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Paeds Vivascardiology

Paeds Vivas · cardiology

Aortic and pulmonary stenosis — branching viva

Branching viva on aortic and pulmonary stenosis: classifying the obstruction by anatomic level, grading severity by echo peak gradient, recognising the duct-dependent critical neonate, matching the intervention to the lesion, and identifying the syndromic associations from bicuspid aortic valve through Noonan and Williams syndromes.

branching clinical structured oral2 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
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Study tools

Target exams

RACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
A four-year-old child has an ejection systolic murmur at the upper left sternal border with a widely split second heart sound and a respiratory-varying ejection click. The examiner asks: what is the diagnosis, how do you classify it, what is the severity, and what is the management — then branches to the balloon valvuloplasty evidence, the dysplastic Noonan valve exception, the distinction from aortic stenosis, the critical neonatal presentation, and finally the Ross procedure for aortic valve replacement.

Opening: classify and grade

The candidate opens by recognising that the murmur at the upper left sternal border with a respiratory-varying ejection click and a widely split second heart sound is the classic presentation of valvar pulmonary stenosis. The click localises the lesion to the valve, its respiratory variation confirms pulmonary rather than aortic origin, and the wide S2 split reflects prolonged right-ventricular ejection. The diagnosis is confirmed by echocardiography, which shows the domed valve with fused commissures and measures the peak systolic gradient. [1] [2]

The candidate classifies the lesion by anatomic level — valvar, subvalvar, or supravalvar — and grades it by peak gradient: mild below 40 mmHg, moderate 40 to 64 mmHg, severe above 64 mmHg. The level predicts the intervention and the syndrome, and the gradient determines the urgency. This dual classification is the framework that earns marks. [1]

Branch 1: balloon valvuloplasty and the evidence

The examiner asks about the management of moderate valvar pulmonary stenosis. The candidate answers that balloon pulmonary valvuloplasty is the first-line and definitive intervention, one of the most successful procedures in congenital interventional cardiology, with long-term outcome data showing sustained gradient reduction and low restenosis rates. The procedure is indicated for moderate and severe gradients and for symptomatic disease. [2]

The examiner probes the complications. The candidate names pulmonary regurgitation as the main late concern, usually well tolerated in childhood but potentially requiring pulmonary valve replacement in adulthood. The candidate contrasts this with aortic valvuloplasty, where aortic regurgitation is the more immediate concern and the VACA Registry data established the predictors of success. [4]

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

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References4ShowHide
  1. [1]Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation, 2019.PMID 30586767
  2. [2]Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol, 2002.PMID 12084585
  3. [3]Affolter JT, Ghanayem NS. Preoperative management of the neonate with critical aortic valvar stenosis. Cardiol Young, 2014.PMID 25647388
  4. [4]McCrindle BW. Independent predictors of immediate results of percutaneous balloon aortic valvotomy in children. Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Am J Cardiol, 1996.PMID 8607410
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