Paeds Vivas · cardiology
Pulmonary hypertension in children — branching viva
Branching viva on pulmonary hypertension in children: the haemodynamic definition, the WHO classification, the echocardiographic and catheter-based diagnosis, the three drug pathways, the Eisenmenger threshold, and the surgical escalations.
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RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
An eight-year-old child collapses while running at school and is found to have a loud, palpable pulmonary component of the second heart sound. The examiner asks: what is your differential, how do you define and confirm pulmonary hypertension, what are the five WHO groups, why does the right ventricle decide the prognosis, and what are the three drug pathways and their targets — then branches to the vasoreactivity test, the Eisenmenger threshold, the bronchopulmonary-dysplasia form, and finally the surgical escalations of septostomy, the Potts shunt, and transplantation.
Branch 1 — Definition and classification
The candidate opens by defining pulmonary hypertension as a mean pulmonary artery pressure above twenty at cardiac catheterisation, with pulmonary arterial hypertension adding a raised resistance and a normal wedge pressure. They then name the five WHO groups and explain why congenital heart disease and bronchopulmonary dysplasia dominate the paediatric caseload. [3] [2]
References7ShowHide
- [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, et al. 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. J Heart Lung Transplant, 2019.PMID 31495407
- [5]Ivy DD, Abman SH, Barst RJ, et al. Pediatric pulmonary hypertension. J Am Coll Cardiol, 2013.PMID 24355636
- [8]Arvanitaki A, Gatzoulis MA, Opotowsky AR, et al. Eisenmenger Syndrome: JACC State-of-the-Art Review. J Am Coll Cardiol, 2022.PMID 35331414
- [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