TY - JOUR
T1 - Heart transplantation outcomes in arrhythmogenic right ventricular cardiomyopathy
T2 - a contemporary national analysis
AU - Giuliano, Katherine
AU - Scheel, Paul
AU - Etchill, Eric
AU - Fraser, Charles D.
AU - Suarez-Pierre, Alejandro
AU - Hsu, Steven
AU - Wittstein, Ilan S.
AU - Kasper, Edward K.
AU - Florido, Roberta
AU - Tandri, Harikrishna
AU - Calkins, Hugh
AU - Choi, Chun Woo
AU - Sharma, Kavita
AU - Kilic, Ahmet
AU - Gilotra, Nisha A.
N1 - Funding Information:
The authors wish to acknowledge funding from Leducq Foundation (Fondation Leducq). The Johns Hopkins ARVD/C Program is supported by the Leonie Wild Foundation, the Leyla Erkan Family Fund for ARVD Research, the Dr Francis P. Chiramonte Private Foundation, the Dr Satish, Rupal, and Robin Shah ARVD Fund at Johns Hopkins, the Bogle Foundation, the Healing Hearts Foundation, the Campanella family, the Patrick J. Harrison family, the Peter French Memorial Foundation, and the Wilmerding Endowments.
Publisher Copyright:
© 2021 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.
PY - 2022/4
Y1 - 2022/4
N2 - 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.
AB - 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.
KW - Arrhythmogenic right ventricular cardiomyopathy
KW - Heart failure
KW - Heart transplantation
KW - Post-transplant outcomes
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U2 - 10.1002/ehf2.13687
DO - 10.1002/ehf2.13687
M3 - Article
C2 - 35132806
AN - SCOPUS:85124522014
SN - 2055-5822
VL - 9
SP - 988
EP - 997
JO - ESC Heart Failure
JF - ESC Heart Failure
IS - 2
ER -