Historically, only a minority of children born with a congenital heart defect survived into adulthood; however, recent advances in surgical treatments and techniques have reversed these statistics. Now, approximately 90 percent of children born with a heart defect live healthy lives through adulthood. This shift in survival rates has produced a relatively new group of patients with a new set of challenges for the medical community—adults with congenital heart disease. Today, adults with congenital heart disease outnumber children with the condition.
Although repairs of a heart defect are corrective, they are not curative. All survivors have an increased risk of developing heart problems as their hearts and bodies age. If you have a complex defect, you have an increased risk of heart failure, stroke, and developing heart rhythm problems and/or heart infections. People with complex defects like valve atresia, tetralogy of Fallot, transposition of the great arteries, or abnormal ventricles need to be evaluated in a specialized center for adult congenital heart disease on a regular basis. Regardless of the complexity of the defect, most people who actively manage their congenital heart disease have few or no ongoing symptoms or limitations on what they can do physically.
Most specialists feel that the single most important thing individuals with congenital heart disease can do is learn about their condition so that they can effectively manage it. No adult should rely on parents, a primary care doctor, or anyone else to manage his or her disease.
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How well the heart compensates for structural abnormalities is, in part, determined by overall physical fitness and health. People with congenital heart disease need to fully embrace a lifestyle that promotes fitness and heart health.
Key guidelines include:
People with congenital heart disease need to adopt a team approach to manage their disease effectively. The team usually consists of three to five people in addition to themselves:
Additional members of the team rotate in and out over time and may include a cardiac surgeon, general surgeon, gynecologist, obstetrician, or genetic counselor. Open communication among team members is fundamental to successfully managing congenital heart disease.
Many aspects of your lifestyle will need to be discussed with the healthcare team, including potential effects of over-the-counter medications, vitamins, herbal preparations, or prescription medications on the heart. You'll also want to discuss potential interactions between any drugs you might be taking for your heart defect and any of the above.
It is important to communicate any changes in your condition or lifestyle to your physician. Fever or other signs of infection, increased fatigue, and shortness of breath all are reasons to contact a physician. Changes in the circulatory system resulting from corrected heart abnormalities can develop over a long period of time, so the first manifestations of these can be very subtle. When in doubt, get it checked out.
It's important for people with heart defects to get regular checkups. The frequency and components of these checkups will vary according to individual circumstances, depending upon your defect(s), age, and overall health. These checkups may include blood tests, standard electrocardiogram, 24-hour ambulatory electrocardiogram, chest X-ray, echocardiogram, or exercise testing.
Children. Periodic heart checkups with a pediatric cardiologist are necessary to evaluate the progression of the condition or verify the continuing success of the treatment. The frequency of checkups depends upon complexity of the defect and treatment plan. More frequent checkups are necessary just after diagnosis, around surgery, and for children with complex defects.
Adults. The frequency at which one sees a cardiologist with expertise in congenital heart disease should be determined on a case-by-case basis by one's health team. For example, a healthy adult with a simple defect, like a small ventricular septal defect or atrial septal defect, may need to see the specialist only once every five years. A more complex defect may require annual consultations. In either case, it is important for proper management of your condition to find a congenital cardiologist who will be available to consult with you and your local physician as necessary. If you have complex heart disease, you should not wait until you have symptoms; you should see your congenital cardiologist on a regular basis.
Endocarditis is a dangerous infection of the endocardium, the lining of the inside of the heart's four chambers. Certain bacteria and other microorganisms that occasionally circulate in the blood may settle and multiply on the heart valves. Abnormal heart valves, where blood flow is turbulent, are especially vulnerable to this kind of infection. Although effective treatments are available, endocarditis can lead to permanent damage of the heart valves or even heart failure.
Most congenital heart defects are associated with increased susceptibility to endocarditis. Individuals at high risk include those who have had endocarditis before, had previous defects in their heart valves or have mechanical valves (such as for aortic valve stenosis), as well as individuals with complex heart abnormalities like single ventricles, tetralogy of Fallot, or transposition of the great arteries. People who have had surgery to construct systemic pulmonary shunts or conduits also are at high risk.
Endocarditis is diagnosed using a combination of blood culture and special echocardiogram. If a child or an adult with congenital heart disease has a fever that cannot be attributed to a specific infection like strep throat or an ear infection, endocarditis should be considered.
Treatment of endocarditis may require four to six weeks or more of intravenous antibiotics and may include surgery. Penicillin (amoxicillin/ampicillin) and gentamicin are the most commonly used antibiotics. Other antibiotics can be used as well: vancomycin, cephalosporin, clindamycin, or azithromycin. Although powerful antibiotics are available, treatment can be difficult and complications of endocarditis may occur.
In order to minimize the risk of developing endocarditis, some people with congenital heart disease will need to take antibiotics prophylactically, prior to certain dental procedures. In some cases the antibiotics will be administered intravenously and may include follow-up doses after the procedure. In other situations, the antibiotics are taken orally, one hour before the procedure. Detailed information on endocarditis treatment can be found on the American Heart Association website.
It's important to note that most cases of endocarditis are not caused by and do not directly follow a dental procedure. More often, the endocarditis follows a minor gum injury or is caused by poor dental hygiene. Regular and comprehensive dental care is imperative for people with congenital heart disease.
Dental procedures requiring endocarditis prophylaxis include:
Individuals can do several things daily to guard against an endocarditic infection:
Although physicians and dentists are aware that people who are susceptible to bacterial endocarditis should be given antibiotics prophylactically prior to procedures, it is a good idea to inform or remind your doctor or dentist of congenital heart disease before any dental, surgical, or special procedures is done.
All children and adults with heart defects must take extra precautions when undergoing a noncardiac surgical procedure. A relatively simple surgery like an appendectomy can be life-threatening for an individual with a complex congenital heart defect like Eisenmenger's syndrome because the hearts and lungs may respond unpredictably to anesthesia or the stress of surgery. Noncardiac surgeries may need to be performed at a special center that has cardiac anesthesia and cardiologists familiar with the complications of congenital heart defects.
During pregnancy, a woman's body goes through many changes, which may bring on or worsen the symptoms of a heart defect. This is especially true in the second and third trimesters. The mother's condition also can put the fetus at risk for poor growth or other problems. Some commonly prescribed heart disease medications pose added risks to the fetus. For some types of heart disease, especially those associated with pulmonary hypertension or significant ventricular dysfunction, pregnancy creates significant risk for the mother. In such cases, doctors would advise the woman not to have a baby.
With proper care, however, most women with congenital heart disease can successfully bear children. Prior to conception, women need to seek medical advice to fully assess the potential risk to the mother and baby. Proper medical advice will require assembling a team including the congenital cardiologist, a high-risk obstetrician, and possibly a local cardiologist and a genetic counselor as well. The multidisciplinary team will determine the best way to manage the pregnancy, the best way to deliver the baby, and whether there's need for special postpartum care for the mother.
In many cases, the team may recommend that the pregnancy be monitored by a high-risk obstetrician, as well as a cardiologist familiar with her condition.
In general, the risk of a fetus developing a heart abnormality is about 6 percent when the mother has congenital heart disease. However, each situation is unique and is largely dependent upon the type of heart defect in the mother, father or siblings. An accurate determination of the risk to the developing fetus may require genetic counseling.
Women with congenital heart defects need to consult their primary care physician, gynecologist, and cardiologist before choosing a birth control agent. Intrauterine devices may be associated with an increased risk of endocarditis. Most women with congenital heart disease can use most of the effective methods of birth control safely. However, women with pulmonary hypertension or complex congenital heart disease, such as Eisenmenger's syndrome, should not use oral contraceptives or other methods of birth control that contain estrogen, because of increased risk of blood clots.
Many people with congenital heart disease are at risk for pulmonary hypertension and heart arrhythmias.
Pulmonary hypertension is a debilitating and often fatal condition in which the blood pressure is elevated in the arteries that supply the lungs. Individuals with congenital heart disease that causes excessive blood flow through the pulmonary blood vessels are susceptible to developing pulmonary hypertension. For example, people with Eisenmenger's syndrome and mitral valve stenosis are at increased risk for developing pulmonary hypertension.
The early symptoms are fatigue and shortness of breath that become worse over time. As the condition progresses, chest pain, blueness of the skin, fainting, swelling of the legs, bulging of the neck veins, loss of appetite, and abdominal pain may develop. It is important to diagnose and treat this condition early on, before irreversible damage to the lungs occurs.
Some people diagnosed with pulmonary hypertension take vasodilators, medications that dilate the blood vessels, to control symptoms. The most effective of these, epoprostenol, is also the most complicated to administer, requiring continuous injection into a vein. In these cases, a permanent venous catheter is inserted, and the patient wears a small pump 24 hours a day. Other vasodilators can be taken orally in pill form or via a specialized inhaler. The blood thinner warfarin also can increase the life span in some people with pulmonary hypertension.
Arrhythmias, abnormal changes in the rhythm of the heartbeat, are not unusual in individuals with congenital heart disease. Arrhythmias may involve the heart beating too fast, too slow, skipping a beat, or adding an extra beat periodically. The symptoms of arrhythmias vary with severity from negligible to sudden death. Most frequently, arrhythmias involve sudden shortness of breath, lightheadedness, or fainting.
Arrhythmias can be lethal; any individual with congenital heart disease who becomes acutely short of breath needs to get an EKG immediately to identify any arrhythmias.
Congenital cardiologists prescribe regular aerobic exercise for most people with congenital heart disease. Aerobic exercise strengthens the heart and circulation and improves overall physical fitness and emotional well-being. When exercising, people with congenital heart disease should always follow a physician's guidelines.
Many parents of children with congenital heart defects worry about the risks of rough play and vigorous activity on their child's heart. However, most children can engage in normal or near-normal activity levels. The parents may need to discuss their concerns with the pediatric cardiologist, who can advise parents on which activities are safe for a child. In certain instances, parents may need to encourage a child to pursue other interests, instead of focusing on the child's limitations. Although every circumstance is unique, most children with congenital heart defects grow up to lead healthy, productive lives.
Individuals with congenital heart disease face tough health issues throughout their lives. Often, they are coping with a lot of uncertainty about how their disease might progress. Family planning can be a critical issue for people with complex defects so as to avoid potentially life-threatening situations in terms of birth control and/or pregnancy. Planning for good health insurance so as to maintain access to one's doctors can be an added layer of stress.
Many of these issues arise during the transition from childhood to adulthood. Learning about one's condition is fundamental in order for preteens and young adults to make wise decisions that ultimately affect their adult lives. Selecting a career that is compatible with their condition and maintains lifelong medical insurance can help alleviate some stress. Proximity to specialized medical centers may need to be a factor when considering where to live.
Despite careful planning, feelings of anxiety and even depression are common among people coping with chronic health conditions, such as congenital heart disease. So if you think you may need help, seek it out. The Adult Congenital Heart Association is a nonprofit organization that seeks to improve the quality of life and extend the lives of adults with congenital heart defects. This national organization serves and supports more than 1 million adults with congenital defects, their families, and the medical community.
Last reviewed on 2/11/2009
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