Prevention of sudden cardiac death in adults with congenital heart disease

Sudden cardiac death is the most common cause of death in adult congenital heart disease (ACHD) patients. When sudden cardiac death occurs it is often at a young age (on average 36 years old). In general it is rare, but there are certain individuals who have a greatly increased risk. It is important to identify those patients; sudden cardiac death is caused by cardiac arrhythmias and can be prevented by placing an implantable defibrillator (ICD), a type of pacemaker that delivers a shock if a life-threatening arrhythmia is detected.
However, we have found that at present the predictive methods for sudden cardiac death in ACHD patients to determine who should receive such an ICD are insufficient.
We have explored new risk factors and validated a new risk assessment model to better predict sudden cardiac death. This risk score model is based on seven risk factors that patients who died of sudden cardiac death had significantly more often than patients with the same condition who were still alive. It turned out that this risk model worked better than the current prediction methods.
In addition, we have conducted research into resuscitation in ACHD patients, where we have shown that patients with less complex congenital heart defects also suffer from life-threatening arrhythmias and that myocardial infarction is not a common cause of cardiac arrest in ACHD patients.
We also investigated what happens when patients with congenital heart disease receive an ICD. In about 25% of patients with an ICD, the device must intervene for a life-threatening heart rhythm disorder. However, a relatively large number of complications, especially from the leads of the device, occur in also about a quarter of the patients. Lastly, another quarter receives an inappropriate shock from the device.