Immunosuppression After Myocarditis Episodes In Patients With Dsp-cardiomyopathy

Abstract

Introduction

Desmoplakin cardiomyopathy (DSP-CMP) is a genetic cardiomyopathy characterized by high risk of ventricular arrhythmias (VAs), heart failure (HF), and recurrent myocarditis. Data regarding the use and impact of immunosuppression on those myocarditis episodes is scarce.

Hypothesis

Immunosuppression treatment in DSP-CMP associated myocarditis may improve clinical outcomes.

Methods

Patients (pts) with DSP-CMP and 1+ episode of myocarditis in the worldwide DSP-ERADOS Network (26 institutions across 9 countries in 3 continents) were enrolled. For each treated episode, immunosuppressive medication regimen and duration (acute vs chronic immunosuppression) were collected. The primary combined outcome was sustained VA and/or HF episodes during follow up. Outcomes of DSP-CMP patients receiving immunosuppression at the time of first myocarditis episode were compared with DSP-CMP patients with untreated myocarditis using a log-rank test.

Results

Of 815 patients in the DSP registry, 153 patients (age at first presentation 31.1±8.7 yo, 72.5% female, 71.2% proband, LVEF 46.7±8.6%) experiencing a total of 260 myocarditis episodes (age at first episode 32.5±7.4 yo; n=60/153 had myocarditis as initial DSP-CMP presentation; 1.7 episodes/pt; n=54 pts with 2+ episodes,) were enrolled in the current study (Table 1). Of these episodes, 79 (30.4%) were treated with immunosuppression, with 23 treated with multiple agents. Seven (4.6%) patients received chronic immunosuppression. Over a median follow up of 4.6 [1.6-8.2] years, 96 primary outcome events (HF: n=31, VA: n=65) where observed in 73 (47.7%) patients. Patients whose first episode was treated with immunosuppression were less likely to meet the combined endpoint of HF/VA than those whose first episode was not treated (6/31 (19.4%) vs 67/122 (54.9%) p<0.001).

Conclusions

In a large, multicenter DSP-CMP registry, we demonstrate that approximately one-third of all myocarditis episodes were treated with immunosuppression, most commonly corticosteroid-based strategies. Patients whose first episode of myocarditis was treated with immunosuppression had a lower rate of subsequent VA and HF events. These findings provide preliminary data to support a prospective trial to confirm the role of immunosuppression in DSP-CMP presenting with myocarditis.