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Middle Aortic Syndrome Secondary To Takayasu Arteritis
Erin E. McIntosh, MD, John Westley Ohman, MD.
Washington University in St. Louis, Saint Louis, MO, USA.
DEMOGRAPHICS: The patient is an 18-year-old female with a history of middle aortic syndrome (MAS) secondary to Takayasu arteritis (TAK), resulting in refractory hypertension and chronic mesenteric ischemia.
HISTORY: This patient initially presented at the age of 11 years with a history of bilateral lower extremity claudication, malaise, abdominal pain, chest pain, weight loss, and multisystem organ failure, including renal failure with need for dialysis temporarily. During that initial hospitalization, she was diagnosed with Takayasu arteritis with middle aortic syndrome. She underwent aortogram, which showed a diffusely hypoplastic thoracoabdominal aorta (4mm in smallest diameter with a gradient of 65mmHg), and subsequent angioplasty and stenting at that time. Medically, she was managed with Remicade and prednisone for TAK and multiple antihypertension agents. However, over the next 5 years, she developed recurrent symptoms and narrowing of her thoracic and infrarenal aorta. Repeat endovascular interventions were performed, including balloon angioplasty and stenting of her thoracic and infrarenal aorta, and right renal balloon angioplasty. Despite these efforts, the continued to have symptoms, including worsening renal function, uncontrolled hypertension, chronic mesenteric ischemia, and lifestyle limiting claudication.
PLAN: Given the ultimately unsuccessful attempts at endovascular treatment in this patient, she was planned for open surgical repair. She underwent sternotomy, exploratory laparotomy, ascending aorta to bilateral common iliac artery bypass, SMA, and bilateral renal artery bypass. The graft was tunneled from the chest to the abdomen in an antepancreatic fashion, due to the significant inflammation in the retroperitoneum, limiting mobilization posterior to the pancreas. Post-operatively, her creatinine improved, as did her hypertension, symptoms of chronic mesenteric ischemia, and claudication.
DISCUSSION: Takayasu arteritis is an immune-mediated large vessel vasculitis, primarily affecting the aorta and its major branches. Middle aortic syndrome is a narrowing of the distal descending and abdominal aorta that can be congenital or acquired, such as secondary to TAK. While there are some reports in the literature of successful endovascular treatment over the long-term, open surgical treatment remains the gold standard for MAS and was ultimately required for the treatment of this patient.
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