Experience with heart transplantation for cardiac tumors

Background: Primary cardiac tumors are rare clinical entities. Benign tumors are often amenable to surgical excision, whereas malignant tumors are seldom resectable. Five patients have been reported to undergo orthotopic heart transplantation for inoperable primary cardiac tumors. We review the clinical course of these five patients and present our institutional experience with three patients who underwent orthotopic heart transplantation as primary therapy for unresectable cardiac tumors. Methods: Retrospective chart review and telephone interview, when possible, were used to gather historical and clinical course data. Results: Of the eight patients who underwent orthotopic heart transplantation for primary cardiac neoplasms, four had malignant tumors (three sarcomas, one lymphoma) and four had locally invasive neoplasms (three fibromas, one pheochromocytoma). For those patients in whom cardiectomy resulted in surgical margins free of tumor (six of eight), orthotopic heart transplantation provided long-term survival (range 14 to 78 months) without tumor recurrence despite therapeutic immunosuppression. The only death in this group, at 6.2 years after operation, was unrelated to tumor recurrence. The patients with tumor identified at the surgical margins died of metastatic disease at 14 and 15 months after the operation in spite of adjuvant chemotherapy. Conclusions: An awareness by clinicians of the presenting clinical picture of these tumors is warranted in view of the potential for cure by resection or transplantation. Patients with benign primary cardiac tumors appear to benefit from the complete resection afforded by cardiectomy and transplantation. The role of transplantation for patients with malignant tumors remains unclear. Additional studies and continued follow-up with serial echocardiography is necessary to further ascertain the role of heart transplantation in the management of patients with primary cardiac tumors.