Acute aortic dissection and pregnancy: Review and meta-analysis of incidence, presentation, and pathologic substrates

Objectives: Pregnancy has been recognized as a predisposing factor for acute aortic dissection (AAD) although its occurrence is quite rare. Currently, no trial and few prospective studies exist about this catastrophic event. The present review and meta-analysis aims to update information on clinical presentation, potential risk factors, treatment, and outcome of acute dissection during pregnancy and puerperium. Methods: A comprehensive search of three databases was performed to identify all patients reported in articles published from January 1987. A proportional single-arm meta-analysis with random-effects model was used to pool these variables: risk factors, pregnancy/postpartum occurrence, surgical characteristics, and outcomes. Results: A total of 11 reports and 85 patients with pregnancy-related AAD were available for this study. The prevalence of connective tissue disorders was 62%, Marfan syndrome being the most common. Out of 76 patients, 46 (61%) had dissection during pregnancy and 30 (39%) during puerperium; 40% of events occurred in primigravidae and 60% in multigravidae. Type A and type B dissection occurred in 67% vs 33% of patients. Surgery was performed in 73% of cases with a maternal and fetal mortality of 23% and 27%, respectively. Conclusions: Throughout pregnancy, AAD is quite rare but fatal, especially in Marfan and Loeys–Dietz syndromes, while isolated bicuspid aortic valve is not a risk factor. Even in Marfan syndrome, pathogenesis and evolution of the disease are still unclear. Occurrence of dissection also during puerperium indicates the need for continuous counselling and aortic size monitoring in women at-risk.