ABSTRACT
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.
Subject(s)
Aortic Aneurysm, Abdominal , Aortic Aneurysm, Thoracic , Aortic Dissection , Blood Vessel Prosthesis Implantation , Connective Tissue Diseases , Endovascular Procedures , Aortic Dissection/diagnostic imaging , Aortic Dissection/genetics , Aortic Dissection/surgery , Aortic Aneurysm, Abdominal/surgery , Aortic Aneurysm, Thoracic/diagnostic imaging , Aortic Aneurysm, Thoracic/genetics , Aortic Aneurysm, Thoracic/surgery , Blood Vessel Prosthesis , Connective Tissue Diseases/complications , Humans , Prosthesis Design , Retrospective Studies , Risk Factors , Time Factors , Treatment OutcomeABSTRACT
PURPOSE: To report endovascular repair of postdissection thoracoabdominal aortic aneurysm (TAAA) in 2 patients with vascular Ehlers-Danlos syndrome (vEDS). CASE REPORTS: Case 1. A 56-year-old vEDS male patient with a 50-mm type III TAAA [history of aortic root repair, hemiarch replacement, and thoracic endovascular aortic repair (TEVAR) for acute type A aortic dissection (TAAD) 7 years ago] was treated by a 2-stage procedure; first, cervical debranching of the left subclavian artery and second TEVAR and t-branch. The postoperative course was uneventful. Follow-up computed tomography angiography (CTA) 3.5 years postoperatively demonstrated aortic remodeling with patency of targeted visceral vessels and no endoleak. Case 2. A 47-year-old vEDS male patient presented with a TAAA (diameter of 67 mm). The patient had a history of aortic valve and arch replacement with elephant trunk for acute TAAD, and consequently a TEVAR and candy-plug procedure after a ruptured false lumen (FL) aneurysm of the descending thoracic aorta. He also had a surgical repair by an aorto-bi-iliac graft. Two years later, CTA demonstrated aneurysmal FL dilatation distally to the candy-plug and he was treated with fenestrated EVAR (F-EVAR). CONCLUSION: Endovascular repair of postdissection TAAA was feasible and safe with good short-term outcome in 2 patients with vEDS.
