Down's syndrome and myocardial reperfusion injury

Down syndrome is known to be an independent risk factor for mortality after surgical repair of congenital heart anomalies. It is also associated with neurodegenerative disease and accelerated aging. The mechanism of the latter features has been attributed to abnormal handling of oxygen-free radicals as well as mitochondrial dysfunction. These properties also place the child with Down syndrome at a risk of an exaggerated myocardial ischemia/reperfusion injury. A 6 month old child with Down syndrome is reported who suffered from obvious clinical ischemia/reperfusion injury following an uncomplicated repair of complete AV canal. Both intraoperative as well as postoperative echocardiography documented a satisfactory technical repair. After resting the heart on ECMO the child's myocardial function returned to normal. The mechanisms by which patients with Down syndrome are at risk of ischemia/reperfusion injury are reviewed. Future studies should focus on specific approaches for myocardial protection in the child with Down syndrome undergoing cardiac surgery.