Clinical Heterogeneity of Recurrent Familial Cardiac Myxoma in PRKAR1A-Related Carney Complex

Vallejos Barrientos A.; Espinoza Alva D.; Davila Flores D.; Montesinos Segura R.; Aguilar Carranza C.; Zamudio Silva R. · JACC: Case Reports (2026) · DOI: 10.1016/j.jaccas.2026.108964

Resumen

Background: Cardiac myxomas are common benign primary cardiac tumors; hereditary forms are strongly associated with the Carney complex. Case Summary: A 42-year-old woman with familial Carney complex and a pathogenic PRKAR1A variant developed recurrent cardiac myxomas involving 3 of the 4 chambers. The first presentation featured left atrial and right ventricular myxomas causing cardioembolic cerebellar stroke. A first recurrence in the left atrium caused severe transmitral obstruction and decompensated heart failure. A second recurrence in the right atrium presented as an infected myxoma. A third recurrence arose from the tricuspid annulus 4 months after resection; because further reoperation after 2 prior sternotomies and a right thoracotomy was considered prohibitive, she was listed for transplantation but died suddenly while awaiting transplant. Discussion: This case highlights the aggressive, recurrent, and clinically heterogeneous phenotype and fatal course of Carney complex–associated cardiac myxoma. Take-Home Message: Early recognition, lifelong surveillance, and timely consideration of advanced strategies are essential in selected patients with recurrent disease.