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A man diagnosed with immunoglobulin G4 (IgG4)-related disease at the age of 65 years underwent abdominal aortic replacement due to an abdominal aortic aneurysm. In the same hospitalization period, a small coronary artery aneurysm was noticed. He was treated with corticosteroids and his serum IgG levels returned to normal. After experiencing sudden chest pain at age 74 years, coronary angiography showed that the size of the aneurysm had increased dramatically. He underwent coronary artery bypass graft and coronary artery resection without using cardiopulmonary bypass. Thus, we conclude that observation of aneurysms in patients with IgG4-related disease is important, even under corticosteroid therapy.

Background Aortic dilatation may occur in some patients even after complete repair of tetralogy of Fallot (TOF). The progression rate of the aortic diameter is so slow, and the incidence of aortic dissection is so low that it is suspected that frequent imaging of the aorta may not be necessary. Case presentation We describe an asymptomatic 41-year-old man with hypertension in whom aortic dilatation was accidentally discovered 39 years after TOF repair. He underwent ambulatory follow-up without any difficulty for 21 years after the repair. Contrast-enhanced computed tomography revealed significant aortic dilatation (maximum diameter of 88 mm at the sinus of Valsalva), and echocardiography revealed severe aortic regurgitation, which seemed to progress during the last 18 years without any evaluation or follow-up. The Bentall procedure was successfully performed using a valved graft, under deep hypothermic circulatory arrest with antegrade cerebral perfusion, and his postoperative course was uneventful. Histopathological examination of ascending aorta specimens revealed severe cystic medial degeneration. Conclusions Keeping in mind that a patient with rapid progression of the aortic dilatation after TOF repair exist, periodic follow-up for evaluation of the aorta is essential in patients with TOF.

We report a case of a 55-year-old male who had been diagnosed with mitral regurgitation and atrial septal defect 5 years earlier. He was referred to our institution because of worsening of mitral regurgitation accompanied by exertional dyspnea. As an echocardiography showed atrioventricular valve regurgitation and ostium primum atrial septal defect, but without ventricular septal defect, he was diagnosed as having partial atrioventricular septal defect (pAVSD). An operation was performed through median sternotomy. The anterior atrioventricular leaflet had a cleft and thickening with calcification. Suturing the cleft could not control the regurgitation. Incomplete coaptation was seen at the edge of the anastomosis site of the cleft, where the severe calcification had been identified. A rough zone including a part of the chordae tendineae was sutured in order to compensate for the gap. The atrioventricular septal defect was closed with an autologous pericardial patch. He was discharged uneventfully on the 24th postoperative day and has been followed up without complications for 1.5 years.