Mediterr J Rheumatol 2016;27(3):102-7
Evolution and management of late onset cardiac involvement in a contemporary systemic sclerosis cohort
Authors Information

1: Rheumatology Unit, First Department of Propaedeutic and Internal Medicine, Athens University Medical School
2: Cardiology Department, Laikon Hospital, Athens, Greece

Objective: To prospectively assess late cardiac involvement in asymptomatic patients with systemic sclerosis (SSc). Patients and Methods: Sixty-six selected patients (60 women, 41 with diffuse SSc), aged 50.3±12.4 years (mean±SD) with at least a 3-year disease duration (8.2±5.9) having left ventricular ejection fraction (LVEF) >55% by ultrasound were followed-up every 3-6 months for 5.5±2.1 years (range 3-10). A 12-lead electrocardiogram and 24-hour heart rate recording were performed at least annually and every 1-3 years, respectively. None were receiving bosentan or iloprost at baseline. Results: Baseline electrocardiographic abnormalities included incomplete (10.6%) and complete right bundle branch block (1.5%), left bundle branch block (6.0%), left anterior hemiblock (6.0%) and LV hypertrophy (1.5%). Baseline LV diastolic dysfunction and systolic pulmonary pressure >40mmHg were also found by ultrasound in 15.1% and 21.2%, respectively. During follow-up, no myocardial infarction or cardiac death occurred, but 4.5% and 2.1% of patients developed pericarditis and atrial fibrillation, respectively, and 6.1% needed pacemaker implantation. Although none of the patients developed heart failure at follow-up end, the mean LVEF decreased significantly. At follow-up end, the use of calcium channel blockers and angiotensin-converting enzyme inhibitors administration was not increased, but 16.7% and 22.7% of patients were on anti-arrhythmic drugs and bosentan, respectively. No major differences in evolution and management between diffuse and limited SSc were noted. Conclusion: Late cardiac involvement is common but relatively benign in a typical contemporary SSc cohort, probably due to prompt diagnosis and improved therapeutic approaches.