Pulmonary vein stenosis

A condition involving narrowing of the pulmonary veins

Pulmonary vein stenosis

Diagram of the heart showing the pulmonary veins.

Pulmonary vein stenosis is a rare but serious condition characterized by the narrowing of one or more of the pulmonary veins. These veins are responsible for carrying oxygenated blood from the lungs to the left atrium of the heart.

Pathophysiology

Pulmonary vein stenosis can occur as a congenital defect or develop later in life. In congenital cases, the condition is present at birth and may be associated with other congenital heart defects. Acquired pulmonary vein stenosis can result from various causes, including surgical interventions, radiation therapy, or inflammatory processes.

The narrowing of the pulmonary veins leads to increased pressure in the pulmonary circulation, which can result in pulmonary hypertension. This increased pressure can cause strain on the right side of the heart, potentially leading to right heart failure.

Symptoms

The symptoms of pulmonary vein stenosis can vary depending on the severity of the condition. Common symptoms include:

• Dyspnea (shortness of breath)
• Hemoptysis (coughing up blood)
• Fatigue
• Chest pain
• Palpitations

In severe cases, patients may experience cyanosis and signs of heart failure.

Diagnosis

The diagnosis of pulmonary vein stenosis typically involves imaging studies such as:

• Echocardiography
• Computed tomography (CT) scan
• Magnetic resonance imaging (MRI)
• Cardiac catheterization

These tests help visualize the structure of the pulmonary veins and assess the degree of stenosis.

Treatment

Treatment options for pulmonary vein stenosis depend on the severity and underlying cause of the condition. They may include:

• Balloon angioplasty
• Stent placement
• Surgical repair or reconstruction

In some cases, lung transplantation may be considered if the condition is severe and other treatments are not effective.

Prognosis

The prognosis for individuals with pulmonary vein stenosis varies. Early detection and treatment are crucial for improving outcomes. In cases where the condition is associated with other congenital heart defects, the overall prognosis depends on the complexity and severity of the associated conditions.

Related pages

• Pulmonary hypertension
• Congenital heart defect
• Heart failure