Adult Congenital Heart Disease (ACHD)

Adult congenital heart disease (ACHD), often called a ‘hole in the heart’, is one of the most common birth defects. While some congenital heart defects don’t require treatment because they are very minor, many patients diagnosed with congenital heart disease often require specialized care.

Furthermore, many patients who had their condition repaired in childhood require on-going follow-up care because they are prone to unique complications that require treatment.

That’s because surgical repairs may deteriorate over time and patients with congenital heart disease may develop acquired cardiovascular disorders. As a result, patients with ACHD often need more specialized cardiac care.

Common follow-up repair procedures for patients with ACHD include:
  • Repairing valves or conduits that have deteriorated over time and are not functioning properly
  • Repair of a recurrent coarctation (re-coarctation) 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 ACHD may be susceptible to other cardiovascular diseases

Patients with congenital heart problems can develop acquired heart disease and other cardiovascular diseases as they age, which can be difficult to treat. Common age-related cardiovascular conditions in these patients may include:

  • Heart Failure
  • Pulmonary Hypertension
  • Heart Arrhythmia
  • Stroke
Septal Defects ('holes in the heart’)

A septal defect can occur between the two lower chambers or ventricles (pumping chambers) of the heart, called a ventricular septal defect, or between the two upper chambers or atria (filling chambers), called an atrial septal defect. With either type, oxygenated blood coming from the lungs gets mixed with deoxygenated blood returning from the body.

A serious complication of septal defects is seen when the direction of the mixing of blood causes the blood supply leaving the heart to contain less oxygen than normal (a shunt, or “septal hole,” that is right to left). A shunt, whether left to right or right to left, makes the heart work harder to distribute the same amount of oxygen to the body.

More commonly, blood from the left heart gets shunted to the right, making the pressure in the right heart go up, overworking the right pumping chamber of the heart making it malfunction over time.

Valve Defects

In heart valve disease, one or more of the valves in your heart do not work properly. Your heart has four valves that keep blood flowing in the correct direction. In some cases, one or more of the valves don't open or close properly.

This can cause the blood flow through your heart to your body to be disrupted. ‘Leaky valves’ allow the blood to flow in the opposite direction, while ‘tight valves’ prevent the blood from flowing forward in the right direction.

Narrow Blood Vessels

Similar to tight valves, blood vessels that are too narrow at a certain point cause the heart to work harder to pump a normal supply of blood. Blood vessels can be connected incorrectly, sending deoxygenated blood to the body or already oxygenated blood back to the lungs.

Symptoms

Common symptoms of adult congenital heart defects include:

  • Shortness of breath, especially during exercise
  • A heart murmur
  • Abnormal heart rhythms (arrhythmias)
  • Swelling of the extremities
  • Tiredness
  • Cyanosis (a blue tint to the lips, skin, or fingernails caused by a lack of oxygen in the blood)
Treatment

Various approaches can be taken in treating and managing a congenital heart defect, depending on the type and severity of the defect. It should be noted that some minor congenital heart defects discovered in adults will never need to be treated or repaired. However, these patients should receive regular cardiac checkups to make sure that the defect does not deteriorate over time.

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