BMPR2 and pulmonary arterial hypertension: ECM changes in PH were discussed by Jandl et al. primarily in relation to PAH42 and were demonstrated in pulmonary hypertension due to left heart disease (PH-LHD),43,44 PH associated with chronic lung diseases.45–47 There is a correlation between BMPR2 abnormalities and the remodeling of specific ECM components.