Congenital Heart Diseases in Adults – Status Report

In the past children with congenital heart disease CHD rarely reached adulthood due to limited treatment options. With the advent and progress of congenital cardiac surgery, and improvements in the medical management, the number of of adults with operated CHD is increasing worldwide. It is predicted that 32,000 new cases of CHD are detected per year and that 85% of infants born with CHD reach adulthood.

The majority of these patients had palliative or reparative surgery during childhood, but 10 % of CHD patients are not diagnosed until they are adults. The increasing number of adults with CHD is related not only to congenital cardiac surgery but also increased awareness and recognition of CHD and increasing numbers of patients with uncorrected CHD coming to medical attention.

The majority of patients with operated CHD is not cured and thus requires ongoing congenital cardiac care because of the high frequency of residua and sequelae. The conditions and their expected survival are shown below.

Survival of Corrected and Uncorrected congenital heart defects

Common Defects in which un-operated adult survival is expected

  1. Bicuspid aortic valve
  2. Pulmonary valve stenosis
  3. Ostium secundum atrial septal defect
  4. Small patent ductus arteriosus
  5. Small ventricular septal defect

Uncommon Defects in which un-operated adult survival is expected

  1. Congenitally corrected transposition of the great arteries (I-TGA)
  2. Ebstein’s anomaly
  3. Congenital pulmonary valve regurgitation
  4. Lutembacher’s syndrome
  5. Sinus of Valsalva aneurysm
  6. Coronary artery anomalies
  7. Single ventricle with pulmonary stenosis

Common Defects in which un-operated adult survival is exceptional

  1. Large ventricle septal defect
  2. Aortic forestation
  3. Tetralogy of Fallot
  4. Complete transportation of the great arteries

Most patients with operated CHD require lifelong informed cardiovascular follow up. Periodic evaluation at an established adult congenital heart disease clinic is recommended. However, regular lifelong follow up is required at a local level and adult cardiologists must be aware of the cardiovascular issues that may arise in adult CHD patients and recognize problems which warrant referral to an adult congenital heart disease clinic. Many patients with important residua or sequelae from their CHD are asymptomatic and the first symptom is often an arrhythmia.

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