Subclinical progression of systemic sclerosis-related cardiomyopathy

Abstract

Aims Cardiac involvement in patients with systemic sclerosis (SSc) is frequent and represents a negative prognostic factor. Recent studies have described subclinical heart involvement of both the right ventricle (RV) and left ventricle (LV) via speckle-tracking-derived global longitudinal strain (GLS). It is currently unknown if SSc-related cardiomyopathy progresses through time. Our aim was to assess the progression of subclinical cardiac involvement in patients with SSc via speckle-tracking-derived GLS. Methods This was a prospective longitudinal study enrolling 72 consecutive patients with a diagnosis of SSc and no structural heart disease nor pulmonary hypertension. A standard echocardiographic exam and GLS calculations were performed at baseline and at follow-up. Results Traditional echocardiographic parameters did not differ from baseline to 20-month follow-up. LV GLS, despite being already impaired at baseline, worsened significantly during follow-up (from –19.8 ± 3.5% to –18.7 ± 3.5%, p = .034). RV GLS impairment progressed through the follow-up period (from –20.9 ± 6.1% to –18.7 ± 5.4%, p = .013). The impairment was more pronounced for the endocardial layers of both LV (from –22.5 ± 3.9% to –21.4 ± 3.9%, p = .041) and RV (–24.2 ± 6.2% to –20.6 ± 5.9%, p = .001). A 1% worsening in RV GLS was associated with an 18% increased risk of all-cause death or major cardiovascular event ( p = .03) and with a 55% increased risk of pulmonary hypertension ( p = .043). Conclusion SSC-related cardiomyopathy progresses over time and can be detected by speckle-tracking GLS. The highest progression towards reduced deformation was registered for the endocardial layers, which supports the hypothesis that microvascular dysfunction is the main determinant of heart involvement in SSc patients and starts well before overt pulmonary hypertension.

Editors’ presentation: Focus on cardiomyopathy and heart failure

“…In this issue of the Journal, Stronati et al investigate the progression of subclinical biventricular cardiac involvement in patients with SSc using speckletracking-derived GLS. 12 At follow-up, conventional echocardiographic parameters did not differ from baseline, but both LV GLS and right ventricle (RV) GLS deteriorated significantly in the absence of overt fibrosis in cardiac magnetic resonance. Moreover, among patients who have experienced major clinical events during follow-up, the data showed a faster progression of RV GLS impairment.…”

Cardiovascular Imaging for Systemic Sclerosis Monitoring and Management

“…Systemic sclerosis patients also show differences in strain imaging via echocardiography as compared to controls. Studies have shown both regional and global LV longitudinal systolic strain (LVLSS) is significantly lower in SSc patients than controls and is most pronounced in the endocardial layer of the LV ( 56 , 57 ). Furthermore, SSc patients with lower global LVLSS were more likely to have elevated C-reactive protein values suggesting greater inflammation.…”

CHLD score, a new score based on traditional risk factor evaluation and long-term cardiovascular outcomes in patients with systemic sclerosis

“…Also, acute myocardial infarction (AMI) in patients with SSc was shown to be associated with higher risk of recurrent adverse cardiovascular events and death, but not bleeding in comparison to other patients with AMI 9 . Of note, patients with SSc and adverse cardiovascular events had much worse prognosis than patients with other systemic connective tissue diseases, such as rheumatoid arthritis or systemic lupus erythematosus 10 The cardiac involvement in SSc is thought to be related to microvascular dysfunction and resultant subclinical systolic dysfunction reflected by impaired left ventricular (LV) global longitudinal strain, which even precede development of pulmonary hypertension 11 . Patients with cardiovascular incidents were characterized by cardiac magnetic resonance alterations, such as early and late gadolinium enhancement 12 .…”

“…The cardiac involvement in SSc is thought to be related to microvascular dysfunction and resultant subclinical systolic dysfunction reflected by impaired left ventricular (LV) global longitudinal strain, which even precede development of pulmonary hypertension 11 . Patients with cardiovascular incidents were characterized by cardiac magnetic resonance alterations, such as early and late gadolinium enhancement 12 .…”