Right ventricular outflow tract obstruction pulmonary supravalvular stenosis
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Right ventricular outflow tract obstruction Microchapters |
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Differentiating Right ventricular outflow tract obstruction from other Diseases |
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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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Overview
Supra valvular pulmonary artery stenosis is defined as stenosis of the pulmonary artery above the level of the valve area.
Anatomy
1. Also called peripheral pulmonary stenosis.
2. May have one or many stenoses of the PA or its branches.
3. Infrequently occurs as an isolated lesion, more commonly occurs as part of Tetralogy of Fallot.
Diagnosis
Physical Examination
1. The murmur is most prominent at the upper left sternal border, axillae, and the posterior lung fields.
Imaging
2008 ACC/AHA Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)[1]
Recommendations for evaluation of patients with supravalvular, branch, and peripheral Pulmonary Stenosis (DO NOT EDIT)[1]
| 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) " |
Treatment
Surgery and Device based Therapy
ACC / AHA Guidelines - Recommendations for interventional therapy in the management of branch and peripheral Pulmonary Stenosis (DO NOT EDIT)
| 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) " |
Follow-Up
ACC / AHA Guidelines - Recommendations for evaluation and follow-up (DO NOT EDIT)
| 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) " |
References
- ↑ 1.0 1.1 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.