Supravalvular Ring Mitral Stenosis - Causes, Symptoms, Diagnosis, and Treatment

Introduction

Supravalvular ring mitral stenosis is a sporadic disease that leads to the formation of a ridge that has a single or double aperture, and it obstructs the regular function of the mitral valve. The disease can be diagnosed with the help of the latest radiological studies, such as magnetic resonance imaging and echocardiography. Difficulty in breathing is one of the most common clinical signs of supravalvular ring mitral stenosis. This disease is a congenital heart anomaly and develops due to abnormal development of the connective tissues of the mitral valve. A patient diagnosed with supravalvular ring mitral stenosis generally appears with more than one cardiovascular disease.

What Are Heart Valves?

The heart has four chambers- two upper sections called the right atrium and left atrium, and two lower sections called the right ventricle and left ventricles. Parallel to four chambers, the heart has four valves- mitral valve, tricuspid valve, aortic valve, and pulmonary valve.

• Valves are a crucial part of the heart’s structure.

• They open and close as the blood passes through them.

• They mainly regulate the entry and exit of blood into the heart’s various chambers.

• The valves have flaps or leaflets that open and close.

• Valves with three leaflets open and close simultaneously.

• In order to avoid any sort of backflow of blood, the leaflets close, and visa verse is why they open.

• The mitral or bicuspid valve allows blood to flow smoothly from the left atrium into the left ventricle. It has two leaflets.

• The tricuspid valve allows blood to flow easily from the right atrium into the right ventricle. It has three leaflets.

• The aortic valve controls smooth blood flow from the left ventricle to the aorta.

• The pulmonary valve has the primary function of allowing smooth blood flow from the right ventricle into the pulmonary artery. It has three leaflets.

What Are the Causes of Supravalvular Ring Mitral Stenosis Diagnosed?

Supravalvular ring mitral stenosis is a rare subtype of mitral stenosis that is congenital in formation. The shape and structure of supravalvular ring mistral stenosis varies from the presence of an extremely thin membrane to the thickening and fibrosis of the opening of the mitral valve. This morphological abnormality may be additionally present in the leaflets of the mitral valve. Shone complex is a condition in which supravalvular ring mitral stenosis is observed along with several other congenital heart defects.

What Are the Signs and Symptoms of Supravalvular Ring Mitral Stenosis?

The development of supravalvular ring mitral stenosis occurs when the fetus is in the womb. Clinical manifestations are not the same in each and every individual. Once the condition is quite advanced, there are some signs and symptoms that a patient may present with.

Mentioned below are a few of the signs and symptoms of supravalvular ring mitral stenosis.

• Shortness of breath.

• Difficulty sitting up.

• Being tired quickly.

• Chest pain.

• Fatigue.

• Dizziness.

• Fainting.

• Fever.

• Irregular heartbeat.

• Fluttering sensation in the chest.

• Lightheadedness.

• Coughing.

• Swollen ankles.

• Bloating of the abdomen.

• Tightness in the chest.

• Insomnia.

• Inability to engage in physical activities.

• Heavy breathing after and while climbing stairs.

• Heart murmurs.

• Palpitations.

How Is Supravalvular Ring Mitral Stenosis Diagnosed?

Supravalvular ring mitral stenosis may be diagnosed during a regular consultation with a physician post a few extra diagnostic tests. A stethoscope can pick up any abnormal heart murmurs and regurgitation sounds. To confirm the presence of valvular regurgitation or valvular stenosis, additional tests such as those mentioned below may be suggested. Physical examination and a detailed account of medical as well as drug history are vital for the diagnosis of supravalvular ring mitral stenosis.

• Electrocardiography.

• Ultrasound of the heart.

• Complete blood picture.

• Doppler echocardiography.

• X-ray of the chest.

• Transesophageal echocardiogram.

• Magnetic resonance imaging of the heart.

• Catheterization.

What Are the Treatment Options for Supravalvular Ring Mitral Stenosis Diagnosed?

As of today, there are not any strong medications that can help in the reversal or betterment of supravalvular ring mitral stenosis. Invasive procedures are the sole treatment option for supravalvular ring mitral stenosis.

Mentioned below are a few of the treatment strategies for supravalvular ring mitral stenosis.

• Open heart surgery.

• Open heart surgery.

• Mechanical valve insertion.

• Bioprosthetic valve repair.

• Mitral clip.

• Balloon valvuloplasty.

• Transcatheter aortic valve replacement or TAVR.

• Transcatheter mitral valve Replacement or TMVR.

What Are the Risks of Supravalvular Ring Mitral Stenosis Surgery?

• Excessive bleeding during the surgery or even after the surgery.

• Blood clots.

• Heart attack.

• Stroke.

• Lung disorders.

• Infection of the blood.

• Pneumonia.

• Pancreatic disorders.

• Breathing difficulty.

• The abnormal rhythm of the heart.

• Incorrect replacement of the heart valve.

What Are the Kinds of Heart Valve Disease?

Heart valve disease is diagnosed when there is a disruptive change in one or more than one valve of the heart. This change brings about an overall reduction in cardiac input. When a valve is damaged or diseased, they do not open and close efficiently and eventually becomes leaky. This phenomenon is called regurgitation. Due to this, the backflow of blood is not stopped, and thus, blood goes back to the chamber it came from. Stenosis is another type of valve disorder where the leaflets turn stiff and narrow. There are other cases where a leaflet is missing, which is generally found in the aortic valve. All of the above leads the heart to work harder, which eventually gives up by being tired, leading to heart failure and sudden cardiac arrest.

Mentioned below are the various types of heart valve diseases.

• Mitral valve stenosis.

• Mitral valve regurgitation.

• Aortic valve stenosis.

• Aortic valve regurgitation.

• Tricuspid valve stenosis.

• Tricuspid valve regurgitation.

• Pulmonary valve stenosis.

• Pulmonary valve regurgitation.

Conclusion

Supravalvular ring mitral stenosis is a congenital defect of the mitral valve of the heart that leads to the formation of abnormal ridges at the apertures of the mitral valve, leading to the narrowing and stiffness of the valve. This, in turn, reduces the functionality of the mitral valve, and hence the cardiac output is drastically reduced. Early diagnosis of supravalvular ring mitral stenosis generally occurs because the condition gets identified when the fetus is in the womb. As of today, surgical correction is the only treatment modality for supravalvular ring mitral stenosis.