OBJECTIVE: To analyze presentation, management, and outcomes of acute aortic dissections with proximal entry tear in the arch. METHODS: Patients enrolled in the International Registry of Acute Aortic Dissection and entry tear in the arch were classified into 2 groups: arch A (retrograde extension into the ascending aorta with or without antegrade extension) and arch B (only antegrade extension into the descending aorta or further distally). Presentation, management, and in-hospital outcomes of the 2 groups were compared. RESULTS: The arch A (n = 228) and arch B (n = 140) groups were similar concerning the presence of any preoperative complication (68.4% vs 60.0%; P = .115), but the types of complication were different. Arch A presented more commonly with shock, neurologic complications, cardiac tamponade, and grade 3 or 4 aortic valve insufficiency and less frequently with refractory hypertension, visceral ischemia, extension of dissection, and aortic rupture. Management for both groups were open surgery (77.6% vs 18.6%; P < .001), endovascular treatment (3.5% vs 25.0%; P < .001), and medical management (16.2% vs 51.4%; P < .001). Overall in-hospital mortality was similar (16.7% vs 19.3%; P = .574), but mortality tended to be lower in the arch A group after open surgery (15.3% vs 30.8%; P = .090), and higher after endovascular (25.0% vs 14.3%; P = .597) or medical treatment (24.3% vs 13.9%; P = .191), although the differences were not significant. CONCLUSIONS: Acute aortic dissection patients with primary entry tear in the arch are currently managed by a patient-specific approach. In choosing the management type of these patients, it may be advisable to stratify them based on retrograde or only antegrade extension of the dissection.