Pulmonic Valvular Stenosis Treatment & Management

Updated: Dec 19, 2016
  • Author: Melanie A Loewenthal, MD; Chief Editor: Robert E O'Connor, MD, MPH  more...
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Treatment

Prehospital Care

If the patient has a large left-to-right shunt, such as patent ductus arteriosus or ventricular septal defect, and is in respiratory distress, diuresis is effective in reducing the cyanosis secondary to pulmonary edema.

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

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Emergency Department Care

Management depends on the degree of stenosis. Therefore, clinical evaluation as well as imaging is will direct management. Cardiology or cardiothoracic consultation is needed if imaging studies indicate right outflow tract obstruction.

Note the following:

  • Severe stenosis, a peak gradient of 60 mm Hg or more, requires emergent dilatation of valves by balloon valvulotomy. [11]
  • Patients with mild pulmonic valvular stenosis usually do not require any treatment.
  • Patients with severe or symptomatic infundibular or supravalvular pulmonary stenosis require prompt intervention. Valvulotomy is standard of care in these patients.
  • Frequently, cyanotic infants with respiratory distress and hypotension/shock undergo a workup as that for septic patients.

Bacterial endocarditis prophylaxis

During the Second Natural History Study of Congenital Heart Defects, 592 patients with pulmonic valvular stenosis were followed for 10,688 person-years; only one patient had an episode of bacterial endocarditis. [37]

Pulmonic valvular stenosis is not specifically mentioned in the 2007 American Heart Association (AHA) guidelines for antibiotic prophylaxis to prevent bacterial endocarditis. This guideline recommends prophylaxis for endocarditis in the 6 months following repair of a congenital heart defect. Additionally, prophylaxis is required for lifetime for individuals who have prosthetic valves. [38]

Hospitalization

Intervention with either balloon angioplasty or valve repair is indicated for patients with peak valve gradients more than 50 mm Hg or for patients with angina, syncope, exertional dyspnea, or presyncope. Corrective options include open heart surgery, balloon angioplasty, percutaneous stenting, percutaneous valve replacement, or percutaneous conduit placement. [6]

Galal et al reported on the case of a pregnant patient with severe pulmonary valve stenosis and exertional dyspnea who underwent balloon dilation using sole echocardiographic guidance to protect the baby from radiation. The primary technical difficulty occurred during catheter advancement across the right ventricular outflow tract into the pulmonary valve, which was overcome by using a wedge balloon catheter over a percutaneous transluminal coronary angioplasty wire. The investigators concluded that pulmonary balloon valvuloplasty can be performed safely using sole transthoracic echocardiography guidance without fluoroscopy. [39]

Patients with severe or symptomatic infundibular or supravalvular pulmonary stenosis require surgical intervention. [6]

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. [6] 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. [2] Frequently, the main and branch pulmonary arteries require augmentation.

Consultations

Consult with a pediatric cardiologist and with an intensivist.

Transfer

Patients with symptomatic pulmonic valvular stenosis should be transferred to a tertiary care center offering pediatric cardiology and pediatric cardiothoracic surgery.

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Activity

Patients should maintain normal physical activity.

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Long-Term Monitoring

Most patients with murmurs are given prophylaxis against infective subacute bacterial endocarditis (SBE). [38] 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. [38]

For patients older than 6 months with a gradient less than 40 mm Hg at the time of diagnosis, follow-up care can safely be performed at intervals of 2 years or more.

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