Myocardial perfusion defects in scleroderma detected by contrast-enhanced cardiovascular magnetic resonance

The authors investigated whether contrast-enhanced cardiovascular magnetic resonance (CMR) imaging may be used to detect early cardiac involvement in patients with systemic sclerosis (SSc).Twenty-six SSc patients (nine with diffuse cutaneous SSc and 17 with limited cutaneous SSc) and 13 sex- and age-matched healthy controls (HC) were studied. Contrast-enhanced CMR allowed the analysis of first-pass images (areas of hypo-enhancement indicating perfusion defects) and delayed images (persistent hyper-enhancement indicating fibrosis). Clinical variables including disease duration and presence of major visceral complications of SSc were investigated in each patient.Perfusion defects were detected in 53.8 \% of SSc patients but in none of the HC. Perfusion abnormalities were detected in 28.6 \% of SSc patients with disease duration less than 2 years and in 29.2 \% of asymptomatic SSc patients. Delayed contrast enhancement was present in 25 \% of SSc patients but not in HC. All patients with delayed contrast enhancement showed first-pass hypoperfusion. Right ventricular wall thickness was significantly increased in all SSc patients when compared to HC (p < 0.001); a similar trend was observed when SSc patients without pulmonary arterial hypertension were analysed (p < 0.04). A trend to lower end-diastolic and end-systolic right ventricular volumes in SSc versus HC was observed (p < 0.05 and p < 0.04, respectively).Myocardial hypoperfusion is common in SSc and occurs early in the course of the disease. Co-localisation of perfusion defects and delayed contrast enhancement indicative of fibrosis suggests that myocardial hypoxia may play a role in the pathogenesis of myocardial fibrosis.