Guidelines for Treating Adults with Congenital Heart Disease (2008)

The American College of Cardiology and the American Heart Association have developed standards for treating adults with congenital heart disease (ACHD). Because there are many different types of congenital heart disease, the guidelines include general recommendations that apply to most patients—which are covered in this summary—as well as specific recommendations for each condition, which can be found in the guidelines document itself. As always, these guidelines give your doctor the flexibility to tailor treatment to your specific clinical needs.

Print out this check list and bring it with you to your next doctor visit so that you can ask questions and have a record of your treatment. When you get home, visit CardioSmart’s Congenital Heart Defects condition center to help remind you of what you talked about with your doctor and what you need to do to take care of yourself or your loved one.

What You Need to Know

Approximately 800,000 adults in the United States have congenital heart disease (CHD).

Experts in congenital heart disease are working to improve the health care system, so that teens and young adults have an easier time making the transition from receiving health care in pediatric cardiology centers to receiving care from specialists in adult cardiology. They have made the following recommendations:

  • Improve access to care by establishing more transition clinics, reaching out to patients who are leaving pediatric care, and improving education on adult congenital heart disease for adult cardiovascular specialists.
  • Establish more regional ACHD centers to coordinate care for ACHD patients and provide a support and referral network for pediatric cardiology and adult cardiology practices, as well as local emergency departments.
  • Keep current clinical records for each ACHD patient on file with the primary care physician, local cardiologist, and regional ACHD center. Patients should also have copies of key medical records.
  • Provide ACHD patients with a medical “passport” that contains information on their medical history, surgeries, medications, and contact information for the local and regional ACHD centers where more detailed records are stored.

If you are an adult patient with CHD, a regional ACHD center should play a key role in your health care throughout life. Experts recommend that:

  • You go to a regional ACHD center for regular follow-up visits at least every 1 to 2 years if you have moderate or complex CHD and every 6 to 12 months if you have very complex ACHD. Even if you have low-risk, simple CHD, you should be evaluated at a regional ACHD center at least once, to determine if future follow-up is needed.
  • If you need to be admitted to a hospital for treatment of a new illness or health problem, you should usually be transferred to a regional ACHD center. This is especially important if you have a new cardiac problem or need surgery with anesthesia, but is important even for noncardiac problems if you have high-risk or complex CHD. If you have low-risk CHD and you are admitted to a regular hospital, your doctor should contact your regional ACHD center for information and advice.
  • You should go to an experienced regional ACHD center if you have moderate or complex CHD and need imaging tests or interventional procedures on your heart and blood vessels, including echocardiography, magnetic resonance imaging, computed tomography, advanced cardiac catheterization, and electrophysiology procedures, which are used to diagnose and treat abnormal heart rhythms.
  • If you have moderate or complex CHD and your doctor does not have experience or training in taking care of patients with ACHD, he or she should team up with a specialist who has advanced ACHD training.

Examples of low-risk, simple CHD include mild pulmonary stenosis and repaired ventricular septal defect. Examples of moderate CHD include coarctation of the aorta, moderate-to-severe pulmonary valve regurgitation, and tetralogy of Fallot. Examples of highly complex CHD include single ventricle, transposition of the great arteries, and any type of CHD that causes cyanosis (not enough oxygen getting to the body’s tissues).

As you strive to live a happy, independent life, you may face special challenges if you have CHD—especially as a teen or young adult. Social workers, psychologists, nurses, and physician assistants should play an important role in helping you and your family deal with the following issues:

  • Independent living.
  • Social relationships and support.
  • Employment.
  • Health and life insurance.
  • Learning problems.
  • Psychological problems, such as depression or anxiety.

Your doctor or other members of your health care team will give you specific information and advice about health issues that are affected by your CHD, including:

  • Exercise and participation in sports.
  • Preventive health habits for overall good health.
  • Genetic testing and counseling.
  • Safe and effective birth control, especially the need for some women with ACHD to avoid estrogen-containing birth control pills.
  • Pregnancy, including the risk of premature delivery and low birth weight; risk to the baby from medications the mother needs to take, such as blood thinners; possible complications during pregnancy and delivery; and importance of preventing blood clots during and after delivery.

Patients with CHD face an increased risk for getting an infection that can spread to the lining of the heart and heart valves—infective endocarditis (IE). Your doctor may:

  • Talk with you about your specific risk for IE and how to prevent it, including the importance of good teeth-brushing and regular visits to the dentist and dental hygienist, effective acne treatment, the need to avoid nail-biting, and whether you should take antibiotics before certain dental procedures or before giving birth.
  • Draw blood to check for infection and start antibiotics if you develop an unexplained fever that may be IE.
  • Examine your heart with echocardiography if you have signs of an infection in your bloodstream, heart valves, or the implanted materials or devices used to surgically repair your CHD.

If you need noncardiac surgery, you are at higher risk than someone who doesn’t have ACHD. Experts recommend the following:

  • Whenever possible, you should have both preoperative testing and surgery at an ACHD regional center with experienced surgeons and anesthesiologists. If not, your doctor should consult with ACHD experts and a cardiac anesthesiologist.
  • If you have certain high-risk forms of CHD, you should always have surgical care at an ACHD regional center, unless it is an absolute emergency.

It’s not uncommon for adults with CHD to have an irregular heart rhythm (arrhythmia), which is caused by problems in the heart’s electrical system. In treating arrhythmias, experts recommend that:

  • You have in-depth evaluation and testing, including a review of your past heart surgeries, before having any procedures to diagnose or treat your arrhythmia. This will give your arrhythmia specialist (electrophysiologist) a clear understanding of your specific heart structure.
  • You go to an experienced ACHD center for procedures like pacemaker implantation or catheter ablation.
  • You and your doctor consider an implantable cardioverter-defibrillator (ICD) if you have ever had your heart beat so fast that you became dizzy or fainted, or had your heart stop beating completely (cardiac arrest).

If your ACHD results in too little oxygen being circulated to your body tissues (cyanosis), this may cause other health problems or medical issues, including:

  • Thickening of the blood, resulting from the body’s attempt to increase oxygen circulation by making extra red blood cells. You may feel headache or fatigue.
  • Excessive bleeding, as a result of problems with blood clotting.
  • Reduced kidney function.
  • Gallstones.
  • Joint and bone problems, especially scoliosis (curvature of the spine).
  • Neurological problems, including the risk of blood clots to the brain.
  • Dehydration on long-distance flights and, possibly, the need for extra oxygen.

If your ACHD results in heart failure (when your heart can’t pump enough blood to meet your body’s needs), you may need a heart, lung or heart/lung transplant. In this case, you should be evaluated and receive care at a major medical center where the doctors, nurses, and other health care workers have experience and training not only in transplantation but also in CHD.

Read the full guidelines in the Journal of the American College of Cardiology