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Congenital Heart Disease

A congenital heart defect is a malformation of the heart or major blood vessels that is present at birth. Because both repaired and unrepaired congenital heart defects frequently lead to problems later on in life, all adults with congenital heart disease need to be evaluated by an adult congenital heart disease specialist and require follow-up cardiac care throughout their lives.

The most common types of congenital heart defects diagnosed for the first time in adulthood are:

  • Septal defects ("holes in the heart") – A septal defect can occur between the two ventricles (pumping chambers) of the heart, which is called a ventricular septal defect, or between the two atria (filling chambers), which is called an atrial septal defect. With either type, oxygenated blood coming from the lungs gets mixed with deoxygenated blood returning from the body. Such holes can lead to complications and in most cases require repair for preventative purposes.
  • Congenital Valve defects – A valve in the heart may be unable to open completely, or unable to close completely, due to a defect of function or shape. These defects force the heart to work harder as it moves blood through the heart.
  • Abnormal blood vessels – Similar to valves to open incompletely, blood vessels that are too narrow at a certain point cause the heart to work harder. In addition, blood vessels can be connected incorrectly, sending deoxygenated blood to the body or already oxygenated blood back to the lungs.

Some specific conditions that may be found in adults include:

  • Marfan Syndrome – This is a genetic disorder that affects the body's connective tissue, the tissue that holds all the body's cells, organs and tissue together. Patients with Marfan Syndrome are at risk of tearing or bursting of their major blood vessels.
  • Ebstein's Anomaly – This defect is caused by a malformed heart valve that does not properly close to keep the blood flowing in the proper direction toward the lungs.
  • Tetralogy of Fallot – This heart defect features four distinct problems: a hole between the lower chambers of the heart, an obstruction between the heart and lungs, the aorta (blood vessel) covers the hole in the lower chambers and the muscle surrounding the lower right chamber becomes overly thickened.

Regardless of the type of problem present, individuals with a congenital heart defect have an increased risk of cardiac problems, including stroke, pulmonary hypertension, heart failure and arrhythmia.

Causes of Congenital Heart Defects

In most cases, the cause of any particular congenital heart defect is unknown. The factors that can increase the chance of having a heart defect include:

  • Family history of birth defects
  • Premature birth
  • Genetic conditions such as Down syndrome
  • Pregnant women exposed to certain prescription medications, rubella (German measles) or uncontrolled diabetes

Special Considerations for Adults with Congenital Heart Disease

Adults with congenital heart defects are different from adults with other heart problems like coronary artery disease. They are typically younger and have unique problems which vary depending on the particular heart condition with which they were born. This means that care for adults with congenital heart disease must be individualized.

Perhaps most important, adults with diagnosed congenital heart defects need routine checkups. These patients need to visit a primary care physician and cardiologist regularly, even when they are feeling well, to prevent problems from occurring.

To develop an individualized care strategy for your congenital heart disease, schedule an appointment with one of the expert cardiologists on the medical staff at Baylor Scott & White Heart and Vascular Hospital, please call .

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