# Successful treatment for distal-arch aortic aneurysm in a cold agglutinin-positive patient via physician-modified thoracic endovascular aortic repair: a case report

## TL;DR

A 76-year-old man with cold agglutinin disease successfully underwent a modified aortic repair procedure instead of open heart surgery.

## Contribution

This is the first reported use of physician-modified TEVAR in a patient with cold agglutinin disease.

## Key findings

- Physician-modified TEVAR was safely performed without complications in a cold agglutinin-positive patient.
- The procedure avoided risks associated with hypothermic cardiopulmonary surgery in cold agglutinin disease.
- Further research is needed to determine the broader applicability of this approach in CAD patients.

## Abstract

Cold agglutinin disease (CAD) is sometimes incidentally detected before cardiovascular surgery. Several methods to prevent complications associated with CAD after cardiac surgery have been reported, but there are no reports of the use of physician-modified TEVAR to date.

A 76-year-old man with an arch aortic saccular aneurysm was scheduled to undergo arch aortic replacement. However, cold agglutinin syndrome was incidentally detected before open heart surgery. The safety of cardiopulmonary surgery under hypothermia for patients with cold agglutinin disease is unknown, as intravascular hemolysis is a source of concern for patients sensitive to cold stimulation. Instead, we performed physician-modified thoracic endovascular aortic repair (3 fenestrations and 1 branch), as the aneurysm in this case was suitable for thoracic endovascular aortic repair (TEVAR). As a result, the patient recovered well without any complication.

The long-term prognosis of physician-modified thoracic endovascular aortic repair remains unclear, and its use is limited to high-risk patients who require open chest surgery. Also, the impact of cold agglutination on stent grafts in CAD patients has not been reported. Despite that situation, this case illustrated that physician-modified TEVAR can be safely performed without significant postoperative complications, such as coagulation-fibrinolytic abnormalities or embolic events. Further studies are needed to establish the indications for this procedure in CAD patients.