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

Katherine Giuliano, Paul Scheel, Eric Etchill, Charles D. Fraser, Alejandro Suarez-Pierre, Steven Hsu, Ilan S. Wittstein, Edward K. Kasper, Roberta Florido, Harikrishna Tandri, Hugh Calkins, Chun W. Choi, Kavita Sharma, Ahmet Kilic, Nisha A. Gilotra

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)988-997
Number of pages10
JournalESC Heart Failure
Volume9
Issue number2
DOIs
StatePublished - Apr 2022

Keywords

  • Arrhythmogenic right ventricular cardiomyopathy
  • Heart failure
  • Heart transplantation
  • Post-transplant outcomes

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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