Overview
Pulmonary stenosis (PS) describes the narrowing of the tricuspid valve leading to obstructed blood flow through the pulmonary valve, which separates the right ventricle and pulmonary artery. This obstruction can lead to increased workload on the right ventricle and right ventricular hypertrophy.
Epidemiology
- Pulmonary valve disease is rare and mostly caused by congenital disorders
Causes
- Tetralogy of Fallot
- Noonan syndrome
- Maternal rubella syndrome
- Infective endocarditis
- Carcinoid syndrome
- Marfan syndrome
Presentation
A key finding in PS is an ejection systolic murmur that is louder on inspiration. Other features include:
- Shortness of breath
- Chest pain
- Exertional syncope
- Sudden cardiac death
- Critical PS – causes cyanosis in neonates
Investigations
Overview
- ECG:
- May show right atrial/ventricular hypertrophy
- Chest X-ray:
- May show dilation of the pulmonary artery
- May show enlargement of the right atrium
- Echocardiogram:
- Identifies PS
Management
Overview
Management involves treating the underlying cause, managing heart failure, and surgery.
Complications
- Infective endocarditis
- Arrhythmia
- Heart failure
Prognosis
- Critical PS is fatal in neonates if untreated
- The prognosis depends on the severity of PS and any resulting right ventricular/atrial remodelling
