Postpartum Aortic Dissection Without Aortic Dilation in a Woman With an ACTA2 Gene Mutation

Pregnancy and the postpartum period are recognized risk factors for aortic dissection in young women. Mutations in the actin alpha 2, smooth muscle (ACTA2) gene also predispose individuals to aortic dissection. We report a case of postpartum aortic dissection without aortic dilatation, in which a pathogenic ACTA2 mutation was identified.

A 36-year-old woman developed sudden-onset back pain 8 days after an uncomplicated vaginal delivery. Computed tomography revealed Stanford type B aortic dissection. She had no history of hypertension, connective tissue disease, or family history of aortopathy. Genetic testing demonstrated a pathogenic ACTA2 mutation. She was managed conservatively with blood pressure control and serial imaging follow-up.

Women with ACTA2 mutations are at high risk for aortic dissection through the postpartum period. Dissection may occur even with a normal aortic diameter; therefore, risk stratification based solely on aortic size is insufficient.

Genetic testing is essential in young women with unexplained aortic dissection.