The following review has been performed to understand The most common connective tissue disorder accounting for thoracic aortic dissections and aneurysms is Marfan syndrome. There are six subtypes of EDS, with subtype IV primarily responsible for vascular complications and arterial rupture. Type IV EDS and Loeys-Dietz syndrome are both caused by an autosomal dominant defect that causes ineffective collagen synthesis, resulting in dissection or aortic aneurysm formation.
Although this study shows that TEVAR can achieve high short-term (1-year) success rates for patients with connective tissue disorders, the lack of long-term data is the weakness of this report. The authors agree that open repair is generally recommended for patients with connective tissue disorder because of concerns with vessel fragility and weakness of the arterial wall. Most authorities recommend open repair to prevent these complications, because a weak arterial wall renders endograft placement a less durable option owing to concerns of proximal or distal aortic degeneration. TEVAR should mainly be used as a bridging treatment for patients who are too high risk for open surgery or those with unstable ruptured thoracic aortic aneurysms or dissections.
Reference link- https://www.jvascsurg.org/article/S0741-5214(20)32045-0/fulltext
