The eligibility of patients with an inherited cardiac disease for therapy with a subcutaneous implantable cardioverter-defibrillator

The subcutaneous implantable cardioverter-defibrillator (S-ICD) is considered especially beneficial in young patients with an inherited cardiac disease, but data on these patient groups is limited.

To evaluate the eligibility of patients with an inherited cardiac disease for therapy with a subcutaneous implantable cardioverter-defibrillator

We retrospectively analysed all patients with an inherited cardiac disease who received an S-ICD in our tertiary center between February 2009 and June 2021. ECG parameters and data on appropriate and inappropriate shocks were collected. Different diagnoses were compared using a chi-square test.

We analysed 109 patients who were diagnosed with IVF (DPP6-based, N=41, 37.6%), HCM (N=36, 33.0%), Brugada syndrome (BrS, N=13, 11.9%), ARVC (N=9, 8.3%), PLN (N=3, 2.8%), LQTS (N=2, 1.8%) and other cardiomyopathies (N=5, 4.6%). Mean follow-up was 5.5±3.4 years. ECG parameters did not significantly change during follow-up. HCM patients were most likely to receive an appropriate shock (10/36, P=0.01), followed by ARVC (2/9, P=0.57) and Brugada patients (1/13, P=0.40). Patients with LQTS had most frequently inappropriate shocks (1/2, P=0.06), followed by BrS (4/13, P=0.008) and HCM patients (4/36, P=0.8).

Patients with an inherited cardiac disease are eligible for S-ICD therapy, but comes with a risk of inappropriate shocks, especially in patients with LQTS and BrS.

Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Dutch Heart Foundation (CVON 2018-10 PREDICT2)