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2012 Fall Young at Heart

Heart & Vascular Institute Physician eNewsletter - Fall 2012

When young patients face aortic valve disease, a desire to maintain an active lifestyle, avoid blood thinning medications and prevent future surgeries all weigh into decisions about what operation is best and when to time that surgery.

Two percent of the population is born with abnormal aortic valves, and the vast majority of those abnormalities are referred to as bicuspid valves—a congenital condition where a person has two leaflets (or cusps) in the valve rather than three. While two-thirds of these patients have a bicuspid valve that will function well for life, others can later suffer from leaky valves that cannot close tightly or stenosis, when valves stiffen and cannot open or close properly.

When a patient is born with a bicuspid valve, more serious issues can surface at any stage in life. “Some complications manifest as children and young adults, which is when we most often see leaky valves, and after patients reach about age 50 and up, they most often present with stenosis,” says Gosta Pettersson, MD, Vice-Chair of Cardiovascular & Thoracic Surgery, Miller Family Heart & Vascular Institute at Cleveland Clinic.

Monitoring these patients carefully throughout their lives is critical, as is educating them about surgical options and the pros and cons of each procedure. “The main issue is that there is no perfect operation for these patients—you can’t tell them, ‘We’ll cure you and you’ll never have a problem again,’” Dr. Pettersson says. “Each surgery has its limitations.”

Creating a Treatment Plan and Managing Expectations

First, physicians must address the timing of the procedure—when should we operate? At what point could the abnormal valve cause damage to the heart that is possibly irreversible or pose an immediate risk in terms of the size of the aorta and risk for aortic dissection and rupture? And, if the patient is a woman, does she plan to have children, and when? “There is risk to becoming pregnant if you have a valve condition,” Dr. Pettersson points out. The question of timing can be different depending on if the valve is leaky or stenotic. Additional consideration is given to symptoms echocardiogram findings, and indications on other examinations. 

Next, there should be discussion about the patient’s expectations for the procedure. “From a mental perspective, young patients want to feel free to do anything and live their lives—they don’t want limitations imposed on them, and they don’t want to accept being sick and that there may be things they can’t do because they have a heart problem,” Dr. Pettersson says. So, it’s difficult to come to terms with the fact that a procedure could put limits on activity or require the use of anti-coagulants—or even necessitate another surgery down the road. (Because they have miles ahead of them, young patients can “wear out” their surgeries, so to speak.)

After aortic valve surgery, patients can live long, healthy lives with little or no complications. But it’s critical that they understand that none of these procedures are perfect. Some factors that can help patients and surgeons determine the best option for aortic valve surgery include: the patient’s age; co-existing medical conditions; surgery risks; risk of blood clots; risk of endocarditis; risk of bleeding; and patient’s lifestyle and personal preferences.

Surgical Options for Aortic Valve Disease

Typically there are two types of surgeries performed for young patients with aortic valve disease. One is to repair and preserve the patient’s own valve. While this can decrease risk of infection and the need for anti-coagulants, the surgery is technically difficult and is only an option for leaky valves, not stenotic valves. Not all repairs remain good and some patients will require another valve replacement within 10 years. Repair of the aortic valve is less often possible and less successful than for the mitral valve. When repair is not possible, the valve has to be replaced. Replacement can be done with a mechanical or tissue valve prosthesis. Mechanical valve prostheses are durable and designed to last a lifetime, but patients must take an anti-coagulant for the rest of their lives to prevent clot formation on the prosthesis. “Even though the mechanical valve structurally is very good, and there is no wear and tear, a few patients will still require another operation later in life because of infection of the valve prosthesis or dysfunction from tissue growing in and interfering with the function,” Dr. Pettersson says, reiterating that even a strong, reliable surgery comes with its disadvantages.

Tissue valve prostheses have a limited lifetime, more so in younger patients compared to older patients. “A younger patient may not get more than seven to 10 years out of a tissue valve, while a patient who is older may get 15 to 20 years out of it,” Dr. Pettersson says. The advantage is that tissue valve prostheses do not require life-long anticoagulant therapy after surgery, unless the patient has other conditions that require these medications.

In addition to these straightforward alternatives, there are more sophisticated tissue valve alternatives. One is use of human valves that have been removed from a donated human heart. These valves are referred to as homografts and are particularly useful when the aortic root has been damaged by infection or previous operations. Another alternative is the Ross procedure. The Ross procedure, also called the “switch procedure,” is generally for patients under the age of 50 who want to avoid taking long-term anticoagulant medications. The patient’s own pulmonary valve is removed and used to replace the diseased aortic valve. The pulmonary valve is then either “switched” with that aortic valve, or replaced with the pulmonary homograft. The idea, Dr. Pettersson explains, is “to put the best valve in the most important position, and the Ross operation may give the patient a perfect aortic valve.” But, not all pulmonary valves can stand up to the pressure in the systemic circulation. They can enlarge and become leaky. “These [procedures] are also not perfect and can require surgeries later in life,” Dr. Pettersson says. “I have lately been reversing failed Ross operations and returning the pulmonic valves to their original positions.”

The key for physicians is to weigh the risks and benefits of every option so patients understand their choices. “It’s important for patients with aortic disease to be seen early on so they can plan and research their different options and gain a better idea and understanding of which route they want to take, when the time an operation is necessary comes,” Dr. Pettersson says.

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This information is provided by Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition.

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