Stenosis of pulmonary artery

Stenosis of the pulmonary artery is a congenital heart defect characterized by the narrowing of the pulmonary artery, the major blood vessel responsible for transporting blood from the heart to the lungs for oxygenation. This condition can lead to various complications, including increased pressure in the right ventricle, reduced blood flow to the lungs, and right heart failure.

Causes

Stenosis of the pulmonary artery is most commonly a congenital condition, meaning it is present at birth. The exact cause of this congenital defect is not well understood, but it is believed to involve a combination of genetic and environmental factors. In some cases, it may occur as part of a larger syndrome, such as Tetralogy of Fallot or Williams syndrome.

Symptoms

Symptoms of pulmonary artery stenosis can vary widely depending on the severity of the condition. Mild cases may be asymptomatic and go undiagnosed until later in life. More severe cases can present in infancy or early childhood with symptoms such as:

• Shortness of breath
• Fatigue
• Cyanosis (a bluish tint to the skin, lips, and nails)
• Heart murmur
• Failure to thrive

Diagnosis

Diagnosis of pulmonary artery stenosis typically involves a combination of physical examination, where a healthcare provider may detect a heart murmur, and imaging studies. These imaging studies can include:

• Echocardiography: An ultrasound of the heart that can visualize the structure and function of the heart and pulmonary artery.
• Cardiac MRI: Provides detailed images of the heart and pulmonary artery and can assess the severity of the stenosis.
• Cardiac catheterization: A procedure where a thin tube is inserted into a blood vessel and guided to the heart to measure pressures, assess the severity of the stenosis, and sometimes treat the condition during the procedure.

Treatment

Treatment for pulmonary artery stenosis depends on the severity of the condition. Mild cases may require no immediate treatment but regular monitoring to assess any progression of the disease. More severe cases may require intervention to relieve the obstruction. Treatment options include:

• Balloon valvuloplasty: A procedure performed during cardiac catheterization where a balloon is inflated at the site of the narrowing to widen the pulmonary artery.
• Surgery: In cases where balloon valvuloplasty is not effective or possible, surgical options may include repair or replacement of the pulmonary valve or reconstruction of the pulmonary artery.

Prognosis

The prognosis for individuals with pulmonary artery stenosis varies depending on the severity of the condition and the presence of other heart defects. With early diagnosis and appropriate treatment, many individuals can lead normal, active lives.

See also

• Congenital heart defect
• Pulmonary hypertension
• Heart valve disease