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Bicuspid Aortic Valve Disease
How common is bicuspid aortic valve?
As many as 1 in 50 people have a
bicuspid aortic valve. It’s twice as common in men and people assigned male at
birth (AMAB) compared with women and people assigned female at birth (AFAB).
People with Turner syndrome have an
increased risk of heart problems including a bicuspid aortic valve.
What are the symptoms of a bicuspid
aortic valve?
You may not have any symptoms of a
bicuspid aortic valve. You could live many years without even knowing your
anatomy is different. But over time, you may develop symptoms of aortic
regurgitation or stenosis. If your child has a bicuspid aortic valve, you may
notice symptoms soon after birth (in severe cases). But it’s more likely that
you won’t notice them until later on.
Symptoms in adults include:
Fatigue (most common).
Chest pain or discomfort.
Dizziness or fainting.
Heart palpitations.
Shortness of breath, especially with
activity.
Symptoms in infants and children
include:
Becoming tired easily.
Chest pain.
Fainting.
Pale skin.
Trouble breathing.
Trouble with feeding or gaining
weight.
Many people don’t realize they have
a bicuspid aortic valve until it’s diagnosed through medical testing. That’s
because symptoms are easy to miss. Your family may notice a change before you
do. One helpful tip is to think about how much activity you can do in a typical
day. Compare it with one year ago, or even six months ago. Do you get tired
more easily? Do you need more time to rest? If so, that may signal your heart
is working harder to keep up.
That’s why it’s so important to keep
up with your medical appointments. Talk with your healthcare provider about any
changes you notice in your daily life.
What causes a bicuspid aortic valve?
Researchers don’t know what causes a
bicuspid aortic valve to form. It’s the most common congenital heart defect
(present at birth). It develops early in pregnancy.
Is a bicuspid aortic valve genetic?
A bicuspid aortic valve can run in
families. If a close family member (parent, child or sibling) has this heart
defect, talk with your healthcare provider. They may want to run some tests to
check your heart anatomy and function.
If you’ve been diagnosed with a
bicuspid aortic valve and are planning a pregnancy, talk with your provider.
You can’t prevent this defect from happening, but you can monitor your
pregnancy and try to diagnose any problems early.
Research will continue to explore
the role of genetic mutations in causing bicuspid aortic valve disease. It
seems to be an inherited heart defect, but researchers don’t yet fully
understand the details.
How is a bicuspid aortic valve
diagnosed?
A bicuspid aortic valve may be
diagnosed during pregnancy through a cardiac prenatal ultrasound. Other times,
it’s diagnosed when a child has other heart problems that cause symptoms.
Testing then reveals the bicuspid aortic valve.
But other people go many years without
knowing they have this condition. The first sign may be a heart murmur that
your provider hears through a stethoscope. After that, your provider may run
imaging tests to check your heart structure and function.
Tests to diagnose a bicuspid aortic
valve
Your provider may run the following
tests to check your heart and diagnose a bicuspid aortic valve:
Echocardiogram (echo).
Transesophageal echocardiogram.
CT scans.
Heart MRI.
What is the treatment for a bicuspid
aortic valve?
About 4 out of 5 people with
bicuspid aortic valve disease need aortic valve surgery. New technology
continues to improve outcomes and reduce complications. Many people can have
minimally invasive techniques instead of traditional open heart surgery. Your
surgeon will either repair or replace your aortic valve. Your surgeon may also
fix other issues (like an aneurysm) at the same time.
Your provider will evaluate your
situation, run some tests and talk with you about your treatment options. Your
treatment will depend on the condition of your aortic valve and aorta. But your
provider will also consider your overall heart health, age and other medical
conditions.
It’s important to have surgery early
enough to prevent permanent damage to your heart. Even if you don’t have
symptoms, your provider may want to fix the problem now to avoid complications
down the road. The best timing is different for everyone. Your medical care
team will discuss your options with you.
Traditional aortic valve surgery vs.
minimally invasive aortic valve surgery
Traditional (open heart) aortic
valve surgery involves a 6- to 8-inch incision down the middle of your sternum
(breastbone). Your sternum is divided so your surgeon can directly access your
heart.
Minimally invasive aortic valve
surgery involves a smaller “J” incision (2 to 4 inches). The incision will be
at the top of your sternum or between your ribs. Your surgeon doesn’t need to
open your whole chest. This procedure reduces blood loss and allows you to
leave the hospital sooner. You may also recover more quickly.
Aortic valve repair vs. aortic valve
replacement
Aortic valve repair fixes your valve
without replacing it. It can be a good option for aortic regurgitation (leaky
valve). But it can’t be used to treat aortic stenosis (narrowing). Aortic valve
repair can often be done through a minimally invasive surgery with the “J”
incision. Your surgeon will reshape your aortic valve cusps to help them open
and close more completely.
Aortic valve replacement is used
when a repair isn’t possible. It involves removing your valve and giving you a
new one. This procedure can be done through traditional open surgery. Or, it
can use the minimally invasive transcatheter aortic valve replacement (TAVR)
method.
There are two main options for your
new valve: biological or mechanical.
Biological valve. This type of valve
is used 80% of the time. It’s made from pig or cow tissue. This valve is safe
and durable. But after ten years, you may need another replacement surgery.
Mechanical valve. This type of valve
is very durable and can last the rest of your life. But you need to take blood
thinners for your whole life to keep it working safely.
Many people prefer a biological valve
so they don’t have to take blood thinners. But it depends on your age, other
medical conditions and personal preferences. Your provider will discuss your
options and help you decide what’s best for you.
Another option for valve replacement
is the Ross procedure (also called the switch procedure). It involves using
your pulmonary valve to replace your aortic valve. This procedure can be a good
option for people under age 50 who want to avoid long-term use of blood
thinners.
Risks of surgery
Generally, valve surgeries have a
low risk of complications. But possible risks include:
Infection.
Blood clots.
Stroke.
Arrhythmia (temporary).
Reduced kidney function (temporary).
About 98% of people who have valve surgery
survive and have a normal life expectancy.
How can I prevent a bicuspid aortic
valve?
There’s no way to prevent a bicuspid
aortic valve. It’s congenital (something you’re born with). But you can make
lifestyle changes that reduce your risk of other heart problems like
atherosclerosis and coronary artery disease. If you have a bicuspid aortic
valve, talk with your provider about managing heart disease risk factors
including:
High blood pressure (hypertension).
High cholesterol (hyperlipidemia).
Diabetes.
Lifestyle factors like smoking,
using tobacco products and alcohol use.