Summary Purpose Patients with pulmonary arterial hypertension (PAH) due to systemic sclerosis (SSc) have high mortality. Left ventricular (LV) peak global longitudinal strain (GLS) is decreased in SSc. It is unknown whether low GLS is due to SSc or PAH. Therefore, our primary aim was to evaluate both LV and right ventricular free wall GLS (RVFW GLS) in SSc, with and without PAH, using cardiac magnetic resonance with feature tracking. Secondary aim was to relate GLS to invasive mean pulmonary arterial pressure (mPAP) and pulmonary vascular resistance (PVR). Methods Thirty‐eight patients with SSc, 19 patients with SSc‐PAH and 19 healthy controls for comparison, were included. Endocardial and epicardial borders were delineated in cine images (short‐axis stack and three long‐axis views) for volumetric and strain calculations. Results Systemic sclerosis‐PAH had lower LV and RVFW GLS than SSc (LV: P = 0·01, RV: P<0·001) and controls (LV: P = 0·02; RV: P<0·001), with no difference between SSc and controls. LV strain correlated with mPAP (R = 0·42, P = 0·03) and PVR (R = 0·52, P = 0·006). RVFW GLS correlated with mPAP (R = 0·68, P<0·001) and PVR (R = 0·59, P = 0·001). ROC curves for predicting PAH had AUC 0·73 for LV strain (P = 0·003) and 0·86 for RVFW GLS (P<0·001). Conclusions Lower GLS is mainly determined by increased pulmonary pressure and not by SSc per se. Low LV and RVFW GLS are indicative of increased mPAP and PVR, which opens for improved non‐invasive methods to select patients eligible for right heart catheterization and to monitor the effects of PAH therapy.

Background Impaired myocardial deformation has been sporadically described in cardiac asymptomatic systemic sclerosis (SSc). We aimed to study myocardial deformation indices in cardiac asymptomatic SSc patients using cardiac magnetic resonance feature tracking (CMR-FT) and correlate these findings to the phenotypic and autoimmune background. Methods Fifty-four cardiac asymptomatic SSc patients (44 females, 56±13 years), with normal routine cardiac assessment and CMR evaluation, including cine and late gadolinium enhancement (LGE) images, were included. SSc patients were compared to 21 sex- and age- matched healthy controls (17 females; 54±19 years). For CMR-FT analysis, a mid-ventricular slice for LV peak systolic radial and circumferential strain and a 4-chamber view for LV/RV peak systolic longitudinal strain were used. Results Twenty-four patients had diffuse cutaneous SSc and 30 limited cutaneous SSc. Thirteen patients had digital ulcers. Median disease duration was 3.6 years. LV ejection fraction was higher in SSc patients compared to controls (62±6% vs. 59±5%, p = 0.01). Four patients had no LGE examination; in the remaining patients LGE was absent in 74%, while 18% had RV insertion fibrosis and 8% evidence of subendocardial infarction. LV longitudinal strain differed in those with insertion fibrosis (-18.0%) and infarction (-16.7%) compared to no fibrosis (-20.3%, p = 0.04). Patients with SSc had lower RV longitudinal strain and strain rate compared to controls (p<0.001 and p = 0.01, respectively). All other strain and strain rate measurements were non-significant between patients and controls. Conclusions In cardiac asymptomatic SSc patients with normal routine functional indices, CMR-FT identifies subclinical presence of insertion fibrosis and/or myocardial infarction by impaired LV longitudinal strain. RV derived longitudinal indices were impaired in the patient group. CMR FT indices did not correlate to the patients’ phenotypic and autoimmune features.

Background: Right ventricular (RV) function is a major determinant of outcome in patients with pulmonary hypertension. Cardiac magnetic resonance (CMR) is gold standard to assess RV ejection fraction (RVEF CMR), however this is a crude measure. New CMR measures of RV function beyond RVEF CMR have emerged, such as RV lateral atrioventricular plane displacement (AVPD lat), maximum emptying velocity (S' CMR), RV fractional area change (FAC CMR) and feature tracking of the RV free wall (FWS CMR). However, it is not fully elucidated if these CMR measures are in parity with the equivalent echocardiography-derived measurements: tricuspid annular plane systolic excursion (TAPSE), S'-wave velocity (S' echo), RV fractional area change (FAC echo) and RV free wall strain (FWS echo). The aim of this study was to compare regional RV function parameters derived from CMR to their echocardiographic equivalents in patients with pulmonary hypertension and to RVEF CMR. Methods: Fifty-five patients (37 women, 62 ± 15 years) evaluated for pulmonary hypertension underwent CMR and echocardiography. AVPD lat , S' CMR , FAC CMR and FWS CMR from cine 4-chamber views were compared to corresponding echocardiographic measures and to RVEF CMR delineated in cine short-axis stack. Results: A strong correlation was demonstrated for FAC whereas the remaining measurements showed moderate correlation. The absolute bias for S' was 2.4 ± 3.0 cm/s (relative bias 24.1 ± 28.3%), TAPSE/AVPD lat 5.5 ± 4.6 mm (33.2 ± 25.2%), FWS 4.4 ± 5.8% (20.2 ± 37.5%) and for FAC 5.1 ± 8.4% (18.5 ± 32.5%). In correlation to RVEF CMR, FAC CMR and FWS echo correlated strongly, FAC echo , AVPD lat , FWS CMR and TAPSE moderately, whereas S' had only a weak correlation.