Pulmonary stenosis is mostly congenital.
Symptoms range from none to profound cyanosis and the potential for sudden death.
Systolic ejection murmur is present and is loudest over left upper sternal border.
Cyanotic patients are treated with oxygen and prostaglandin E1 prior to diagnostic testing.
The diagnosis is confirmed and severity classified by echocardiography.
Mild pulmonary stenosis is a benign condition requiring sequential cardiac follow-up but no therapy.
Percutaneous balloon pulmonary valvuloplasty (PBPV) is indicated in moderate to severe/critical lesions.
Surgical valvotomy is reserved for treatment failure and contraindication to PBPV.
Pulmonary stenosis (PS) obstructs the blood flow from the right ventricle (RV) into the pulmonary bed, resulting in a pressure gradient greater than 10 mmHg across the pulmonary valve during systole. In 80% to 90% of all cases PS is found at the level of the valve, but it can also occur below the level of the valve or distally in the pulmonary arteries. PS is commonly associated with other forms of congenital heart disease. The increased subpulmonary ventricular pressure may lead to hypertrophy of the RV proportional to the degree of stenosis. Clinical symptoms range from none in mild PS to profound cyanosis and the potential for sudden death in critical PS.
History and exam
Key diagnostic factors
- pathological systolic ejection murmur with or without a systolic click
Other diagnostic factors
- rheumatic fever
- chest pain
- dysmorphic features of Noonan syndrome
- dysmorphic features of Noonan syndrome with multiple lentigines
- dysmorphic features of Williams syndrome
- dysmorphic features of Alagille's syndrome
- failure to thrive
- signs of right heart failure
- right ventricular heave
- systolic thrill
- black ethnicity
- Noonan syndrome
- Noonan syndrome with multiple lentigines
- Alagille syndrome
- Williams syndrome
- congenital rubella syndrome
- carcinoid syndrome
- infectious endocarditis
- myocardial tumours
- external compression
1st investigations to order
- chest x-ray
- echocardiography with Doppler assessment
Investigations to consider
- diagnostic cardiac catheterisation
- Hb and Hct
- pulse oximetry
- arterial blood gas
- cardiac MRI
- cardiac CT
- exercise stress testing
severe to critical disease
- Innocent murmur
- Straight back syndrome
- Idiopathic dilation of pulmonary artery
- Guideline on management and re-interventional therapy in patients with congenital heart disease long-term after initial repair
- Guidelines for cardiovascular intervention in adults with congenital heart disease
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