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Summary: Current concepts in the pathophysiology and predisposing conditions of acute aortic dissection in children, adolescents, and young adults are presented. Timely diagnosis is required for this life-threatening condition. Most children and adolescents with aortic dissection have congenital cardiovascular anomalies. Certain heritable disorders involving connective tissue also predispose to this disorder. Newer associations include cocaine abuse and weight lifting. To facilitate early diagnosis, the salient physical findings of the known predisposing conditions are reviewed. Clinical presentation and diagnostic imaging of aortic dissection are briefly summarized. Physicians working in an acute care setting, particularly in the emergency room, should be aware of disorders predisposing to acute aortic dissection in the pediatric and young adult population. Practitioners conducting school or college preparticipation sports evaluations can make use of such information in their assessment of risk for sudden death. Clin Pediatr. 2000;39:71-80 [ABSTRACT FROM AUTHOR]