Pulmonic Stenosis (Pulmonary Stenosis) Treatment & Management

Updated: Dec 14, 2020
  • Author: Priya Pillutla, MD; Chief Editor: Richard A Lange, MD, MBA  more...
  • Print
Treatment

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]

Next:

Activity

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.

Previous