Paeds · cardiology
Aortic and pulmonary stenosis
Also known as congenital aortic stenosis · congenital pulmonary stenosis · valvar aortic stenosis · valvar pulmonary stenosis · bicuspid aortic valve · critical aortic stenosis · critical pulmonary stenosis
A fellowship approach to congenital aortic and pulmonary stenosis: classifying the obstruction by anatomic level (valvar, subvalvar, supravalvar), grading severity by echo peak gradient, recognising the duct-dependent critical neonate, and matching the intervention to the lesion — balloon valvuloplasty as first-line for valvar disease, the Ross procedure for the aortic valve that needs replacement, and syndromic associations from bicuspid aortic valve through Noonan and Williams syndromes.
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- A neonate with critical aortic stenosis is duct-dependent for systemic perfusion — start prostaglandin E1 at 0.01 to 0.05 mcg per kg per minute before the duct closes, because ductal closure produces shock and acidosis within hours
- Critical pulmonary stenosis is duct-dependent for pulmonary blood flow — the duct closing produces profound cyanosis, and prostaglandin E1 is the bridge to balloon valvuloplasty
- Syncope or chest pain on exertion in a child with aortic stenosis signals severe obstruction with subendocardial ischaemia — it is a risk factor for sudden cardiac death and an indication for urgent intervention
- A bicuspid aortic valve is the commonest congenital cardiac lesion and may be silent in childhood — any systolic ejection click and upper-sternal-border murmur in a well child needs an echocardiogram
- Supravalvar aortic stenosis in Williams syndrome is associated with coronary artery abnormalities and a risk of sudden death under anaesthesia or sedation
- A dysplastic pulmonary valve (thickened, immobile leaflets) as seen in Noonan syndrome responds poorly to balloon valvuloplasty and often needs surgical valvotomy
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- General and Community Paediatrics
- Cardiology
- Neonatal medicine
- Cardiology - recognise, investigate and manage valvar aortic and pulmonary stenosis and identify critical and syndromic forms
- Neonatal medicine - recognise duct-dependent left or right heart obstruction and initiate prostaglandin therapy
- Renewed curriculum for first-year trainees from 2027 - Learning goal 6: Clinical management - essential general paediatrics
- Cardiology 10-12
- Neonatal medicine 10-12
- Clinical Applications
- Long Cases
- Short Cases
- Cardiology - valvar and outflow-tract obstruction
- Neonates and infants with cardiac disorders: critical obstruction
- 4. Professional skills and knowledge: Patient management
- Foundation of Practice (FOP)
- Theory and Science (TAS)
- Applied Knowledge in Practice (AKP)
- Clinical
- Cardiology short case
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- General Pediatrics Content Outline - D22 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 murmur heard on the routine newborn or six-week check, a systolic ejection click at the upper left sternal border in an asymptomatic toddler, or a grey, collapsed neonate with a duct that has just closed — these are the three faces of congenital outflow-tract obstruction that a general paediatrician will meet. The fellowship task is to classify the level, grade the severity, recognise the duct-dependent infant, and time the intervention so that the child is neither exposed to unnecessary procedures nor lost to preventable complications. [3] [1]
Classify every stenotic lesion by where along the outflow tract the obstruction sits. Valvar — at the valve leaflets, the commonest form, first-line treatment is balloon valvuloplasty. Subvalvar — below the valve, usually a discrete membrane or tunnel, needs surgical resection. Supravalvar — above the valve at the great-vessel level, syndromic with Williams syndrome (aortic) or peripheral pulmonary stenosis (Alagille, Noonan, rubella). The level predicts the syndrome, the echo view, and the intervention. [1] [8]
Overview & Definition
Congenital aortic stenosis and congenital pulmonary stenosis are fixed anatomical narrowings of the left and right ventricular outflow tracts respectively, present from birth and graded by the pressure gradient they impose on ventricular ejection. They are among the commonest forms of congenital heart disease, together accounting for a substantial fraction of all structural cardiac lesions, and they span a spectrum from a silently mild murmur detected at a routine check to a duct-dependent critical obstruction that kills the neonate within hours of ductal closure. [3] [1]
The definition that earns marks is anatomical, not haemodynamic. The lesion is named for the level at which it sits — valvar, subvalvar, or supravalvar — and the severity is a separate, graded axis measured by echocardiographic peak systolic gradient. This dual classification matters because the level determines the intervention and the syndrome, while the severity determines the urgency. A bicuspid aortic valve with a gradient of 25 mmHg is observed; the same valve at 80 mmHg with syncope is an interventional emergency. [1] [4]
The clinical importance of the topic rests on three pillars. First, these are common lesions that every paediatrician will encounter as a murmur, and the ability to distinguish a flow murmur from a pathological ejection systolic murmur with a click is a core skill. Second, the critical neonatal forms are time-sensitive emergencies that demand prostaglandin therapy before the duct closes. Third, the long-term trajectory — restenosis after intervention, aortic root dilation in bicuspid aortic valve, right-ventricular failure in untreated pulmonary stenosis, and the lifelong need for cardiology follow-up — is what makes these conditions chronic, not acute, problems. [2] [3]
References12ShowHide
- [1]Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF. 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]Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J, 2021.PMID 32860028
- [3]Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol, 2002.PMID 12084585
- [4]Spaziani G, Girolami F, Arcieri L, et al. Bicuspid Aortic Valve in Children and Adolescents: A Comprehensive Review. Diagnostics (Basel), 2022.PMID 35885654
- [5]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
- [6]Affolter JT, Ghanayem NS. Preoperative management of the neonate with critical aortic valvar stenosis. Cardiol Young, 2014.PMID 25647388
- [7]Zhu MZL, Konstantinov IE, Wu DM, et al. Aortic valve repair versus the Ross procedure in children. J Thorac Cardiovasc Surg, 2023.PMID 37169064
- [8]Linglart L, Gelb BD. Congenital heart defects in Noonan syndrome: Diagnosis, management, and treatment. Am J Med Genet C Semin Med Genet, 2020.PMID 32022400
- [9]Twite MD, Stenquist S, Ing RJ. Williams syndrome. Paediatr Anaesth, 2019.PMID 30811742
- [10]Devanagondi R, Peck D, Sagi J, et al. Long-Term Outcomes of Balloon Valvuloplasty for Isolated Pulmonary Valve Stenosis. Pediatr Cardiol, 2017.PMID 27826708
- [11]Rowland DG, Hammill WW, Allen HD. Natural course of isolated pulmonary valve stenosis in infants and children utilizing Doppler echocardiography. Am J Cardiol, 1997.PMID 9036756
- [12]Latson LA. Critical pulmonary stenosis. J Interv Cardiol, 2001.PMID 12053395