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. 2022 Apr;9(2):988-997.
doi: 10.1002/ehf2.13687. Epub 2022 Feb 8.

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

Katherine Giuliano  1 , Paul Scheel 3rd  2 , Eric Etchill  1 , Charles D Fraser 3rd  1 , Alejandro Suarez-Pierre  3 , Steven Hsu  2 , Ilan S Wittstein  2 , Edward K Kasper  2 , Roberta Florido  2 , Harikrishna Tandri  2 , Hugh Calkins  2 , Chun W Choi  1 , Kavita Sharma  2 , Ahmet Kilic  1 , Nisha A Gilotra  2
Affiliations

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

Katherine Giuliano et al. ESC Heart Fail. 2022 Apr.

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.

Keywords: Arrhythmogenic right ventricular cardiomyopathy; Heart failure; Heart transplantation; Post-transplant outcomes.

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Conflict of interest statement

H.T. is a consultant for Abbott. K.S. is a consultant and advisory board member and received honoraria from Novartis, Janssen, Bayer, Novo Nordisk, and Bristol Myers Squibb. H.C. is a consultant for Medtronic Inc., Biosense Webster, Pfizer, StrideBio, and Abbott. N.A.G. is a consultant and advisory board member for scPharmaceuticals, Inc. The remaining authors have no disclosures or possible conflicts of interest to report.

Figures

Figure 1
Figure 1
Study population of heart transplantation patients from the Organ Procurement and Transplantation Network database. ARVC, arrhythmogenic right ventricular cardiomyopathy; CM, cardiomyopathy.
Figure 2
Figure 2
The number of transplants performed per year for arrhythmogenic right ventricular cardiomyopathy (ARVC) (columns) and the percentage of total transplants (line graph) have significantly increased over the study period (P < 0.001).
Figure 3
Figure 3
Kaplan–Meier survival estimates demonstrated (A) improved survival and (B) improved survival free of graft failure after heart transplantation for patients with primary diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC) vs. non‐ARVC dilated, restrictive, and hypertrophic cardiomyopathy aetiologies (both P < 0.001 by log‐rank test).
See this image and copyright information in PMC

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