P622Sudden death in congenital heart disease. a study from a specialist UK referral centre

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Abstract

Background

Congenital heart disease (CHD) is rising in prevalence due to surgical techniques allowing prolonged survival. However there are complications associated with grown up CHD including cardiac failure, complications of surgery, arrhythmias and sudden death.

Method

In a retrospective study, 77/2000 (3.9%) cases died a sudden cardiac death with congenital cardiac lesions.

Results

Simple CHD cases were atrial/ventricular septal defect (ASD/VSD, n=23), aortic stenosis (AS, n=3), Noonan syndrome (n=3) and Williams syndrome (n=2). Complex CHD cases were double outlet right ventricle (n=10), Tetralogy of Fallot (n=6), transposition of the great vessels (TGV, n=4), congenitally corrected TGV (n=2), atrioventricular septal defect (AVSD, n=4), anomalous pulmonary venous drainage (n=2), Ebstein anomaly (n=1) and hypoplastic left heart syndrome (n=1). There were cases of morphologically normal hearts with a functional bicuspid aortic valve (BAV) and no complications (n=3), healed VSD (n=2) and small ASD (n=1). Aortic CHD cases included coarctation of the aorta (n=4), sinus of Valsalva aneurysm (n=1) and aortic dissection with BAV (n=1). Complications of bacterial endocarditis occurred with AS (n=1) and AVSD (n=1). Finally there were cases of sudden death with pulmonary hypertension (n=2) secondary to CHD and ischaemic heart disease with CHD (n=2).

Conclusions

Sudden death can occur in a wide range of complex and simple CHD even with corrective surgery. Patients can also die from complications such as bacterial endocarditis. Aortic dissections can occur linked to bicuspid valve and coarctation. There was a proportion of cases of sudden death with a morphologically normal heart with potentially bystander congenital lesions suggesting the importance of channelopathy in such cases. Finally, pulmonary hypertensive changes are also a cause of sudden death in this population.

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