Background/Purpose: To determine the prevalence of echocardiographic abnormalities and to identify associated clinical and laboratory features in a large systemic sclerosis (SSc) cohort.

Methods: The sample comprised 117 patients with SSc (ACR/EULAR 2013 criteria) treated at a tertiary university hospital that does not attend pediatric populations. In the routine clinical management of these patients, a transthoracic Doppler echocardiogram (TTE) is performed every 1 or 2 years. In each study, we investigated the presence of myocardial systolic dysfunction, diastolic myocardial dysfunction, valve disease, and pulmonary arterial hypertension (PAH) defined as a systolic pulmonary artery pressure (PAP) ≥ 40 mmHg and tricuspid regurgitation velocity (TRV) greater than 2.5 m/s.

Results: The sample included 104 women (89%) and 13 (11%) were men, with a mean age of 59 ± 15 years (mean ± standard deviation; range 19–86) and a median disease duration of 6.5 years (interquartile range [IQR], 25th-75th: 3–14 yrs). Twenty one patients (18%) had diffuse cutaneous scleroderma, eighty-nine (76%) had limited cutaneous involvement, 1 (0.8%) had a systemic sclerosis sine scleroderma, and six (5%) had prescleroderma.

The main TTE findings observed were:

– Left ventricular (LV) systolic dysfunction was present in 5.1% (6/117) of subjects, whereas LV diastolic dysfunction was present in 11.1% (13/117). Factors independently associated with LV diastolic dysfunction on multivariable analysis included arterial hypertension, disease duration, advanced age, and the limited cutaneous SSc subtype.

– Subclinical valve disease: 10.2% (12/117) of patients had valve dysfunction involving the mitral and/or the aortic valves. Valve dysfunction was mild or moderate in all cases, except in 3 patients (2.56%) who presented severe aortic stenosis. The age of these three patients were 52, 59 and 78 yrs. Antiphospholipid (APL) antibodies were positive in only 1 of these patients.

– TTE criteria for PAH: 11.1% (13/117). Right heart catheterization (RHC) confirmed the diagnosis of PAH in all cases except one (false positive). The median disease duration at PAH diagnosis was 6 years (IQR: 1.5 -14.5 yrs). Factors independently associated with PAH on multivariable analysis included the limited cutaneous SSc subtype and more severe peripheral vascular disease (Raynaud’s phenomenon and digital ulcers).

Conclusion: In addition to the presence of PAH, it is also relatively common the presence of LV diastolic dysfunction (11.1%) and subclinical valve disease (10.2%).

The high frequency of severe aortic stenosis observed in our cohort (its prevalence in the general population is 0.3-0.5%) deserves a case-control study to investigate whether this complication is related or not to the activity of the disease.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/prevalence-of-echocardiographic-abnormalities-in-patients-with-systemic-sclerosis/