A Case of an Abdominal Aortic Dissection in a Hemodynamically Stable Marfan Syndrome Patient Presenting without Pain

Abstract EN

Introduction.

Marfan syndrome (MFS) is a rare connective tissue disorder attributed to a defect in the fibrillin-1 gene.

Aortic aneurysms and dissection are common causes of morbidity and mortality in Marfan syndrome.

Case Report.

A 43-year-old female with a history of MFS and a 4.0 cm dilated ascending aorta presented to her cardiologist reporting that since a C-section two years prior, the left side of her abdomen painlessly protruded when standing.

An outpatient CT scan of the abdomen/pelvis noted a 5.5 cm abdominal aortic dissection, and she was directed to the hospital.

Repeat CT scan of the abdomen/pelvis revealed a 5.6 cm dissecting aneurysm of the infrarenal abdominal aorta.

The patient was admitted to the ICU and started on a nitroglycerin drip to maintain systolic blood pressure less than 110 mmHg.

The patient underwent repair of her abdominal aortic dissection via a retroperitoneal approach, and she tolerated the procedure well.

She was started on metoprolol tartrate 12.5 mg BID and aspirin 81 mg postoperatively.

She was safely discharged with follow-up care.

Conclusion.

This case stresses the importance of having a low threshold to obtain imaging in a MFS patient with protruding abdomen, even though the patient may not have pain and be hemodynamically stable.