Branched stent-graft used for type IV thoracoabdominal aneurysm in abdominal aortic agenesis

Thrombophilia suspected

• Weight : 40kg
• Height : 140cm (<165cm)
• GFR : 74,6 ml/min

Custom-made triple-branched endovascular graft (Cook Medical) deployed from a trans-apical access

Angiogram perioperative

Trans-apical access by anterior thoracotomy

• Transoesophageal echocardiogram control
• Hybrid suite with fusion imaging capabilities
• Heparinization systemic

Stenting of the target vessels by right axillary artery

• 8Fr introducer
• Covered stents (COVERA­™ BD)

• RRA: 7mm diameter
• CT: 9mm diameter
• SMA: 8mm diameter

ACT : 238-240 seconds

Post operative period

Total thrombosis of the stent-graft and mesenteric portal veinous system

• Prosthetic bypass between ascending aorta and right hepatic, SMA and right renal arteries

Multiple complications

• D+2 Haemostasis for haemorrhage, right colectomy and cholecystectomy
• D+4 Making of an ileocolostomy, 60cm resection of necrotic bowel, evacuation of hematoma of the mesentery
• Persistant thrombocytopenia (Platelets 7 g/l) of multi-factorial origin (peripheral consumption on multiple thromboses, splenomegaly on portal thrombosis, inflammation, spinal stupor)
• Kidney failure KDIGO 3 dialysis
• Grade E pancreatitis
• Digestive bleeding (peristomial, gastric ulcers)
• D+12 MOF and died

Discussion

Agenesis of the abdominal aorta and iliac arteries is an extremely rare congenital vascular anomaly, associated with various long-term morbidities.

To treat thoracoabdominal aortic aneurysm, open procedures are more complex and associated with high mortality and morbidity rates

In the presented case, the patient was evaluated in a multidisciplinary team. High risk of pelvic necrosis under ECC and under-recognized or misdiagnosed hemophilia contraindicated open repair. An endovascular repair was considered to be the optimal solution.

The absence of an adequate femoral or iliac access to deliver a branched or fenestrated endograft is usually considered an absolute contraindication for endovascular repair.

Alternative routes and dedicated stent-graft designs have been advocated.

1. Transapical and axillary access to deploy standard straight thoracic stent-grafts has been previously demonstrated in patients with aorto-iliac occlusion
   ^{Malik, K., Poletto, G., Zhao, Y., & Civilini, E. (2019). Unconventional Endovascular Access for Symptomatic Thoracic Aortic Ulcer with Infrarenal Aortic Occlusion—A Case Report. Annals of Vascular Surgery.}
2. Only one other case use of a custom-made triple- branched endovascular graft deployed from a transaxillary access to treat a aneurysm associated with pararenal aortic occlusion

• Postoperative course was uneventful
  ^{Bertoglio, L., Lopes, A. C., Rinaldi, E., Bossi, M., Berchiolli, R. N., Ferrari, M., & Chiesa, R. (2022). Transaxillary Tri-Branch Aortic Endovascular Graft Repair of Recurrent Thoracoabdominal Aneurysm With Pararenal Aortic Occlusion. Journal of Endovascular Therapy}

Conclusion

The use of this custom-made tri-branched device, combined with the transapical access, allowed to treat this aneurysm through a less invasive approach, despite the unfortunate outcome.

Transapical and axillary access have allowed to extend the number of eligible patients to endovascular approach.

Further validation and longer follow-up times would be required to prove the effective durability of this visceral aortic stump

• Post operative anticoagulation management more aggressive?

References

• Bertoglio, L., Lopes, A. C., Rinaldi, E., Bossi, M., Berchiolli, R. N., Ferrari, M., & Chiesa, R. (2022). Transaxillary Tri-Branch Aortic Endovascular Graft Repair of Recurrent Thoracoabdominal Aneurysm With Pararenal Aortic Occlusion. Journal of Endovascular Therapy. 152660282211026.
• Helmy, A., Catarino, P., Dunning, J. H., Hayes, P. D., Goon, S., & Winterbottom, A. (2018). Branched Thoraco-Abdominal Aortic Aneurysm Repair with Branch Access Through a Transapical Left Ventricular Approach. CardioVascular and Interventional Radiology.
• Malik, K., Poletto, G., Zhao, Y., & Civilini, E. (2019). Unconventional Endovascular Access for Symptomatic Thoracic Aortic Ulcer with Infrarenal Aortic Occlusion—A Case Report. Annals of Vascular Surgery.