Survival is improved in pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) patients and is related to right ventricular (RV) function. In this study we aimed to investigate the prognostic role of RV mechanics in Eisenmenger Syndrome (ES) compared with other causes of PAH using speckle-tracking imaging.

We performed a prospective cohort study. We recruited 80 consecutive adult PAH patients: 39 patients with ES and noncomplex CHD (mean age 41,5±15,6 years) and 41 patients with group 1 PAH (mean age 58,9±17,8). Clinical assessment, BNP and echocardiography were realised at the time of inclusion and then clinical outcomes were collected during the regular follow-up. Normal strain values were obtained from 37 control volunteers. On inclusion, 53 patients (66.3%) were in functional class III or higher. Mean BNP was lower in ES (p<0,001) but TAPSE (p=0,6) and peak RV longitudinal strain (p=0,9) were similar in both groups. Global longitudinal LV strain was midly reduced in ES (–16,2±5 vs –17,9±8%, p=0,03), whereas no difference was found for circumferential strain. Mean RV transverse strain was higher in ES patients (27,3±17 vs 14±11%; p<0,001) but still reduced in comparison with normal values (44,9±16%, p<0,001). Whereas normal RVs present a base to apex transverse strain gradient, ES patients more frequently present a predominant apical function (p<0,001). Over a median follow-up of 20,6 months, 32 patients were hospitalized for PAH and 16 died from cardio-pulmonary causes. On multivariate survival analysis (including age, NYHA class and advanced therapies), peak transverse strain (HR 0.92 [0.87–0.98]) and CHD (HR 0.17 [0.05–0.6]) were associated and were the strongest predictors of event-free survival.

The increased RV transverse strain and predominant apical function in ES could explain their survival advantage as transverse strain is a strong independent predictor of outcomes in PAH.