Summary

Pulmonary Valve Stenosis is a condition that affects the pulmonary valve in the heart. The valve can develop a set of conditions, one of them being Pulmonary Valve Stenosis. This affects blood-flow to the lungs and reduces the availability of oxygen-rich blood to rest of the body. This leads to various symptoms in the body. In most cases, medication and minimally-invasive procedures will resolve the condition, while in some cases, surgery may be required. In this article, we will learn more.

Introduction

Most of us are aware of the working of the heart. The heart has 4 chambers – 2 upper (called atria) and 2 lower (called ventricles). Impure blood from all over the body arrives at the right ventricle. The right ventricle contracts to push this impure blood into the pulmonary artery. This artery takes the blood to the lungs where gas-exchange happens and the blood gets purified. The purified blood then comes to the left atrium, then left ventricle, from where it’s pumped through the aorta to rest of the body. This cycle repeats again and again, for the entire lifespan of the person.

There are valves that regulate this flow of blood into the major arteries and veins, from the concerned chambers of the heart. The Pulmonary Valve is one of them, and lies at the start of the pulmonary artery. It’s a tricuspid valve meaning, it has 3 flaps (like 3 petals of a flower). When blood has to be pumped into the pulmonary artery, these flaps open, and allow blood-flow. Once that is done, they close tightly on each other to prevent back-flow of blood.

Pulmonary Valve Stenosis (PVS) is one of the 3 conditions that affect the Pulmonary Valve (other 2 being atresia and regurgitation). In this condition, the 3 flaps that make up the valve do not open fully. They have either become thick so they strangle the opening, or they have become stiff, so they don’t open freely. So, the opening available for blood-flow to the lungs, gets reduced (this is what is called stenosis). As a result of PVS, the entire volume of blood present in the right ventricle does not enter the pulmonary artery and reach the lungs.

As a direct consequence, the quantity of purified blood that is available for rest-of-the-body reduces, leading to various symptoms and conditions. Also, now, the right ventricle tries to pump harder (contracts more tightly) to clear the existing volume of blood. Over time, this causes the muscles in the walls of the right ventricle to become hard and thick.

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Also Read: Pulmonary valve endocarditis: a case report

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Causes and Risk Factors

The heart-valves are formed inside the foetus in the first 8 weeks of pregnancy. According to cardiologists, most often, PVS is caused by genetic reasons, irrespective of what the mother did or did not do during pregnancy.

• German measles, also called Rubella: This is a contagious viral infection that is marked by red rashes on the body. A pregnant woman who contracts Rubella increases the risk of PVS in the baby.
• Noonan syndrome: This is a genetically inherited condition that is caused by altered DNA. It is marked by wide eyes, low ears, short stature and many problems with the heart’s structure and/or function, in the growing child.
• Rheumatic fever: Strep throat is a bacterial infection that causes itchy and painful throat. One of its complications is rheumatic fever which is an inflammatory condition. Rheumatic fever in turn can cause permanent damage to the heart and valves of the heart. It also increases the person’s risk of developing PVS in the later years.
• Carcinoid syndrome: Carcinoid tumours are a kind of neuroendocrine tumour that is marked by the release of the hormone serotonin in the blood. Carcinoid syndrome refers to a set of symptoms seen in people with carcinoid tumours. Some people having this syndrome develop carcinoid heart disease which is known to damage heart valves and cause PVS.
• Williams syndrome: Also called Williams-Beuren syndrome, this is a congenital disorder. Babies born with this condition have pronounced facial features while growing up, slow mental development and heart defects such as PVS.
• Infections: Some infections that affect the heart, such as endocarditis, can damage heart valves and eventually cause PVS.
• Previous Heart Surgery: Surgical procedures that involve the heart, especially those that affect the pulmonary valve or nearby tissues, can result in postoperative PVS.
• Idiopathic: In some cases, the cause of PVS is not clear as there is no underlying condition which could have triggered it.

Symptoms

• Heart murmur: a swooshing sound that can be heard with a stethoscope
• Rapid heart-rate and palpitations
• Unexplained fatigue
• Dyspnoea: Shortness of breath, especially during activity
• Chest pain (angina)
• Fainting (syncope)
• An enlarged or prominent jugular vein
• Cyanosis: a bluish tint to the skin
• Difficulty in exercising
• Under-development in children
• Swelling in the hands, feet, legs, belly, or face

Complications

• Endocarditis: This is a bacterial infection of the inner lining of the heart. It is both a risk factor for PVS as well as a complication from PVS.
• Arrhythmias: Or irregular heartbeats. If the PVS is severe, the resulting arrhythmias can be life-threatening.
• Right ventricular hypertrophy (RVH): As mentioned earlier, the right ventricle pumps blood harder now. This strains the muscles of its walls, and they become thick over time.
• Heart failure: Because of RVH, the thickened muscle wall becomes thick and loses its flexibility. The right ventricle cannot expand or contract as efficiently as before. As a result, it fails to pump blood properly, eventually leading to heart-failure.
• Pregnancy complications: A pregnant woman with PVS faces the risk of premature delivery, C-section deliveries, low-birth-weight baby, etc.

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Also Read: Signs of Heart-valve Disease, treatment and follow-up

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Diagnosis

• Electrocardiogram (ECG/EKG)
• Echocardiogram
• Chest X-ray
• MRI / CT scan
• Cardiac catheterization

Treatment

1. Medications – to overcome the symptoms or complications from PVS
2. Surgical procedures – to correct the original problem

Medications

• Diuretics: This helps remove the excess fluid in the blood, which happens due to heart-valve defects
• Prostaglandins: These drugs help improve blood flow
• Blood thinners: These drugs reduce clotting risk
• Drugs that overcome arrhythmias or irregular heart rhythms

Surgical procedures

• Balloon Valvuloplasty: Similar to balloon angioplasty done to clear blockages in the coronary arteries. A catheter with a deflated balloon is inserted into the body and threaded all the way up to the Pulmonary valve. The balloon is inflated, which helps push open the constricted valve. After that, the balloon is deflated and the catheter pulled out of the body.
• Pulmonary valve replacement: If balloon valvuloplasty has not worked out after a couple of attempts, an open-heart surgery is inevitable. In this, the defective valve is replaced by a new valve that is either a mechanical, or biological one (taken from the heart of a cow or pig). People who have undergone this procedure must take antibiotics before surgeries or some dental procedures. This will help prevent infections such as endocarditis.

If you or a loved one is dealing with heart valve problems, understanding the available treatment options is crucial for effective management. At Kauvery Hospital, with branches in Chennai, Hosur, Salem, Tirunelveli, and Trichy, our team of expert cardiologists and surgeons are committed to providing comprehensive care. We offer the latest in diagnostic and treatment solutions to ensure the best possible outcomes for your heart health.

Kauvery Hospital is globally known for its multidisciplinary services at all its Centers of Excellence, and for its comprehensive, Avant-Grade technology, especially in diagnostics and remedial care in heart diseases, transplantation, vascular and neurosciences medicine. Located in the heart of Trichy (Tennur, Royal Road and Alexandria Road (Cantonment), Chennai (Alwarpet & Vadapalani), Hosur, Salem, Tirunelveli and Bengaluru, the hospital also renders adult and pediatric trauma care.

Chennai Alwarpet – 044 4000 6000 •  Chennai Vadapalani – 044 4000 6000 • Trichy – Cantonment – 0431 4077777 • Trichy – Heartcity – 0431 4003500 • Trichy – Tennur – 0431 4022555 • Hosur – 04344 272727 • Salem – 0427 2677777 • Tirunelveli – 0462 4006000 • Bengaluru – 080 6801 6801

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