Cardiovascular magnetic resonance patterns of biopsy proven cardiac involvement in systemic sclerosis

Background: to determine morphological and functional cardiovascular magnetic resonance (cmr) patterns in histopathologically confirmed myocardial involvement in patients with systemic sclerosis (ssc). methods: twenty patients (6 females; mean age 41 ± 11 years) with histopathologically proven cardiac involvement in ssc in the years 2008-2016 were retrospectively evaluated. morphological,functional and late gadolinium enhancement (lge) images were acquired in standard angulations at 1.5 t cmr. pathologies were categorized: 1) pericardial effusion; 2) pathologic left (lv) or right ventricular (rv) contractility (hypokinesia,dyssynchrony,and diastolic restriction); 3) reduced left (lv-ef) and right ventricular ejection fraction (rv-ef); 4) fibrosis and/or inflammation (positive lge); 5) rv dilatation. 95 % confidence intervals (ci) were calculated for appearance of pathologic ef and rv dilatation. results: seven patients (35 %) had positive cmr findings in three categories,9 patients (45 %) in four categories and 4 patients (20 %) in five categories. the distribution of pathologic findings was: minimal pericardial effusion in 7 patients (35 %),moderate pericardial effusion >5 mm in nine patients (45 %); abnormal lv or rv contractility in 19 patients (95 %),reduced lv or rv function in 14 patients (70 %; 95 % ci: 51-88 %),pathologic lge in all patients,rv dilatation in 6 patients (30 %; 95 % ci: 15-54 %). conclusions: cmr diagnosis of myocardial involvement in ssc requires increased attention to subtle findings. pathologic findings in at least three of five categories indicate myocardial involvement in ssc. © 2016 the author(s).