Myocardial Work Evaluation in Patients With PAH

Pulmonary arterial hypertension (PAH) is a rare hemodynamic disease characterized by pre-capillary pulmonary hypertension (PH), due to pulmonary vascular remodeling. Despite the recent development of effective therapies, right ventricular (RV) function remains a key determinant of survival as right heart failure remains the main cause of death among patients with PAH. According to ESC/ERS guidelines, RV function should be evaluated in patients with PAH at baseline (by echocardiography or cardiac MRI) to assess one-year mortality risk, thereby impacting on patient treatment. However, few RV function markers address both the systolic function of the RV and its afterload (i.e., RV-pulmonary artery coupling), which seems necessary in PAH, where the disease is characterized by a progressive increase in pulmonary artery pressures and subsequently, an alteration in RV systolic function. Noninvasive myocardial work is a promising new tool for the evaluation of RV systolic function developed to help differentiate reduced myocardial performance due to increased afterload versus reduced myocardial contractibility. It was first developed for left ventricular assessment but can be adapted to the RV using pressure-strain loops. Very few data on its use on RV function are available but the results are promising. However, its value in patients with PAH and its evolution after PAH treatment initiation has never been evaluated. Thus, the aim of this article is to evaluate the association of RV myocardial work parameters at baseline and at first reevaluation with estimated one-year mortality risk in patients with PAH.