Table 2.
Classification systems to categorise patients with congenital heart disease according to the severity of the disease
| Classification system | Prevalence | ||
| Initial diagnosis (n = 629) | |||
| Task Force 1 of the 32nd Bethesda conference of the American College of Cardiology1 | 164 (26.1%) | ||
| • Mild (for example, isolated aortic valve disease, closed ASD without residua) | 365 (58.0%) | ||
| • Moderate (for example, coarctation of the aorta, tetralogy of Fallot) | 100 (15.9%) | ||
| • Severe (for example, Fontan operation, Eisenmenger, double outlet ventricle) | |||
| Illness course (n = 629) | |||
| Disease severity index15 | |||
| • Low: maximum 1 cardiovascular operation or 1 catheterisation procedure | 404 (64.2%) | ||
| • Moderate: more than 1 cardiovascular operation or catheterisation | 203 (32.3%) | ||
| • High: persistent cyanosis, <92% oxygen saturation at rest, or single ventricle physiology | 22 (3.5%) | ||
| Current functional status (n = 627) | |||
| NYHA functional class16 | |||
| • Class I: patients with cardiac disease but without resulting limitation of physical activity; ordinary physical activity does not cause undue fatigue, palpitation, dyspnoea, or anginal pain | 511 (81.5%) | ||
| • Class II: patients with cardiac disease resulting in slight limitation of physical activity; they are comfortable at rest; ordinary physical activity results in fatigue, palpitation, dyspnoea, or anginal pain | 85 (13.6%) | ||
| • Class III: patients with cardiac disease resulting in major limitation of physical activity; they are comfortable at rest; less than ordinary activity causes fatigue, palpitation, dyspnoea, or anginal pain | 26 (4.1%) | ||
| • Class IV: patients with cardiac disease resulting in inability to carry on any physical activity without discomfort; symptoms of heart failure or the anginal syndrome may be present even at rest; if any physical activity is undertaken, discomfort increases | 5 (0.8%) | ||
| Ability index17 (n = 628) | |||
| • Class 1: normal life (full time work or school, pregnancy poses no health risk) | 524 (83.4%) | ||
| • Class 2: able to work (intermittent symptoms, interference with life, pregnancy possible) | 78 (12.4%) | ||
| • Class 3: unable to work (limitation of all activities, pregnancy poses health risk) | 24 (3.8%) | ||
| • Class 4: extreme limitation (dependent, almost house bound) | 2 (0.3%) | ||
| Congenital heart disease functional index18 (n = 628) | |||
| • Class 1: no surgery, good clinical status, medical follow up not strictly necessary | 24 (3.8%) | ||
| • Class 2: with or without surgery, functionally perfect, postoperative normalisation of clinical condition, medical check up every 3 to 5 years, competitive sports permitted | 221 (35.2%) | ||
| • Class 3: with or without surgery, functionally good, medical restrictions, medical check up every 1 to 2 years, recreational sports permitted | 355 (56.5%) | ||
| • Class 4: with or without surgery, moderate functional status, functioning at own pace, medical check up every year | 23 (3.7%) | ||
| • Class 5: with or without palliative surgery, bad functional status, cyanosis, medical check up every 6 to 12 months | 5 (0.8%) | ||
| Left ventricular ejection fraction | Median: 62 (IQR: 15) | ||
ASD, atrial septal defect; IQR, interquartile range; NYHA, New York Heart Association.