Recurrent Atrial Myxoma in a Patient With Carney Complex 26 Years After Heart Transplantation

Carney complex, a familial syndrome caused by germline PRKA1A mutation, predisposes to recurrent cardiac myxomas. In refractory cases, orthotopic heart transplantation (OHT) can be considered for assumed curative treatment of recurrent myxoma. We present a case of recurrent cardiac myxoma arising from residual native right atrial tissue in a woman with Carney complex 26 years after OHT for recurrent myxoma. To our knowledge, this represents the first reported case of recurrent cardiac myxoma after OHT in a patient with Carney complex. Surgical ablation of remaining atrial tissue during OHT for myxoma may reduce the risk of recurrence, and lifelong surveillance after myxoma resection in Carney complex may be indicated. This case highlights a rare genetic syndrome that can cause recurrent cardiac myxomas. If OHT is performed in the context of Carney complex, attention to surgical and surveillance strategies to prevent and detect recurrence is key.