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Adult Congenital Heart Disease (ACHD)

What is Adult Congenital Heart Disease?

People who were treated for a congenital heart defect when they were little may think they do not need to follow-up with a cardiologist. That is not always true. Many have been told they were “fixed. Really,, they have only been repaired. Those who were treated as a child may have complications at different times during their life. These issues may will not be the same for everyone. Routine follow-up with a adult congenital heart cardiologist is vital in reducing the potential problems these patients may have as they get older.

There are about 50 different categories of congenital heart defects, so the problems that may occur vary from one condition to another. Each patient should ask their cardiologist what symptoms to look for.

What to Expect During a Visit

A typical congenital heart patient visit includes an echo ECG, a health history, and a physical exam. The patient can expect to see three or four different congenital heart providers during the visit.

At each visit, the patient and cardiologist discuss when to schedule the next visit. They talk about what future testing to include in the treatment plan.

Adult Congenital Heart Disease Treatment

Patients with congenital heart disease can have many symptoms. Treatment is based on the symptoms. Examples may include:

For a patient whose heart beats fast, all the time or occasionally, treatment options may include:

  • Medicines - control the heart rate or to prevent the abnormal rhythm
  • Ablation procedure - the electrical circuit causing the rapid heartbeat is interrupted
  • Pacemaker - a small, battery-powered medical device designed to electrically stimulate the heart muscle in an effort to restore the heart rhythm towards normal
  • Defibrillator - a small battery-powered device placed in the chest to detect and stop irregular heartbeats

For a patient whose valve problem becomes severe, treatment options include:

  • Valve repair
  • Valve replacement

For a patient who has had a heart infection called infective endocarditis, treatment may include:

  • Antibiotics
  • Heart surgery, if needed

For a patient with a very weak heart muscle, treatment may include:

  • Medications
  • Treatment of the condition causing the weak heart muscle (for example, a very leaky or narrow valve)
  • A pacemaker procedure called “cardiac resynchronization”
  • Mechanical circulatory support and/or heart transplantation

Who Should Receive Genetic Screening?

Some patients may have congenital heart disease. This is related to a genetic condition.

Knowing if a patient has a genetic condition is important to improve their healthcare treatment. It also helps patients understand the possible risks to other people in their family.

Patients with the following issues may be helped by a genetic screening:

  • Problems with growth such as short stature.
  • Another type of birth defect in addition to a congenital heart defect.
  • Hearing loss or major problems with vision.
  • Learning disabilities or developmental delay
  • Conditions that don’t have a known cause in a patient such as a major kidney problem, a thyroid disorder, or a mental health diagnosis.
  • A family history of any of these concerns or a family history of congenital heart disease.

Patients with a heart defect who are interested in talking about pregnancy may benefit from genetic counseling and / or screening.

Group 1—Simple Congenital Heart Disease
These patients can usually be cared for in the general medical community.

Unrepaired conditions:

  • Isolated small atrial septal defect (ASD)
  • Isolated small ventricular septal defect (VSD)
  • Mild pulmonic stenosis
  • Isolated dextrocardiano other heart problems

Repaired conditions:

  • Patent ductus arteriosus (PDA)
  • Secundum atrial septal defect (ASD)
  • Isolated ventricular septal defect (VSD)

Repaired or unrepaired conditions:

  • Isolated aortic valve disease
  • Isolated mitral valve disease
  • Isolated mitral valve disease
Group 2—Congenital Heart Disease of Moderate Severity
These patients should be seen periodically at adult congenital heart disease centers.

Repaired or Unrepaired:

  • Anomalous Left Coronary Artery from Pulmonary Artery (ALCAPA)
  • Anomalous pulmonary venous drainage (partial or total)
  • Atrioventricular (AV) canal/septal defects (partial or complete)
  • Ostium primum or sinus venosus ASDs
  • Coarctation of the aorta
  • Ebstein's anomaly
  • Infundibular right ventricular outflow obstruction (moderate to severe)
  • Pulmonary valve regurgitation (moderate to severe)
  • Pulmonic valve stenosis (moderate to severe)
  • Sinus or Valsalva fistula/aneurysm
  • Subvalvar or supravalvar aortic stenosis
  • Tretalogy of Fallot
  • Ventricular septal defect (VSD) with any valve problems and/or obstructions
Group 3—Congenital Heart Disease of Great Complexity
These patients should be seen regularly at adult congenital heart disease centers.

Repaired or Unrepaired:

  • Congenitally corrected transposition of the great arteries (ccTGA or I-TGA)
  • Double outlet ventricle
  • Mitral atresia
  • Pulmonary atresia (all forms)
  • Pulmonary vascular obstructive diseases
  • Shone's syndrome
  • Single ventricle—all forms (ie, double-inlet ventricle; HLHS, HRHS, common/primitive ventricle)
  • Transposition of the great arteries (d-TGA)
  • Tricuspid atresia
  • Truncus arteriosus/hemitruncus
  • Other abnormalities of AV connections (ie, crisscross heart, isomerism, heterotaxy syndromes)

All patients who have undergone the following procedures:

  • Arterial switch procedure
  • Blalock-Taussig shunt
  • Any conduit(s), valved or nonvalved
  • Double-switch procedure
  • Fontan procedure
  • Mustard procedure
  • Norwood procedure
  • Rastelli procedure
  • Senning procedure
  • All patients with Eisenmenger syndrome
  • All patients who are cyanotic "blue"

Last Updated 04/2022

Reviewed By Amy Rich, MSN, APRN, CNP

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