Medical expert of the article
New publications
Isolated pulmonary artery stenosis: symptoms, diagnosis, treatment
Last reviewed: 07.07.2025
All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
Isolated pulmonary artery stenosis accounts for 6 to 8% of all congenital heart defects. Most often, the narrowing is located in the area of the pulmonary artery valves and is represented by a diaphragm with a central or eccentric opening with a diameter of 1 to 10 mm.
Due to the narrowing, a pressure gradient is formed between the right ventricle and the pulmonary artery. The pressure in the right ventricle increases, tricuspid valve insufficiency occurs, right ventricular failure develops with an enlarged liver and increasing edema. If the oval window is open, blood can be discharged through it under high pressure. In this case, diffuse cyanosis occurs.
On examination, the heart area is visually unchanged, the boundaries of relative cardiac dullness are slightly expanded. Systolic murmur is heard in the second-third intercostal space on the left.
[ 1 ]
How to recognize isolated stenosis of the pulmonary artery?
The ECG reveals a deviation of the electrical axis of the heart to the right, signs of overload of the right ventricle, often an incomplete block of the right bundle branch of His, and signs of overload of the right atrium.
Radiographically, the pulmonary pattern is depleted; the size of the heart depends on the size of the right-left shunt (if present) and on the overload of the heart cavities.
The most noticeable echocardiographic sign of heart defect is an enlargement of the right ventricle and thickening of the interventricular septum. In addition, a violation of the opening of the pulmonary valve is revealed, the thickened cusps of which bulge in an arc during systole into the dilated pulmonary trunk. Doppler echocardiography shows turbulent blood flow on the valve, the presence of a pressure gradient.
Cardiac catheterization and angiocardiography are indicated only in cases of balloon valvuloplasty.
Differential diagnosis should first of all be carried out with an atrial septal defect, since the systolic murmur in the second intercostal space on the left in both cases is caused by a narrowing of the outflow tract of the right ventricle.
What do need to examine?
How to examine?
Treatment of isolated pulmonary artery stenosis
Drug treatment is ineffective. In newborns, prostaglandins can be used to maintain pulmonary blood flow. Surgical treatment involves transluminal balloon valvuloplasty. The intervention can be performed at any age, and also repeatedly. Open-heart surgery is indicated for severe valve dysplasia and infundibular stenosis requiring correction.
Использованная литература