Table 3.

Clinical and Hemodynamic Characteristics of Major Etiologies of Pediatric Pulmonary Hypertension

	Population/risk factors/clinical features	Echocardiography findings	Treatment and prognosis
Idiopathic and hereditary PAH (IPAH/HPAH)	IPAH – PAH with no disease known to be assocated with it HPAH – Familial history of PAH or known genetic mutation associated with PAH	mPAP > 20 mmHg, PAWP or LVEDP ≤ 15 mmHg, PVRI ≥ 3Wu×m2	Pulmonary vasodilator therapy
PAH-CHD	ASD, VSD, PDA, AV canal defects, TGA, Eisenmenger’s syndrome, single ventricle physiology	Elevated PVRI in addition to presence of shunt lesions with affect pulmonary and systemic flow	Catheterization to perform AVT and assess for operablity, selective pulmonary vasodilators, surgical repair
BPD-PH	Prematurity, low birthweight, growth restriction, mechanical ventilation	Elevated PVR and TRJV, flattened IVS, RV hypertrophy, suspicion for PVS should trigger CTA or cardiac catheterization	Oxygen, ventilatory management, selective pulmonary vasodilators
PPHN	Neonatal population, meconium aspiration, maternal SSRIs, pneumonia, RDS, CDH, pulmonary hypoplasia, renal dysplasias	Suprasystemic PA and RV pressures after birth	Oxygen, acid-base balance, surfactant, mechanical ventilation, iNO, milrinone, inhaled and subcutaneous prostaglandins. ECMO in medically refractory PPHN
PH from CDH	Neonates and infants with CDH	Elevated PVR and mPAP, along with possible presence of LV dysfunction	Pulmonary selective vasodilators
PH from left heart disease	Older children with CHD, history of repair of coarctation of aorta, VSD repair, heart transplant, HLHS and its variants, cardiomyopathies, LV systolic or diastolic dysfunction	mPAP > 20 mmHg, PAWP or LVEDP > 15 mmHg, PVRI < 3 Wu×m2	Treatment of left heart disease, surgical repair in children with CHD after assesing operability

PH, pulmonary hypertension; RA, right atrium; RV, right ventricle; LA, left atrium; LV, left ventricle; PAH, pulmonary arterial hypertension; CHD, congenital heart disease; ASD, atrial septal defect; VSD, ventricular septal defect; PDA, patent ductus arteriosis; HLHS, hypoplastic left heart syndrome; CDH, congenital diaphragmatic hernia; SSRI, selective serotonin reuptake inhbitor; AV, atrioventricular; TGA, transposition of great arteries; BPD, bronchopulmonary dysplasia; PPHN, persistent pulmonary hypertension of the newborn; RDS, respiratory distress syndrome, iNO, inhaled nitric oxide; ECMO, extracorporeal membrane oxygenation, PAWP, pulmonary arterial wedge pressure; LVEDP, left ventricular end-diastolic pressure; PVRI, pulmonary vascular resistance index; mPAP, mean pulmonary artery pressure; TRJV, tricuspid regurgitant jet velocity; IVS, interventricular septum; PVS, pulmonary vein stenosis; CTA, computed tomography with angiography.