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Cardiac Myxomas in Carney Complex: Single Institution Multidecade Experience.

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Cardiac myxomas affect over 60% of Carney complex (CNC) patients, with over half experiencing recurrence. Regular echocardiograms and surgical removal are key for managing these tumors in CNC patients.

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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.