The Royal Free Hospital experience of ICD implantation in systemic sclerosis patients between 2008 and 2018

Introduction: Systemic Sclerosis (SSc) manifests as inflammation, vasculopathy and fibrosis of the skin and visceral organs. Cardiac involvement, defined as either pericarditis, congestive heart failure, severe arrhythmias, and/or atrioventricular conduction abnormalities, was found to be the leading cause of death (36%) in a large cohort of SSc patients, followed over 10 years. Literature reports 4–6% of SSc patients die of sudden cardiac death (SCD). We described SSc patients with ICD implants, under the care of the Royal Free Hospital (RFH) Scleroderma Service. RFH is a national tertiary referral centre for SSc patients and the largest UK cohort. Methods: A retrospective study was conducted on patients registered under the RFH Scleroderma Service who had ICDs implanted between 2008 and 2018. All medical notes available were reviewed. We recorded their demographics, scleroderma profile, cardiac biomarkers, NYHA class, ECG, echocardiography, cardiac MRI findings, ICD complications, ICD activations and date of death/last follow up. Results: ICDs were implanted in 24 SSc patients between 2008 and 2018. The mean age was 48 ± 13 (range 18–74). Fifteen of the 24 patients had diffuse cutaneous systemic sclerosis (dcSSc) and nine of these had an overlap with another autoimmune condition (6 of these 9 had myositis). Of the nine limited cutaneous systemic sclerosis (lcSSc) patients, four had overlap (two of the four had myositis). Thirteen patients had an ICD implanted for primary prevention and three of these patients went on to have appropriate shocks for VT. All three patients had an ejection fraction (EF) less than 35%. Two of these three patients had Late Gadolinium Enhancement (LGE) on their MRI. All three had NT-proBNP raised above the upper reference range of 236 ng/L. Eleven patients had an ICD implant for secondary prevention, three had further appropriate shock therapy for VT. Two of the three patients had an EF