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

Abstract

BackgroundTo determine morphological and functional cardiovascular magnetic resonance (CMR) patterns in histopathologically confirmed myocardial involvement in patients with systemic sclerosis (SSc).MethodsTwenty 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.ResultsSeven 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 %).ConclusionsCMR 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.Electronic supplementary materialThe online version of this article (doi:10.1186/s12968-016-0289-3) contains supplementary material, which is available to authorized users.

Mid-term development of the right ventricle in competitive athletes

“…This evaluation has not the same quantitative value as T 1 mapping and does not replace T 1 mapping but gives an overview over potential diffuse myocardial contrast agent uptake. We have not found pathologically diffuse contrast agent uptake in our study cohort, whereas these findings are common in cardiac involvement of systemic and storage diseases (18).…”

Early myocardial damage and microvascular dysfunction in asymptomatic patients with systemic sclerosis: A cardiovascular magnetic resonance study with cold pressor test

“…We found a different prevalence of patients with isolated nT1 elongation (30%, in most cases with concomitant increase in the T2 value) and combined nT1/ECV increase (42.5%). These tissue signal abnormalities could express a combination of low grade inflammation (increase of nT1 and T2) and diffuse myocardial fibrosis (expanded ECV) [ 8 , 30 ], that might precede the contractile impairment occurring at late stage of SSc-related cardiopathy[ 30 ].…”

Update on assessment and management of primary cardiac involvement in systemic sclerosis

“…In a recent large study, diastolic dysfunction was more predominant than systolic, and several factors such as primary heart disease, vascular lung disease, and PAH contributed to it (82). Also, other studies have shown reduced RV ejection fraction and RV dilatation (77), further enhancing the notion of a primary RV impairment in SSc. Altered RV ejection fraction has been reported as a poor prognostic factor in PAH and other clinical conditions, such as heart failure, reduced LVEF, or ischemic cardiomyopathy (85).…”