Introduction. Aortic dissection is the most common acute aortic pathology. Acute ascending aortic dissection is a life-threatening condition and a surgical emergency which requires prompt visualization of the aortic anatomy.Presentation of the case. Concurrent aortic dissection and Marfan syndrome (MFS) followed by the aneurysm of the aorta which required supracoronary aortic replacement (2012), Bentall procedure ( 2014) and urgent open thoracic descending aorta replacement (2017) are described in the case report.Discussion. Patients with the aortic dissection may have certain underlying conditions, such as MFS, which can predispose to dissection. The aortic pathology is registered in almost 65-100% of the patients with MFS. The prevalence of Type A aortic dissection among the patients with MFS at the age of 60 is approximately 50%. The most common pattern of repair is the proximal ascending aortic repair followed by the descending thoracic aneurysm surgery. The open surgery technique, as well as the aortic dissection or aneurysm repairs, may be safely used in the patients with MFS.
Conclusion.Anatomical features of the aorta in the patients with MFS are vitally important when carrying out the analysis of the computed tomography images; in particular, the schedule of the computed tomography follow-up for the patients with MFS should be customized to each case.