Aortic Valve Disease

Types of Aortic Valve Disease

In aortic valve disease, the aortic valve does not work properly. A diseased or damaged aortic valve can cause insufficient blood flow to the rest of the heart and body.

Types of aortic valve disease we treat at University Hospitals Harrington Heart & Vascular Institute’s Valve and Structural Heart Disease Center include:

• Aortic Valve Regurgitation
• Aortic Valve Stenosis
• Bicuspid Aortic Valve

Aortic Valve Regurgitation

In aortic valve regurgitation, the aortic valve does not close fully. As a result, some of the blood pumped out of the heart’s main pumping chamber (the left ventricle) leaks backward. This leakage can decrease the heart’s ability to efficiently pump blood to the rest of the body, often resulting in fatigue, shortness of breath and other symptoms.

Symptoms of Aortic Valve Regurgitation

Aortic valve regurgitation often develops gradually. People with the condition usually do not present with symptoms for years, so that some individuals are unaware they have the condition. However, aortic valve regurgitation can occur suddenly at times, usually when the affected valve becomes infected.

Signs and symptoms of aortic valve regurgitation include:

• Fatigue and weakness, particularly during increased levels of physical activity
• Shortness of breath during exercise or when you lie down
• Heart murmur
• Irregular pulse (arrhythmia)
• Chest pain (angina), discomfort or tightness, often increasing during exercise
• Lightheadedness or fainting
• Rapid, fluttering heartbeat (palpitations)
• Swollen feet and ankles

Causes of Aortic Valve Regurgitation

Common causes of aortic valve regurgitation include:

• Congenital heart valve disease: Congenital heart disease occurs as a baby’s heart develops during pregnancy. Congenital heart defects that involve the heart valves include conditions such as bicuspid valve.
• Aortic Stenosis (aortic valve narrowing): In aortic stenosis, the flaps of the aortic valve become stiff, preventing the valve from opening and closing fully.
• Endocarditis: Endocarditis is inflammation of the lining inside the heart’s chambers and valves. Usually caused by infection, this life-threatening condition can damage the aortic valve.
• Rheumatic fever: Rheumatic fever is an autoimmune disease that causes inflammation of the body’s tissues, including the heart and joints. Heart inflammation brought on by rheumatic fever can cause the aortic valve to stiffen and narrow, resulting in blood leakage. An abnormal heart valve caused by rheumatic fever is referred to as rheumatic heart disease.
• Other diseases: Certain rare diseases can enlarge the aorta and the aortic valve and lead to regurgitation. These conditions include Marfan syndrome, a connective tissue disease. In addition, certain autoimmune conditions, such as lupus, can cause aortic valve regurgitation.
• Injury to the aorta: An aortic tear (dissection) or traumatic chest injury can cause backward leakage of blood through the aortic valve.

Risk Factors for Aortic Valve Regurgitation

Conditions that make people more likely to develop aortic valve regurgitation include:

• Certain heart conditions present at birth (congenital heart disease)
• Advancing age
• History of infections that affect the heart
• Having aortic valve stenosis and certain other heart valve conditions
• High blood pressure

Aortic Valve Regurgitation Diagnosis

To diagnose aortic valve regurgitation, your doctor will perform a physical exam, and discuss your symptoms and your family medical history. Your doctor may order additional testing to diagnose aortic valve regurgitation and determine the cause of the condition, including:

• Electrocardiogram (ECG): A test that assesses heart rhythm.
• Transthoracic echocardiogram (TTE): An ultrasound of the heart to diagnose and evaluate disease.
• Transesophageal echocardiogram (TEE): An ultrasound of the heart taken from the esophagus to assess condition severity and check for tearing of the aorta, if needed.
• CT or MRI: May be used to assess for tearing of the aorta; MRI can also be used to assess condition severity and heart function if echocardiogram images are not satisfactory.
• Chest X-ray: To identify enlargement of the heart and/or aorta.
• Cardiac catheterization, aortography, or coronary angiography: May be recommended before aortic valve surgery or when initial testing produces mixed results.
• Stress testing: Used to assess how well the heart pumps blood under physical stress

Aortic Valve Regurgitation Treatment

Aortic valve regurgitation treatment depends on the severity of the condition, whether or not symptoms are present, and if the condition is worsening. Goals of treatment are to ease symptoms and prevent the development of complications. If you are experiencing mild or no symptoms, your doctor may monitor your condition via regular follow-up appointments and recommend healthy lifestyle changes. You may require regular echocardiograms to check that your aortic valve regurgitation hasn’t become more severe.

Treatments for aortic valve regurgitation include:

• Medication: You may need to take medication to treat symptoms of aortic valve regurgitation or reduce risk of complications. In addition, you may need to take medication to lower blood pressure.
• Surgery or other procedures: Surgery for aortic valve regurgitation includes:
  • Aortic valve repair: In repairing an aortic valve, surgeons may separate valve flaps (cusps) that have fused, reshape or remove excess valve tissue so that the cusps can close tightly, or patch holes in a valve.
  • Aortic valve replacement: In aortic valve replacement, a surgeon removes the diseased valve and replaces it with a mechanical valve or a valve made from pig, cow or human heart tissue (biological tissue valve). Transcatheter aortic valve replacement (TAVR), also called transcatheter aortic valve implantation (TAVI), is a minimally invasive, nonsurgical procedure in which a long, thin tube (catheter) is threaded into a blood vessel through the groin and guided inside the heart to replace a narrowed aortic valve with a biological tissue valve.

Bicuspid Aortic Valve

The aortic valve usually has three cusps (flaps) that open and close with each heartbeat, making blood flow in the correct direction. A bicuspid aortic valve has only two cusps. A bicuspid aortic valve is a condition that some people have at birth (a congenital heart condition).

A bicuspid aortic valve can lead to the following heart problems:

• Narrowing of the aortic valve (aortic valve stenosis)
• Backward flow of blood (aortic valve regurgitation)
• Enlarged aorta (aortopathy): In some individuals, a bicuspid aortic valve can lead to an enlarged aorta, which increases the risk of tearing in the lining of the aorta.

Bicuspid Aortic Valve Symptoms

A bicuspid aortic valve usually doesn’t cause signs or symptoms until adulthood. However, infants with the condition may experience symptoms. Symptoms of a bicuspid aortic valve include:

• Chest pain
• Shortness of breath
• Difficulty exercising

Causes of Bicuspid Aortic Valve

A bicuspid aortic valve develops during the early weeks of pregnancy. Bicuspid aortic valve is the most common congenital heart defect. The cause of the condition is unclear. Bicuspid aortic valve tends to run in families.

Diagnosis of Bicuspid Aortic Valve

Medical tests for other health conditions may reveal the presence of a bicuspid aortic valve. While some bicuspid aortic valves are silent, others produce a heart murmur; the heart murmur is often the first sign of a defective aortic valve.

To confirm a diagnosis of a bicuspid aortic valve, an echocardiogram is performed. This test uses sound waves to create video images of the heart in motion to evaluate valve function, anatomy and blood flow through the heart. If the presence of a bicuspid aortic valve is confirmed, a computed tomography (CT) scan is often done to check for an enlarged aorta.

Treatment of Bicuspid Aortic Valve

Children and adults with a bicuspid aortic valve require regular doctor’s checkups and echocardiograms to check for stiffening (stenosis) or leaking (regurgitation) of the valve or an enlarged aorta. Treatment depends on the severity of the condition. Although there are no medications designed to treat a bicuspid heart valve, your doctor may prescribe other medications to treat related heart problem, including high blood pressure medication.

You may need surgery to repair or replace your aortic valve. Surgery will depend on your specific condition and symptoms:

• Aortic valve replacement: In aortic valve replacement, a surgeon removes the defective valve and replaces it with a mechanical valve or a valve made from pig, cow or human heart tissue (biological tissue valve). Sometimes a biological tissue valve replacement procedure that uses the patient’s own pulmonary valve is performed.
• Aortic root and ascending aorta surgery: During this procedure, a surgeon removes the enlarged section of the aorta located near the heart and replace it with a synthetic tube (graft) that is sewn into place. The aortic valve can be repaired or replaced during this operation.
• Balloon valvoplasty: If a bicuspid valve has caused aortic stenosis, your doctor may recommend this procedure. In balloon valvoplasty, the doctor inserts a catheter with a balloon on the tip into an artery in your groin and navigates it to the aortic valve. The balloon is inflated to expand the opening of the valve. After the balloon is deflated, the catheter and balloon are removed.