Causes

Narrow entrance to the pulmonary artery

prevent the closure of the foramen ovale

Pulmonary stenosis affects females slightly more than males.

Symptoms

Newborns with critical pulmonary stenosis therefore will have cyanosis (blue discoloration of the lips and nail beds) due to lower oxygen levels in their blood.

Regurgitation

Peripheral edema

Cyanosis intensifies with crying in children with this defect.

It is not alleviated by the administration of 100% oxygen.

Right-sided Heart Failure

Exercise intolerance and fatigue

Heart murmur

Labs/Testing

The echocardiogram is the most important non-invasive test to detect and evaluate pulmonary valve stenosis.

Cardiac catheterization is an invasive technique that enables physicians to accurately measure the degree of pulmonary stenosis that is present.

Treatment

Low sodium diet if heart failure is present

Diuretics, such as furosemide, to address heart failure

Angiotensin-converting enzyme (ACE) inhibitors

For these conditions surgical pulmonary valvotomy (opening of the valve), partial valvectomy (removal of a portion of the leaflet), and possibly a transannular patch (patch from the right ventricle to pulmonary artery) may be required during the open-heart surgery repair.
Oxygen

Cardiac observation and antibiotic prophylaxis for children who are asymptomatic

Interventional catheterization or surgical management for severe stenosis; PGE1 infusion keeps ductus arteriosus open

Valvuloplasty with dilation of valve to decrease pressure

	Created by Kalyn S over 6 years ago

Note on Congenital Heart Disease, created by Kalyn S on 16/03/2018.