Abstract
Cardiac involvement in systemic sclerosis (SSc) is considerably frequent in autopsy, but the early identification is clinically difficult. Recent advantages in cardiac magnetic resonance (CMR) enabled to detect myocardial fibrotic scar as late gadolinium enhancement (LGE). We aimed to examine the prevalence and distribution of LGE in patients with SSc, and associate them with clinical features, electrocardiographic abnormalities and cardiac function. Forty patients with SSc (58 ± 14 years-old, 35 females, limited/diffuse 25/15, disease duration 106 ± 113 months) underwent serological tests, 12-lead electrocardiogram (ECG) and CMR. Seven patients (17.5 %) showed LGE in 26 segments of left ventricle (LV). LGE distributed mainly in the basal to mid inter-ventricular septum and the right ventricular (RV) insertion points, but involved all the myocardial regions. More patients with LGE showed NYHA functional class II and more (71 vs. 21 %, p < 0.05), bundle branch blocks (57 vs. 6 %, p < 0.05), LV ejection fraction (LVEF) < 50 % (72 vs. 6 %, p < 0.01), LV asynergy (43 vs. 0 %, p < 0.01) and RVEF < 40 % (100 vs. 39 %, p < 0.01). There was no difference in disease duration, disease types, or prevalence of positive autoimmune antibodies or high serum NT-proBNP level (>125 pg/ml). When cardiac involvement of SSc was defined as low LVEF, ECG abnormalities or high NT-proBNP, the sensitivity, specificity positive and negative predictive values of LGE were 36, 92, 71 and 72 %, respectively. We could clarify the prevalence and distribution of LGE in Japanese patients with SSc. The presence of LGE was associated with cardiac symptom, conduction disturbance and impaired LV/RV contraction.
Highlights
Systemic sclerosis (SSc) is characterized by vascular changes and fibrosis of the skin and internal organs
The presence of late gadolinium enhancement (LGE) was associated with cardiac symptom, conduction disturbance and impaired left ventricle (LV)/right ventricular (RV) contraction
There was no significant correlation between %LGE volume and left ventricular ejection fraction (LVEF), RVEF, LVEDVI or RVEDVI
Summary
Systemic sclerosis (SSc) is characterized by vascular changes and fibrosis of the skin and internal organs. Cardiac involvement in SSc was assumed to be derived from impairment of the microcirculation and primary myocardial fibrosis, and from ischemic damage due to coronary atherosclerosis [6,7,8]. Previous studies have suggested that tissue Doppler echocardiography is useful for detection of the depressed contractility [3], and serum N-terminal-pro brain natriuretic peptide (NT-proBNP) can be a surrogate marker of cardiac involvement [10]. The values of cardiac magnetic resonance (CMR) are suggested for the early detection of cardiac involvement in SSc. Cine-CMR can assess cardiac morphology and function with high spatial resolution, and late gadolinium enhancement (LGE)-CMR can differentiate fibrotic scar from normal myocardium [11, 12]. There are no such data in Asian patients, and only few studies have examined the values of LGE by comparison with echocardiographic findings and serum NT-proBNP level [13, 14]
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