Narayana Health Care

What is Pulmonary Valve Stenosis?

Pulmonary valve stenosis is a rare kind of heart valve defect. Heart is the vital organ of the body that pumps blood all through the body and collects back deoxygenated blood returning from different parts of the body.  The heart has 4 chambers 2 upper atrium and 2 bottom ventricles. The atrium and ventricles are separated by valves, which provide unidirectional blood flow. Left ventricle opens in the aorta that supplies oxygenated blood to the body via other arteries.

• Left ventricle and aorta are separated by an Aortic Valve.
• The left atrium and left ventricle are separated by mitral valve
• Right atrium and right ventricle are separated by tricuspid valve
• A valve between the right ventricle and the pulmonary artery is called pulmonary valve

Each valve has three cusps except mitral valve which has two cusps.

In case of pulmonary valve stenosis, the pulmonary valve is shrunk so that it does not open normally. Due to this, the flow of deoxygenated blood that goes through a pulmonary artery to lungs for oxygenation is restricted. The defect may not always present with severe manifestations and the patient usually lives a normal life.

Symptoms

• Mostly asymptomatic
• Heart murmur
• Bluish discolouration of skin
• Chest Pain
• Inflated jugular vein
• Palpitations
• Anaemia
• Generalized weakness
• Breathing distress
• Heart failure
• Episodes of unconsciousness
• Rarely death

Causes

• Unknown cause
• Congenital malformations
• Rheumatic fever
• Carcinoid tumors of the digestive system

Complications of pulmonary valve stenosis

• Overworking of right ventricle causing its enlargement or hypertrophy. Prolonged overworking of the heart may lead to permanent heart damage. Deoxygenated blood flowing through the body gives it a bluish discolouration and difficulty in breathing. Still left untreated it may lead to heart failure and in rare cases death
• An amplified risk of heart infection

Diagnosis

• History
• Physical Examination with a stethoscope- Abnormal heart sounds (rumbling or snapping) are heard at a stethoscope examination. This is presented as a heart murmur
• Electrocardiogram or 24 hour Holter monitoring
• Chest X-Ray
• Echocardiogram
• Transesophageal echocardiogram – A small device inserted orally to look at heart closely
• Cardiac catheterization – Minimally invasive procedure where a tube is inserted and used to create a much clearer picture

Treatment

A lot of people may not require any treatment at all. Symptomatic treatment is given until normal life is not disturbed.

• Medication management

1. Prostaglandins
2. Blood Thinners

• Management of high blood pressure- Beta-blockers

1. Diuretics
2. Antiarrhythmic drugs

• Percutaneous Intervention

1. Pulmonary Valvuloplasty by inserting a catheter with an attached balloon up to your heart at the location of a pulmonary valve and then inflated to keep it open.

• Surgical options for valve replacement include:

1. Mechanical valve — a long-lasting valve made of durable materials
2. Tissue valve (which may include human or animal donor tissue) last 10-20 years
3. Ross Procedure — “Borrowing” your healthy valve and moving it into the position of the damaged valve aortic valve
4. Newer surgery options like donor valve implantation- Human donor valves, only used in conditions like infective endocarditis

Most cases do not even require any management. A few may require above management options. A healthy lifestyle, heart-friendly diet and mild physical activity will take long you a long way forward.

Dr. Sajal Gupta, Consultant – Cardiology, Dharamshila Narayana Superspeciality Hospital, Delhi