Endovascular treatment of aortic aneurysms and dissections in patients with genetically triggered aortic diseases

Connective tissue disease (CTD) syndromes involve the ascending, aortic arch, and thoracoabdominal aorta and are associated with higher risk of aortic aneurysm or dissection. Currently, vascular societies generally recommend open repair as the first option for aortic disease in patients with CTD. However, the implementation of endovascular techniques for patients with CTD with aortic pathologies seems to have increased in recent years, mainly in patients of high surgical risk or in urgent situations. Endovascular treatment of aortic arch pathologies in patients with CTD have been feasible in experienced centers; however, the evidence is scarce. Thoracic endovascular aneurysm repair in patients with CTD is more evident; in 15 studies, 304 patients with CTD were treated with thoracic endovascular aneurysm repair with high technical success rates (88% to 100%) and a low early mortality rate (1.6%). During the median follow-up, 33 patients died and 64 patients underwent a re-intervention. In 6 studies, 26 patients with CTD were treated with fenestrated/branched endovascular aneurysm repair for thoracoabdominal aortic aneurysm, with a technical success rate of 100%, without early mortality and morbidity. The endovascular approach to thoracoabdominal aortic aneurysm, especially in post-dissection patients, mandates adjunctive techniques to achieve false lumen thrombosis with various approaches; in our experience, the Candy-Plug technique has been proven to be technically feasible with good outcomes. Endovascular treatment of aortic pathologies in patients with CTD seems to be feasible and safe in high-risk and urgent patients. Re-intervention remains an issue. The constant development of endovascular techniques and devices may provide improved mortality and morbidity outcomes.