Abstract 16777: Do Not Clown Around With Intracardiac Myxomas: A Clinical Case of Carney Complex

Introduction: Intracardiac myxomas are associated with cardiovascular mortality most frequently due to embolism and obstruction leading to heart failure. One of the most feared complications of intracardiac myxomas are sudden cardiac death, as these tumors are often identified incidentally. Carney Complex (CNC) is a rare disorder characterized by cardiac myxomas, psammomatous melanotic schwannomas, abnormal skin pigmentation, and endocrine disorders.

Clinical Case: A 55 year old female with a past medical history of hypertension presented to the hospital with syncopal episodes and intermittent vision loss. Head computed tomography scan noted a large extra-axial mass present within the left cerebellopontine angle. A transthoracic echocardiogram showed a severely dilated left atria containing a large atrial mass (7.9 x 5.0 cm) that was causing obstruction of mitral inflow resulting in peak velocity 246 cm/S with a mean gradient of 12 mmHg. The patient was also noted to have dispersed epithelioid blue nevi present on skin examination. The patient met major criteria for diagnosis of Carney Complex.

Conclusion: Carney complex (CNC) is an extremely rare autosomal dominant syndrome with less than 1,000 cases reported worldwide. Patients with familial history of intracardiac myxomas, multiple myxomas, characteristic skin findings, or endocrinopathies should be evaluated for CNC. Patients with cardiac myxoma secondary to CNC typically have a recurrence risk of roughly 30%. Patients with CNC should be screened with biannual transthoracic echocardiograms after resection and patients with suspected CNC should be worked up for additional tumor burden and endocrinopathies.