Cardiovasc Surg. 1986; 91: 389-396PubMed Google Scholar]. We recently treated a 40-year-old man who presented with an embolic stroke. He had a family history of cardiac myxoma and underwent resection of a right atrial myxoma 7 years earlier.Echocardiography revealed multiple intracardiac masses in the left atrium (Fig 1A , arrow), right atrium, and right ventricle (Fig 1B, arrow). An ultrasound examination of the scrotum showed multiple bilateral testicular calcifications (Figs 2A, 2B), thought to be calcifying Sertoli cell tumors. A computed tomography scan of the abdomen showed no abnormalities, and the 24-hour urine cortisol level was within the normal reference range. Genetic evaluation revealed a heterozygous mutation in the PRKAR1A gene.Fig 2View Large Image Figure ViewerDownload (PPT)The patient underwent excision of 4 cardiac myxomas (left atrium, right atrium, and right ventricle; Fig 3) using cardiopulmonary bypass and standard aortic and bicaval cannulation. His postoperative course was unremarkable, and he was dismissed on the fourth postoperative day.Fig 3View Large Image Figure ViewerDownload (PPT) Cardiac myxomas have many manifestations, including an association with endocrine overreactivity, unusual testicular tumors, and manifestations in Carney's complex [1McCarthy P.M. Piehler J.M. Schaff H.V. et al.The significance of multiple, recurrent, and “complex” cardiac myxomas.J Thorac Cardiovasc Surg. 1986; 91: 389-396PubMed Google Scholar]. We recently treated a 40-year-old man who presented with an embolic stroke. He had a family history of cardiac myxoma and underwent resection of a right atrial myxoma 7 years earlier. Echocardiography revealed multiple intracardiac masses in the left atrium (Fig 1A , arrow), right atrium, and right ventricle (Fig 1B, arrow). An ultrasound examination of the scrotum showed multiple bilateral testicular calcifications (Figs 2A, 2B), thought to be calcifying Sertoli cell tumors. A computed tomography scan of the abdomen showed no abnormalities, and the 24-hour urine cortisol level was within the normal reference range. Genetic evaluation revealed a heterozygous mutation in the PRKAR1A gene. The patient underwent excision of 4 cardiac myxomas (left atrium, right atrium, and right ventricle; Fig 3) using cardiopulmonary bypass and standard aortic and bicaval cannulation. His postoperative course was unremarkable, and he was dismissed on the fourth postoperative day.