Paeds · cardiology
Pulmonary hypertension in children
Also known as pulmonary hypertension · pulmonary arterial hypertension · PAH · paediatric pulmonary hypertension · Eisenmenger syndrome · idiopathic pulmonary arterial hypertension · IPAH
A fellowship approach to pulmonary hypertension in children: a mean pulmonary artery pressure above twenty is never normal, the WHO group decides the cause, the cardiac catheter measures the resistance, and the right ventricle decides the prognosis. The thread runs from the syncope or the loud second heart sound through the echocardiogram and the catheter to combination therapy with phosphodiesterase-5 inhibitors, endothelin receptor antagonists, and prostacyclin analogues, and on to the surgical escalations of septostomy, the Potts shunt, and transplantation.
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Red flags
- A child with exertional syncope, presyncope, or chest pain has pulmonary hypertension until the echocardiogram shows the tricuspid regurgitation velocity and the right ventricle
- A loud, palpable pulmonary component of the second heart sound is the bedside signature of a raised pulmonary pressure — it is not an innocent finding
- The right ventricle decides survival: right ventricular failure is the mode of death, so every management decision protects the right ventricle, not just the pressure number
- Down syndrome, congenital diaphragmatic hernia, and bronchopulmonary dysplasia carry a high burden of pulmonary hypertension and must be screened, not assumed well
- Eisenmenger physiology is irreversible pulmonary vascular disease from an unrepaired shunt — repair must happen before the resistance crosses the operative threshold
- Never give a pulmonary vasodilator in isolation when the cause is left heart disease — you will flood the lungs and worsen pulmonary oedema
Life stages
Care settings
Clinical exam formats
Board mappings
- Cardiology - recognise and investigate pulmonary hypertension
- Neonatal medicine - persistent pulmonary hypertension of the newborn
- General and Community Paediatrics
- Cardiology - investigate and manage pulmonary hypertension and Eisenmenger syndrome
- Congenital heart disease - interpret echocardiography and arrange cardiac catheterisation
- Renewed curriculum for first-year trainees from 2027 - Learning goal 6: Clinical management - essential general paediatrics
- Cardiology 10-12
- Neonatal and congenital cardiac 10-12
- Clinical Applications
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- Short Cases
- 8. Cardiology - pulmonary hypertension and Eisenmenger syndrome
- 1. Neonates and infants with cardiology: pulmonary vascular disease
- 4. Professional skills and knowledge: Patient management
- Foundation of Practice (FOP)
- Theory and Science (TAS)
- Applied Knowledge in Practice (AKP)
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- General Pediatrics Content Outline - D15 Cardiology (5%)
- General Pediatrics Content Outline - D01 Newborn (3%)
- General Pediatrics Content Outline - Universal Task 4: Management and Treatment
- Patient Care 4: Clinical Reasoning
- Patient Care 5: Patient Management
- Medical Knowledge 1: Congenital anomalies and genetics
- Systems-Based Practice 1: Patient Safety
- Medical Expert
- Pediatrics: Foundations EPA #8 - Communicating assessment findings and management plans to patients and/or families
- Pediatrics: Cardiology applied domain
A four-year-old is brought in after collapsing while running, with an examination that reveals a loud pulmonary component of the second heart sound; or a teenager with a ventricular septal defect repaired late now has cyanosis and a decreasing exercise tolerance, the Eisenmenger physiology; or an ex-preterm infant with bronchopulmonary dysplasia fails to wean from oxygen and has a rising tricuspid regurgitation velocity on echocardiography. The fellowship task in each is the same: recognise the raised pulmonary pressure, confirm it at catheter, assign the group, and protect the right ventricle. [5] [9]
Hold the pathway as five moves. Suspect the lesion in any child with syncope, a loud second heart sound, exertional dyspnoea, or unexplained failure to thrive. Scan with echocardiography to estimate the pressure and assess the right ventricle. Catheterise to measure the mean pulmonary artery pressure, the wedge pressure, and the indexed pulmonary vascular resistance. Classify by the WHO group, because the group decides the cause and the therapy. Escalate by combination therapy, protecting the right ventricle with prostacyclin, septostomy, the Potts shunt, or transplantation as the risk demands. [1] [4]
Overview & Definition
Pulmonary hypertension is defined haemodynamically at cardiac catheterisation as a mean pulmonary artery pressure above twenty millimetres of mercury. The sixth World Symposium refined this in 2018, and the 2022 European guideline carries the same threshold, separating pulmonary arterial hypertension from the other groups by adding a raised resistance and a normal wedge pressure. [3] [12]
Pulmonary arterial hypertension, the first group, requires a pulmonary artery wedge pressure of fifteen or less and a pulmonary vascular resistance above two Wood units in adults, with an indexed resistance above three Wood units per square metre in children. The wedge pressure separates a problem in the pulmonary arteries, where it is normal, from a problem downstream in the left heart, where it is raised. This single number is the safeguard against giving a vasodilator to a child whose true problem is left heart disease. [2] [1]
The condition is distinguished from persistent pulmonary hypertension of the newborn, which is a transitional circulation problem of the first days of life and is addressed on its own page. The child with established pulmonary hypertension presents weeks, months, or years later, and the resistance is fixed rather than transitional. The distinction matters because the management, the prognosis, and the counselling diverge sharply between the two. [5] [1]
References12ShowHide
- [1]Abman SH, Hansmann G, Archer SL, et al. Pediatric Pulmonary Hypertension: Guidelines From the American Heart Association and American Thoracic Society. Circulation, 2015.PMID 26534956
- [2]Rosenzweig EB, Abman SH, Adatia I, et al. Paediatric pulmonary arterial hypertension: updates on definition, classification, diagnostics and management. Eur Respir J, 2019.PMID 30545978
- [3]Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, Williams PG, Souza R. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J, 2019.PMID 30545968
- [4]Hansmann G, Koestenberger M, Alastalo TP, et al. 2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT. J Heart Lung Transplant, 2019.PMID 31495407
- [5]Ivy DD, Abman SH, Barst RJ, Berger RMF, Bonnet D, Fleming TP, Haworth SG, Rosenzweig EB, Schulze Neick I, Steinhorn RH, Beghetti M. Pediatric pulmonary hypertension. J Am Coll Cardiol, 2013.PMID 24355636
- [6]Beghetti M, Berger RM, Schulze-Neick I, et al. Diagnostic evaluation of paediatric pulmonary hypertension in current clinical practice. Eur Respir J, 2013.PMID 23563261
- [7]Beghetti M, Schulze-Neick I, Berger RM, et al. Haemodynamic characterisation and heart catheterisation complications in children with pulmonary hypertension: Insights from the Global TOPP Registry (tracking outcomes and practice in paediatric pulmonary hypertension). Int J Cardiol, 2016.PMID 26583838
- [8]Arvanitaki A, Gatzoulis MA, Opotowsky AR, et al. Eisenmenger Syndrome: JACC State-of-the-Art Review. J Am Coll Cardiol, 2022.PMID 35331414
- [9]del Cerro MJ, Sabaté Rotés A, Cartón A, et al. Pulmonary hypertension in bronchopulmonary dysplasia: clinical findings, cardiovascular anomalies and outcomes. Pediatr Pulmonol, 2014.PMID 23788443
- [10]Barst RJ, Ivy DD, Gaitan G, et al. A randomized, double-blind, placebo-controlled, dose-ranging study of oral sildenafil citrate in treatment-naive children with pulmonary arterial hypertension. Circulation, 2012.PMID 22128226
- [11]Lammers AE, Apitz C, Michel-Behnke I, et al. A guide to echocardiographic assessment in children and adolescents with pulmonary hypertension. Cardiovasc Diagn Ther, 2021.PMID 34527541
- [12]Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J, 2016.PMID 26320113