A Mother-Son Case of Non-Marfan Syndrome with Acute Aortic Dissection on the Same Day / 日本心臓血管外科学会雑誌

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.

A Case of Aortic Valve Re-replacement for Starr-Edwards Ball Valve 45 Years after Implantation / 日本心臓血管外科学会雑誌

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.

Graft Replacement with Partial Extra-corporeal Circulation of Descending Thoracic and Abdominal Aortic Aneurysms in Marfan Syndrome Combined with Severe Left Ventricular Dysfunction and Mitral Regurgitation / 日本心臓血管外科学会雑誌

We report on a rare case of Marfan syndrome with giant dissecting descending thoracic and abdominal aortic aneurysms associated with poor left ventricular function and severe mitral regurgitation. Before the anesthetic induction, a partial extra-corporeal circulation was established to prevent a collapse of the circulation. Descending aortic graft replacement and following abdominal aortic graft replacement were performed safely using the partial extra-corporeal circulation to relief the afterload for the severely deteriorated left ventricle with severe mitral regurgitation. Intra-aortic balloon pumping was also promptly used to assist the poor circulation in the postoperative period. Despite the admission to a specialized institute, he died from irreversible heart failure with a developing renal failure. Even for a difficult patient with Marfan syndrome with severe left ventricular dysfunction and mitral regurgitation, graft replacement was feasible with meticulous perioperative circulatory management using partial extra-corporeal circulation and intra-aortic balloon pumping. However, a prompt registration for heart transplantation and an aortic surgery concomitant with implantation of left ventricular assisted device should have been considered to save the patient.

A Case of Coronary Artery Bypass Grafting in a Patient with Wolff-Parkinson-White Syndrome / 日本心臓血管外科学会雑誌

A 54-year-old man with unstable angina and Wolff-Parkinson-White (WPW) syndrome was admitted. Coronary angiography showed 90% stenosis of the left main trunk and 75% stenosis of the obtuse marginal branch. Coronary artery bypass grafting under cardioplegic arrest was done emergently. The left internal mammary artery graft was anastmosed to the left anterior descending artery, and a saphenous vein graft was used as a sequential bypass graft to the high lateral branch and obtuse marginal branch. Immediately after weaning from cardiopulmonary bypass, paroxysmal supraventricular tachycardia (PSVT) requiring electrical cardioversion was occurred, and catheter ablation was performed on the first postoperative day. There are controversus concerning the strategies of surgical treatment for unstable angina concomitant with WPW syndrome. Coronary bypass operation may trigger PSVT in patients with WPW syndrome. The optimal timing of perioperative catheter ablation needs further discussion.