The Current State of Endovascular Aortic Repair in Heritable Thoracic Aortic Disease

Heritable thoracic aortic disease (HTAD) is associated with the development of aortic aneurysms or dissections. These patients often suffer their initial aortic event at a younger age, compared to those without HTADs. Although genetic aortopathy can affect any segment of the aorta, there are characteristic segments for specific HTADs. This population is at increased risk of aortic-related mortality, and early identification and screening of HTAD has been shown to positively impact long-term survival. The standard of aortic care in this population has historically involved open repair. However, the associated morbidity and mortality of open thoracoabdominal aortic repair led to the expansion of endovascular aortic repair (EVAR) in HTAD, despite concerns of chronic radial force from the aortic endograft and the bridging stents to the renovisceral target vessels. EVAR in HTAD has evolved from thoracic EVAR in a prior open thoracic graft to fenestrated-branched EVAR of the aortic arch and thoracoabdominal aorta. Contemporary data demonstrate high technical success and low perioperative morbidity. The EVICTUS study elucidated midterm outcomes of endovascular repair in genetic aortopathy (n = 171) with similar midterm survival (median follow-up 4.7 years) in patients with Marfan syndrome and Loeys-Dietz syndrome, compared to open repair. Despite over half of patients undergoing secondary interventions to maintain sac exclusion, there was a low rate of open conversion during the study period. Guidelines currently advise endovascular repair in HTAD only in emergent interventions as a bridge to open repair or to avoid open surgery in reoperative fields. Long-term outcomes must be defined and compared to open repair if endovascular repair in HTAD is to ever be recommended in the guidelines. Staged strategies are now preferred for these extensive aortic repairs in HTAD, often combining open and endovascular techniques. Thus, multidisciplinary aortic teams with expertise in both techniques at high-volume centers are essential. Finally, endograft technology and implantation techniques must continue to evolve and to be tailored to this unique population.