Cardiac Myxomas in Carney Complex: Single Institution Multidecade Experience

Area of Science:

• Cardiology
• Oncology
• Genetics

Background:

• Carney complex (CNC) is a rare genetic disorder associated with multiple tumors.
• Cardiac myxomas are a significant manifestation of CNC.
• This study details the surgical experience with cardiac myxomas in CNC patients.

Purpose of the Study:

• To present the surgical experience with cardiac myxomas in patients with Carney complex.
• To analyze the incidence, characteristics, recurrence patterns, and outcomes of cardiac myxomas in CNC.
• To evaluate the long-term survival and recurrence-free survival after surgical management.

Main Methods:

• Retrospective review of institutional data for patients diagnosed with Carney complex (CNC).
• Data collection included clinical, surgical, and recurrence information from electronic medical records (1970-2023).
• Analysis of 38 CNC patients, focusing on the 24 who developed cardiac myxomas.

Main Results:

• Cardiac myxomas occurred in 63.1% of CNC patients, with a median age of onset at 39 years.
• The majority of myxomas were found in the left atrium (52.7%).
• Over half (54.1%) of patients experienced recurrence, with a median time to first recurrence of 7.5 years. Freedom from tumor recurrence was 16.7% at 10 years. Long-term survival was 100% at 10 and 15 years.

Conclusions:

• Cardiac myxomas are highly prevalent in Carney complex patients, with a substantial recurrence rate.
• Consistent echocardiographic monitoring is crucial for early detection of primary and recurrent cardiac myxomas.
• Surgical excision remains the primary therapeutic strategy for cardiac myxomas in the context of CNC.