Right ventricular outflow tract obstruction pulmonary supravalvular stenosis

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Right ventricular outflow tract obstruction Microchapters


Patient Information


Anatomy of Pulmonary Valve


Pulmonary valve stenosis
Pulmonary subvalvular stenosis
Pulmonary supravalvular stenosis
Pulmonary atresia



Differentiating Right ventricular outflow tract obstruction from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis


History and Symptoms

Physical Examination


Chest X Ray


Cardiac Catheterization

Pulmonary Angiography


Indications For Surgery


Pre-Operative A/P

Post-Operative A/P

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Special Scenarios

Pulmonary artery conduits/Prosthetic Valves

Double-Chambered Right Ventricle

Case Studies

Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Associate Editor-in-Chief: Keri Shafer, M.D. [3]

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Supra valvular pulmonary artery stenosis is defined as stenosis of the pulmonary artery above the level of the valve area.


1. Also called peripheral pulmonary stenosis. [1] [2] [3]

2. May have one or many stenoses of the PA or its branches. [4]

3. Infrequently occurs as an isolated lesion, more commonly occurs as part of Tetralogy of Fallot. [5] [6]


Physical Examination

1. The murmur is most prominent at the upper left sternal border, axillae, and the posterior lung fields.[7] [8] [2] [9][10] [5] [2] [11]

2008 ACC/AHA Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[12]

Recommendations for Evaluation of Patients With Supravalvular, Branch, and Peripheral Pulmonary Stenosis (DO NOT EDIT)[12]

Class I
"1. Patients with suspected supravalvular, branch, or peripheral PS should have baseline imaging with echocardiography- Doppler plus 1 of the following: MRI angiography, CT angiography, or contrast angiography. (Level of Evidence: C)"
"2. Once the diagnosis is established, follow-up echocardiography- Doppler to assess RV systolic pressure should be performed periodically, depending on severity. (Level of Evidence: C)"

Recommendations for Interventional Therapy in the Management of Branch and Peripheral Pulmonary Stenosis (DO NOT EDIT)[12]

Class I
"1. Percutaneous interventional therapy is recommended as the treatment of choice in the management of appropriate focal branch and/or peripheral pulmonary artery stenosis with greater than 50% diameter narrowing, an elevated RV systolic pressure greater than 50 mm Hg, and/or symptoms. (Level of Evidence: B)"
"2. In patients with the above indications for intervention, surgeons with training and expertise in CHD should perform operations for management of branch pulmonary artery stenosis not anatomically amenable to percutaneous interventional therapy. (Level of Evidence: B)"

Recommendations for Evaluation and Follow-Up (DO NOT EDIT)[12]

Class I
"1. Patients with peripheral PS should be followed up every 1 to 2 years, on the basis of severity, with a clinical evaluation and echocardiography-Doppler to evaluate RV systolic pressure and RV function. (Level of Evidence: C)"
"2. Discussion with a cardiac surgeon with expertise in CHD should take place before percutaneous peripheral pulmonary artery interventions are undertaken. (Level of Evidence: C)"


  1. Pierpont ME, Basson CT, Benson DW, Gelb BD, Giglia TM, Goldmuntz E; et al. (2007). "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. 115 (23): 3015–38. doi:10.1161/CIRCULATIONAHA.106.183056. PMID 17519398.
  2. 2.0 2.1 2.2 Dweck MR, Boon NA, Newby DE (2012). "Calcific aortic stenosis: a disease of the valve and the [[myocardium]]". J Am Coll Cardiol. 60 (19): 1854–63. doi:10.1016/j.jacc.2012.02.093. PMID 23062541. URL–wikilink conflict (help)
  3. Frantz C, Stewart KM, Weaver VM (2010). "The extracellular matrix at a glance". J Cell Sci. 123 (Pt 24): 4195–200. doi:10.1242/jcs.023820. PMC 2995612. PMID 21123617.
  4. Tamura T, Horiuchi H, Imai M, Tada T, Shiomi H, Kuroda M; et al. (2015). "Unexpectedly High Prevalence of Acquired von Willebrand Syndrome in Patients with Severe Aortic Stenosis as Evaluated with a Novel Large Multimer Index". J Atheroscler Thromb. 22 (11): 1115–23. doi:10.5551/jat.30809. PMID 26269004.
  5. 5.0 5.1 Galli D, Manuguerra R, Monaco R, Manotti L, Goldoni M, Becchi G; et al. (2016). "Understanding the structural features of symptomatic calcific aortic valve stenosis: A broad-spectrum clinicopathologic study in 236 consecutive surgical cases". Int J Cardiol. 228: 364–374. doi:10.1016/j.ijcard.2016.11.180. PMID 27866029.
  6. Roberts WC (1973). "Valvular, subvalvular and supravalvular aortic stenosis: morphologic features". Cardiovasc Clin. 5 (1): 97–126. PMID 4272665.
  7. Baumgartner H, Hung J, Bermejo J, Chambers JB, Evangelista A, Griffin BP; et al. (2009). "Echocardiographic assessment of valve stenosis: EAE/ASE recommendations for clinical practice". J Am Soc Echocardiogr. 22 (1): 1–23, quiz 101-2. doi:10.1016/j.echo.2008.11.029. PMID 19130998.
  8. Schmoldt A, Benthe HF, Haberland G, Sinelnikova EM, Dvoretskova TV, Kagan ZS, Odom DG, Brady JT, Kenyon G, Carter DC, Mekler LB, Dougherty EM, Vaughn JL, Reichelderfer CF (September 1975). "Digitoxin metabolism by rat liver microsomes". Biochem. Pharmacol. 24 (17): 1639–41. doi:10.1007/bf01732356. PMC 5922622. PMID 10.
  9. Cor triatrium https://radiopaedia.org/articles/cor-triatriatum (2016) Accessed on November 29, 2016
  10. Sosland RP, Vacek JL, Gorton ME (2007). "Congenital mitral stenosis: a rare presentation and novel approach to management". J Thorac Cardiovasc Surg. 133 (2): 572–3. doi:10.1016/j.jtcvs.2006.10.025. PMID 17258606.
  11. Galante A, Pietroiusti A, Vellini M, Piccolo P, Possati G, De Bonis M; et al. (2001). "C-reactive protein is increased in patients with degenerative aortic valvular stenosis". J Am Coll Cardiol. 38 (4): 1078–82. PMID 11583885.
  12. 12.0 12.1 12.2 12.3 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). "ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". J Am Coll Cardiol. 52 (23): e1–121. doi:10.1016/j.jacc.2008.10.001. PMID 19038677.

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