PROGRESSION OF VENTRICULAR WALL THICKENING AFTER LIVER-TRANSPLANTATION FOR FAMILIAL AMYLOIDOSIS

Background. Familial amyloidosis (FAP) is characterized by the progres sion of neurologic and cardiac impairment ultimately leading to death within 7 to 15 years after the onset of the disease. Liver transplanta tion represents the only definitive therapy for this disease and has b een performed since 1990. Methods. To determine the effect of liver tr ansplantation on disease progression, electrocardiography and Doppler echocardiography were performed and blindly analyzed on 11 patients wi th FAP who were followed 0.8 to 8.6 years before liver transplantation and 0.8 to 4.1 years after liver transplantation. Results. After live r transplantation, five patients showed progression of left ventricula r wall thickening with increased left ventricular mass, and three of t hese five showed a reduction in electrocardiographic voltage despite a bolition of the mutant protein hom the serum. Of the five patients sho wing progressive wall thickening, four had the transthyretin variant G lu 42 Gly and one patient had the Ala 36 Pro variant; none of the rema ining six patients, all of whom possessed the Val 30 Met variant, show ed echocardiographic changes. Although 9 of the 11 patients have shown symptomatic improvement in neurologic symptoms, 1 patient has develop ed heart failure and a second patient has suffered a sudden cardiac de ath. Conclusions. After liver transplantation, patients with FAP shoul d have regular clinical evaluations including electrocardiographic and echocardiographic examinations to look for continued deterioration in heart structure or function.