Complex and Successful Management of a Symptomatic Isolated Abdominal Aortic Aneurysm in a Pregnant Woman with Marfan Syndrome

Abstract

Aortopathies associated to Marfan syndrome (MFS) are important causes of maternal death during pregnancy. We present a 27-year-old and 24-week pregnant MFS woman who arrived to the emergency department with increasing abdominal pain; an obstetric ultrasound showed an Abdominal Aortic Aneurysm (AAA), a multislice computed tomography angiography (CTA) confirmed and demonstrated a 7.3 centimeter (cm) infrarenal AAA without evidence of dissection. A multidisciplinary committee determined that an open repair would lead to a significantly high maternal-fetal morbidity and mortality. Although endovascular repair (EVAR) in MFS patients remains controversial, an urgent bridge therapy was considered to be the best option. She was transferred to the angiography suite for EVAR to prevent AAA rupture and ensure a satisfactory pregnancy. The patient and fetus presented no complications during the procedure and were discharged 3 days later. She continued her pregnancy without eventualities and an elective C-section was performed on week 36. A CTA imaging at 12 months revealed type 1A and 3 endoleaks, we decided to perform endograft explant and a definitive open repair, there were no complications during the procedure, the patient is currently asymptomatic. Our case illustrates a complex decision and management that successfully avoid morbidity and mortality of a MFS mother and her product; additionally, this experience reinforces the need for lifelong and close surveillance in these patients.

Evidence on the need for early identification of asymptomatic true abdominal aortic aneurysm in pregnancy: A case report

“…A list of previously published relevant case reports is presented in Table 1. [4][5][6][7][8][9][10] Understanding the risk factors of abdominal aortic dilatation and making predictive diagnosis is the key point. The risk factors of AAA include age (over 40), male sex, smoking, family history of AAA, presence of other cardiovascular disease, hypertension, hyperlipemia, and inherited disorders.…”