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# Pulmonary valve stenosis

The valve between the [right ventricle](https://durgical.com/en/anatomy/right-ventricle/) and the lung artery is narrow, so the heart works harder. Usually present at birth and treated with a balloon.

Updated 2026-11-01

## Structures involved

Tap one to see what it is and open it in 3D.

- [Pulmonary anterior cusp](https://durgical.com/en/anatomy/anterior-semilunar-leaflet-of-pulmonary-valve/)
- [Pulmonary left cusp](https://durgical.com/en/anatomy/left-semilunar-leaflet-of-pulmonary-valve/)
- [Pulmonary right cusp](https://durgical.com/en/anatomy/right-semilunar-leaflet-of-pulmonary-valve/)
- [Pulmonary trunk](https://durgical.com/en/anatomy/pulmonary-trunk/)
- [Right ventricle](https://durgical.com/en/anatomy/right-ventricle/)
- [Pulmonary arteries](https://durgical.com/en/anatomy/right-pulmonary-artery/)
- [Right atrium](https://durgical.com/en/anatomy/right-atrium/)
- [Interatrial septum](https://durgical.com/en/anatomy/interatrial-septum/)
- [Valve rings (fibrous skeleton)](https://durgical.com/en/anatomy/fibrous-rings-of-the-heart-valves/)

## Why it happens

- [Hypertrophy: physiologic and pathologic](https://durgical.com/en/general-pathology/hypertrophy-physiologic-and-pathologic/)
- [Murmurs and the hemodynamics of valve disease](https://durgical.com/en/physiology/murmurs-and-the-hemodynamics-of-valve-disease/)
- [Preload, afterload and contractility](https://durgical.com/en/physiology/preload-afterload-and-contractility/)

## What it is

Pulmonary valve stenosis is narrowing of the pulmonary valve that obstructs flow from the right ventricle to the pulmonary trunk. It is almost always congenital: most often a thin, dome-shaped valve with fused commissures, less often a thick, dysplastic valve. Obstruction can also lie below the valve (subvalvular, as in tetralogy of Fallot) or above it (supravalvular or branch pulmonary artery stenosis).

## Who gets it

Isolated pulmonary valve stenosis accounts for roughly 8–10% of congenital heart disease and is often found as a murmur in an otherwise well child. Acquired forms are rare (carcinoid heart disease, rheumatic disease). Many mild cases are first detected at routine child or school check-ups.

## Causes

- Congenital fusion of the valve commissures (dome-shaped valve)
- Dysplastic, thickened valve, typical of Noonan syndrome
- Part of complex defects such as tetralogy of Fallot
- Congenital rubella (mainly peripheral pulmonary artery stenosis) and Williams or Alagille syndrome (supravalvular and branch stenosis)
- Acquired: carcinoid heart disease, rarely rheumatic disease or external compression

## Risk factors

- Noonan syndrome and other RASopathies
- Maternal rubella in pregnancy
- Family history of congenital heart disease

## Pathophysiology

The narrowed valve creates a pressure gradient between the right ventricle and the pulmonary trunk. The right ventricle compensates by concentric hypertrophy, and jet flow causes post-stenotic dilation of the pulmonary trunk. Mild stenosis rarely progresses, while severe stenosis eventually reduces right ventricular compliance and output, raises right atrial pressure and may open a patent [foramen ovale](https://durgical.com/en/anatomy/foramen-ovale/) with right-to-left shunting and cyanosis. In critical neonatal stenosis, lung blood flow depends on the ductus arteriosus.

## Classification

- By level: valvular (most common), subvalvular, supravalvular, branch pulmonary artery stenosis
- By valve type: dome-shaped (thin, fused commissures) vs dysplastic
- By peak Doppler gradient: mild, moderate, severe
- Critical pulmonary stenosis of the newborn (ductus-dependent pulmonary blood flow)

## Symptoms and signs

- Most mild and moderate cases are asymptomatic and found because of a murmur
- Exertional dyspnoea and fatigue in severe stenosis
- Exertional chest pain or syncope (fixed right ventricular output)
- Right-heart failure signs (oedema, hepatomegaly) in long-standing severe disease
- Cyanosis and poor feeding in critical neonatal stenosis

## Red flags

- Cyanotic newborn with a systolic murmur (critical stenosis, ductus-dependent)
- Syncope or chest pain on exertion
- Signs of right ventricular failure

## Diagnosis

**Clinical**
Crescendo-decrescendo systolic ejection murmur at the left upper sternal border, preceded by an ejection click that softens with inspiration (the only right-sided sound that does so); widely split S2 with a soft P2; right ventricular heave in severe cases. Look for features of Noonan syndrome (short stature, webbed neck, hypertelorism).

**Tests**
ECG: normal when mild; right [axis](https://durgical.com/en/anatomy/axis/) deviation and right ventricular hypertrophy when severe. Pulse oximetry in newborns.

**Imaging**
Echocardiography confirms the level and morphology of obstruction and measures the peak gradient, right ventricular size and function and any atrial shunt. [Chest](https://durgical.com/en/anatomy/region/chest/) X-ray may show a prominent [pulmonary trunk](https://durgical.com/en/anatomy/pulmonary-trunk/) (post-stenotic dilation) with normal or reduced lung markings. Cardiac MRI or CT for branch pulmonary artery stenosis.

## Differential diagnosis

- Atrial septal defect (flow murmur, fixed split S2)
- Innocent pulmonary flow murmur in children
- Tetralogy of Fallot
- Ventricular septal defect
- Aortic stenosis (murmur radiates to the carotids)

## Goal of treatment

Relieve significant obstruction early, before right ventricular hypertrophy and failure develop, and keep pulmonary blood flow adequate in critical neonatal disease.

## Treatment

**Acute**
Critical neonatal stenosis: prostaglandin E1 infusion to keep the ductus arteriosus open until the valve is opened.

**Procedure**
Percutaneous balloon pulmonary valvuloplasty is the first-line treatment for moderate to severe valvular stenosis with a dome-shaped valve.

**Surgical**
Surgical valvotomy, partial valvectomy or valve replacement for dysplastic valves, subvalvular or supravalvular obstruction, or failed balloon dilation.

**Follow-up**
Mild stenosis: periodic clinical review and echocardiography; after intervention, follow-up for pulmonary regurgitation and restenosis. Nursing after catheterisation: puncture site, distal pulses and rhythm monitoring.

## Complications

- Right ventricular hypertrophy and failure
- Pulmonary regurgitation after valvuloplasty or surgery
- Arrhythmias and, rarely, sudden death in severe disease
- Infective endocarditis (uncommon)
- Right-to-left shunt through a patent foramen ovale with cyanosis

## Prognosis

Mild stenosis has an excellent outlook and seldom progresses. After balloon valvuloplasty long-term results are very good; some patients later need treatment of pulmonary regurgitation.

## Prevention

- Rubella vaccination before pregnancy
- Prenatal ultrasound and newborn pulse oximetry screening for critical disease

## Test yourself

Think of the answer, then tap the question to see it.

## Which genetic syndrome is classically associated with a dysplastic pulmonary valve?

Noonan syndrome.

## How does the pulmonary ejection click change with inspiration?

It becomes softer, unlike other right-sided sounds.

## What is first-line treatment for a dome-shaped stenotic pulmonary valve?

Percutaneous balloon valvuloplasty.

## What chest X-ray finding results from jet flow through the valve?

Post-stenotic dilation of the pulmonary trunk.

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## Related conditions

- [Noonan syndrome](https://durgical.com/en/conditions/noonan-syndrome/)
- [Tetralogy of Fallot](https://durgical.com/en/conditions/tetralogy-of-fallot/)
- [Atrial septal defect](https://durgical.com/en/conditions/atrial-septal-defect/)
- [Carcinoid syndrome](https://durgical.com/en/conditions/carcinoid-syndrome/)
- [Rubella and congenital rubella syndrome](https://durgical.com/en/conditions/rubella-and-congenital-rubella-syndrome/)
- [Tricuspid regurgitation and tricuspid valve disease](https://durgical.com/en/conditions/tricuspid-regurgitation-and-tricuspid-valve-disease/)
- [Heart failure](https://durgical.com/en/conditions/heart-failure/)
- [Infective endocarditis](https://durgical.com/en/conditions/infective-endocarditis/)
- [Pulmonary hypertension and cor pulmonale](https://durgical.com/en/conditions/pulmonary-hypertension-and-cor-pulmonale/)

Educational information, not medical advice. For any symptom, see a health professional.

Sources: [Pulmonic valve stenosis](https://medlineplus.gov/ency/article/001096.htm) (MedlinePlus, US National Library of Medicine, 2026).

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Representative model, for educational purposes only.

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Source: https://durgical.com/en/conditions/pulmonary-valve-stenosis/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/conditions/pulmonary-valve-stenosis/