Multicenter cohort analysis of cardiac amyloidosis patients treated with heart transplant.
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Argirò Alessia, Colio Federica, Garofalo Manuel, Longhi Simone, Potena Luciano, Saturi Giulia, Allinovi Marco, Aloisio Alessio, Porcari Aldostefano, Sheikh Awais, Fontana Marianna, Gillmore Julian, García-Pavía Pablo, Gómez Bueno Manuel, Menale Silvia, Moody William E, Thornton Caitlin, Herrador Lorena, González-Costello José, de Frutos Fernando, Di Nora Concetta, Vendramin Igor, Imazio Massimo, Merlo Marco, Sinagra Gianfranco, Urey Marcus Anthony, Bui Quan M, Masri Ahmad, Alqabani Mohammad, Chen Zhongli, Chen Liang, Gasperetti Alessio, Milani Paolo, Palladini Giovanni, Cattadori Barbara, Cipriani Alberto, Sinigiani Giulio, Gerosa Gino, Palmiero Giuseppe, Limongelli Giuseppe, Musumeci Beatrice, Tini Giacomo, Pieroni Maurizio, Perfetto Federico, Cappelli Francesco
Аннотация
METHODS: This retrospective multicenter study involving 14 referral centers included 113 patients with cardiac amyloidosis who underwent HT: 57 with transthyretin amyloidosis (ATTR) (22 with wild-type ATTR and 35 with variant ATTR) and 56 with light chain amyloidosis (AL).
RESULTS: Compared with patients with ATTR, patients with AL amyloidosis showed more severe hemodynamic compromise before HT, with a lower cardiac index (1.7 L/min/m2 [interquartile range, 1.4-2.0 L/min/m2] vs 1.9 L/min/m2 [interquartile range, 1.6-2.4 L/min/m2]; P = .027) and higher right atrial pressure. Post-transplant infections occurred in 23 patients with ATTR (40%) and 24 with AL (43%). New-onset neuropathy occurred in 11% of patients, while neuropathy progression was more frequent in ATTR compared with AL (23% vs 7.1%; P = .044). Median follow-up was 4.6 years in ATTR and 5.4 years in AL. Five-year survival was similar in both groups (77% in AL vs 83% in ATTR). Sepsis was the leading cause of death (n = 11, 29% of deaths). Among patients with ATTR, 9 received tafamidis before HT and 7 after HT. Eight patients with ATTR received patisiran post-HT (1 pre-HT), and 3 received diflunisal post-HT. In AL, 70% received bortezomib-based therapy while 28% received daratumumab-based therapy. In AL, hematologic responses improved after HT, with the complete response rate increasing from 42% before HT to 54% after HT.
CONCLUSIONS: HT is an effective treatment for carefully selected patients with cardiac amyloidosis, with comparable unadjusted survival between AL and ATTR. These findings warrant confirmation in prospective studies.