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A Case of an Abdominal Aortic Dissection in a Hemodynamically Stable Marfan Syndrome Patient Presenting without Pain
Hussein Al-Mohamad1, Kara Stout2, Taryn Bolling2
1Department of Cardiology, Largo Medical Center, Largo, FL, USA.
Abstract:
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.
Insights
Marfan syndrome (MFS) patients with abdominal protrusion require prompt imaging. Early diagnosis of abdominal aortic dissection in MFS is crucial for timely intervention and improved outcomes.
Area of Science:
- Cardiovascular Medicine
- Genetics
- Vascular Surgery
Background:
- Marfan syndrome (MFS) is a genetic connective tissue disorder.
- Aortic aneurysms and dissection are significant causes of mortality in MFS patients.
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