ImmunoglobulinG4 (IgG4)-related sclerosing disease can occur in various organs, rarely in cardiovascular lesions. We report a case of IgG4-related cardiac tumor which was concomitant with aortic dissection. A 72-year-old woman visited our hospital with chief complaints of difficulty swallowing and weight loss. A tumor was found in the pericardium adjacent to the left ventricle on echocardiography, and a dissecting aneurysm of the ascending aorta with a maximum of 60 mm in the short diameter was noted on contrast CT. There had been no episodes related to the onset of aortic dissection such as chest pain, and chronic dissection was also identified on diagnostic imaging. A tumor biopsy was performed via a left lateral thoracotomy for tumor tissue diagnosis. Histopathologically, the tumor showed no malignant findings and we identified infiltration of IgG4-positive plasmacytes. The IgG4 level in blood exceeded the reference level, but no findings of IgG4-related disease were observed in other organs. Coronary artery CT showed the left circumflex branch of the coronary artery to run over the tumor. It was thus judged to be difficult to surgically resect the tumor. Oral steroid administration was started to reduce the size of the tumor. However, due to an enlarging trend of the aortic aneurysm involving the ascending aorta on CT at 1 month, the patient underwent ascending aorta replacement. Although infiltration of IgG4-positive plasmacytes was found in the outer lining of the resected arterial wall, the association between IgG4 and the onset of aortic dissection was unclear.

Patients with an aortic root pseudoaneurysm communicating to the right atrium are rare. A 67-year-old woman underwent ascending aorta and total aortic arch replacement for acute type A aortic dissection at our institute 9 years prior to the current presentation. She was transported to our emergency department with complaints of chest pain, palpitations, and cold sensation. A continuous murmur was heard at the right sternal margin. Contrast-enhanced computed tomography (CT) and ultrasonic cardiography showed a huge pseudoaneurysm at the proximal anastomotic site and an aorto-right atrial fistula. Ascending aortic replacement with concomitant direct closure of the fistula was successfully performed. The patient was discharged in good condition on the 14th postoperative day. Careful follow-up with CT is important after acute type A aortic dissection repair.

We report a 48-year-old man who underwent hybrid aortic repair for visceral aortic patch (VAP) aneurysm. He had undergone descending thoracic aortic repair for post-dissection aneurysm at the age of 25, ascending aorta and proximal aortic arch aneurysm repair at the age of 27, and residual thoracoabdominal dissecting aortic aneurysm repair with VAP reconstruction at the age of 28. During 20 years of follow-up, the VAP gradually enlarged and eventually reached 70×61 mm in diameter. Considering a possible severe adhesion after 2 previous left thoracotomies, we planned a 2-staged hybrid aortic repair. First, we performed reno-visceral debranching and as a second stage operation, endovascular aortic repair was performed successfully 39 days after the first-stage operation.