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

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 resultsThe 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.

Legacy Effect of Endurance Training in a Sexagenarian Heart Transplant Recipient: A Case Report

“…Although ARVC remains a rare disease and does not represent the majority of pathologies requiring HT, heart failure has increasingly been recognized as a later-stage manifestation of ARVC, which has led to more HTs to appropriately treat (8). Recently, researchers reviewed a registry of HT recipients from January 1994 through February 2020 to compare ARVC patients with non-ARVC patients who had dilated, restrictive, and hypertrophic cardiomyopathies (8). Consistent with other published reports, they found that ARVC patients had significantly less comorbidities (e.g., diabetes and cigarette use) when compared with non-ARVC patients and a significantly higher rate of survival (8).…”

Continuous-flow left ventricular assist device treatment for arrhythmogenic right ventricular cardiomyopathy complicated by advanced biventricular failure – University of Tokyo experiences

“…But, there was no patient who was implanted with a mechanical circulatory support device in this cohort. Guiliano et al reported 252 HTx wait-listed patients extracted from the UNOS database between 1994 and 2020 ( 14 ). They comprised only 0.4% of the entire HTx listed patients, but the percentage of all HTx performed for the primary indication of ARVC increased from 0.04% in 1994 to 0.73% in 2019.…”

“…Six patients (2.4%) had VADs on listing: one with LVAD, two with a TAH, and three with BiVAD. By the time of transplantation, a total of 15 patients (7.9%) had VADs: six with LVADs, two with RVADs, four with TAHs, and three with BiVAD ( 14 ).…”

NT-proBNP is superior to novel plasma biomarkers for predicting adverse outcome in arrhythmogenic right ventricular cardiomyopathy

“…Currently, due to widespread use of implantable cardioverter-defibrillators (ICDs) to prevent sudden cardiac death, an increasing number of ARVC patients develop heart failure [8]. In advanced stages of the disease, heart transplantation should be considered but selecting optimal time for this treatment remains challenging because of the predominantly right ventricular pathophysiology [9][10]. Numerous novel biomarkers have been recently studied in cardiology, and the results suggest that some of them will be introduced into routine clinical practice.…”