Pseudoaneurysm with a fistula to the right ventricle late after surgical repair of type A aortic dissection in a patient with systemic lupus erythematosus

Abstract

Background: Pseudoaneurysm with a shunt to the right ventricle after aortic repair for acute aortic dissection is an extremely rare and life-threatening condition. Surgical treatment is unavoidable, but surgery is complicated, and there are some pitfalls. This study describes the reoperation performed in a patient at a high surgical risk by clarifying the shunt site using multimodality imaging before surgery.

Case presentation: A 69-year-old woman with a history of systemic lupus erythematosus (SLE) and Sjogren's syndrome presented with a pseudoaneurysm 1 year after emergency surgery for acute type A aortic dissection. Eight years after the first surgery, she experienced sudden chest pain and presented to the emergency department. Her dyspnea worsened; therefore, echocardiography and three-dimensional computed tomography (3DCT) were performed, and a pseudoaneurysm and shunt to the right ventricle were identified. The medical team attempted to close the shunt with a percutaneous catheter but was unsuccessful, and she was referred to our department for surgical treatment. The pseudoaneurysm originating from the proximal side of the aorta was large (diameter = 55 mm), and echocardiography-gated 3DCT identified the shunt from the pseudoaneurysm to the right ventricle. First, extracorporeal circulation was initiated, and resternotomy was performed. We could not insert the left ventricular venting tube from the right side because of the pseudoaneurysm size. Instead, the tube was inserted from the left atrial appendage. We found a half-circumferential disengaged anastomosis around the proximal anastomosis, which formed the large pseudoaneurysm leading to a fistula in the right ventricle. We closed the fistula and performed a Bentall operation. The patient had a good postoperative course and was discharged on postoperative day 21. She continued treatment for SLE and Sjogren's syndrome, and her inflammatory reaction improved.

Conclusions: We performed a Bentall operation and fistula closure with resternotomy in a patient with type A aortic dissection with SLE and Sjogren's syndrome. Multimodal imaging is essential in defining the pseudoaneurysm and the fistula surrounding the anatomy while ensuring their resolution and guiding the approach for operation.

Keywords: Aortic dissection; Echocardiography-gated three-dimensional computed tomography; Fistula; Pseudoaneurysm; Sjogren’s syndrome; Systemic lupus erythematosus.

Fig. 1

Preoperative chest X-rays. A cardiothoracic ratio of 60% is noted

Fig. 2

Preoperative 3DCT images. a Preoperative 3DCT showing a true saccular aneurysm on the lesser curvature side (black arrow) and a proximal anastomotic pseudoaneurysm (red arrow). b The pseudoaneurysm (white arrow) is in close contact with the sternum. c ECG-gated 3DCT is used to visualize the pseudoaneurysm (red arrow) and the previously used artificial graft (“prior graft”). The pseudoaneurysm is not in direct contact with the right coronary artery, with a small distance between them. d The pseudoaneurysm (red arrow) and shunt (yellow arrow) are near the right heart structures. e The Valsalva without the aortic dissection (yellow arrow) is shown with the pseudoaneurysm (red arrow). 3DCT, three-dimensional computed tomography; ECG, echocardiography; SVC, superior vena cava; rt upper PV, right upper pulmonary vein; RA; right atrium. RV; right ventricle; RCA, right coronary artery

Fig. 3

Transesophageal cardiac ultrasonography showing the pseudoaneurysm (red arrow). The shunt (yellow arrow) is indicated. RA, right atrium; RV, right ventricle; LA, left atrium

Fig. 4

Operative findings. a Intraoperative view showing the pseudoaneurysm (black arrow). At the ST junction, the artificial graft and aortic wall are separated by half of the posterior wall. b Intraoperative view showing the pseudoaneurysm’s (black arrow) proximity to the shunt (yellow arrow). RA, right atrium; LA, left atrium

Fig. 5

Postoperative ECG-gated 3DCT showing no pseudoaneurysm and a normal right coronary artery. ECG, echocardiography; 3DCT, three-dimensional computed tomography