Cardiac tissue characterization and the diagnostic value of cardiovascular magnetic resonance in systemic connective tissue diseases.

OBJECTIVE: Accurate diagnosis of cardiovascular involvement in connective tissue diseases (CTDs) remains challenging. We hypothesized that cardiovascular magnetic resonance (CMR) demonstrates cardiac lesions in symptomatic CTD patients with normal echocardiography. METHODS: CMR from 246 CTD patients with typical cardiac symptoms (TCS; n = 146, group A) or atypical cardiac symptoms (ATCS; n = 100, group B) was retrospectively evaluated. Group A included 9 patients with inflammatory myopathy (IM), 35 with sarcoidosis, 30 with systemic sclerosis (SSc), 14 with systemic lupus erythematosus (SLE), 10 with rheumatoid arthritis (RA), and 48 with small vessel vasculitis. Group B included 25 patients with RA, 20 with SLE, 20 with sarcoidosis, 15 with SSc, 10 with IM, and 10 with small vessel vasculitis. CMR was performed by 1.5T; left ventricular ejection fraction, T2 ratio (edema imaging), and late gadolinium enhancement (LGE; fibrosis imaging) were evaluated. Acute and chronic lesions were characterized as LGE positive plus T2 ratio >2 and T2 ratio ≤2, respectively. According to LGE, lesions were characterized as diffuse subendocardial, subepicardial, and subendocardial/transmural due to vasculitis, myocarditis, and myocardial infarction, respectively. A stress study by dobutamine echocardiography or stress, nuclear, or adenosine CMR was performed in CTD patients with negative rest CMR. RESULTS: Abnormal CMR was identified in 32% (27% chronic) and 15% (12% chronic) of patients with TCS and ATCS, respectively. Lesions due to vasculitis, myocarditis, and myocardial infarction were evident in 27.4%, 62.6%, and 9.6% of CTD patients, respectively. Stress studies in CTD patients with negative CMR revealed coronary artery disease in 20%. CONCLUSION: CMR in symptomatic CTD patients with normal echocardiography can assess disease acuity and identify vasculitis, myocarditis, and myocardial infarction.

Cardiovascular magnetic resonance imaging in asymptomatic patients with connective tissue disease and recent onset left bundle branch block.

BACKGROUND-AIM: Recent LBBB in connective tissue diseases (CTDs) is challenging, due to high incidence of underlying pathology that may remain undetected, due to limitations of imaging tests. We hypothesized that cardiovascular magnetic resonance (CMR) may be of diagnostic value in CTDs with recent LBBB and normal echocardiogram. PATIENTS-METHODS: 26 CTDs, aged 32 ± 7 yrs (19 F) and 26 controls without CTDs, aged 60 ± 4 yrs (10 F) with recent LBBB and normal echo were evaluated by CMR. The CTDs included 6 sarcoidosis (SRC), 4 systemic sclerosis (SSc), 6 systemic lupus erythematosus (SLE), 6 rheumatoid arthritis (RA) and 4 inflammatory myopathies (IM). CMR was performed by 1.5T. LVEF, T2 ratio (oedema imaging) and late gadolinium enhancement (LGE) (fibrosis imaging) were evaluated. Acute and chronic lesions were characterised by T2>2 and positive LGE and T2<2 and positive LGE, respectively. According to LGE, lesions were characterised as diffuse subendo-, subepicardial/intramural not following and subendocardial/transmural following the distribution of coronaries, indicative of vasculitis, myocarditis and myocardial infarction, respectively. RESULTS: CTDs were younger (p<0.001), with higher incidence of abnormal CMR (42.31 vs 30.77%, p=NS), including dilated cardiomyopathy (11.54%), diffuse subendocardial fibrosis (11.54%), myocardial infarction (7.69%) and acute myocarditis (11.54%) vs dilated cardiomyopathy (19.23%), myocardial infarction (7.69%) and acute myocarditis (3.85%), detected in non-CTDs. CONCLUSIONS: In CTDs with recent LBBB, CMR documented acute and chronic cardiac pathology, particularly myocarditis. CMR should be considered as an adjunct to conventional diagnostic workup in both patient groups, more so in CTDs.