Cardiac Myxomas in Carney Complex: Single Institution Multidecade Experience

Background: We present our surgical experience with cardiac myxomas in the setting of Carney complex (CNC).

Methods: We searched our institutional data explorers to identify patients diagnosed with CNC. We gathered clinical, surgical, and recurrence data from electronic medical records. In total, 38 patients with CNC were documented from 1970 through 2023.

Results: Cardiac myxomas developed in 24 patients (63.1%) in the setting of CNC. The median age of onset for cardiac myxoma occurrence was 39.0 years (interquartile range [IQR], 25.0-56.0 years). Most patients were females (62.5%), and all underwent surgery. A total of 42 myxomas (52.7%) were extracted from the left atrium, 12 (15.0%) from the right ventricle, 11 (13.7%) from the right atrium, and 6 (7.5%) from the left ventricle. Among the 24 myxoma patients, 13 (54.1%) experienced at least 1 myxoma recurrence. The median time for the first myxoma recurrence was 7.5 years (IQR, 3.8-10.0 years). There were 27 recurrences (52.9%) from the same chamber, 11 (29.4%) from different chambers, and the localizations in 9 (17.6%) were undocumented. The freedom from tumor recurrence was 100% (95% CI, 100%-100%), 66.7% (95% CI, 44.7%-99.5%), and 16.7% (95% CI, 4.7%-59.1%) at 1, 5, and 10 years, respectively. The long-term survival was 100% at 10 and 15 years.

Conclusions: Cardiac myxomas developed in nearly two-thirds of CNC patients (63.1%) in this study, and more than half (54.1%) experienced recurring instances. Consistent monitoring through echocardiograms is essential for detecting asymptomatic first-time occurrences or recurrences. Surgical removal remains the key treatment method for managing cardiac myxomas associated with CNC.