The Seven Year Itch? Promising long-term Survival after Heart Transplant in a Patient with Transthyretin-Amyloidosis

A 53 year-old male patient presented with exertional dyspneoa and massive left ventricular hypertrophy. Endomyocardial biopsy with Congo red staining and immunohistochemistry revealed transthyretin amyloid deposition. By genetic testing Val20Ile mutation was observed in the transthyretin gene, thus confirming diagnosis of hereditary transthyretin amyloidosis. Due to clinical and hemodynamic worsening the patient was referred for heart transplantation that was performed successfully within one year. The postoperative course was unremarkable except for two episodes of graft rejection without any hemodynamic deterioration that were treated with steroids. The patient is doing very well for more than seven years without any evidence of amyloid recurrence in the graft.