Congenital Heart Disease in Adults

(Also Called ‘Bicuspid Aortic Valve Disease’, ‘Congenital Heart Disease’)

Congenital heart disease is a type of defect in one or more structures of the heart or blood vessels that occur before birth.

• Congenital heart defects occur while the fetus is developing in the uterus.
• They affect 8 to 10 out of every 1,000 children. Congenital heart defects may produce symptoms at birth, during childhood and sometimes not until adulthood.
• About 500,000 adults in the United States have grown into adulthood with congenital heart disease. This number increases by about 20,000 each year.

Bicuspid (two-flaps) Aortic Valve

Bicuspid aortic valve disease is a congenital (from birth) valve disease that affects the aortic valve. Instead of the normal three leaflets or cusps (see below), the bicuspid aortic valve has only two. Without the third leaflet, the valve may be:

• stenotic – stiff valves that can not open or close properly
• leaky – not able close tightly

This is the condition that I have been living with.  Interestingly enough, I have known all my life that I have a “Heart Murmur”.

A heart murmur is an extra or unusual sound heard during a heartbeat. Murmurs range from very faint to very loud. They sometimes sound like a whooshing or swishing noise. Normal heartbeats make a “lub-DUPP” or “lub-DUB” sound. This is the sound of the heart valves closing as blood moves through the heart. Doctors can hear these sounds and heart murmurs using a stethoscope.

My doctor, Dr. Gerald Jensen, has always listened carefully to my heart, but when I went for my check up, he was noticeably more attentive to the sounds he was hearing in the stethoscope.  He listened on my chest, then my back, which was all normal.  But when he listened in my neck, wrists, and ankles, I knew that this time was different.  He asked if I had experienced any fatigue, shortness of breath, dizziness, numbness in my feet and hands, any chest pains.  My answer was yes to all.  Oddly enough I had been having all of these symptoms (consistent with heart disease), but NEVER connected the dots in my mind, and concluded “ah-ha”, I have heart disease.

This occurs more frequently in some family members. About 1/4 of patients may have some enlargement of the aorta above the valve. Bicuspid aortic valve disease affects about 2 percent of the population. Eighty-five percent of children with congenital aortic stenosis have a bicuspid aortic valve.

Inside the Heart

The human heart is divided into four chambers.  These chambers are separated from each other and the rest of the circulatory system by valves.  The valves open and close as the heart beats to allow blood to flow in only one direction.  Diseases, age, and birth defects may cause the valves to stop working properly.  A defective heart valve does not open or close fully, which can cause serious problems.  Some defective heart valves can be treated with surgery or drugs, but others need to be replaced.

Heart valves can be replaced with mechanical heart valves or tissue heart valves.  Mechanical heart valves are made from synthetic materials that can be put into the body.  There are several designs of mechanical heart valves that all operate differently.  Tissue heart valves are real heart valves taken from another person or even other animals.  Tissue heart valves are commonly taken or made from porcine (pigs) and bovine (cows) heart tissues.  Mechanical heart valves can be superior to tissue valves because they will not wear out over time.

Dr. Bibler explained to us that a mechanical valve is much longer lasting, (possibly for life) whereas the tissue valves would most likely wear out with age.  The tissue valve would probably have to be replaced again within 10-15 years.  So in my case, since I am only 50 years old, the best option would be to use a mechanical replacement, and I should be good to the end of my life.  The picture above is the exact same device that Dr. Bibler will use for my heart.