Aortic root pathology and sudden death in youth: review of anatomical varieties

Post-mortem findings in 6 young people who died suddenly are reported; ages ranged from 9 to 28 years (average 17). All had practised sport, and 2 died during strenuous exercise. Three cases had been under observation for mild aortic stenosis, while no cardiovascular disorders had been suspected in the others. Autopsy studies ruled out coronary atherosclerotic diseases, myocardial disarray or myocarditis as well as any extracardiac cause of death in every case. A stenotic bicuspid aortic valve was found in 2; supravalvar aortic stenosis and incomplete isolation of the left coronary ostium in 1; anomalous origin of the right coronary artery from the left sinus of Valsalva in 1, and dissecting aneurysm of the ascending aorta in 2. These findings show that congenital anomalies of the aortic root (valve, wall, coronary ostia) are at risk for abrupt complications. Early detection of this frequently silent pathology by non-invasive techniques is mandatory to avoid effort and sporting activities and thus prevent sudden death.