Sudden death in children with unoperated heart disease

One would hope that most significant cardiovascular malformations would be detected early in life, preferably at an asymptomatic stage. However, despite health screening some significant problems go unrecognised,"20 and may cause sudden death. Deaths from obstructive left heart malformations are unusual but they do occur.4 Postoperative deaths in such patients were ascribed to "arrhythmia" in the study by

Silka and colleagues, but syncope in aortic valve stenosis is probably not primarily arrhythmic in origin.w21 Congenital abnormalities of the coronary arteries are rarely diagnosed in life and prodromal syncope is uncommon. The most frequently recognised abnormalities after sudden death are anomalous origin of the left or right coronary arteries from the contralateral aortic sinus, with a proximal course between the aorta and the main pulmonary artery.w22 w23 Although the prevalence of such anomalies is hard to define precisely, the risk of sudden death is probably high enough to warrant surgical treatments24 Sudden death in other specific conditions, such as Williams syndrome, is also reported and may be caused, in some cases at least, by coronary artery stenosis or malformation^25

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