Adult Congenital Heart Disease (ACHD)
Congenital heart disease is the most common form of birth defect in the United States. Many patients diagnosed with congenital heart disease require specialized care, and even patients who had their condition repaired in childhood require follow-up care because they are prone to unique complications that require treatment. Surgical repairs may deteriorate over time and patients with congenital heart disease may develop acquired cardiovascular diseases and — the combination of these two requires specialized care.
Adults who have had surgical procedures as a child to correct or treat a congenital heart defect require follow-up care because these surgical treatments may deteriorate over time and can become less effective. Some common follow-up repair procedures include:
- Repairing valves or conduits that are outdated or not functioning properly
- Repair of a recurrent coarctation (recoarctation) of the aorta
- Inserting a balloon or stent to repair obstructions that develop in the systemic or pulmonary veins after atrial repair for transposition of the great arteries
- Treating complications from Fontan procedures
Patients who did not receive treatment for congenital heart disease as a child may require surgical or medical treatment to manage their condition as adults.
Patients with congenital heart problems can develop acquired heart disease and other cardiovascular diseases as they age, which can be more complicated to treat than for adults without a congenital heart disease (CHD). Common age-related cardiovascular conditions may include:
General adult cardiologists may be less familiar with patients diagnosed with heart failure and a congenital heart disease because heart failure associated with a congenital heart disease frequently presents differently, with symptoms that are unique compared to the rest of the population. As an example, adults diagnosed with CHD often develop right-sided heart failure (in adults without CHD, heart failure is commonly on the left side). Developing heart failure on the right side of the heart requires different treatments than left-side heart failure.
It is recommended that adults diagnosed with congenital heart disease seek treatment from specialized physicians, to prevent heart failure and to treat it should it develop.
Pulmonary hypertension is more common in patients diagnosed with congenital heart diseases than in the general population. Pulmonary hypertension is high blood pressure in the lungs and can cause limitations in a patient’s life as the disease progresses.
Adults diagnosed with congenital heart disease may require different and unique interventions to manage their pulmonary hypertension, and may require routine treatment and appointments with a specialized pulmonologist. In rare cases, a lung transplant may be necessary for patients in the end-stage of the disease.
Arrhythmias, or irregular heart rhythms, may develop over time in patients with congenital heart disease. These may be caused by conduction abnormalities from prior surgical incisions or scarring of heart tissue after repair operations. Certain congenital heart conditions and procedures may be associated with arrhythmia:
- Fontan procedure
- Mustard/Senning procedure
- Tetralogy of Fallot
- Ebstein’s anomaly
- Atrial septal defects
Treatment for arrhythmia may include medication, a pacemaker or surgical treatment.
Women diagnosed with congenital heart disease may face more risks during their pregnancy than others. During pregnancy, a woman’s cardiovascular system changes, which may cause more complications for the mother and her unborn child. Pregnancy may not be recommended for women with some forms of congenital heart disease. Women diagnosed with congenital heart disease planning to have children are encouraged to see a physician specialized in adult congenital heart disease to evaluate the risks and potential complications of pregnancy.
Johns Hopkins Adult Congenital Heart Disease Center
The Johns Hopkins Adult Congenital Heart Disease Center provides lifelong heart care for patients diagnosed with congenital heart diseases.
Can I exercise after being diagnosed with ACHD?
Exercising and training for adults and children with congenital heart disease may be difficult depending on the patient’s defect, medical history and current condition. A physician can provide guidelines that establish safe limits based on the level of physical exertion and the patient’s condition.