Middle aortic coarctation

Abstract

Background: Middle aortic coarctation (MAC), a variant of middle aortic syndrome, is a rare entity with only ∼200 cases described in the literature. It classically presents with early onset and refractory hypertension, abdominal angina, and lower extremity claudication. Although endovascular repair has been described for focal stenoses, open bypass remains the standard to restore abdominal inflow and correct renovascular hypertension.

Methods: We describe an unusually late presentation of MAC in a 52-year-old man from El Salvador with refractory hypertension since age 8 requiring 5 antihypertensive medications. He presented with acute chest pain and severe hypertension. He denied mesenteric and peripheral vascular symptoms. Distal pulses were not palpable. Creatinine was 1.9 mg/dL (peak 4.0 mg/dL). Computed tomography angiography demonstrated coarctation of the descending thoracic aorta with extensive collateralization.

Results: After control of blood pressure, the patient's singular anatomy dictated a descending thoracic aorta-infrarenal aortic bypass using a 16-mm woven Hemashield tube graft via a left fifth-interspace anterolateral thoracotomy and left retroperitoneal incision. The graft was tunneled through the posterior left hemidiaphragm. Patient had excellent recovery, with decrease in antihypertensive medications (5 to 2), restoration of all distal pulses, and no neurologic complications. Postoperative creatinine was 0.9 mg/dL.

Conclusions: MACs are rare entities with presentation usually in youth or adolescence, comprising only 0.5-2% of all aortic coarctation cases. Etiologies include congenital, acquired, inflammatory, and infectious causes. If untreated, most patients do not survive past the fourth decade because of the sequelae of renovascular hypertension including myocardial infarction, heart failure, intracranial hemorrhage, and aortic rupture. Depending on technical considerations, open surgical bypass remains the standard repair for MAC.