Heart transplantation outcomes in arrhythmogenic right ventricular cardiomyopathy: a contemporary national analysis

ESC Heart Failure

卷:9

Alejandro Suarez‐Pierre¹  Chun W. Choi²  Eric Etchill²  Katherine Giuliano²  Charles D. Fraser III²  Ahmet Kilic²  Paul Scheel III³  Edward K. Kasper³  Kavita Sharma³  Harikrishna Tandri³  Nisha A. Gilotra³  Ilan S. Wittstein³  Hugh Calkins³  Steven Hsu³  Roberta Florido³ 

[1] Department of Surgery University of Colorado Aurora CO USA;

[2] Division of Cardiac Surgery Johns Hopkins University School of Medicine Baltimore MD USA;

[3] Division of Cardiology Johns Hopkins University School of Medicine Baltimore MD USA;

关键词: Arrhythmogenic right ventricular cardiomyopathy;    Heart transplantation;    Heart failure;    Post‐transplant outcomes;   

DOI  :  10.1002/ehf2.13687

来源: DOAJ

【 摘 要 】

Abstract Aims Heart failure is an increasingly recognized later stage manifestation of arrhythmogenic right ventricular cardiomyopathy (ARVC) that can require heart transplantation (HT) to appropriately treat. We aimed to study contemporary ARVC HT outcomes in a national registry. Methods and results The United Network for Organ Sharing registry was queried for HT recipients from 1/1994 through 2/2020. ARVC patients were compared with non‐ARVC dilated, restrictive, and hypertrophic cardiomyopathy HT patients (HT for ischaemic and valvular disease was excluded from analysis). Post‐HT survival was assessed using Kaplan–Meier estimates. A total of 189 of 252 (75%) waitlisted ARVC patients (median age 48 years, 65% male) underwent HT, representing 0.3% of the total 65 559 HT during the study time period. Annual frequency of HT for ARVC increased significantly over time. ARVC patients had less diabetes (5% vs. 17%, P < 0.001), less cigarette use (15% vs. 23%, P < 0.001), lower pulmonary artery and pulmonary capillary wedge pressures, and lower cardiac output than the 33 659 non‐ARVC patients (P < 0.001). Ventricular assist device use was significantly lower in ARVC patients (8% vs. 32%, P < 0.001); 1 and 5 year post‐HT survival was 97% and 93% for ARVC vs. 95% and 82% for non‐ARVC HT recipients (P < 0.001). On adjusted multivariable Cox regression, ARVC had decreased risk of post‐HT death compared with non‐ARVC aetiologies (hazard ratio 0.48, 95% confidence interval 0.28–0.82, P = 0.008). Patients with ARVC also had lower risk of death or graft failure than non‐ARVC patients (hazard ratio 0.51, 95% confidence interval 0.32–0.81, P = 0.004). Conclusions In the largest series of HT in ARVC, we found that HT is increasingly performed in ARVC, with higher survival compared with other cardiomyopathy aetiologies. The right ventricular predominant pathophysiology may require unique considerations for heart failure management, including HT.

【 授权许可】

Unknown