Cardiac involvement in systemic sclerosis and the value of Late Gadolinium Enhancement (LGE) in Cardiac Magnetic Resonance (CMR)

Abstract

Background: Cardiac involvement in systemic sclerosis (SSc) affects the prognosis of the disease, but the early diagnosis is difficult due to the subclinical manifestation. We assessed the cardiac involvement in SSc using CMR, bio-markers and other imaging modalities. Methods: Thirty eight consecutive patients with SSc (58±13 years, M/F: 5/33, limited/diffuse: 23/15, disease duration: 5-516 (mean, 107) months) underwent CMR, 12-lead ECG, and echocardiogram. Results: (1) Fourteen patients were symptomatic (NYHA class II-IV), and 3 showed low left ventricular ejection fraction (LVEF<50%). Eight patients had conduction disturbance (3 atrio-ventricular block and 6 bundle branch block) and 3 had pulmonary arterial hypertension (PAH). The serum NT-pro BNP level ranged from 5 to 9506 pg/ml (mean 447 pg/ml), and the troponin I level was high in 12 patients (≥0.015 ng/ml). (2) Seven patients (18%) had LGE in LGE-CMR located at mid-ventricular septum (5 striated, 1 patchy, and 1 trans-mural patterns) (Figure). (3) Patients with LGE had higher NYHA classes and NT-proBNP levels, and longer QRS duration than those without LGE (1850±3449 vs. 79±45 pg/ml, and 106.6±28.5 vs 89.2±16.0 ms, p<0.05). (4) Patients with LGE also showed lower LVEF and right ventricular (RV) EF (47.6±16.5% vs. 63.8±8.4% and 24.7±5.8% vs. 42.9±10.5%, p<0.05), larger LA volume (23.2±2.0 vs. 30.8±7.1 ml/m2, p<0.05) and higher tricuspid regurgitation pressure gradient (33.3±7.1 vs. 23.0±5.4 mmHg, p<0.05). ![Figure][1] Representative CMR images Conclusions: Cardiac involvement in SSc was considerably frequent, and the presence of LGE correlated well with conduction disturbance, LV and RV functional impairment, and PAH. When a patient was symptomatic or showed a high NT-proBNP level, CMR should be examined for the early detection of cardiac involvement. [1]: pending:yes