Guide to Aortic Valve Disease

Aortic Regurgitation | Aortic Stenosis | Bicuspid Aortic Valve | Aortic Valve Repair & Replacement | TAVR | Next Steps

What is the Aortic Valve?

The aortic valve is one of the valves of the heart. It is normally tricuspid (with three leaflets), although in 1% of the population it is found to be congenitally bicuspid (two leaflets). It lies between the left ventricle and the aorta.

What is the function of the Aortic Valve?

During ventricular systole (heart beat- emptying phase), pressure rises in the left ventricle (one of the chambers of the heart). When the pressure in the left ventricle rises above the pressure in the aorta, the aortic valve opens, allowing blood to exit the left ventricle into the aorta (the oxygen rich blood which comes from the lungs). When ventricular systole ends, pressure in the left ventricle rapidly drops (the relaxation or the filling phase of the heart). When the pressure in the left ventricle decreases, the aortic pressure forces the aortic valve to close. So the aortic valve is essentially the barrier between body and heart, which is why it is so important.

What diseases affect the Aortic Valve?

Basically, there are two typical processes that can affect the aortic valve and one congenital condition that will affect it.

  • Aortic stenosis, in which the valve fails to open fully, thereby obstructing blood flow out from the heart.
  • Aortic insufficiency, also called aortic regurgitation, in which the aortic valve is incompetent and blood flows passively back to the heart in the wrong direction.
  • Bicuspid Aortic Valve, the most common congenital abnormality of the heart. In this condition, instead of three cusps, the aortic valve has two cusps. This condition is often undiagnosed until later in life when the person develops symptomatic aortic stenosis.

Aortic Regurgitation (Insufficiency)

What is Aortic Regurgitation (Insufficiency)?

This condition occurs when your heart's aortic valve doesn't close tightly. Aortic valve regurgitation allows some of the blood that was just pumped out of your heart's main pumping chamber (left ventricle) to leak back into it. The leakage of blood may prevent your heart from efficiently pumping blood out to the rest of your body. The body does not get enough blood, so the heart has to work harder to make up for it.

What Causes Aortic Insufficiency?

There are a variety of causes of aortic regurgitation, ranging from congenital heart defects to complications of infectious illnesses. Major causes include:

  • congenital heart defects,
  • deterioration of the valve with age,
  • endocarditis,
  • rheumatic fever,
  • rare diseases such as Marfan syndrome that that can damage the aortic valve,
  • trauma (damage to the aorta near the site of aortic valve), and
  • enlargement of the aorta because of high blood pressure.

Is this a sudden disease?

Aortic valve regurgitation can develop suddenly or over decades. When it develops over years it is called chronic aortic regurgitation, whereas for sudden onset, it is called acute aortic regurgitation.

Do Symptoms of Aortic Regurgitation Appear Early On?

Aortic valve regurgitation develops gradually, and your heart compensates for the problem over time. You may have no signs or symptoms for many years, and you may even be unaware that you have this condition.

What are the causes of sudden aortic insufficiency?

Aortic insufficiency can be caused by complications with a replacement (prosthetic) aortic valve; trauma to the heart valve or aorta itself; endocarditis, which is an infection of the heart; aortic dissection, which means that the inner layer of the aorta separates from the middle layer.

How does aortic insufficiency affect my heart?

In aortic valve regurgitation, some blood leaks back into the left ventricle instead of flowing onward to the rest of your body after being pumped into the aorta. This forces the left ventricle to hold more blood. In response, this chamber of your heart may enlarge and thicken. At first, these adaptations help the left ventricle pump blood with more force. But eventually these changes weaken the left ventricle — and your heart overall in time

What are the signs and symptoms of Aortic Insufficiency?

  • Fatigue and weakness with increase in your activity level
  • Shortness of breath with exertion or when you lie on your back
  • Chest pain (angina), discomfort or tightness, often increasing during exercise
  • Swollen ankles and feet (edema)
  • Heart palpitations — sensations of a rapid, fluttering heartbeat
  • Irregular pulse (arrhythmia)
  • Heart murmur
  • Fainting

How is Aortic Insufficiency diagnosed?

Echocardiography (your heart's ultrasonography) is the gold standard in diagnosing valvular heart disease. After valvular heart disease has been diagnosed by echocardiography, catheterization may be performed to monitor pressures inside your heart as well as to check for additional coronary artery disease.

Should I limit my exercise?

Patients with aortic regurgitation should have detailed counseling about physical activity. Isometric exercise, weight lifting, and heavy exertional activities, which involve strenuous arm work, should be specifically prohibited because of the reflex increase in peripheral vascular resistance that occurs with arm exercise. In contrast, rhythmic, low-resistance, large muscle group exercise such as bicycling reduces peripheral resistance, and should be encouraged for fitness and a sense of well-being. Infective endocarditis prophylaxis is no longer required based on the new ACC/AHA guidelines.

How is Aortic Insufficiency Treated?

Once aortic valve regurgitation becomes severe, surgery is often required to repair or replace the aortic valve.

When is Surgery Recommended for Aortic Insufficiency?

Aortic valve surgery for chronic severe aortic regurgitation is indicated for those with symptom onset, asymptomatic patients with left ventricular dysfunction, patients undergoing cardiac surgery, and patients with preserved ventricular function but a left ventricular end-systolic dimension more than 50 to 55 mm or end-diastolic dimension more than 70 to 75 mm.

Aortic Stenosis

What is Aortic Stenosis?

Aortic stenosis occurs when the heart's aortic valve narrows. This narrowing prevents the valve from opening fully, which obstructs blood flow from your heart into your aorta and onward to the rest of your body.

What happens when the Aortic Valve Narrows?

This narrowing prevents the valve from opening fully, which obstructs blood flow from your heart into your aorta and onward to the rest of your body. When the aortic valve is obstructed, your heart needs to work harder to pump blood to your body. Eventually, this extra work limits the amount of blood it can pump and may weaken your heart muscle, leading to symptoms.

What causes Aortic Valve Stenosis?

Calcific aortic stenosis and congenital bicuspid aortic valve stenosis account for the overwhelming majority of aortic stenosis cases, followed by less common conditions, such as rheumatic aortic stenosis and congenital aortic stenosis.

What is calcific Aortic Stenosis?

In older adults, mild thickening, calcification, or both of a trileaflet aortic valve without restricted leaflet motion (aortic sclerosis) affects about 25% of the population older than 65 years. Calcific aortic stenosis, however, affects approximately 2% to 3% of those older than 75 years.

Does everyone who has sclerosis develop Aortic Stenosis?

No, not all patients with aortic sclerosis go on to develop obstructive aortic valve disease.

Can you explain congenital Aortic Stenosis?

Yes, in summary it is due to bicuspid aortic valve (2 leaflets instead of 3). Congenital bicuspid aortic valve stenosis is a major common cause of aortic stenosis; the approximate overall incidence of an anatomic bicuspid aortic valve is 1% to 2% of the population. Of these, about one half will develop aortic stenosis and up to one third will develop aortic regurgitation. Aortic stenosis caused by a congenital bicuspid aortic valve affects men more often than women.

What are the symptoms of Aortic Stenosis?

Aortic valve stenosis signs and symptoms typically develop when narrowing of the valve is severe and can include:

  • Chest pain (angina) or tightness
  • Feeling faint or fainting with exertion
  • Shortness of breath, especially with exertion
  • Fatigue, especially during times of increased activity
  • Heart palpitations — sensations of a rapid, fluttering heartbeat
  • Heart murmur

How do symptoms of aortic stenosis develop?

As the aortic valve becomes narrower, the pressure increases inside the left heart ventricle. This causes the left heart ventricle to become thicker, which decreases blood flow and can lead to chest pain. As the pressure continues to rise, blood may back up into the lungs, and you may feel short of breath. Severe forms of aortic stenosis prevent enough blood from reaching the brain and rest of the body. This can cause light-headedness and fainting.

Is this a sudden disease?

No. Aortic valve stenosis may not produce warning signs right away, making it difficult to detect at first. The condition is often discovered during a routine physical when a doctor hears an abnormal heart sound (heart murmur). This murmur may occur long before other signs and symptoms develop.

How is Aortic Stenosis diagnosed?

Echocardiography (your heart's ultrasonography) is the gold standard in diagnosing valvular heart disease. After valvular heart disease has been diagnosed by echocardiography, catheterization may be performed to monitor pressures inside your heart as well as to check for additional coronary artery disease.

My doctor tells me that I have Aortic Stenosis, what should I do now?

Patients with aortic stenosis fall into one of four categories of severity: mild, moderate, severe, or critical. Asymptomatic patients with aortic stenosis should have medical follow-up (see a cardiologist every 3 to 6 months) with regular inquiry about changes in exercise tolerance or other symptoms. Patients should stop smoking and may be treated for high cholesterol.

Can Aortic Stenosis be treated with medications?

Unfortunately no. To date, no medical therapy exists for the treatment of calcific aortic stenosis. The possible impact of secondary prevention measures, particularly lipid lowering with statins, on the progression of aortic stenosis has been investigated. Hypertension occurs in up to 40% to 50% of patients with calcific aortic stenosis and should be managed appropriately, because untreated hypertension may lead to earlier onset of symptoms. Antihypertensive medications should be titrated slowly, and vasodilators should be used with caution with severe aortic stenosis.

What are the treatment options for Aortic Stenosis?

Surgery to repair or replace the valve is the preferred treatment for adults or children who develop symptoms. Even if symptoms are not very bad, the doctor may recommend surgery. People with no symptoms but worrisome results on diagnostic tests may also require surgery.

Bicuspid Aortic Valve

What is Bicuspid Aortic Valve?

As we mentioned when describing the aortic valve, the normal aortic valve has three small flaps or leaflets that open widely and close securely to regulate blood flow, allowing blood to flow from the heart to the aorta and preventing blood from flowing backwards into the heart. In bicuspid aortic valve disease, the valve has only two leaflets. With this deformity, the valve doesn't function perfectly, but it may function adequately for years without causing symptoms or obvious signs of a problem.

What causes Bicuspid Aortic Valve?

Bicuspid aortic valve is present at birth (congenital). An abnormal aortic valve develops during the early weeks of pregnancy, when the baby's heart develops. The cause of this problem is unclear, but it is the most common congenital heart disease. It often runs in families and is associated with other disorders.

Which disorders are concomitant with Bicuspid Aortic Valve?

A bicuspid aortic valve often exists in babies with coarctation of the aorta, and other diseases in which there is a blockage to blood flow on the left side of the heart. Both disorders are seen in most of the patients with Turner Syndrome.

What is the incidence of Bicuspid Aortic Valve in the general population?

About 2% of the population has bicuspid aortic valve disease, and it is twice as common in males as in females.

What happens if I have Bicuspid Aortic Valve?

In time, the abnormal valve can leak or become narrowed.

Is Bicuspid Aortic Valve diagnosed at birth?

Most of the time, bicuspid aortic valve is not diagnosed in infants or children because it causes no symptoms.

Are there any complications related to Bicuspid Aortic Valve?

Yes, Heart Failure, Aortic Aneurysm and Dissection are the major complications related to Bicuspid Aortic Valve.

What are the symptoms of Bicuspid Aortic Valve?

Symptoms may include tiring easily, chest pain, difficulty breathing, rapid irregular heartbeat (palpitations), loss of consciousness (fainting), and pale skin.

How is Bicuspid Aortic Valve treated?

A bicuspid aortic valve may be repaired by reshaping the aortic valve leaflets, allowing the valve to open and close more completely. Bicuspid aortic valve repair may be an option to treat leaking valves, but it cannot be used to treat a stenotic or narrowed bicuspid aortic valve. Bicuspid aortic valve repair can be performed using a minimally invasive surgical technique.

What treatments are available for infants with bicuspid aortic valve?

If there is stenosis due to bicuspid aortic valve, it can be opened through cardiac catheterization. A fine tube (catheter) is directed to the heart and into the narrow opening of the aortic valve. A balloon attached to the end of the tube is inflated, to make the opening of the valve larger.

Aortic Valve Repair and Replacement

What are the surgical options for Aortic Valve Disease?

There are two types of aortic valve surgery: aortic valve repair and aortic valve replacement. Surgical options for either aortic stenosis or insufficiency include the implantation of a mechanical valve, a tissue valve, or your own valve (the Ross procedure). Aortic valve surgery can be performed using traditional heart valve surgery or minimally invasive approaches.

What kinds of valves are used in Aortic Valve Replacement Surgery?

Mechanical valves, Biological Valves, and Homograft Valves are used in aortic valve replacement surgery.

What is a mechanical valve?

Mechanical valves are made completely of mechanical parts, which are non-reactive and tolerated well by the body. The bileaflet valve is used most often. It consists of two pyrolite (qualities similar to a diamond) carbon leaflets in a ring covered with polyester knit fabric.

Do I have to take any anti-coagulants if I get a mechanical valve implanted?

All patients with mechanical valve prostheses need to take an anticoagulant medication, such as warfarin (Coumadin), for the rest of their life to reduce the risk of blood clotting and stroke.

What is a biological valve?

At NewYork-Presbyterian/Columbia, the majority of aortic valves are replaced with a bioprosthesis. Biological valves (also called tissue or bioprosthetic valves) are made of tissue, but they may also have some artificial parts to provide additional support and allow the valve to be sewn in place. Biological valves can be made from pig tissue (porcine), cow tissue pericardial (bovine), or pericardial tissue from other species.

Do I have to take any anti-coagulants if I get a biological valve implanted?

No. These valves allow patients to avoid lifetime use of anticoagulants (blood thinning medications).

What is a Homograft?

A homograft (also called allograft) is an aortic or pulmonic valve that has been removed from a donated human heart, preserved, treated with antibiotics, and frozen under sterile conditions. Homografts are ideal valves for aortic valve replacement, especially when the aortic root is diseased or endocarditis (infection) is present.

How is the type of replacement valve chosen?

The type of valve used typically depends on the severity of symptoms and other individual health concerns, such as age and the use of blood thinners.

What is traditional heart valve surgery?

During traditional aortic valve surgery, a surgeon makes a 6- to 8-inch incision down the center of your sternum, and part or total of the sternum (breastbone) is divided to provide direct access to your heart. The surgeon then repairs or replaces your abnormal heart valve or valves.

What is minimally invasive aortic valve surgery?

Minimally invasive aortic valve surgery is performed through smaller, 2- to 4-inch incisions. Minimally invasive surgery may reduce blood loss, trauma, and length of hospital stay. Most patients who require isolated aortic valve surgery are candidates for minimally invasive aortic valve surgery, but your surgeon will review your diagnostic tests and determine if you are a candidate for this type of surgery.

How serious is an enlarged aorta?

Aortic valve disease is often associated with enlargement (aneurysm) of the ascending aorta, the initial portion of the aorta (the main blood vessel in the body that originates from the aortic valve). If the enlargement of the aorta is substantial (usually above 4.5 or 5 cm in diameter), this part of the aorta may need to be replaced.

I have an enlarged aorta in addition to aortic valve disease. What kind of surgery I will need?

The replacement of the aorta itself is done at the same time as aortic valve repair or replacement. In patients who have a leaky aortic valve and an enlarged aorta, a special procedure (the David procedure) can be performed. The David procedure allows surgeons to repair the aortic valve and simultaneously replace the enlarged ascending aorta. Another option is replacement of the entire aortic root and aortic valve with a combination of a mechanical valve with an attached tube graft. Also called the modified Bentall operation, this approach is often used in younger patients or in those patients who wish to avoid reoperation. Anticoagulation therapy is required.

Transcatheter Aortic Valve Replacement (TAVR)

Can aortic valve surgery be done minimally invasively?

Yes, there is a minimally invasive approach called transcatheter aortic valve replacement, or TAVR (sometimes also called transcatheter aortic valve implantation, or TAVI).

What happens during the TAVR procedure?

During the TAVR procedure, a thin tube called a catheter is advanced through an artery in the leg (the femoral artery) or through the chest wall and left ventricle to the aortic valve, where a tissue valve with metal stent scaffolding is positioned and deployed.

Who is a candidate for transcatheter aortic valve replacement, or TAVR?

TAVR is a proven, effective option for people dealing with severe aortic stenosis.

Next Steps

If you are in need of help for an aortic condition, we’re here for you. Call us now at (844) 792-6782 or fill out our online form to get started today.

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