Table 1.

Definition of Pulmonary Hypertension Adapted from the European Pediatric Pulmonary Vascular Disease Network (EPPVDN) modeled on the 6th WSPH definitions (233–235)

1. Pulmonary hypertension

a. mPAP > 20 mmHg in children > 3 months at sea level

2. Precapillary PH (e.g., pulmonary arterial hypertension)

a. mPAP > 20 mmHg

b. PAWP or LVEDP < 15 mmHg

c. PVRI ≥ 3 Wu×m2

d. Diastolic TPG ≥ 7 mmHg

3. Isolated postcapillary PH in adults (predominantly LV diastolic dysfunction)

a. mPAP > 20 mmHg

b. PAWP or LVEDP > 15 mmHg

c. PVRI < 3 Wu×m2

d. Diastolic TPG < 7 mmHg

4. Combination of precapillary and postcapillary PH in adults

a. mPAP > 20 mmHg

b. PAWP or LVEDP > 15 mmHg

c. PVRI ≥ 3 Wu×m2

5. Pulmonary arterial hypertension

a. mPAP > 20 mmHg

b. PAWP or LVEDP ≤ 15 mmHg

c. PVRI ≥ 3 Wu×m2 plus criteria for Group 1 PH

6. Idiopathic PAH (IPAH)–PAH with no underlying disease known to be associated with PAH

7. Heritable PAH (HPAH)–PAH with no known underlying disease but with positive family history or positive genetic testing of the index patient

8. Eisenmenger syndrome–Patient with longstanding pulmonary hypertension, supra-systemic PVR and PAP, and accordingly, right-to-left cardiovascular shunting with systemic hypoxemia (e.g., unrepaired VSD or PDA)

9. Pulmonary hypertensive vascular disease For biventricular circulations: mPAP > 20 mmHg and PVR index ≥ 3 WU×m2 For circulations with cavopulmonary anastomosis (e.g., Fontan physiology): Mean TPG > 6 mmHg (calculate mPAP minus mLAP or PAWP) or PVR index > 3 WU×m2

PH, pulmonary hypertension; mPAP, mean pulmonary artery pressure; PAWP, pulmonary arterial wedge pressure; LVEDP, left ventricular end-diastolic pressure; PVRI, pulmonary vascular resistance index; TPG, transpulmonary gradient; PAH, pulmonary arterial hypertension; PVR, pulmonary vascular resistance; VSD, ventricular septal defect; PDA, patent ductus arteriosus; WU, Wood units mLAP, mean left atrial pressure.

Adapted, with permission, from Rosenzweig EB, et al., 2019 (506).