Letter to the Editor Myocardial perfusion-fibrosis pattern in systemic sclerosis assessed by cardiac magnetic resonance ☆ Sophie Mavrogeni a, ⁎, Konstantinos Bratis a , Kees van Wijk d , Efthymios Stavropoulos c , David Hautemann d , Johan H.C. Reiber b , Genovefa Kolovou a a Onassis Cardiac Surgery Center, Athens, Greece b Leiden University Medical Center, Leiden, The Netherlands c Athens Naval Hospital, Athens, Greece d Medis medical imaging systems, Leiden, The Netherlands article info Article history: Received 3 December 2011 Accepted 17 December 2011 Available online 9 January 2012 Keywords: Scleroderma Perfusion cardiovascular magnetic resonance Myocardial fibrosis Coronary artery disease Systemic sclerosis (SSc) is a connective tissue disease character- ized by vascular and fibrotic lesions of skin and internal organs. Focal hypoperfusion contributes to myocardial fibrosis, documented by pathology. SSc heart involvement carries a poor prognosis (1–3). Stress echocardiography and single-photon emission computed tomography (SPECT) detect perfusion defects (PDs) (4–6). Stress car- diac magnetic resonance imaging (CMR) has been also applied to de- tect PDs in coronary artery disease (7,8). Our aim was to evaluate myocardial perfusion-fibrosis in SSc using CMR stress perfusion and late gadolinium enhancement (LGE). Seven asymptomatic SSc (2 M/5 F), aged 51 ± 2 yrs, and 2 limited (L)/5 diffuse (D), without pulmonary hypertension were evaluated. Exclusion criteria were: known CAD and CMR contraindications. SSc received 10 mg/day prednisone, without vasodilator or immunosup- pressive treatment. Biochemical and inflammatory indexes were nor- mal at examination time. Raynaud phenomenon was present in all, while digital ulcers in 5/7 (71%). SSc were compared with 7 controls and 5 patients with coronary artery disease (CAD). Written consent form was obtained from all. Stress CMR was performed by 1.5 T magnet using 140 mg/kg/min adenosine for 4 min (9–13). 0.1 mmol/kg Gd-DTPA was given during the first-pass perfusion sequence (IR balanced Turbo Field Echo,TR 2.8 ms, TE 1.38 ms, FA 45, slice thickness 8 mm, preparation pulse delay 200 ms). A rest perfusion was performed using the same proto- col. LGE was obtained 10 min later (3D-Turbo field echo sequence, TR 5.1 ms, TE 2.5 ms, FA 15, slice thickness 8 mm). Two readers, unaware of patients' profile, evaluated the images. A consensus was used for discordant grades. PDs were assessed by both visual and parametric analysis. Quantification was performed using delineation of endo and epicardial LV borders throughout the first-pass perfusion images (QMass MR, Medis medical imaging systems, Leiden, Netherlands). Stress and rest perfusion slopes were derived using Fermi-fitting of signal intensity vs time and normalized to LV blood pool slope. A Myocardial Perfusion Reserve Index (MPRI) was calculated for each patient, defined as ratio of stress to rest. Intra and inter-observer var- iability were 0.88 and 0.85, respectively. Nonsegmental, subendocardial perfusion defects were identified in all SSc and segmental, subendocardial in 3/5 CAD. The MPRI in SSc was lower compared to both CAD and controls (1.2 ± 0.5 vs 1.8±0.2 vs 2.46±0.3, p b 0.001). Although the lowest MPRI values were identified in diffuse SSc, reduced MPRI was also found in recent, limited SSc. LGE equal to 16% and 10.4 ± 2% of myocardial mass was assessed in 1/7 SSc and in 3/5 CAD, respectively. LGE in SSc was dif- fuse. However, in CAD, LGE followed the distribution of coronaries (LAD in our patients). Perfusion defects were seen in all SSc with Ray- naud and digital ulceration. Patient data are presented in Table 1 and representative images in Fig. 1. The frequency of CAD in SSc is similar to the general population. However, small arteries are involved more frequently than epicardial coronaries. The likelihood of coronary vasospasm is also higher. Pa- thology proved that the typical lesion is the impairment of microcir- culation, due to fibrinoid necrosis of intramural coronaries (14). Repeated ischemia–reperfusion leads to irreversible fibrosis, unre- lated to epicardial coronaries. Follansbee et al. (3) documented the presence of normal coronaries in SSc with stress PDs suggesting that microcirculation's resistance accounts for abnormal perfusion. PDs improved after nifedipine, nicardipine and captopril have been reported in SSc by SPECT, suggesting that these defects were revers- ible (11–13). International Journal of Cardiology 159 (2012) e56–e58 ☆ This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. ⁎ Corresponding author at: 50 Esperou Street, 175-61 P.Faliro, Athens, Greece. Tel./ fax: +30 210 98 82 797. E-mail address: soma13@otenet.gr (S. Mavrogeni). 0167-5273/$ – see front matter © 2011 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2011.12.039 Contents lists available at SciVerse ScienceDirect International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard