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

Abstract

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.

Systemic sclerosis: An update in 2016

“…Among 22 patients with scleroderma and pulmonary interstitial disease who had a pulmonary histology, 64% had a NSIP pattern and 36% a UIP pattern, with a median survival of 15 versus 3 years, respectively [14]. The presence of an extensive pulmonary fibrosis, defined by lesions extent above 20% on high resolution-CT scan and/or FVC below 70%, was associated with a 5-year mortality rate of 60% [15].…”

“…Myocardial damages are most often linked to ischemic injury associated to disorders of the coronary microcirculation that may lead to myocardial fibrosis. Up to 60% of patients showed perfusion defects on myocardial scintigraphy [15]. In a large international series of 3,656 SSc patients, 24-27% of patients had palpitations, 11-13% a conduction block, 15% a diastolic dysfunction and 5% a reduced left ventricular systolic function [6].…”

“…arterioles and inter-lobular arched, with adventitial fibrosis and intraglomerular thrombosis. The main risk factors of scleroderma renal crisis are a diffuse and rapidly progressive cutaneous form, the presence of anti-RNA polymerase III antibodies, a new onset of anemia, a recent cardiovascular event, the administration of high doses of corticosteroids (> 15mg per day) and a recent development of scleroderma (<4 years) [15,16].…”

Diagnostic criteria, severity classification and guidelines of systemic sclerosis

“…Therefore, myocardial scintigraphy is also useful for detecting cardiac lesions. Papagoras et al 336 . reported that reduced blood flow was found on stress myocardial perfusion scintigraphy in 60% (21/35) of asymptomatic SSc patients, and scintigraphy is considered useful for the early diagnosis of myocardial damage that is caused by SSc.…”

“…335 Therefore, myocardial scintigraphy is also useful for detecting cardiac lesions. Papagoras et al 336 reported that reduced blood flow was found on stress myocardial perfusion scintigraphy in 60% (21/ 35) of asymptomatic SSc patients, and scintigraphy is considered useful for the early diagnosis of myocardial damage that is caused by SSc. Only a limited number of facilities are able to implement MRI and myocardial perfusion scintigraphy, but implementing these tests in the available facilities is recommended.…”