Heart involvement in systemic sclerosis: a combined echocardiographic and scintigraphic study

The aim of this study is to investigate systemic sclerosis (SSc) patients without clinically evident heart disease for cardiac abnormalities. SSc patients and age- and sex-matched healthy controls from the hospital staff underwent transthoracic echocardiography for the assessment of the left ventricle (LV) morphology and function and estimation of the pulmonary artery systolic pressure (PASP). Patients further underwent stress-rest myocardial perfusion imaging (MPI) scintigraphy by single-photon emission computed tomography (SPECT). Thirty-seven patients were included (33 women, 19 with diffuse, and 18 with limited SSc). LV hypertrophy was more common in SSc patients than controls (24.3 vs 0 %, p = 0.001). Impaired LV relaxation was found in 45.9 % of patients and 40.5 % controls (p = 0.639). Excluding patients with arterial hypertension, LV hypertrophy was still found in 23.1 % and LV relaxation impairment in 38.5 %. PASP over 30 mmHg was found in 13 patients (35.1 %), 11 of whom had no history of pulmonary arterial hypertension (PAH). Of 35 patients who underwent SPECT, 21 patients (60 %) exhibited reversible LV perfusion defects. Their mean age was 51.8 years; four patients were younger than 40 years old and eight patients younger than 50 years. In all cases, ischemia was graded as mild or moderate and in a single case, graded as significant. Subclinical heart involvement is common in SSc patients even in the younger age groups. LV hypertrophy and impaired relaxation, raised PASP, and ischemia on MPI with SPECT are found in a significant proportion of SSc patients. Careful screening of SSc patients for potential heart involvement and consultation by a cardiologist may be of value.