Abstract #1371: Assessment of Heart Involvement in Systemic Sclerosis by Cardiac Magnetic Resonance Imaging in 50 Patients
Laura K. Hummers, MD
Authors:
A. Hachulla, D. Launay, V. Gaxotte, M. Lambert, S. Morell-Dubois, C. Lions, V. Queyrel, H Charlanne, P. Hatron, J. Beregi, E. Hachulla
Background:
This study examined cardiac MRI in unselected consecutive patients with scleroderma. Patients with known pulmonary or cardiac disease were not excluded.
Results:
Fifty consecutive patients were enrolled. The mean age was 43 years, 32 had limited cutaneous disease and 18 diffuse disease. They were mostly female (43/50) with a mean disease duration of 11 years. Subjects were systematically evaluated with MRI, PFT, echocardiogram and 19/50 patients also had a right heart catheterization (RHC). Sixteen patients had PAH (by cath), 24 had evidence of interstitial lung disease. None of the subjects without PAH had any clinical evidence of heart involvement. MRI imaging was performed with a 1.5 Tesla magnet and T2, Cine, perfusion and delayed enhancement imaging was obtained. 78% of subjects had some abnormality by MRI (excluding valvular lesions). 12% of patients had increase in T2 signal intensity suggestive of inflammation. 26% had thinning of the myocardium possibly suggestive of microvascular insults. 6% had left ventricular dilatation and 20% had right ventricular dilatation. Another 20% had delayed contrast enhancement which was linear in appearance possibly consistent with fibrosis. Global and segmental hypokinesis was seen in 4 and 28% of subjects respectively and 18% had pericardial effusions. No perfusion abnormalities were noted. Diastolic dysfunction was noted in 30% of patients. No difference in abnormalities were seen when subjects with limited and diffuse disease were compared except those with diffuse disease had more right ventricular hypertrophy. No difference was noted when those with and without PAH were compared. Those with longer disease duration were noted to have more frequent delayed enhancement.
Editorial Comment:
The heart has been somewhat of a black box in scleroderma. While most cardiac involvement is subclinical, the development of clinically apparent heart involvement is associated with a very poor prognosis. A greater understanding of the pathologic processes in the heart in scleroderma could advance treatment options for those with symptomatic disease and we may learn more how “asymptomatic” abnormalities may be contributing to other pathologic processes such as pulmonary hypertension. As our assessment tools become more sophisticated, our capability to non-invasively assess the heart will greatly improve. There will need to be further validation of the abnormalities noted by MRI with pathology. This study was also limited by a lack of a control group. However, this study is an early indicator that MRI may be a valuable tool to study the heart in scleroderma.
