Background—: Cardiac involvement in systemic sclerosis (scleroderma [SSc]) adversely affects long-term prognosis, often remaining undetectable despite close clinical examination and 2-dimensional echocardiographic monitoring. Speckle-derived strain of the right ventricle (RV) was utilized to detect occult abnormalities in regional and global contractility in SSc patients. Methods and Results—: A total of 138 SSc patients with technically adequate echocardiograms was studied and compared with 40 age- and sex-matched healthy non-SSc controls. Standard assessment of RV chamber function included tricuspid annular plane systolic excursion and fractional area change. RV longitudinal systolic speckle-derived strain was assessed in the basal, mid, and apical free wall. Tricuspid annular plane systolic excursion was not different between groups ( P =0.307). Although fractional area change was lower in SSc patients than in controls (mean, 48.9 versus 55; P =0.002), the mean fractional area change was still within the normal range (>35). In contrast, RV longitudinal systolic speckle-derived strain measures were significantly different between groups, both globally (−20.4% versus −17.7%; P =0.005) and regionally: they were decreased in the apex (−8.5% versus −17.1%; P <0.0001) and mid segments (−12.4% versus -20.9%; P <0.0001), and increased in the base (−32.2% versus −23.3%; P =0.0001) for the SSc group. The regional difference in the base compared with the apex was significantly greater for SSc than for controls ( P <0.0001 for interaction). The differences observed in regional strain between SSc and control were unchanged after adjusting for RV systolic pressure. Conclusions—: Speckle-derived strain reveals a heterogenous pattern of regional heart strain in SSc that is not detected by conventional measures of function, suggestive of occult RV myocardial disease.

Question addressed Echocardiography is not currently considered as providing sufficient prognostic information to serve as an integral part of treatment goals in pulmonary arterial hypertension (PAH). We tested the hypothesis that incorporation of multiple parameters reflecting right heart function would improve the prognostic value of this imaging modality. Methods and Main Results We pooled individual patient data from a total of 517 patients (mean age 52±15 years, 64.8% females) included in seven observational studies conducted at five European and United States academic centers. Patients were subdivided into three groups representing progressive degrees of RV dysfunction based on a combination of echocardiographic measurements: group 1 (low-risk): normal tricuspid annular plane systolic excursion (TAPSE) and non-significant tricuspid regurgitation (TR) (n=129); group 2 (intermediate-risk): normal TAPSE and significant TR or impaired TAPSE and non-dilated inferior vena cava (IVC) (n=256); group 3 (high-risk): impaired TAPSE and dilated IVC (n=132). The 5-year cumulative survival rate was 82% in group 1, 63% in group 2, and 43% in group 3. Low-risk patients had better survival rates than intermediate-risk patients (log-rank χ²: 12.25 p<0.001) and intermediate-risk patients had better survival rates than high-risk patients (log-rank χ²: 26.25 p<0.001). Inclusion of other parameters such as right atrial area and pericardial effusion did not provide added prognostic value. Answer to the question The proposed echocardiographic approach integrating the evaluation of TAPSE, TR grade and IVC is effective in stratifying the risk for all-cause mortality in PAH patients, outperforming the prognostic parameters suggested by current guidelines.

Autoimmune rheumatic diseases (ARDs) involve multiple organs including the heart and vasculature. Despite novel treatments, patients with ARDs still experience a reduced life expectancy, partly caused by the higher prevalence of cardiovascular disease (CVD). This includes CV inflammation, rhythm disturbances, perfusion abnormalities (ischaemia/infarction), dysregulation of vasoreactivity, myocardial fibrosis, coagulation abnormalities, pulmonary hypertension, valvular disease, and side-effects of immunomodulatory therapy. Currently, the evaluation of CV involvement in patients with ARDs is based on the assessment of cardiac symptoms, coupled with electrocardiography, blood testing, and echocardiography. However, CVD may not become overt until late in the course of the disease, thus potentially limiting the therapeutic window for intervention. More recently, cardiovascular magnetic resonance (CMR) has allowed for the early identification of pathophysiologic structural/functional alterations that take place before the onset of clinically overt CVD. CMR allows for detailed evaluation of biventricular function together with tissue characterization of vessels/myocardium in the same examination, yielding a reliable assessment of disease activity that might not be mirrored by blood biomarkers and other imaging modalities. Therefore, CMR provides diagnostic information that enables timely clinical decision-making and facilitates the tailoring of treatment to individual patients. Here we review the role of CMR in the early and accurate diagnosis of CVD in patients with ARDs compared with other non-invasive imaging modalities. Furthermore, we present a consensus-based decision algorithm for when a CMR study could be considered in patients with ARDs, together with a standardized study protocol. Lastly, we discuss the clinical implications of findings from a CMR examination.

Diastolic dysfunction (DD) identified on echocardiography predicts mortality after cardiac surgery, however the most useful diastolic parameters for assessment and the association of DD with prolonged mechanical ventilation, ICU re-admission, and hospital length of stay are not established. We included patients that underwent coronary artery bypass grafting (CABG), aortic valve replacement (AVR) or a combined procedure (CAB-AVR) from 2010 to 2016, and who had preoperative transthoracic echocardiography (TTE) at our institution within 6 months of the operation. Diastolic function was graded using the transmitral E and A waves and the septal tissue Doppler velocity. We performed logistic regression to assess the association of grade of DD with a composite endpoint of death, prolonged mechanical ventilation, ICU readmission during hospitalization, and hospital length of stay longer than 14 days. Between 2010 and 2016, 577 patients were eligible for inclusion. DD was common, with 42% of the cohort manifesting grade II or grade III DD. Rates of death and prolonged ventilation increased across grades of DD and across quartiles of increasing LV filling pressure, assessed by the E/e’ ratio. Adjusting for age, sex, procedure, systolic and diastolic function, both systolic (odds ratio 0.68 95% CI 0.55–0.85 per inter-quartile increase in LVEF) and diastolic function (odds ratio 1.31 95% CI 1.04–1.66 per increasing DD grade) both independently predicted outcome. Diastolic dysfunction is common among patients undergoing cardiac surgery and is associated with death, prolonged mechanical ventilation, and prolonged hospital and ICU length of stay independent of systolic dysfunction.