BMPR2 and pulmonary arterial hypertension: Among the disease-specific factors, it is worth mentioning that BMPR2 mutation carriers and scleroderma patients with PAH display reduced afterload-adjusted RV ejection fraction [42] and depressed sarcomeric function [43], respectively, compared to patients with idiopathic PAH, whereas patients with Eisenmenger syndrome have higher RV contractility and less diastolic stiffness in comparison to those with idiopathic PAH [44].