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Article in Japanese | WPRIM | ID: wpr-886208


It is well known that Marfan syndrome is a genetic disorder characterized by congenital abnormality of connective tissue, in which the associated prognosis is affected by aortic lesions, and in particular the onset of aortic dissection, with some reports of familial aortic dissection. Meanwhile, it has been reported that in the absence of genetic disorder, aortic dissection occurring in two or more individuals within a family is rare. We experienced a mother-son case of non-Marfan syndrome that developed aortic dissection on the same day, and thus report our findings. Case 1 (son) was a 32-year-old man with no particular medical history observed. He had experienced epigastric pain in the early morning and come to the emergency room in our hospital, where contrast-enhanced computed tomography (CT) led to diagnosis of acute aortic dissection (Stanford type B). He was admitted to the intensive care unit, and received treatment by anti-hypertensive therapy. Case 2 (mother) was a 61-year-old woman who was prescribed internal medicines for hypertension. She had been informed that her son was hospitalized for aortic dissection, and had visited the hospital to see him. While visiting her son in the hospital room, she developed sudden chest pain and remarkable cold sweats, and upon undergoing contrast-enhanced CT, she was diagnosed with acute aortic dissection (Stanford type A). Then emergency hemiarch replacement was performed. In the family tree, there were some cases of cerebrovascular disease found on the mother's side. Pathology findings of the aorta for the mother revealed cystic medial necrosis and genetic screening subsequently performed revealed TGFBR2 mutation. Both subjects exhibited good progress, with the son discharged from hospital after three weeks, and the mother discharged 14 days after surgery.

Article in Japanese | WPRIM | ID: wpr-758250


This patient is a 72-year-old-man who had undergone aortic valve replacement using a Starr-Edwards Ball Valve to treat aortic valve stenosis when he was 28 years old. In April 2015, he was admitted with cardiac failure of NYHA III. Echocardiography showed a remarkable increase of aortic valve pressure gradient and progressive change in mitral valve stenosis and tricuspid valve regurgitation. The Starr-Edwards Ball Valve was replaced using a CEP MAGNA EASE prosthesis, the mitral valve was replaced using a CEP MAGNA MITRAL EASE prosthesis with tricuspid annuloplasty using the MC3 ring. Cloth wear of the Starr-Edwards Ball Valve cage and all-round pannus formation under the valve seat was found at the operation, and the cause of the higher pressure gradient may have been the pannus. The postoperative period of this case following the initial aortic valve implantation of the Starr-Edwards Ball Valve is the longest known in Japan as far as we could discover.

Article in Japanese | WPRIM | ID: wpr-378648


<p>A 79-year-old woman complained of chest pain during hemodialysis. At 65 years of age, she underwent total arch replacement for acute aortic dissection. The following year, she underwent thoracic endovascular aortic repair and Y-graft replacement of a thoracoabdominal aortic aneurysm. Since the age of 73 years, she has been on hemodialysis for chronic renal failure. At 77 years of age, she underwent a modified Bentall procedure and mitral valve annuloplasty for a huge pseudoaneurysm at the aortic root and for mitral regurgitation. One year after the surgery, computed tomography (CT) showed a pseudoaneurysm around the aortic root. Although asymptomatic at the time of her follow-up visit, she developed chest pain during hemodialysis 20 months following the Bentall procedure. CT revealed progressive dilatation of the aortic root pseudoaneurysm. Coronary angiography showed severe stenosis of the right coronary artery. During the redo operation, findings indicated the presence of oozing type of bleeding from the needle hole from the previous artificial graft. We experienced a rare case of a huge aortic root pseudoaneurysm due to oozing from the needle hole at the artificial graft.</p>