Pulmonary Valve Disease

Overview

Pulmonary valve disease refers to any condition affecting the valve between the heart's right lower chamber and the pulmonary artery, the large blood vessel that carries blood to the lungs. The pulmonary valve normally has three leaflets and opens and closes with each heartbeat to keep blood flowing in one direction toward the lungs. When the valve cannot open fully or does not close properly, the workload on the right lower chamber increases and serious problems can develop over time.

Pulmonary valve disease is less common than left-sided valve conditions. The most frequent type is pulmonary valve stenosis, which in the great majority of cases is a congenital condition present from birth. Pulmonary valve regurgitation most often develops as a consequence of surgical repair of congenital heart disease or as a complication of another cardiac condition.

The course of pulmonary valve disease depends greatly on its type and severity. In mild cases, monitoring alone is sufficient. In moderate to severe disease, intervention is needed. With early diagnosis and appropriate treatment, most people can lead long and active lives.

Types

• Pulmonary valve stenosis. The most common type. The valve leaflets are thickened, stiffened, or fused together and cannot open fully. Less blood passes from the right lower chamber to the pulmonary artery, and the right lower chamber must work harder to overcome the resistance. Over time, its walls thicken. Pulmonary valve stenosis is almost always congenital in origin. When mild to moderate, it can go undetected for decades without causing symptoms.
• Pulmonary valve regurgitation. The valve does not close completely and blood leaks back from the pulmonary artery into the right lower chamber. The right lower chamber must pump this extra volume with every beat and gradually enlarges. This form most often develops after surgical repair of congenital heart conditions such as tetralogy of Fallot, in which the pulmonary valve is widened or removed as part of the procedure. Pulmonary hypertension and rheumatic fever are less common causes.
• Pulmonary valve atresia. A rare and severe congenital anomaly in which the pulmonary valve never develops or is completely blocked. This condition requires treatment immediately after birth.

Symptoms

The symptoms of pulmonary valve disease vary considerably depending on the type and severity of the condition. Mild stenosis or regurgitation may produce no symptoms for many years.

• Shortness of breath. This may occur particularly during physical exertion. When the right lower chamber is overworked or unable to pump enough blood, breathing can become more difficult.
• Fatigue and weakness. When the body receives less blood than it needs, a persistent sense of exhaustion may develop. Physical activities that were previously manageable can feel increasingly demanding.
• Palpitations or irregular heartbeat. As the right lower chamber enlarges under increased strain, rhythm disturbances can develop. The heart may feel as though it is racing, fluttering, or beating irregularly.
• Swelling in the legs and ankles. When right heart failure develops, fluid can accumulate in the body.
• Dizziness or lightheadedness. In significant stenosis, reduced blood flow from the right lower chamber can affect circulation and produce dizziness.
• Fainting. In severe pulmonary stenosis, particularly during exertion, insufficient cardiac output can cause loss of consciousness. This symptom should always be taken seriously.
• A bluish tinge to the lips or fingertips. In very severe pulmonary stenosis or pulmonary valve atresia, inadequate oxygenation of the blood can cause visible bluish discoloration. This is a serious sign requiring immediate medical attention.

When to Seek Medical Care

See a doctor if you notice any of the following.

• Shortness of breath during activity or at rest
• A noticeable and unexplained decline in exercise capacity
• Palpitations or a sensation of irregular heartbeat
• Swelling in the legs or ankles
• Dizziness or lightheadedness

Call emergency services immediately if you experience any of the following.

• Fainting or nearly fainting
• Sudden, severe shortness of breath
• Sudden, severe chest pain
• A sudden bluish discoloration of the lips or fingertips

Causes

• Congenital valve abnormalities. The most common cause of pulmonary valve disease. The leaflets may develop with fewer than three components, be fused, or be abnormally thick from birth. Tetralogy of Fallot, pulmonary valve atresia, and other complex congenital heart conditions also affect the pulmonary valve.
• Regurgitation following congenital heart surgery. In conditions such as tetralogy of Fallot, surgical repair often requires widening or removing the pulmonary valve to relieve obstruction. Pulmonary regurgitation is a frequent consequence of these procedures. Long-term follow-up after childhood heart surgery is essential for this reason.
• Pulmonary hypertension. Elevated pressure in the lung vessels places increased demand on the pulmonary valve and can over time contribute to regurgitation.
• Rheumatic fever. A rare cause of pulmonary valve disease. When it does occur, other valves are almost always affected as well.
• Infective endocarditis. Pulmonary valve infection is much less common than infection of the left-sided valves, but it should not be overlooked, particularly in people who use intravenous drugs.
• Carcinoid syndrome. Hormones secreted by certain digestive system tumors can accumulate on the heart valves and cause pulmonary valve damage. This is an uncommon but recognized cause.

Risk Factors

• A history of congenital heart disease. The majority of people with pulmonary valve disease have an underlying congenital cardiac condition.
• Prior congenital heart surgery. People who underwent operations for tetralogy of Fallot or similar conditions in childhood need ongoing monitoring for pulmonary regurgitation in adult life.
• Pulmonary hypertension. Elevated pressure in the lung vessels from any cause places increased strain on the pulmonary valve.

Diagnosis

• Medical history and physical examination. The onset and progression of symptoms are discussed. A history of congenital heart disease or prior heart surgery is particularly important. When the doctor listens to the heart, a murmur associated with pulmonary valve disease may be heard. A characteristic ejection murmur during systole, the phase when the heart contracts, is typical of pulmonary stenosis.
• Echocardiogram (heart ultrasound). The cornerstone of diagnosis and monitoring. It shows the structure and motion of the valve leaflets, quantifies the degree of stenosis or regurgitation, assesses the size, wall thickness, and function of the right lower chamber, and provides an estimate of pulmonary artery pressure.
• Cardiac MRI. Provides highly precise measurements of the right lower chamber's size and function. It can quantify the volume of pulmonary regurgitation accurately, which is not always possible with echocardiography alone. Cardiac MRI plays a particularly important role in the evaluation of patients with congenital heart disease and in guiding the timing of surgery.
• Electrocardiogram (ECG). Used to identify electrical changes associated with right lower chamber hypertrophy or enlargement.
• Chest X-ray. Can show enlargement of the right side of the heart and dilation of the pulmonary artery.
• Computed tomography. Used to image the pulmonary artery and major vessels in detail. It may be particularly useful for evaluating the complex anatomy of congenital cardiac abnormalities.

Treatment

Treatment of pulmonary valve disease is determined by the type, severity, and whether symptoms are present.

• Monitoring. In mild to moderate pulmonary valve stenosis, monitoring alone may be sufficient for many years. Regular echocardiography and clinical review track the state of the right lower chamber and assess whether the stenosis is progressing.
• Medications. Medications cannot correct the underlying valve problem. However, when right heart failure symptoms develop, diuretics can help relieve fluid accumulation. Rhythm disturbances may require specific medications, and anticoagulation is needed when atrial fibrillation is present.
• Balloon pulmonary valvuloplasty. The preferred first interventional treatment for pulmonary stenosis. It does not require open heart surgery. A thin catheter passed through a blood vessel in the groin is guided to the pulmonary valve, where a small balloon is inflated to push the leaflets apart and widen the opening. This approach produces excellent results, particularly when the leaflets are not heavily calcified.
• Surgical valve repair or replacement. Considered when balloon valvuloplasty is not suitable or has not been successful. In complex congenital anomalies, the anatomy of the valve determines the specific surgical approach.
• Transcatheter pulmonary valve implantation. In suitable patients who have previously undergone surgery for congenital heart disease and have since developed pulmonary regurgitation, a new pulmonary valve can be delivered through a catheter without open heart surgery. This approach is particularly valuable for reducing the risk associated with repeated open heart operations.

Lifestyle and Follow-up

Pulmonary valve disease requires lifelong monitoring. This is especially true for people who underwent heart surgery for congenital disease in childhood and have entered adult life. Follow-up for this group is ideally conducted at centers with specific expertise in adult congenital heart disease.

Echocardiography and, when needed, cardiac MRI are used at regular intervals to assess the right lower chamber and valve function. The frequency of follow-up depends on the type and severity of the condition.

Rhythm disturbances are a common complication of pulmonary valve disease and require regular ECG monitoring. Changes in exercise capacity are also an important indicator to watch for between appointments.

Inform your dentist and every treating doctor about your pulmonary valve condition or any prior heart surgery. Some patients may be advised to take antibiotics before dental procedures and certain surgical interventions to reduce the risk of valve infection.

Do not wait for a scheduled appointment if new symptoms develop. Contact your doctor promptly if breathlessness, palpitations, swelling, or a decline in exercise capacity appears or worsens.