Multidisciplinary Management of Heritable Aortopathy in Pregnancy Complicated by Postpartum Acute Type A Dissection

A 33-year-old patient at 20 weeks of gestation presented with a dilated aortic root and abdominal aorta, and a diagnosis of Loeys-Dietz syndrome was made.

What is the incidence of pregnancy-related aortic dissection in patients with heritable thoracic aortic disease (HTAD)? What are the key management principles for pregnant patients with HTAD?

A shared plan was made by a multidisciplinary team of maternal fetal medicine, cardio-obstetrics, and cardiothoracic surgery, consisting of imaging surveillance, blood pressure control, scheduled cesarean delivery, and future elective aortic surgery. After an uncomplicated delivery, the patient experienced an acute type A dissection requiring staged total aortic replacement, including aortic root and arch replacement, thoracoabdominal aortic replacement, and endovascular aortic repair.

Patients with HTAD are at an increased risk of pregnancy-related aortic dissection. Key management principles include multidisciplinary evaluation, imaging surveillance, blood pressure control, and delivery with cardiothoracic surgical backup.