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Surgery for Adult Congenital Heart Disease

Baylor Scott & White Heart and Vascular Hospital – Dallas

Adults with congenital heart defects have increased risk of cardiac problems

According to the American Heart Association, about nine of every 1,000 babies born in the US have a congenital heart defect, a malformation of the heart or major blood vessels present at birth. This problem occurs as the baby's heart is developing during pregnancy. Congenital heart problems are the most common birth defects and can range from simple to complex.

The cause of any particular congenital heart defect is usually unknown. However, factors that may increase the chance of a heart defect include a family history of birth defects, premature birth or genetic conditions such as Down syndrome. Pregnant women exposed to certain prescription medications, those who get rubella (German measles) and women with uncontrolled diabetes are at greater risk. Some congenital heart problems can be watched by the baby's doctor and managed with medicines, while others will require surgery.

Both repaired and unrepaired congenital heart defects frequently lead to problems later on in life. Individuals with a congenital heart defect have an increased risk of cardiac problems, including stroke, pulmonary hypertension, heart failure and arrhythmia.

Adults with congenital heart defects are different from adults with other heart problems such as coronary artery disease. They are typically younger and have unique problems that vary depending on their particular heart condition present at birth. Adults with congenital heart disease should be evaluated by an adult congenital heart disease specialist and receive follow-up cardiac care throughout their lives.

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Surgical treatment options

Surgical treatment options for the most common types of congenital heart problems include atrial septal defect repair, ventricular septal defect repair and tricuspid valve surgery.

Atrial septal defect
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When too much blood passes through the lungs, oxygen-rich blood fails to circulate throughout the body, and this causes increased pressure and stress in the lungs. Atrial septal defect (ASD) is a hole between the two upper chambers of the heart - the right and left atria - leading to abnormal blood flow through the heart. Some people may not experience symptoms and appear healthy; however, if the ASD is large, symptoms could include breathing difficulties.

If heart surgery is required to close this defect, surgeons on the medical staff at Baylor University Medical Center, part of Baylor Scott & White Health, in Dallas offer surgical options including minimally-invasive surgery.

Ventricular septal defect
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Ventricular septal defect is a condition in which a hole occurs in the ventricular septum, a dividing wall between the two lower chambers of the heart (the right and left ventricles). Blood from the left ventricle will flow back into the right ventricle due to higher pressure in the left ventricle, and this causes an extra volume of blood to be pumped into the lungs by the right ventricle. This can create congestion in the lungs, leading to possible breathing problems.

Depending on the severity of any heart or lung problems, surgery may be indicated. Adult congenital heart treatment surgery may include an open-heart procedure to patch the hole between the ventricles.

Tricuspid valve disease
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Another type of congenital heart disease involves the heart's tricuspid valve. Located between the right upper chamber (atrium) and lower chamber (ventricle) of the heart, this valve helps control blood flow, moving it in the appropriate direction. The tricuspid valve has three leaflets, or flaps, that control blood flow and direction.

When the valve doesn't function properly, a person may have tricuspid valve disease, a relatively rare form of valve disease compared to other types of valve problems. In the case of tricuspid stenosis, the valve leaflets are stiff and do not open all the way, restricting blood flow. In tricuspid regurgitation, leaflets do not close completely and blood leaks backwards across the valve instead of flowing into the ventricle; the heart must pump harder to move blood through the body.

If tricuspid valve disease is relatively mild, a physician may prescribe medications to treat the symptoms to help control heart failure or manage an irregular heart rhythm. If a valve condition is severe or becomes worse over time, surgery may be needed to repair or replace the faulty valve.

Coarctation of the aorta (CoA)
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The heart's aorta is the primary blood supplier to the body. If the aorta is narrowed or constricted, too little blood will flow to the lower part of the body, leading to increased blood pressure. This condition is known as coarctation of the aorta (CoA). If severe symptoms of high blood pressure develop surgery may be necessary. A congenital heart defect, symptoms may not be present at birth but can develop as early as the first week of life.

Repair of the narrowed vessel might include cardiac catheterization, a minimally invasive surgical procedure to insert and guide a flexible tube (catheter) into the heart's aorta using an inflated balloon or stent to open the area. Another treatment option is surgical repair in an operating room. The narrowed area is surgically removed or enlarged.

Coronary artery anomalies
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Coronary artery defects present at birth, such as incorrect connections between the heart and the blood vessels that carry blood to and from the heart, may not be diagnosed until adulthood. These defects, or coronary artery anomalies, are associated with congenital heart disease. Individuals with coronary artery anomalies have an increased risk of cardiac problems including stroke, pulmonary hypertension, heart failure, and arrhythmia.

Treatment for an abnormal coronary artery will vary depending on the particular condition, and may include medicines or surgery to fix the defect.

Hypertrophic cardiomyopathy
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When the part of the heart that separates the right and left ventricle - called the ventricular septum - becomes abnormally thick, it's difficult for blood to flow in and out of the heart. This condition is known as hypertrophic cardiomyopathy. Treatment includes medications and minimally-invasive procedures such as a myomectomy, in which the surgeon removes part of the septum.

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