Outcomes of Thoracic Endovascular Aortic Repair for Thoracic Aortic Disease in Patients with Connective Tissue Disorders: Insights from the Vascular Quality Initiative.

BACKGROUND

Open surgical repair continues to be the standard of care for treating thoracic aortic aneurysms (TAAs) and type B aortic dissection (TBAD) in patients with connective tissue disorders (CTDs). Data on the safety and durability of thoracic endovascular aortic repair (TEVAR) in this patient population remains limited. This study aims to evaluate the perioperative and mid-term outcomes of TEVAR in patients with CTDs.

METHODS

Patients with CTD who underwent TEVAR for TAA or TBAD between 2014 and 2024 were identified in the Vascular Quality Initiative. Patients with ruptured aneurysms, trauma, or those converted to open surgery were excluded. CTD were defined as any documented clinical or genetic history of Marfan syndrome, Loeys-Dietz syndrome, or Ehlers-Danlos syndrome. Patients were stratified and compared by aortic pathology (TAA versus TBAD) and CTD pathology (Marfan syndrome versus Loeys-Dietz/Ehlers-Danlos syndrome). Kaplan-Meier analysis was used to evaluate and compare the mid-term event rates of survival, aortic reintervention, aneurysmal degeneration, and dissection propagation. Aneurysmal degeneration was defined as a ≥5 mm increase in aortic diameter from baseline, whereas dissection propagation included antegrade and/or retrograde dissection.

RESULTS

330 patients (median age 50 years [interquartile range [IQR], 35-61]) with CTD underwent TEVAR during the study period, of which 85% (n = 281) had Marfan syndrome, 13% (n = 42) had Loeys-Dietz syndrome, and 2% (n = 7) had Ehlers-Danlos syndrome. TEVAR was performed for TBAD in 63% (n = 208) of patients and TAA in 37% (n = 122), with 8% (n = 25) of procedures performed in an emergent setting. Median hospital stay was 5 days (IQR, 3-9). Perioperative mortality, spinal cord ischemia (SCI), and stroke each occurred in 2.1% (n = 7) of patients, and reintervention was required in 10.9% (n = 36). Patients undergoing TEVAR for TBAD had a longer hospital stay (6 days vs 5 days, P = 0.017) and higher perioperative mortality (3.4% vs 0.0%, P = 0.039) compared with TAA. Marfan syndrome patients had a significantly lower rate of perioperative mortality (1.4% vs 6.1%, P = 0.036) than those with Loeys-Dietz syndrome or Ehlers-Danlos syndrome. At 18-month follow up, overall survival was 88.1% (95% confidence interval [CI], 84.2%-92.0%), with reintervention occurring in 30.8% (95% CI, 23.9%-37.7%), aneurysmal degeneration in 40.2% (95% CI, 29.8%-50.6%), and dissection propagation in 20.4% (95% CI, 10.8%-30.0%). Mid-term outcomes were similar between TBAD and TAA for survival (P = 0.369), reintervention (P = 0.896), aneurysmal degeneration (P = 0.151), and dissection propagation (P = 0.810). Patients with Marfan syndrome had a higher mid-term survival compared to patients with Loeys-Dietz or Ehlers-Danlos syndrome (91.0% vs 71.6%, P = 0.001), with no difference observed in reintervention (P = 0.190), aneurysmal degeneration (P = 0.496), or dissection propagation (P = 0.445).

CONCLUSION

TEVAR in patients with CTD is associated with acceptable perioperative morbidity and mortality but carries a substantial risk of early and mid-term aortic-related complications and reintervention. TEVAR should be used cautiously in this population and primarily reserved for emergency situations or carefully selected patients.