Descriptive analysis of patients with systemic sclerosis: pilot cohort study

Abstract Background In systemic sclerosis (SSc) primary heart involvement, although not exhaustively studied, is frequent and may have prognostic consequences. Purpose This pilot cohort study reports a descriptive analysis of patients’ evaluation in a Nuclear Cardiology Unit. Methods Prospective study. Between 2012 and 2016, 62 patients (age 56.8 ± 12.9 years, men 12.9%) with SSc referred from the Autoimmune Systemic Diseases Unit for cardiac evaluation were prospectively analyzed. All patients were studied with gated Single-Photon Emission Computed Tomography Myocardial Perfusion Imaging (gSPECT-MPI) during rest, exercise and cold test. Ventricular synchrony was assessed by Emory Cardiac ToolboxTM program, and coronary anatomy was evaluated by coronary computed tomography angiography (CCTA) in all patients. Statistical analyses were performed using STATA 18. StataCorp, College Station, TX, USA. Results Most patients were women (86.9%). The prevalence of arterial hypertension (55.7%) and hypercholesterolemia (39.3%) was high, in contrast, the prevalence of diabetes mellitus (4.9%), tobacco (8.2%) and chronic renal failure (3.2%) was low. The prevalence of angina (19.4%), dyspnea (35.5%) and the clinical history of cardiac event (myocardial infarction n=5, heart failure n=2, unstable angina n=4, coronary revascularization n=1, pericardial diseases n=2, syncope n=1) was high (19.3%). A quarter of the patients had pulmonary arterial hypertension. Electrical conduction disorder was observed in 27.4% of patients (left bundle branch block, right bundle branch block, and left anterior hemiblock), and no patient had a pacemaker. Myocardial ischemia (MIs) (Summed Difference Score ≥ 2) was observed in 24.2% of patients who underwent SPECT exercise-rest and in 22.6% of patients who underwent SPECT cold-rest test. Five percent of patients without MIs in the SPECT cold-rest test presented MIs in the SPECT exercise-rest test, and 8.5% of patients without MIs in the SPECT exercise-rest test had it in the SPECT cold-rest test. The overall agreement for MIs tests was 85.5%. Left ventricular ejection fraction 50% was observed in 12% of patients. Ventricular dyssynchrony (BD 51º) was observed in 64.5% of patients. A calcium score 100 AU and a calcium score ≥ 400 AU was observed in 33.9% and 11.5% of patients, respectively. Coronary artery disease was detected in 29% of patients, of which 44% presented significant coronary stenosis (≥ 50%). Conclusion This pilot and descriptive study reveals that patients with SSc have cardiac involvement in a non-negligible percentage of the cases and may benefit of a comprehensive evaluation using Nuclear Cardiology techniques among others tests.