Early assessment of sub-clinical cardiac involvement in systemic sclerosis (SSc) using delayed enhancement cardiac magnetic resonance (CE-MRI)

Objectives

Systemic sclerosis heart involvement (SHI) is one of systemic sclerosis (SSc) most frequent complications, both in diffuse (dcSSc) and limited (lcSSc) cutaneous forms of disease. Nowadays, SHI is a major factor decreasing SSc survival rate because, when clinically evident, is associated with 70% of mortality at 5 years. SHI shows different forms, primary and/or secondary. Primary myocardial SHI is characterized by fibrosis. Aim of our study is to assess the presence and pattern of fibrosis as detected by cardiac magnetic resonance (CMR) in systemic sclerosis.

Methods

In this study, we used CE-MRI (contrast enhanced-MRI) in 58 female SSc patients. Images were evaluated to obtain functional parameters and to see presence, location and pattern (nodular, linear or diffuse) of myocardial LE, sign of fibrosis. CE-MRI findings were correlated with patients clinical setting.

Results

Myocardial fibrosis was detected in 25 of 58 patients (43%). The main finding observed in 16 of these 25 patients was a late enhancement showing a linear pattern, without coronary distribution and sparing the sub-endocardial myocardial layers. A patchy nodular enhancement pattern was observed in 9 patients (36%). Patients with linear pattern presented dcSSc, on the contrary patients with nodular LE displayed the lcSSc form.

Conclusions

This study shows that CE-MRI is a reliable technique to detect SHI earlier than other methods. SHI increase passive myocardial stiffness, proportional to collagen deposition degree, leading to cardiac remodelling with possible development of heart failure, even with normal ejection fraction. An early treatment of SHI might improve SSc patients outcome.