Pulmonic Valvular Stenosis Treatment & Management

Updated: Aug 17, 2023
  • Author: Victoria Zaccone, MD; Chief Editor: Barry E Brenner, MD, PhD, FACEP  more...
  • Print
Treatment

Approach Considerations

Surgical management is the definitive treatment for pulmonic valvular stenosis (PVS). Patients with congestive heart failure may benefit from anticongestive therapy.

Interventions for pulmonic valvular stenosis include balloon dilatation, stenting, and pulmonic valve replacement. [32, 33, 34, 35]

Current guidelines recommend balloon valvuloplasty as the treatment of choice for all patients who can anatomically undergo the procedure. [22, 30] The American Heart Association (AHA) and American College of Cardiology (ACC) have established the following criteria [30] :

  • Asymptomatic patients with peak doppler gradient >60 mm Hg are recommended for balloon valvotomy.

  • Symptomatic patients with a peak doppler gradient >50 mm Hg and a domed pulmonic valve are recommended for balloon valvotomy.

When surgical valve repair is required, it is typically in the setting of severe stenosis along with concomitant severe pulmonary regurgitation, hypoplastic annulus, subvalvular or supravalvular stenosis, or in a patient with dysplastic valves. Additionally, it may be considered when the patient is already undergoing a concurrent cardiac surgery.

Outcomes after balloon or surgical valvulotomy are generally excellent. Stenosis usually does not recur, and right ventricular hypertrophy often regresses. [11] A 2007 study presented long-term follow-up data on 90 adult patients who had pulmonary balloon valvuloplasty. Outcome data was excellent, and the study supports the use of balloon angioplasty. [36]

Next:

Prehospital Care

Oxygen should be administered to any patient in respiratory distress. Oxygen may also assist with pulmonary artery vasodilation, thus increasing pulmonary blood flow. Use of oxygen may reduce pulmonary artery pressure in patients with a reactive pulmonary vasculature, thus increasing pulmonary blood flow.

Previous
Next:

Emergency Department Care

Clinical evaluation and echocardiography will direct management. Patients presenting in extremis will need rapid stabilization, and it is recommended that transfer to a regional congenital heart disease center is initiated. [2]

Hospitalization

Intervention with either balloon angioplasty or valve repair is indicated for patients with severe or symptomatic infundibular or supravalvular pulmonic valvular stenosis (PVS). This is seen clinically with peak valve gradients more than 50 mm Hg, or for patients experiencing angina, presyncope, syncope, or exertional dyspnea. Corrective options include open heart surgery, balloon angioplasty, percutaneous stenting, percutaneous valve replacement, or percutaneous conduit placement. [11]

In infants, critical pulmonic valvular stenosis may present with near pulmonary atresia (a cyanotic lesion) with a small and often inadequate right ventricle. These patients survive because of a patent ductus arteriosus. Pulmonary valve atresia or critical pulmonic valvular stenosis with inadequate right ventricle requires a shunt (usually modified Blalock-Taussig or central shunt) after the ductus is kept patent pharmacologically with prostaglandin E1. [11] Definitive repair may not be possible if the right ventricle is hypoplastic, requiring a single ventricular palliation, such as the Fontan procedure, or a variation, such as a direct right atrial appendage to main pulmonary artery anastomosis. [17] Frequently, the main and branch pulmonary arteries require augmentation.

Patients with infundibular or supravalvular pulmonic stenosis, if severe, require operative and invasive surgical interventions.

A surgical approach is often preferred in patients with Noonan syndrome because of the degree of immobility that is often present. [37]

Patients who require such interventions should be transferred to a tertiary care center that specializes in congenital heart disease (CHD).

Previous
Next:

Complications

Postprocedure complications are rare. Infective endocarditis of the pulmonary valve is very rare. During the Second Natural History Study of Congenital Heart Defects, 592 patients with pulmonic valvular stenosis (PVS) were followed for 10,688 person-years; only one patient had an episode of bacterial endocarditis. [38] Current guidelines do not recommend for prophylaxis with antibiotics. [2]

Previous
Next:

Long-Term Monitoring

In the unoperated, asymptomatic patient with pulmonic valvular stenosis (PVS), ACC/AHA guidelines outline the following [2] :

  • Initial evaluation for all patients includes two-dimensional (2D) echocardiography, chest radiography, and electrocardiography (ECG).

  • In mild pulmonic valvular stenosis, follow up exams every 5 years with 2D echocardiography and ECG are recommended.

  • In moderate to severe pulmonic valvular stenosis, follow up exams every 2-5 years with 2D echocardiography are recommended.

Most patients with murmurs are given prophylaxis against infective subacute bacterial endocarditis (SBE). [39] Opinions differ about the need for SBE prophylaxis recommendations for patients with pulmonic valvular stenosis because of the extremely low incidence of endocarditis in this relatively large subpopulation. [39] Current ACC/AHA guidelines do not advise prophylaxis for pulmonic valvular stenosis. [2]

Previous