Heart involvement in systemic sclerosis: Evolving concept and diagnostic methodologies

Abstract

Heart involvement, including primary myocardial involvement, is very common in systemic sclerosis. There is strong evidence that primary myocardial involvement is related to repeat focal ischaemic injury causing subsequent irreversible myocardial fibrosis. Clinically evident cardiac involvement is recognized to be a poor prognostic factor; thus preclinical identification is highly encouraged. The severity of heart involvement has been confirmed recently. Echocardiography, including pulsed tissue Doppler echocardiography, is the cornerstone of routine heart assessment. Myocardial perfusion may be assessed by single photon emission computed tomography. If available, cardiac magnetic resonance imaging should be considered as it allows simultaneous measurement of ventricular volumes and function and myocardial perfusion, and assessment of possible inflammation and/or fibrosis. Biological variables, such as B-type natriuretic peptides, are highly relevant, valuable markers of global heart involvement in systemic sclerosis and should be considered for screening of patients and/or research purposes.

Hematopoietic Stem Cell Transplantation for Systemic Sclerosis: If You Are Confused, Remember: “It Is a Matter of the Heart”

“…In addition to performing echocardiogram with right ventricular assessment (TAPSE) and right heart catheterization, we also prescreen patients with SSc by cardiac MRI for assessment of ventricular volumes and function and inflammation and/or fibrosis 20 . In Figure 1 cardiac MRI performed on a patient 18 years of age with a 24-month history of SSc who was excluded from HSCT despite normal right heart catheterization shows extensive intramyocardial fibrosis that increases risk of arrhythmias 21 .…”

Complications and Mortality in Systemic Vasculitis – Vasculogenic Clinicopathologic Entities in Rheumatoid Arthritis and Progressive Systemic Sclerosis Autopsy Patients

“…Studies confirm the increased risk of cardiovascular diseases in RA [4][5][6][7]. The cardiovascular complications-beside renal and pulmonary ones -are important in the mortality of SSc patients as well [8][9][10].…”

“…Severity of A-SV was determined histologically in one of our previous study [9]: Atherosclerosis (Ath) -was diagnosed in RA patients only in cases, when it was present macroscopically as a "severe" atherosclerotic process (characterized by occlusive thrombosis or sclerotic ulcers) or, (mTb) in 6 (3.73%) of 161 patients. Tb was associated with A-SV in 9, and active mTb in 4 of 21 cases.…”

“…The basic disease, complication(s) and associated diseases of 11 SSc patients with A-SV and FIP are summarized in Table 3. A-SV: Systemic vasculitis of autoimmune origin (complication with lethal outcome in 11 of 11 patients; accompanied with fibromuscular intimal proliferation -FIP in 10 of 11 patients) Severity of A-SV was determined histologically in one of our previous study: [9] Cl+: Clinically recognized "vasculitis" (Ad litteram -"explicit verbis") in 2 (18.18%) of 11 patients.…”

Determinants of exercise-induced pulmonary arterial hypertension in systemic sclerosis

“…It seems however, that the incidence of EIPH may overestimate the percentage of onset of resting PH during follow-up [9]. Some recent studies have underlined that the origin of EIPH in SSc could be secondary, not only to pulmonary vasculopathy, but also to myocardial [10] and/or pulmonary impairment [11,12]. Therefore, a post-capillary involvement in EIPH has been hypothesized in the literature [13,14].…”