A Gravid Case of Type B Aortic Dissection in a Young Marfanís Patient
Lauren Grimsley, David J Minion
University of Kentucky, Lexington, KY
INTRODUCTION: We present a case with longitudinal follow up of a young patient with Marfan syndrome complicated by Type B aortic dissection and pregnancy.
METHODS: Patient was a female in her mid-twenties with Marfan syndrome and an aortic root of 42 mm. As she desired pregnancy, she underwent early valve sparing aortic root replacement with re-implantation of her coronary arteries. Despite this, she developed a Type B dissection involving the origin of her left subclavian artery approximately 3 years later. She was initially managed medically, but her aortic diameter enlarged from 3.7 cm to 4.8 cm in one monthís time. Therefore, open arch replacement was performed with debranching of her bovine innominate and left carotid arteries in anticipation of TEVAR. She became pregnant shortly thereafter and was noted to have further degeneration of her aorta to 5.3 cm. After consultation with a high risk obstetrician, surgery proceeded. TEVAR with left subclavian retro-sandwich was performed using the Dacron arch graft for the proximal landing zone. She ultimately delivered a healthy baby. Post-delivery scan showed thrombosis of her false lumen in the thoracic aorta with slight regression in diameter. At two years follow up, her thoracic aorta continued to regress, but her paravisceral aorta had enlarged to 5.3 cm from distal entry tears. Further, she was desiring additional children. Therefore, open replacement of her paravisceral and infrarenal aorta was performed via a thoracoabdominal approach. For this stage, the TEVAR stent was used as the proximal neck for the multi-branch Dacron graft.
RESULTS: The patient recovered uneventfully with full neurologic function.
CONCLUSIONS: Marfan syndrome is a challenging aortopathy that often requires multiple interventions throughout a patientís lifetime. While endovascular repair is not considered the optimal method of primary aortic treatment, it can play a role as part of a hybrid approach. While open grafts can serve as the proximal (or distal) neck for endovascular repair, endovascular grafts can similarly serve as the proximal (or distal) neck for open repair.
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