Unique Pulmonary Hypertensive Vascular Diseases Associated with Heart and Lung Developmental Defects

Although pediatric pulmonary hypertension (PH) shares features and mechanisms with adult PH, there are also some significant differences between the two conditions. Segmental PH is a unique subtype of unclear and/or multifactorial pathophysiological mechanisms, often associated complex congenital heart disease (CHD), atresia ventricular septal defect, aortopulmonary collateral arteries. Some cases CHD, single ventricle after Fontan operation, show pathological changes in small peripheral arteries vascular resistance similar to those observed arterial (PAH). This condition termed as hypertensive (PPHVD). Recent advances genetics have identified genes responsible for PAH developmental defects lungs, such TBX4 SOX17. Targeted therapies been developed; however, their effects on lung remain be established. Real-world data analyses anatomy, pathophysiology, genetics, molecular biology PPHVD using nationwide international registries, should conducted order improve treatments prognosis patients these types PH.