Enlarged right-sided dimensions and fibrosis of the right ventricular insertion point on cardiovascular magnetic resonance imaging is seen early in patients with pulmonary arterial hypertension associated with connective tissue disease.

OBJECTIVES To describe the findings of cardiovascular magnetic resonance (CMR) imaging in patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD) and in consecutive patients with systemic sclerosis (SSc) without PAH. METHODS The study comprised nine consecutive patients who were admitted for right heart catheterization (RHC) under a suspicion of CTD-PAH and 25 consecutive patients who were admitted for evaluation because of a clinical suspicion of SSc. In addition to the regular assessment, they also underwent examination by CMR. RESULTS CMR measurements of right ventricular (RV) volumes and function showed severe pathology in patients with CTD-PAH. Patients with SSc without PAH had similar but much less severe findings. Right ventricular end-diastolic volume (RVEDV) and right ventricular ejection fraction (RVEF) were abnormal in all patients with CTD-PAH. In eight out of nine patients with CTD-PAH, fibrosis was seen in the RV insertion point, probably caused by increased tension, but only in one of the consecutive SSc patients. This patient was diagnosed with CTD-PAH 20 months later. CONCLUSIONS In CTD-PAH, CMR shows severe changes in RV volumes and function, but also fibrosis in the RV insertion point. Similar abnormalities, although much less severe, may be seen at diagnosis of SSc. Further evaluation is warranted to determine whether these findings are of value in screening for early signs of PAH in SSc.