Background: It is known that children with previously diagnosed heart defects die suddenly. The causes of death are often unknown.
Objective: The aim of the study was to identify all infants and children within the Netherlands with previously diagnosed heart disease who had a sudden unexpected death (SUD), and to identify the possible cause of death.
Design: Retrospective, cross-sectional study.
Patients and setting: All children (<19 years) with a previously diagnosed heart defect and SUD between January 1990 and June 2001 in seven out of eight tertiary centres in the Netherlands were identified using the hospital databases. We excluded patients receiving compassionate care. Diagnoses, clinical status and circumstances of death were sought from case notes and post mortem reports. Deaths were classified as of cardiac or non-cardiac origin.
Results: We identified 150 cases of SUD (89 male) at a median age of 2.3 years (range 18 days-18.9 years); 49/150 patients (33%) were </=1 year. Diagnostic categories included left to right shunts (N=34, 14 </=1 year), cyanotic lesions (N=38, 13 <==1 yar), cardiomyopathy (N=11, 2 </=1 year), univentricular heart (N=24, 10 </=1 year), obstructive lesions (N=11, 3 </=1 year), arrhythmia (N=8, 0 </=1 year), and miscellaneous defects (N=18, 5 </=1 year). 108/150 patients (72%) had been operated of whom 61 (57%) had corrective surgery. Of the infants with SUD, 32/49 (65%) had undergone surgery (11 corrective); 76/101 older children had undergone previous surgery (50 corrective). 114/150 children (76%) died of a cardiac cause. Causes of death were arryhythmia (59), heart failure (25), shunt occlusion (10), pulmonary hypertensive crisis (8) and acute myocardial infarction (4). 30/49 infants (61%) died of a cardiac cause; causes of death included arrythmia (10), heart failure (8), shunt occlusion (7), acute myocardial infarction (2).
Conclusions: SUD in children with heart defects were predominantly of cardiac origin. Pumpfailure and arrhythmias were the terminal events in a significant number of patients in the entire population.