Highlights & Basics
- Pulmonary stenosis is mostly congenital.
- Symptoms range from none to profound cyanosis and the potential for sudden death.
- Systolic ejection murmur is present and is loudest over left upper sternal border.
- Cyanotic patients are treated with oxygen and prostaglandin E1 prior to diagnostic testing.
- The diagnosis is confirmed and severity classified by echocardiography.
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Citations
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. J Am Coll Cardiol. 2018 Aug 10 [Epub ahead of print].[Abstract][Full Text]
Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA Guideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2021 Feb 2;143(5):e35-71.[Abstract][Full Text]
European Society of Cardiology. 2020 ESC guidelines for the management of adult congenital heart disease (previously grown-up congenital heart disease). Aug 2020 [internet publication].[Full Text]
1. Burch M, Sharland M, Shinebourne E, et al. Cardiologic abnormalities in Noonan syndrome: phenotypic diagnosis and echocardiographic assessment of 118 patients. J Am Coll Cardiol. 1993 Oct;22(4):1189-92.[Abstract][Full Text]
2. Ruckdeschel E, Kim YY. Pulmonary valve stenosis in the adult patient: pathophysiology, diagnosis and management. Heart. 2019 Mar;105(5):414-22.[Abstract][Full Text]
3. Marchini F, Meossi S, Passarini G, et al. Pulmonary valve stenosis: from diagnosis to current management techniques and future prospects. Vasc Health Risk Manag. 2023;19:379-90.[Abstract][Full Text]
4. Keith A. The Hunterian lectures on malformations of the heart. Lancet. 1909;2:359.
5. Oka M, Angrist GM. Mechanism of cardiac valvular fusion and stenosis. Am Heart J. 1967;74:37-47.
6. Pierpont ME, Basson CT, Benson DW Jr, et al. Genetic basis for congenital heart defects: current knowledge. A scientific statement from the American Heart Association Congenital Cardiac Defects Committee, Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. Circulation. 2007;115:3015-3038.[Full Text]
7. Sood E, Newburger JW, Anixt JS, et al. Neurodevelopmental outcomes for individuals with congenital heart disease: updates in neuroprotection, risk-stratification, evaluation, and management: a scientific statement from the American Heart Association. Circulation. 2024 Mar 26;149(13):e997-1022.[Abstract][Full Text]
8. Garson A, Bricker JT, Fisher DJ, eds. The science and practice of pediatric cardiology. 2nd ed. Baltimore, MD: Lippincott, Williams & Wilkins; 1998 (Vol 1):1207-56.
9. Latson LA. Critical pulmonary stenosis. J Interv Cardiol. 2001 Jun;14(3):345-50.[Abstract][Full Text]
10. Botto LD, Correa A, Erickson JD. Racial and temporal variations in the prevalence of heart defects. Pediatrics. 2001 Mar;107(3):E32.[Abstract]
11. Moyssakis IE, Rallidis LS, Guida GF, et al. Incidence and evolution of carcinoid syndrome in the heart. J Heart Valve Dis. 1997;6:625-630.[Abstract]
12. Rowland DG, Hammill WW, Allen HD, et al. Natural course of isolated pulmonary valve stenosis in infants and children utilizing Doppler echocardiography. Am J Cardiol. 1997 Feb 1;79(3):344-9.[Abstract]
13. 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. J Am Coll Cardiol. 2018 Aug 10 [Epub ahead of print].[Abstract][Full Text]
14. Otto CM, Nishimura RA, Bonow RO, et al. 2020 ACC/AHA Guideline for the management of patients with valvular heart disease: executive summary: a report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2021 Feb 2;143(5):e35-71.[Abstract][Full Text]
15. European Society of Cardiology. 2020 ESC guidelines for the management of adult congenital heart disease (previously grown-up congenital heart disease). Aug 2020 [internet publication].[Full Text]
16. American College of Radiology. ACR appropriateness criteria®: congenital or acquired heart disease. 2023 [internet publication].[Full Text]
17. Drossner DM, Mahle WT. A management strategy for mild valvar pulmonary stenosis. Pediatr Cardiol. 2008 May;29(3):649-52.[Abstract]
18. Marelli A, Beauchesne L, Colman J, et al. Canadian Cardiovascular Society 2022 guidelines for cardiovascular interventions in adults with congenital heart disease. Can J Cardiol. 2022 Jul;38(7):862-96.[Abstract][Full Text]
19. Hayes CJ, Gersony WM, Driscoll DJ, et al. Second natural history study of congenital heart defects: results of treatment of patients with pulmonary valvar stenosis. Circulation. 1993 Feb;87(suppl 2):I28-37.[Abstract]
20. Wash KP. Interventional paediatric cardiology. BMJ. 2003 Aug 16;327(7411):385-8.[Abstract][Full Text]
21. Canobbio MM, Warnes CA, Aboulhosn J, et al. Management of pregnancy in patients with complex congenital heart disease: a scientific statement for healthcare professionals from the American Heart Association. Circulation. 2017 Feb 21;135(8):e50-e87.[Abstract][Full Text]
22. Tinker J, Howitt G, Markman P, et al. The natural history of isolated pulmonary stenosis. Br Heart J. 1965 Mar;27:151-60.[Full Text]
23. Gudausky TM, Beekman RH 3rd. Current options, and long-term results for interventional treatment of pulmonary valvar stenosis. Cardiol Young. 2006 Oct;16(5):418-27.[Abstract]
24. Stanger P, Cassidy SC, Girod DA, et al. Balloon pulmonary valvuloplasty: results of the Valvuloplasty and Angioplasty of Congenital Anomalies registry. Am J Cardiol. 1990 Mar 15;65(11):775-83.[Abstract]
25. Peterson C, Schilthuis JJ, Dodge-Khatami A, et al. Comparative long-term results of surgery versus balloon valvuloplasty for pulmonary valve stenosis in infants and children. Ann Thorac Surg. 2003 Oct;76(4):1078-82.[Abstract]
26. John AS, Jackson JL, Moons P, et al. Advances in managing transition to adulthood for adolescents with congenital heart disease: a practical approach to transition program design: a scientific statement from the American Heart Association. J Am Heart Assoc. 2022 Apr 5;11(7):e025278.[Abstract][Full Text]
