Surgical Care

Traditionally, pulmonic stenosis (pulmonary stenosis) (PS) was treated by surgical valvotomy. Since its introduction in 1982, however, percutaneous balloon valvuloplasty has become the initial intervention in children, adolescents, and adults with congenital valvar PS.

Balloon valvuloplasty should be considered in any symptomatic patient with a transvalvular peak gradient over 50 mmHg (mean >30 mmHg) and in asymptomatic patients with a peak gradient over 60 mmHg (mean >40 mmHg). It is critical to ensure that no more than moderate pulmonic valve regurgitation is present prior to valvuloplasty. Providers should be aware of the possibility of "suicidal RV," in which there is severe, dynamic right ventricular outflow tract (RVOT) obstruction acutely following relief of valvar PS. This can be managed by beta blockers and volume expansion.

Occasionally, balloon valvuloplasty is not successful. These patients tend to have valvular dysplasia (eg, Noonan syndrome) or a hypoplastic pulmonic valve annulus and, therefore, may require surgical valvotomy. A 2020 case report from Japan was the first to describe successful balloon pulmonary angioplasty intervention for Noonan syndrome with pulmonary artery stenosis.^[8] The clinicians used a strategy of careful morphologic evaluation with computed tomographic angiography and used scoring balloons over multiple sessions in a graded approach.

Pulmonary artery balloon angioplasty with or without placement of an expandable metal stent can be used to treat supravalvular PS and PPS. Expandable metal stents can overcome an obstruction successfully; however, the need for stent reexpansion as the individual grows remains problematic.

The American Heart Association/American College of Cardiology (AHA/ACA)^[2]and European Society of Cardiology (ESC)^{[9, 10]} adult congenital heart disease guidelines may be reviewed for further details regarding diagnosis and management. (See also the Guidelines section.) The AHA/ACC^[11] and ESC^[12] guidelines on the management of patients with valvular heart disease are also available.

Infective endocarditis prophylaxis

The 2017 American Heart Association (AHA)/American College of Cardiology (ACC) focused update of the 2014 AHA/ACC guideline for the management of patients with valvular heart disease no longer recommends antibiotic prophylaxis for isolated PS.^[13]

Pregnancy

Avoidance of vigorous exercise in pregnancy is recommended, especially during the second half of pregnancy in patients with moderate or severe pulmonic stenosis (pulmonary stenosis) (PS). One study found that pregnant patients with PS had favorable outcomes and low maternal and fetal complications. This is in contrast to left heart obstructive lesions such as aortic and mitral stenosis.^[14]

Athletes

Athletes with mild PS stenosis (peak gradient < 30 mmHg) and normal right ventricular (RV) function have no activity limitations.

Athletes treated by operation or balloon valvuloplasty with only mild residual gradients (< 30 mmHg) have no activity restrictions.

Those with moderate (30-50 mmHg) or severe (>50 mmHg) PS can participate in low-intensity competitive sports; their treatment should be directed by the criteria discussed in Treatment.

Guidelines

Ruckdeschel E, Kim YY. Pulmonary valve stenosis in the adult patient: pathophysiology, diagnosis and management. Heart. 2019 Mar. 105 (5):414-22. [QxMD MEDLINE Link].
[Guideline] Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, et al. 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). Circulation. 2008 Dec 2. 118(23):e714-833. [QxMD MEDLINE Link].
Heaton J, Kyriakopoulos C. Pulmonic stenosis [updated 2020 Aug 10]. StatPearls [Internet]. [Full Text].
Odenwald T, Taylor AM. Pulmonary valve interventions. Expert Rev Cardiovasc Ther. 2011 Nov. 9(11):1445-57. [QxMD MEDLINE Link].
Shaath G, Mutairi MA, Tamimi O, Alakhfash A, Abolfotouh M, Alhabshan F. Predictors of re-intervention in neonates with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum. Catheter Cardiovasc Interv. 2011 Sep 27. [QxMD MEDLINE Link].
Mourad Jarrar, Fethi Betbout, Mohamed Ben Farhat, Faouzi Maatouk Habib Gamra, Faouzi Addad, Sonia Hammani, et al. Long-term invasive and noninvasive results of percutaneous balloon pulmonary valvuloplasty in children, adolescents, and adults. American Heart Journal. November, 1999. 138, Issue 5:950-954. [Full Text].
Earing MG, Connolly HM, Dearani JA, Ammash NM, Grogan M, Warnes CA. Long-term follow-up of patients after surgical treatment for isolated pulmonary valve stenosis. Mayo Clin Proc. 2005 Jul. 80 (7):871-6. [QxMD MEDLINE Link].
Ko S, Komuro J, Katsumata Y, et al. Peripheral pulmonary stenosis with Noonan syndrome treated by balloon pulmonary angioplasty. Pulm Circ. 2020 Oct-Dec. 10 (4):2045894020954310. [QxMD MEDLINE Link]. [Full Text].
[Guideline] Baumgartner H, De Backer J, Babu-Narayan SV, et al, for the ESC Scientific Document Group . 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J. 2020 Aug 29. ehaa554. [QxMD MEDLINE Link]. [Full Text].
[Guideline] Baumgartner H, De Backer J. The ESC clinical practice guidelines for the management of adult congenital heart disease 2020. Eur Heart J. 2020 Nov 14. 41 (43):4153-4. [QxMD MEDLINE Link]. [Full Text].
[Guideline] Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP 3rd, Guyton RA, et al. 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation. 2014 Jun 10. 129(23):e521-643. [QxMD MEDLINE Link].
[Guideline] Vahanian A, Alfieri O, Andreotti F, Antunes MJ, Barón-Esquivias G, Baumgartner H, et al. Guidelines on the management of valvular heart disease (version 2012). Eur Heart J. 2012 Oct. 33(19):2451-96. [QxMD MEDLINE Link].
[Guideline] Rick A. Nishimura, Catherine M. Otto, Robert O. Bonow, Blase A. Carabello, John P. Erwin, Lee A. Fleisher, et al. 2017 AHA/ACC Focused Update of the 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 3/15/2017. 1-59.
Hameed AB, Goodwin TM, Elkayam U. Effect of pulmonary stenosis on pregnancy outcomes--a case-control study. Am Heart J. November 2007. 154:852. [QxMD MEDLINE Link]. [Full Text].
Wang J, Zhou Y, Wang Q, Du B, Wu Y, Chen Q, et al. Elabela: a novel biomarker for right ventricular pressure overload in children with pulmonary stenosis or pulmonary atresia with intact ventricular septum. Front Cardiovasc Med. 2020. 7:581848. [QxMD MEDLINE Link]. [Full Text].

Media Gallery

Pulmonic Stenosis. Echocardiogram of a patient with severe pulmonic stenosis. This image shows a parasternal short-axis view of the thickened pulmonary valve.
Pulmonic Stenosis. Echocardiogram of a patient with severe pulmonic stenosis. This image shows a Doppler scan of the peak velocity (5.2 m/s) and gradients (peak 109 mmHg, mean 65 mmHg) across the valve.
Pulmonic Stenosis. Echocardiogram of a patient with severe pulmonic stenosis. This image shows moderately severe pulmonary insufficiency (orange color flow) is also present.
Pulmonic Stenosis. This is an echocardiogram of a patient with severe pulmonic stenosis. The first segment shows the parasternal short-axis view of the thickened pulmonary valve. The second segment shows the presence of moderate pulmonary insufficiency (orange color flow). AV = aortic valve; PA = pulmonary artery; PI = pulmonary insufficiency; PV = pulmonary valve.