Acute type A aortic dissection in adolescents and young adults under 30 years of age: demographics, aetiology and postoperative outcomes of 139 cases

OBJECTIVES The prevalence and aetiology of acute aortic dissection type A (AADA) in patients = 30 years is unknown. The aims of this clinical study were to determine the prevalence and potential aetiology of AADA in surgically treated patients <= 30 years and to evaluate the respective postoperative outcomes in this selective group of patients in a large multicentre study.METHODS Retrospective data collection was performed at 16 participating international aortic institutions. All patients = 30 years at the time of dissection onset were included. The postoperative results were analysed with regard to connective tissue disease (CTD).RESULTS The overall prevalence of AADA = 30 years was 1.8% (139 out of 7914 patients), including 51 (36.7%) patients who were retrospectively diagnosed with CTD. Cumulative postoperative mortality was 8.6%, 2.2% and 1.4%. Actuarial survival was 80% at 10 years postoperatively. Non-CTD patients (n = 88) had a significantly higher incidence of arterial hypertension (46.6% vs 9.8%; P < 0.001) while AADA affected the aortic root (P < 0.001) and arch (P = 0.029) significantly more often in the CTD group. A positive family history of aortic disease was present in 9.4% of the study cohort (n = 13).CONCLUSIONS The prevalence of AADA in surgically treated patients = 30 years is <2% with CTD and arterial hypertension as the 2 most prevalent triggers of AADA. Open surgery may be performed with good early results and excellent mid- to long-term outcomes.