Pulmonary Stenosis

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Definition Aetiology Pathophysiology Risk factor Sign and Symptoms Investigations Management

Pulmonary stenosis is a congenital cardiac abnormality in which the pulmonary valve, which regulates blood flow from the right ventricle of the heart to the lungs, is constricted or obstructed (see figure 1).

It is generally unknown what causes pulmonary stenosis, but it is assumed that an abnormal development of the heart during foetal development is to blame. Hence why, the majority of instances, pulmonary stenosis is a congenital cardiac abnormality, which means it is present at birth. 

However, it is also possible for pulmonary stenosis to develop later in life as a result of other heart disorders or acquired diseases.

In pulmonary stenosis, the pulmonary valve, which regulates blood flow from the right ventricle of the heart to the lungs, is constricted or obstructed. This restriction can hinder the correct flow of oxygen-rich blood to the body and result in a variety of cardiac and lung alterations.

As the right ventricle pumps blood via the constricted valve, the ventricle's pressure rises. This elevated blood pressure can result in hypertrophy, or thickening, of the right ventricular muscle. The thickened muscle may not be able to adequately pump blood, resulting in decreased blood flow throughout the body.

The increased pressure in the right ventricle increases the blood pressure in the pulmonary artery, which supplies the lungs. This increased pressure might cause the pulmonary blood veins to tighten, making blood flow more difficult. This can lead to pulmonary hypertension, which can damage blood vessels in the lungs and make it more difficult for oxygen to reach the body's tissues.

In severe cases of pulmonary stenosis, decreased blood supply to the body can result in symptoms including shortness of breath, fast breathing, and exhaustion. The illness can progress to cardiac failure and other fatal problems if left untreated.

• Genetics: Chromosomal abnormalities such as Down syndrome, Turner syndrome, and DiGeorge syndrome have been linked to an increased incidence of pulmonary stenosis.

• Gender: Pulmonary stenosis is more prevalent in females compared to males.

• Environmental factors: Illnesses or toxin exposure during pregnancy

• Rheumatic fever: is an acquired condition that can damage the heart valves, notably the pulmonary valve.

• Breathlessness: is a frequent symptom of pulmonary stenosis that can occur during physical activity or during rest.

• Tachypnea: As the right ventricle works harder to pump blood via the constricted valve, rapid breathing may occur.

• Tiredness: Reduced blood supply to the body can result in weariness and weakness.

• Chest pain: Some individuals may have chest discomfort or pain, typically during physical exertion.

• Cyanosis is a deficiency of oxygen in the blood that causes the skin, lips, and nails to turn blue or purple.

• A heart murmur is an odd sound heard during a heartbeat that might be generated by a pulmonary valve that is constricted.

• Ankle/feet swelling: The backflow of blood from the right side of the heart and lungs can result in edoema of the legs, ankles, and feet.

• Echocardiogram

• Electrocardiogram (ECG).

• Chest X-ray

• Cardiac catheterization is an invasive procedure in which a catheter is placed into a blood vessel in the arm or leg and led to the heart in order to assess the pressure in the heart and pulmonary artery.

• Magnetic Resonance Imaging (MRI)

• Balloon valvuloplasty is a minimally invasive treatment in which a tiny balloon is inflated within the valve in order to enlarge the orifice and enhance blood flow. The procedure is performed under general anaesthesia.

• Surgery: In moderate to severe cases of pulmonary stenosis, surgical intervention may be required. The most frequent surgical approach includes removing the constricted valve and replacing it with a new one.

• If a patient has a minor case of pulmonary stenosis and is asymptomatic, he or she may be constantly followed to ensure that the condition does not worsen.