Pulmonary artery stenosis

Overview

Pulmonary artery stenosis is a heart defect that causes a narrowing of the pulmonary artery, the large blood vessel that takes blood from the right ventricle of the heart to the lungs. This narrowing may force the heart to pump harder, leading to an enlarged heart and high blood pressure in the right side of the heart. In mild cases, there may be no symptoms.

This condition often is associated with other medical problems, such as a congenital heart defect (a heart defect that's present since birth), a genetic abnormality or an infection during pregnancy. Some children and young adults develop these narrowed lung arteries after birth due to a blood-clotting problem.

Signs & symptoms

Children and adults with mild pulmonary artery stenosis often don't have symptoms. The first sign of the condition often is a heart murmur, an extra sound heard during a chest examination. If the condition is severe, symptoms may include:

• Episodes of pain in the chest causing difficulty breathing
• Low energy
• Poor feeding
• Rapid breathing
• Tiring early with exercise
• Turning blue, a condition called cyanosis

Diagnosis

Because there may be no obvious symptoms of pulmonary artery stenosis, the first sign of the condition often is a heart murmur, an extra sound heard during a chest examination. To diagnose pulmonary artery stenosis, the following tests may be performed:

• Echocardiogram, a test that uses sound waves to create a moving picture of the heart
• Electrocardiogram (ECG or EKG), a test that records the electrical activity of the heart
• Exercise stress test to test the effect of exercise on the heart
• Magnetic resonance imagining (MRI) or computed tomography (CT) scan may be performed to better see the restricted area and surrounding blood vessels

Treatment

Treatment for pulmonary artery stenosis will vary depending on the severity of the condition and age of the patient.

Babies, children and young adults may be treated in our Pediatric Cardiac Catheterization Laboratory with a procedure called balloon angioplasty. This procedure involves inserting a catheter — a thin, flexible, plastic tube — into a small incision in the leg and threading it through a blood vessel to the heart.

A balloon at the tip of the catheter is inserted into the narrow opening of the artery and inflated to stretch the artery. Occasionally, a metal tube, called a stent, is placed in the artery to keep it expanded. This procedure takes three to five hours, depending on the number of blocked arteries. Patients stay overnight in the hospital and usually go home the next day.

The possibility of a complication due to the procedure is very low. But if a complication does occur, it can be serious, such as bleeding into the lung, which can be life-threatening.

In some cases, the pulmonary artery stenosis is unusually thick and angioplasty with stent repair is not effective. In that case, surgery may be required.