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.

Huge Pseudoaneurysm due to the Needle Hole of the Artificial Graft in the Bentall Procedure / 日本心臓血管外科学会雑誌

<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>

A Case of Successful Repair with Aortic Tailoring for Chronic Type B Aortic Dissection / 日本心臓血管外科学会雑誌

A 64-year-old woman with dyspnea on exertion was referred to our hospital. CT revealed type B aortic dissection with 7cm of aneurysm including a thrombus in the false lumen at the distal aortic arch. Four intimal tears at the distal aortic arch were closed directly during hypothermic circulatory arrest, and the descending thoracic aorta was tailored without a prosthetic graft after fixation of the dissecting adventitia to the intima at the distal portion of the false lumen. The postoperative course was uneventful and this patient was discharged on the 22nd postoperative day. Three years after surgery, the postoperative CT revealed no evidence of dilatation of the descending thoracic aorta as far as the abdominal aorta although the dissection of thoracoabdominal aorta remained. This technique is effective as an surgical option for chronic type B aortic dissection to minimize operative stress and complications.

In Situ Pulmonary Valve Replacement in the Tetralogy of Fallot / 日本心臓血管外科学会雑誌

Ten patients, aged 3 to 43 years, with the tetralogy of Fallot underwent <i>in situ</i> pulmonary valve replacement (PVR) 13 times. The implanted valves were a St. Jude Medical prosthesis (3 times) and a bioprosthetic valve (10 times). In 5 patients PVR was performed at the time of radical repair and in the remaining 5 patients PVR was performed after radical repair. Three patients underwent re-PVR at 6 to 13 years after the first PVR. There was one operative death in re-PVR 14 years after the first PVR and one patient died from congestive heart failure 4 years after PVR. In the patients with the tetralogy of Fallot, the rate of PVR in those who had undergone open Brock's operation were significantly higher than that of the patients without open Brock's operation (p<0.05). Actuarial survival rates at 5 years and 10 years were 88.9% and 88.9%, respectively. Rates of freedom from reoperation at 5 years and 10 years were 88.9% and 59.3%, respectively. Although the early operative results are satisfactory, re-PVR is mandatory in the future. Thus the indications of PVR should be considered carefully.

Cerebral Embolism Following Attempted Balloon Occlusion of a Ruptured Abdominal Aortic Aneurysm / 日本心臓血管外科学会雑誌

The case presented is a 76-year-old woman with a ruptured abdominal aortic aneurysm. We tried to pass a Fogarty balloon catheter from the left subclavian artery for proximal occlusion of the ruptured aneurysm but failed to inset the balloon into the descending aorta. Although the aneurysm was safely replaced with a gelatine coated dacron graft, she developed cerebral embolism and never regained consciousness and died two months later. Balloon insertion through the subclavian artery may cause complication through dislodgement of atheromatous plaque and may induce cerebral embolism.