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 ventricleA loud, palpable pulmonary component of the second heart sound is the bedside signature of a raised pulmonary pressure — it is not an innocent findingThe right ventricle decides survival: right ventricular failure is the mode of death, so every management decision protects the right ventricle, not just the pressure numberDown syndrome, congenital diaphragmatic hernia, and bronchopulmonary dysplasia carry a high burden of pulmonary hypertension and must be screened, not assumed wellEisenmenger physiology is irreversible pulmonary vascular disease from an unrepaired shunt — repair must happen before the resistance crosses the operative thresholdNever give a pulmonary vasodilator in isolation when the cause is left heart disease — you will flood the lungs and worsen pulmonary oedema

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Cardiology - recognise and investigate pulmonary hypertensionNeonatal medicine - persistent pulmonary hypertension of the newbornGeneral and Community PaediatricsCardiology - investigate and manage pulmonary hypertension and Eisenmenger syndromeCongenital heart disease - interpret echocardiography and arrange cardiac catheterisationRenewed curriculum for first-year trainees from 2027 - Learning goal 6: Clinical management - essential general paediatricsCardiology 10-12Neonatal and congenital cardiac 10-12Clinical ApplicationsLong CasesShort Cases8. Cardiology - pulmonary hypertension and Eisenmenger syndrome1. Neonates and infants with cardiology: pulmonary vascular disease4. Professional skills and knowledge: Patient managementFoundation of Practice (FOP)Theory and Science (TAS)Applied Knowledge in Practice (AKP)ClinicalHistoryCommunicationVideoGeneral Pediatrics Content Outline - D15 Cardiology (5%)General Pediatrics Content Outline - D01 Newborn (3%)General Pediatrics Content Outline - Universal Task 4: Management and TreatmentPatient Care 4: Clinical ReasoningPatient Care 5: Patient ManagementMedical Knowledge 1: Congenital anomalies and geneticsSystems-Based Practice 1: Patient SafetyMedical ExpertPediatrics: Foundations EPA #8 - Communicating assessment findings and management plans to patients and/or familiesPediatrics: Cardiology applied domain

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The fellowship answer

Pulmonary hypertension in children is a mean pulmonary artery pressure above twenty millimetres of mercury, and the fellowship skill is to assign the WHO group, measure the pulmonary vascular resistance at catheterisation, and protect the right ventricle while escalating therapy. The clinical thread runs from the child with exertional syncope or a loud second heart sound through the echocardiogram that estimates the pressure to the cardiac catheter that measures the resistance, and on to combination therapy with a phosphodiesterase-5 inhibitor, an endothelin receptor antagonist, and a prostacyclin analogue. [1] [2]

The mechanism turns on a pulmonary vascular bed that has narrowed, so the right ventricle pumps against a higher afterload and eventually fails. Three forces drive the narrowing: vasoconstriction, vascular remodelling with medial hypertrophy and intimal proliferation, and in-situ thrombosis. The three drug pathways reverse these forces — nitric oxide is restored by phosphodiesterase-5 inhibitors, prostacyclin is replaced by analogues, and the excess endothelin is blocked by receptor antagonists. The right ventricle, not the pressure number, decides who lives, which is why a child in right ventricular failure is escalated to parenteral prostacyclin, septostomy, the Potts shunt, and finally transplantation. [1] [4]

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]

The five moves — Suspect, Scan, Catheterise, Classify, Escalate

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]

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You have read the opening of this topic. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.

References12Show ledger

[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

Pulmonary hypertension in children

Also known as pulmonary hypertension · pulmonary arterial hypertension · PAH · paediatric pulmonary hypertension · Eisenmenger syndrome · idiopathic pulmonary arterial hypertension · IPAH

high12 referencesUpdated 13 July 202626 min readVerification in progress

Practise this topic

Overview & Definition

Paediatrics Fellowship Pro

You have read the opening of this topic. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.

References12Show ledger

[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