BACKGROUND: While thoracic endovascular aortic repair (TEVAR) for type B aortic dissection is widely used, its use in cases of type A acute aortic syndrome remains limited. This meta-analysis was conducted to determine the outcomes of TEVAR for retrograde type A intramural hematoma (IMH) and aortic dissection with intimal disruption located in the descending aorta. METHODS: Systematic searches were conducted up to April 2024. Pooled proportions of perioperative and late outcomes were computed. Additionally, standard mean differences (SMD) with 95% confidence intervals (CI) in aortic dimensions following TEVAR were calculated. Kaplan-Meier curves, derived from individual patient data extracted from the studies, were utilized to elucidate long-term all-cause mortality. RESULTS: A systematic review identified 13 non-randomized studies encompassing 288 patients. Pooled proportions revealed short-term mortality at 1%, with incidences of spinal cord injury, stroke, new intimal tear/dissection in the ascending aorta, aortic rupture, necessity for aortic re-intervention, and late mortality each recorded at 1%, 1%, 3%, 1%, 6%, and 7%, respectively. Substantial reductions in the diameters of the ascending aorta, descending aorta, IMH/false lumen of the ascending aorta, and IMH/false lumen of the descending aorta were observed post-TEVAR (SMD [95% CI] = 1.11 [0.82-1.39], 1.02 [0.58-1.47], 3.06 [2.39-3.74], and 2.77 [2.28-3.26], respectively). The anticipated 5-year survival rate stood at 91.1%. CONCLUSION: This meta-analysis suggests TEVAR may offer a viable and safe therapeutic option for retrograde type A acute aortic syndrome. Nevertheless, definitive conclusions are hindered by the scarcity of available data.