Aortic Dissection in Hospitalized Children and Young Adults: A Multiinstitutional Study

Objective.To describe the incidence, characteristics, and outcomes of hospitalized children and young adults with aortic dissection (AD). Design.Retrospective review. Setting.The Pediatric Health Information System database, a multiinstitutional administrative database. Patients.All index cases of patients <30 years old hospitalized with AD between January 2004 and June 2011. Outcome Measures.The primary outcome measure was inpatient mortality. Results.Of 3800964 hospitalizations, AD was identified in 124 (<1%), accounting for 110 patients (69% male, P = .003) at a median age of 12.9 (interquartile range 3.9-16.8) years with a bimodal distribution in infancy and late adolescence. Associated diagnoses included congenital heart disease (CHD) (38%), trauma (24%), connective tissue disease (CTD) (16%), and isolated hypertension (HTN) (8%). Common CHD diagnoses included aortic arch (24%) and valve (21%) disease, hypoplastic left heart syndrome (10%), and transposition of the great arteries (10%). CHD patients were younger and more likely to undergo inpatient non-AD-related cardiovascular procedures compared with other diagnostic groups (P < .001 for both). Marfan and Ehlers-Danlos syndrome were present in 72% and 11% of CTD patients, respectively. Overall in-hospital mortality in patients with AD was 13% compared with 1% in the database population (odds ratio 12.0, 95% confidence interval 6.9-21.1). By diagnostic category, mortality was 22% in HTN, 22% in CTD, 12% in CHD, and 4% in trauma. Conclusions.AD is rare in children and young adults but most commonly occurs in CHD and CTD and in males. AD is associated with high inpatient mortality.