• Biomolecules & Therapeutics
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• v.28 no.1
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• pp.98-103
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• 2020

Marfan syndrome (MFS), a connective tissue disorder caused by mutations in the fibrillin-1 (Fbn1) gene, has vascular manifestations including aortic aneurysm, dissection, and rupture. Its vascular pathogenesis is assumed to be attributed to increased transforming growth factor β (TGFβ) signaling and blockade of excessive TGFβ signaling has been thought to prevent dissection and aneurysm formation. Here, we investigated whether galunisertib, a potent small-molecule inhibitor of TGFβ receptor I (TβRI), attenuates aneurysmal disease in a murine model of MFS (Fbn1^C1039G/+) and compared the impact of galuninsertib on the MFS-related vascular pathogenesis with that of losartan, a prophylactic agent routinely used for patients with MFS. Fbn1^C1039G/+ mice were administered galunisertib or losartan for 8 weeks, and their ascending aortas were assessed for histopathological changes and phosphorylation of Smad2 and extracellular signal-regulated kinase 1/2 (Erk1/2). Mice treated with galunisertib or losartan barely exhibited phosphorylated Smad2, suggesting that both drugs effectively blocked overactivated canonical TGFβ signaling in Fbn1^C1039G/+ mice. However, galunisertib treatment did not attenuate disrupted medial wall architecture and only partially decreased Erk1/2 phosphorylation, whereas losartan significantly inhibited MFS-associated aortopathy and markedly decreased Erk1/2 phosphorylation in Fbn1^C1039G/+ mice. These data unexpectedly revealed that galunisertib, a TβRI inhibitor, showed no benefits in aneurysmal disease in MFS mice although it completely blocked Smad2 phosphorylation. The significant losartan-induced inhibition of both aortic vascular pathogenesis and Smad2 phosphorylation implied that canonical TGFβ signaling might not prominently drive aneurysmal diseases in MFS mice.

• Journal of Chest Surgery
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• v.29 no.11
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• pp.1212-1217
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• 1996

From Dec. 1993 to May 1995, 9 male and 5 female patients ranging in age from 25 to 65 years, were operated on for ascending aorta and/or aortic arch diseases. Six patients had acute aortic dissection, type A(ruptured in 4 cases); four had ruptured ascending aortic aneurysm; three had annuloaortic ectasia(ruptured in 1 cases); one had aortic arch aneurysm. The diagnostic procedures were echo cardiography and dynamic CT scan in all patients having acute dissection or rupture. The aortic angiography was performed in two cases. Indications for operations were rupture in five cases, acute aortic dissection in five cases, severe congestive heart failure in two cases, progressive aortic insufficiency in one case and impending rupture in one case. The emergent repair was performed in ten cases(71%). The surgical treatment consisted of 6 Cabrol operations, a Cabrol operation combined with arch replacement, a modified Bentall operation, 4 replacement of ascending aorta, a replacement of aortic arch, and a replacement of ascending aorta and aortic arch. Complications were a hypoxic encephalopathy, two atrial fibrillations, a sternal deheiscence, and a mediastinitis. Two early mortality(14%) were due to intractable bleeding and multiple organ failure, and one late mortality(7%) was due to ventricular arrhythmia. In eleven survivors, follow-up period was from 2 months to 12 months and the course was uneventful.

• Journal of Chest Surgery
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• v.32 no.6
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• pp.532-535
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• 1999

Background: The purpose of this study was to evaluate and analyze the surgical techniques and postoperative complications in patients undergoing operations for descending thoracic aortic aneurysms. Material and Method: The data of 22 major operations between March 1987 and August 1997 were retrospectively reviewed. Result: There were 18 men and 4 women with a mean age of 49 years (range 33 years to 82 years). The cause of the aneurysm was aortic dissection in 13 patients, atherosclerosis in 3, mycotic in 3, trauma in 2 and uncertain in 1. The operative techniques were resection and graft replacement in 16, axillofemoral bypass graft in 2, femorofemoral bypass graft in 2, exclusion, aneurysmorrhaphy in 1 and transfemoral stent insertion in 1. During the operation, 16 cases were performed under total aortic clamp. Among the 16 patients, femorofemoral bypass was used in 14 cases and previously made shunt in 2 cases. The mean total aortic clamp time was 91 minutes and the mean extracorporeal circulation time was 116 minutes. One death occurred in an excluded patient on the 52 postoperative day due to a rupture of the aneurysm. Postoperative complications were paraplegia in 1 case, acute renal failure in 1 case and acute respiratory failure in 1 case. Conclusion: Although surgical treatment of the descending thoracic aneurysm has many postoperative complications, good surgical results can be achieved with a proper patient selection and fine surgical techniques.

• Journal of Chest Surgery
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• v.35 no.4
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• pp.274-282
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• 2002

Background: Paravalvular leakage or false aneurysm developed after isolated aortic valve replacement(AVR) for aortic regurgitation(AR) associated with Behcet's disease is one of the most serious complications, and requires subsequent reoperations. We describe the surgical result of homograft aortic root replacement(ARR) for AR associated with Behcet's disease. Material and Method: From January 1992 to December 2001, 6 patients with AR associated with Behcet's disease underwent 7 ARR with homograft and 1 Ross operation. Five patients were male and one was female. The grafts used for ARR were 5 aortic and 2 pulmonic homografts. Ages at operation ranged from 27 to 51 years(mean, 37$\pm$9 years). Two patients underwent ARR with aortic homograft at the first operation. In the remaining 4 patients, ARR using a homograft was performed for paravalvular leakage that developed after AVR, and the mean interval from AVR to ARR was 21 $\pm$29 months(range, 5 to 73.3 moths, median, 7.6 months). Result: There was no early death. All patients were followed up for an average of 18.9$\pm$24.0 months(range, 1.9 to 68.9 months, median, 8.4 months). Two of 4patients who had undergone ARR after AVR required subsequent reoperations for false aneurysm of the ascending aorta and failure of pulmonary homograft. One patient underwent re-replacement of the aortic root, ascending aorta and partial aortic arch with an aortic homograft, the other underwent Ross operation. Conclusion: This study suggests that aortic root replacement using a homograft in aortic regurgitation with Behcet's disease may provide good clinical results and decrease the incidence of paravalvular leakage or false aneurysm after aortic valve replacement. However, the adequate perioperative management and complete removal of the inflarrunatory tissue at operation were also important for the good long-term results.

• Journal of radiological science and technology
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• v.30 no.3
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• pp.205-212
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• 2007

To evaluate the feasibility and efficacy of a pulsatile aortic aneurysm phantoms for in-vitro study. The phantoms consisted of a pulsating motor part(heart part) and an aortic aneurysm part, which mimicked true physiologic conditions. The heart part was created from a high-pressured water pump and a pulsatile flow solenoid valve for the simulation of aortic flow. The aortic aneurysm part was manufactured from paper clay, which was placed inside a acrylic plastic square box, where liquid silicone was poured. After the silicone was formed, the clay was removed, and a silicone tube was used to connect the heart and aneurysm part. We measured the change in pressure as related to the opening time(pulse rate, Kruskal-Wallis method) and pressure before and after the stent-graft implantation(n = 5, Wilcoxon's signed ranks test). The changes in blood pressures according to pulse rate were all statistically significant(p<0.05). The systolic/diastolic pressures at the proximal aorta, the aortic aneurysm, and the distal aorta of the model were $157.80{\pm}1.92/130.20{\pm}1.92$, $159.40{\pm}1.14/134.00{\pm}2.92$, and $147.20{\pm}1.480/129.60{\pm}2.70\;mmHg$, respectively, when the pulse rate was 0.5 beat/second. The pressures changed to $161.40{\pm}1.34/90.20{\pm}1.64$, $175.00{\pm}1.58/93.00{\pm}1.58$, and $176.80{\pm}1.48/90.80{\pm}1.92\;mmHg$, respectively, when the pulse rate was 1.0 beat/second, and $159.40{\pm}1.82/127.20{\pm}1.48$, $166.60{\pm}1.67/138.00{\pm}1.87$, and $161.00{\pm}1.22/135.40{\pm}1.67\;mmHg$, respectively, when it was 1.5 beat/second. When pulse rate was set at 1.0 beat/second, the pressures were $143.60{\pm}1.67/90.20{\pm}1.64$, $147.20{\pm}1.92/84.60{\pm}1.82$, and $137.40{\pm}1.52/88.80{\pm}1.64\;mmHg$ after stent-graft implantation. The changes of pressure before and after stent-graft implantation were statistically significant(p<0.05) except the diastolic pressures at the proximal(p =1.00) and distal aorta(p=0.157). The aortic aneurysm phantoms seems to be useful for the evaluation of the efficacy of stent-graft before animal or clinical studies because of its easy reproducibility and ability to display a wide range of pressures.

• Journal of Chest Surgery
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• v.15 no.2
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• pp.222-229
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• 1982

The aneurysmal dilatation of ascending aorta with the aortic regurgitation presents typical surgical problems. Over the years, various surgical procedures had been used for the management of the dilated segment of sending aorta and the aortic regurgitation. The surgical technique Is still in the state of evolution. The one method is the super coronary replacement of the ascending aorta with vascular graft and replacement of the aortic valve with preservation of the coronary ostia as advocated by Miller and his colleague at Stanford University, so called conventional technique". The other is the replacement of aortic valve and the dilated segment of the ascending aorta using a composite graft and transplantation of the coronary ostia as described by Bentall and DeBono in 1968. The controversy appears to evolve around 3 technical problems. One is bleeding from the grafted area. Two is later development of the aneurysmal dilatation of the subcoronary aortic wall when non-composite graft is employed. Three is a management of the coronary arteries. The purpose of this article is to present our experience with 7 cases of annuloaortic ectasia in whom both of these surgical techniques at that employed and to review some of the problems that encountered during the management of these patients .

• Journal of Chest Surgery
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• v.28 no.10
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• pp.906-911
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• 1995

From January 1991 to January 1995, 11 patients with aortic diseases underwent various surgical repairs. The age at operation ranged from 26 years to 63 years[ mean=50.9 years . The disease entities included 8 aortic dissections[ type I in 4, type II in 2 and type III in 2 cases , 2 Marfan`s syndrome with annuloaortic ectasia and 1 desecending thoracic aortic aneurysm The operative procedures we tried were 3 Bentall`s operation, 5 graft replacement of ascending aorta, and 3 graft interposition in descending thoracic aorta.Overall hospital mortality rate is 36.3%[4/11 . And causes of death are pump weaning failure in 2 cases and multiorgan failure in 2 cases. It was that 2 sternal dehiscence & mediastinitis, 1 acute renal failure, 2 hypoxic brain damages and 2 postoperative psychosis were complicated. Recently we tried surgical repair of aortic dissection five out of 6 cases using total circulatory arrest with deep hypothermia at 14$^{\circ}C$. Total circulatory arrest time ranged from 18 to 26 minutes[ mean 22.2 minutes , and mean aortic cross-clamping time was 48.2 minutes. One of 5 patient died on the 7th postoperative day due to multiorgan failure. Mortality of patients with TCA was 20%[1/5 , and it of remainders was 50%[3/6 . Our result for surgical repair using total circulatory arrest with deep hypothermia is satisfactory on the basis of our clinical data.

• Journal of Chest Surgery
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• v.47 no.6
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• pp.552-555
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• 2014

Thoracic endovascular aortic repair has become a widespread alternative treatment option for thoracic aortic aneurysm. The debranching of arch vessels may be required to provide an acceptable landing zone for an endovascular stent graft. We report a case where the bypass graft used in the thoracic endovascular aortic repair procedure compressed the left internal jugular vein, causing acute thrombotic occlusion.

• Journal of Chest Surgery
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• v.47 no.6
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• pp.536-540
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• 2014

Due to its low prevalence and because there is lack of awareness about it, Loeys-Dietz syndrome is often misdiagnosed as Marfan syndrome, which has similar skeletal abnormalities and aortic pathology. However, the differential diagnosis between these two connective tissue diseases is critical because they correspond to different surgical indications and surgical decision-making. We report two cases of successful thoracoabdominal aortic replacement in patients with previously undiagnosed Loeys-Dietz syndrome.

• Journal of Chest Surgery
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• v.26 no.11
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• pp.838-844
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• 1993

Rupture of aortic is for the most part a terminal disorder and requires prompt and aggressive treatment. Most of the patients who develop this condition outside the hospital die before appropriate therapy can be instituted. Thus prompt treatment, sometimes innovative, resulted in a highly significant survival rate. We experionced 21 aortic rupture due to mycotic aneurysm and another 3 due to chest trauma. Operation was done in 11 cases, and mortality was 3 of 11 cases. In another 10 cases, operation was not performed and all of them were expired. We confirmed diagnosis using only non invasive technic as ultrasonogram and computed tomography and invasive technic like aniography was absolutely avoided. Preoperative management was aimed to lower blood pressure for the purpose of preventing srcondary bleeding, and operantions erer porformed promptly if possible.