Background and Objective. Although combined heart and liver transplantation (CHLT) is an accepted therapeutic strategy in non-Val30Met TTR-related amyloidosis (ATTR), Centers with an unrestricted access to this program are few and long term results are poorly defined. We report results of our transplantation strategy in ATTR: stage I neuropathy and severe cardiomyopathy (mean left ventricular wall thicknesV•13 mm), even in the absence of heart failure or systolic dysfunction. Methods. Between 1993 and December 2012, 15 patients with ATTR underwent CHLT and were evaluated for survival and incremental risk factors for mortality. Results and Discussion. 13 patients were males (86,7%) with a median age of 50 years (range 38-60), mean left ventricular wall thickness of 19 mm (range 13-27). They underwent CHLT a mean 3.8 years (range 1-8) from disease onset and after 0.7 years on the waiting list. The most frequent mutation was Gln89 (8 patients, 53%), followed by Asn23 and Leu68 (2 patients each) and Ala49, Gln54 and Thr 59 were each present in one patient. After a mean follow up of 5,1 years (range 0,8 – 13,1), survival was 93,3% at 1 year and 71,3% at 10 years. Three patients died: 1 Gln89 with renal injury 58 days after transplant for multi organ failure and 2 (Gln89 and Ala49) with mBMI < 700 at transplant died respectively 556 and 1727 days after CHLT for progression of autonomic dysfunction. Conclusions. CHLT improves midterm survival in patients with non-Val30Met mutations, mild neuropathy and cardiomyopathy with/without hemodynamic impairment.

Long-term results of combined heart and liver transplant in ATTR: A single centre experience

MASTROROBERTO, MARIANNA;ERCOLANI, GIORGIO;PILATO, EMANUELE;RAPEZZI, CLAUDIO;PINNA, ANTONIO DANIELE
2014

Abstract

Background and Objective. Although combined heart and liver transplantation (CHLT) is an accepted therapeutic strategy in non-Val30Met TTR-related amyloidosis (ATTR), Centers with an unrestricted access to this program are few and long term results are poorly defined. We report results of our transplantation strategy in ATTR: stage I neuropathy and severe cardiomyopathy (mean left ventricular wall thicknesV•13 mm), even in the absence of heart failure or systolic dysfunction. Methods. Between 1993 and December 2012, 15 patients with ATTR underwent CHLT and were evaluated for survival and incremental risk factors for mortality. Results and Discussion. 13 patients were males (86,7%) with a median age of 50 years (range 38-60), mean left ventricular wall thickness of 19 mm (range 13-27). They underwent CHLT a mean 3.8 years (range 1-8) from disease onset and after 0.7 years on the waiting list. The most frequent mutation was Gln89 (8 patients, 53%), followed by Asn23 and Leu68 (2 patients each) and Ala49, Gln54 and Thr 59 were each present in one patient. After a mean follow up of 5,1 years (range 0,8 – 13,1), survival was 93,3% at 1 year and 71,3% at 10 years. Three patients died: 1 Gln89 with renal injury 58 days after transplant for multi organ failure and 2 (Gln89 and Ala49) with mBMI < 700 at transplant died respectively 556 and 1727 days after CHLT for progression of autonomic dysfunction. Conclusions. CHLT improves midterm survival in patients with non-Val30Met mutations, mild neuropathy and cardiomyopathy with/without hemodynamic impairment.
Amyloid: Insoluble, but Solvable
Mastroroberto, M; Ercolani, G; Grigioni, F; Pilato, E; Berardi, S; Longhi, S; Benni, E; Sama, C; Rapezzi, C; Pinna, AD
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/11585/599445
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