BACKGROUND: Restrictive cardiomyopathy (RCM) represents a spectrum of disorders with a common physiology but divergent etiologies. RCM commonly leads to progressive heart failure and the need for heart transplantation (HTx). Pediatric RCM is a more homogeneous disorder with post-HTx outcomes comparable to those for non-RCM patients. However, post-HTx outcomes in adult RCM patients have not been studied to date. METHODS: Demographic, clinical and survival outcomes of 38,190 adult HTx-only recipients from 1987 to 2010 were acquired from the registry of the United Network of Organ Sharing. The study population included 544 RCM patients (1.4%) and 37,646 non-RCM patients (98.6%). RCM diagnoses included idiopathic (n = 227, 42%), amyloid (n = 142, 26%), sarcoid (n = 81, 15%), radiation/chemotherapy (XRT) (n = 35, 6%) and other (n = 59, 11%). RESULTS: Follow-up began at the time of HTx (74±64 months). During the follow-up period, 224 (41%) patients in the RCM group died, whereas 18,791 (50%) in the non-RCM group died. Crude 1-, 5- and 10-year survival for RCM patients was 84%, 66% and 45%, and for non-RCM patients was 85%, 70% and 50%, respectively. The overall unadjusted hazard ratio of RCM vs non-RCM for all-cause mortality was 1.07 (confidence interval [CI] 0.93 to 1.22). Multivariate Cox proportional hazards regression analysis yielded a hazard ratio of 1.06 (CI 0.91 to 1.25). RCM subgroup analysis showed decreased survival at 1, 5 and 10 years in the XRT (71%, 47% and 32%) and amyloid (79%, 47% and 28%) patient groups. The unadjusted hazard ratio for the XRT and amyloid subgroups vs RCM for all-cause mortality was 1.81 (p = 0.002) and 1.85 (p = 0.0004), respectively. CONCLUSIONS: Outcomes for RCM patients post-HTx are comparable to those of non-RCM patients. However, RCM subgroup analysis suggests increased mortality for XRT and amyloid subgroups. Further analysis is warranted to understand the contributing factors.
BACKGROUND: Restrictive cardiomyopathy (RCM) represents a spectrum of disorders with a common physiology but divergent etiologies. RCM commonly leads to progressive heart failure and the need for heart transplantation (HTx). Pediatric RCM is a more homogeneous disorder with post-HTx outcomes comparable to those for non-RCM patients. However, post-HTx outcomes in adult RCM patients have not been studied to date. METHODS: Demographic, clinical and survival outcomes of 38,190 adult HTx-only recipients from 1987 to 2010 were acquired from the registry of the United Network of Organ Sharing. The study population included 544 RCM patients (1.4%) and 37,646 non-RCM patients (98.6%). RCM diagnoses included idiopathic (n = 227, 42%), amyloid (n = 142, 26%), sarcoid (n = 81, 15%), radiation/chemotherapy (XRT) (n = 35, 6%) and other (n = 59, 11%). RESULTS: Follow-up began at the time of HTx (74±64 months). During the follow-up period, 224 (41%) patients in the RCM group died, whereas 18,791 (50%) in the non-RCM group died. Crude 1-, 5- and 10-year survival for RCM patients was 84%, 66% and 45%, and for non-RCM patients was 85%, 70% and 50%, respectively. The overall unadjusted hazard ratio of RCM vs non-RCM for all-cause mortality was 1.07 (confidence interval [CI] 0.93 to 1.22). Multivariate Cox proportional hazards regression analysis yielded a hazard ratio of 1.06 (CI 0.91 to 1.25). RCM subgroup analysis showed decreased survival at 1, 5 and 10 years in the XRT (71%, 47% and 32%) and amyloid (79%, 47% and 28%) patient groups. The unadjusted hazard ratio for the XRT and amyloid subgroups vs RCM for all-cause mortality was 1.81 (p = 0.002) and 1.85 (p = 0.0004), respectively. CONCLUSIONS: Outcomes for RCM patients post-HTx are comparable to those of non-RCM patients. However, RCM subgroup analysis suggests increased mortality for XRT and amyloid subgroups. Further analysis is warranted to understand the contributing factors.
Authors: Mirela Tuzovic; Eric H Yang; Arnold S Baas; Eugene C Depasquale; Mario C Deng; Daniel Cruz; Gabriel Vorobiof Journal: Curr Oncol Rep Date: 2017-07 Impact factor: 5.075
Authors: David K C Cooper; Martin Wijkstrom; Sundaram Hariharan; Joshua L Chan; Avneesh Singh; Keith Horvath; Muhammad Mohiuddin; Arielle Cimeno; Rolf N Barth; John C LaMattina; Richard N Pierson Journal: Transplantation Date: 2017-07 Impact factor: 4.939
Authors: Marcus V Simões; Fabio Fernandes; Fabiana G Marcondes-Braga; Philip Scheinberg; Edileide de Barros Correia; Luis Eduardo P Rohde; Fernando Bacal; Silvia Marinho Martins Alves; Sandrigo Mangini; Andréia Biolo; Luis Beck-da-Silva; Roberta Shcolnik Szor; Wilson Marques Junior; Acary Souza Bulle Oliveira; Márcia Waddington Cruz; Bruno Vaz Kerges Bueno; Ludhmila Abrahão Hajjar; Aurora Felice Castro Issa; Felix José Alvarez Ramires; Otavio Rizzi Coelho Filho; André Schmidt; Ibraim Masciarelli Francisco Pinto; Carlos Eduardo Rochitte; Marcelo Luiz Campos Vieira; Cláudio Tinoco Mesquita; Celso Dario Ramos; José Soares-Junior; Minna Moreira Dias Romano; Wilson Mathias Junior; Marcelo Iório Garcia Junior; Marcelo Westerlund Montera; Marcelo Dantas Tavares de Melo; Sandra Marques E Silva; Pedro Manoel Marques Garibaldi; Aristóteles Comte de Alencar Neto; Renato Delascio Lopes; Diane Xavier de Ávila; Denizar Viana; José Francisco Kerr Saraiva; Manoel Fernandes Canesin; Glaucia Maria Moraes de Oliveira; Evandro Tinoco Mesquita Journal: Arq Bras Cardiol Date: 2021-09 Impact factor: 2.000