Summary
Pediatric pulmonary hypertension is defined by an elevated mean pulmonary arterial pressure beyond the neonatal transition, and in children it is most often tied to congenital heart disease or developmental lung disease rather than the idiopathic disease seen in adults [1][4]. The 2015 American Heart Association and American Thoracic Society guideline used a resting mean pulmonary arterial pressure above 25 mmHg, and the hemodynamic threshold was subsequently lowered to above 20 mmHg to align pediatric and adult definitions [1][4]. The preterm infant with bronchopulmonary dysplasia is a distinct and increasingly common phenotype that every neonatal and cardiology provider now sees [1][5].
- Screen preterm infants with established bronchopulmonary dysplasia by echocardiogram at 36 weeks postmenstrual age, because pulmonary hypertension in this group carries substantial mortality [1][5].
- Early pulmonary vascular disease in the first weeks of life predicts later bronchopulmonary dysplasia and pulmonary hypertension, so risk is present before the classic 36-week timepoint [6].
- Echocardiography is the first-line screen, but cardiac catheterization remains the reference standard for confirming the diagnosis and testing vasoreactivity before targeted therapy [1].
- Classification follows the pediatric pulmonary hypertensive vascular disease framework, which separates the pediatric causes that do not map cleanly onto the adult groups [2][4].
Caution. The absence of an elevated pulmonary artery pressure estimate does not exclude pulmonary hypertension, and a measurable tricuspid regurgitation jet is not always present. In the absence of a measurable jet, the guideline directs attention to qualitative findings including right atrial enlargement, right ventricular hypertrophy, and septal configuration. Read those alongside right ventricular size and function, never the jet alone [1].
Diagnosis
The hemodynamic threshold was lowered to a mean pulmonary arterial pressure above 20 mmHg for all ages beyond the first months of life, aligning pediatric and adult definitions [4]. Pulmonary arterial hypertension additionally requires a pulmonary vascular resistance index at or above 3 Wood units times m squared and a pulmonary arterial wedge pressure at or below 15 mmHg [1][4].
| Parameter |
Threshold |
Role |
| Mean PAP |
> 20 mmHg (2015 guideline: > 25 mmHg) |
Defines pulmonary hypertension beyond the neonatal period [1][4] |
| PVR index |
≥ 3 WU·m² |
Distinguishes pre-capillary pulmonary arterial hypertension [1][4] |
| PAWP |
≤ 15 mmHg |
Separates pre-capillary from post-capillary disease [4] |
| TR jet velocity |
Estimates systolic PAP |
First-line noninvasive screen; absent in a large minority [1] |
Estimate systolic pulmonary artery pressure from the tricuspid regurgitation jet and right atrial pressure at the bedside before committing to catheterization.
Estimate systolic PAP from the TR jet in TR Jet PASP →
Right ventricular function carries more prognostic weight than the pressure estimate itself. Tissue Doppler and strain indices detect right ventricular dysfunction earlier than fractional area change, and they should be indexed to normal pediatric ranges rather than read as raw values [1].
Interpret tissue Doppler velocities against pediatric norms in TDI Z-Scores →
Risk stratification
Management follows a risk-based approach in which low-risk children are treated with oral monotherapy and higher-risk children receive earlier combination therapy [2]. The pediatric determinants of risk differ from the adult six-minute-walk-centered model and include growth failure, syncope, right ventricular dysfunction, and rising natriuretic peptides [1][2].
The European Paediatric Pulmonary Vascular Disease Network consensus provides the pediatric-specific risk framework that this stratification rests on [2][3].
Score pediatric pulmonary hypertension risk in EPPVDN Risk →
At catheterization, the pulmonary-to-systemic vascular resistance relationship guides operability in congenital heart disease and the response to acute vasodilator testing. A pulmonary vascular resistance index that falls appropriately with vasodilator challenge supports treatment and, in shunt lesions, operability [1].
Work through resistances and the Qp:Qs relationship in PVR/SVR →
The preterm and bronchopulmonary dysplasia phenotype
Pulmonary hypertension complicates a substantial share of moderate-to-severe bronchopulmonary dysplasia and independently raises mortality in that population [1][5]. The mechanism is developmental. Disrupted alveolar and vascular growth reduces the cross-sectional area of the pulmonary vascular bed and raises resistance, rather than the remodeling of a fully formed bed seen in older children [5][6].
Because early pulmonary vascular disease is detectable before 36 weeks and predicts the later diagnosis, serial echocardiographic surveillance in the highest-risk preterm infants is reasonable rather than waiting for a single screening timepoint [6]. Confirmed pulmonary hypertension in this group warrants evaluation for the treatable contributors that amplify it, including left-sided filling abnormalities, aortopulmonary collaterals, pulmonary vein stenosis, and a hemodynamically significant patent ductus arteriosus [1][5].
References
- Abman SH, Hansmann G, Archer SL, et al. Pediatric pulmonary hypertension: guidelines from the American Heart Association and American Thoracic Society. Circulation. 2015;132(21):2037-2099. doi:10.1161/CIR.0000000000000329
- Rosenzweig EB, Abman SH, Adatia I, et al. Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management. Eur Respir J. 2019;53(1):1801916. doi:10.1183/13993003.01916-2018
- Hansmann G, Apitz C, Abdul-Khaliq H, et al. Executive summary. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension (EPPVDN). Heart. 2016;102(Suppl 2):ii86-ii100. doi:10.1136/heartjnl-2015-309132
- Simonneau G, Montani D, Celermajer DS, et al. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019;53(1):1801913. doi:10.1183/13993003.01913-2018
- Hansmann G, Sallmon H, Roehr CC, et al. Pulmonary hypertension in bronchopulmonary dysplasia. Pediatr Res. 2021;89(3):446-455. doi:10.1038/s41390-020-0993-4
- Mourani PM, Sontag MK, Younoszai A, et al. Early pulmonary vascular disease in preterm infants at risk for bronchopulmonary dysplasia. Am J Respir Crit Care Med. 2015;191(1):87-95. doi:10.1164/rccm.201409-1594OC