Acute Aortic Dissection during Pregnancy: Hideous Clinical Quandaries with Young Lives on the Line—The Role of Hereditary Genetic Syndromes

Objective: Acute aortic dissection is a rare but frequently fatal aortic catastrophe with high morbidity and mortality. Especially in pregnant patients, acute dissection is often misdiagnosed putting two lives on the line. Due to its scarcity, only case reports have been reported. The aim of this study is to analyze the time of aortic dissection during the course of pregnancy and the outcome of emergency surgery in pregnant women with and without hereditary connective tissue disorder. Methods: We retrospectively reviewed all acute aortic dissections (type A and B) who underwent emergency aortic surgery at our institution between 1994 and 2022 and identified 13 patients with acute aortic dissection during pregnancy or directly postpartum. Mann-Whitney U and Fisher's exact tests were used for statistical analysis. Results: Of the 13 included patients, 5 had a genetic syndrome. These patients were significantly younger at the time of dissection and at an earlier stage of pregnancy (second trimester). Even though operative and in-house mortality was zero, we lost one patient on postoperative day 14 due to rupture of the aortic root after transfer to another hospital. Survival of neonates was 77% including two aborted pregnancies. Conclusions: Surgical treatment of acute aortic dissection during pregnancy can be performed with excellent operative mortality for the mothers and satisfying survival of their neonates. In patients with genetic syndrome, dissection occurs during the early second trimester, whereas non-syndromic patients experience acute dissection in the late third trimester. Long-term follow-up is essential for timely re-intervention, if needed.