Surgical management of an acquired aortic interruption suspected from severe aortic coarctation in an adult with massive collaterals

BACKGROUND: Interrupted aortic arch (IAA) is a severe congenital anomaly typically fatal in infancy without surgical intervention. Adult presentation of isolated IAA is exceedingly rare and usually relies on an extensive compensatory collateral vascular network. CASE PRESENTATION: We present the case of a 25-year-old man who presented with malignant hypertension. Computed tomography angiography with 3D reconstruction revealed complete occlusion of the aortic arch distal to the left subclavian artery, accompanied by a massive collateral circulation derived from dilated intercostal and internal mammary arteries. Open surgical repair was performed via a left posterolateral thoracotomy. Intraoperative findings identified an atretic fibrous cord at the occlusion site, suggesting an extreme progression of severe coarctation of the aorta into an acquired interruption. An 18-mm Dacron tube graft was successfully interposed between the arch and descending aorta. Crucially, this was performed as an off-pump extra-anatomic bypass without cardiopulmonary bypass. The postoperative course was uneventful. At 4 months, his blood pressure was significantly improved, though a small residual gradient persisted. CONCLUSION: This case suggests that severe coarctation can progress to an acquired atresia mimicking IAA. Off-pump extra-anatomic bypass is a safe and effective strategy expected to alleviate refractory hypertension and prevent long-term cardiovascular and cerebrovascular complications in heavily collateralized adults.