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Objective: The aim of this study was to disclose the differences of surgical outcomes between Marfan syndrome with mutations in fibrillin-1 gene and Loeys-Dietz syndrome with mutations in transforming growth factor-beta receptor 1 and 2., Methods: We reviewed 368 patients aged less than 50 years who underwent surgery for thoracic aortic diseases between 1988 and 2019, and enrolled 99 patients with Marfan syndrome (26.9%; 57 men, 33 ± 7.5 years) and 24 patients with Loeys-Dietz syndrome (6.5%; 13 men, 28 ± 11 years)., Results: Freedom from all causes of mortality was similar between the 2 groups (P = .40, log-rank). The cumulative incidence of reintervention was significantly lower in the Marfan syndrome group than in the Loeys-Dietz syndrome group (P = .016, Gray). The cumulative incidence of first aortic arch reoperation for aortic arch aneurysm was significantly lower in the Marfan syndrome group than in the Loeys-Dietz syndrome group (P < .001, Gray). The cumulative incidence of first aortic root reoperation for aortic root aneurysm (P = .57, Gray) and first descending aorta reoperation for descending aortic aneurysm (P = .76, Gray) was similar between the 2 groups. The cumulative incidence of aortic dissection after initial surgery was significantly lower in Marfan syndrome than in Loeys-Dietz syndrome (P = .0059, Gray)., Conclusions: Loeys-Dietz syndrome with mutations in transforming growth factor-beta receptor 1 and 2 revealed higher rates of reoperation, and more specifically the arch reoperation was higher in those with Loeys-Dietz syndrome than those with Marfan syndrome. Aggressive arch surgery in the initial operation on the proximal aorta is recommendable in Loeys-Dietz syndrome to avoid additional aortic events. In Marfan syndrome, this is controversial in patients without dissection because of a low possibility to expand., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: The surgical indications for acute type A aortic dissection (AAAD) in patients in cardiopulmonary arrest remain controversial. Outcomes of AAAD for patients who underwent cardiopulmonary resuscitation (CPR) were evaluated., Methods: Between 2004 and 2018, of the 519 patients who underwent AAAD repair, 34 (6.6%) required CPR before or on starting AAAD repair. The patients were divided into 2 groups, survivors (n = 13) and nonsurvivors (n = 21), to compare the early operative outcomes, including mortality and neurological events., Results: The major cause of cardiovascular collapse requiring CPR was aortic rupture/cardiac tamponade (n = 21 [61.8%]), followed by coronary malperfusion (n = 12 [35.3%]) and acute aortic valve regurgitation (n = 3 [8.8%]). There were 3 (23.1%) patients in the survivors group and 11 (52.4%) in the nonsurvivors group who required ongoing CPR at the beginning of AAAD repair (P = .039). Of these patients, 1 survivor and 6 nonsurvivors could not achieve return of spontaneous circulation after pericardiotomy (P = .045). Although the duration from onset or arrival to the operating room was similar (P = .35 and P = .49, respectively), overall duration of CPR was shorter in survivors (10 minutes [range, 7.5-16 minutes] vs 16.5 minutes [range, 15-20 minutes]; P = .044). All survivors without any neurological deficits showed return of spontaneous circulation after pericardiotomy. Multivariate regression modeling showed that CPR duration >15 minutes was a significant risk factor for in-hospital mortality (P = .0040)., Conclusions: CPR duration beyond 15 minutes may be a contraindication for AAAD repair. Moreover, we should reconsider surgery for patients who cannot achieve return of spontaneous circulation after pericardiotomy., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: This study aimed to review the clinical results of young adult patients with aortic disease associated with mutations in the fibrillin-1 gene (FBN1) and disclose the histologic differences between the ascending and descending aortas., Methods: Between 2012 and 2015, 94 patients aged less than 50 years underwent surgery for thoracic aortic diseases. Forty-two patients (44.7%) had FBN-1 mutations. Of these, 40 patients (42.5%) with surgical specimens for histologic evaluation were included in the study. With the histologic results including the specimen sampled at their previous operations, cystic medial necrosis was classified into 3 grades according to the degree of the cystic area., Results: Thirty-nine patients (97.5%) had aortic root dilatation (Z ≥2), and 13 patients (32.5%) had ectopia lentis. Thirty-nine patients (97.5%) fulfilled the diagnostic criteria for Marfan syndrome. There were no in-hospital deaths. The majority (27/29: 93.1%) of the specimens of the ascending aorta revealed cystic medial necrosis pattern. With grade III being the most severe condition, these cases were classified into grade I (n = 2), grade II (n = 5), and grade III (n = 20). In contrast, only 6 specimens (6/17: 35.3%) of the descending aorta showed a cystic medial necrosis pattern that was classified into grade I (n = 2) and grade III (n = 4), (P < .00001)., Conclusions: Fewer specimens of the descending aorta revealed cystic medial necrosis compared with those of the ascending aorta. This difference might influence the characteristic aortic disease in Marfan syndrome associated with FBN-1 mutations., (Copyright © 2019 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Total arch replacement (TAR) is an established standard surgical procedure. We report >1000 cases of TAR using a 4-branched graft with antegrade cerebral perfusion (ACP) during a 15-year period., Methods: Since May 2001, 1005 patients who underwent total aortic replacement (mean age 69.8 ± 11.2 years; range, 9-92 years; 744 male) underwent TAR with a 4-branched graft. All surgeries were performed under hypothermia with ACP. There were 252 emergent operations for acute aortic dissection or aneurysm rupture. Concomitant operations included coronary arterial bypass grafting in 196 patients, aortic valve repair or replacement in 64, and aortic root replacements in 38., Results: The operation time was 482 ± 171 minutes, cardiopulmonary time was 254 ± 94 minutes, cardiac ischemia time was 145 ± 51 minutes, ACP time was 160 ± 47 minutes, and lower body circulatory arrest time was 62 ± 16 minutes. The hospital mortality rate was 5.2%. The permanent neurological dysfunction rate was 3.6% and temporary neurological dysfunction rate was 6.4%. There were no spinal cord complications. The 5-year survival rate was 80.7% and 10-year survival rate was 63.1%. Fifteen patients (1.5%) underwent reoperation for the arch grafts because of a pseudoaneurysm (11 patients), hemolysis (3 patients), and infection (1 patient)., Conclusions: TAR using a 4-branched graft with ACP could be accomplished with acceptable short- and long-term results., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: Since 2012, we have routinely applied balloon protection of the proximal left subclavian artery to prevent embolic events through the left vertebral artery during debranching thoracic endovascular aortic repair. This study aimed to study the effectiveness of balloon protection of the proximal left subclavian artery., Methods: We reviewed the medical records of 157 patients who underwent debranching thoracic endovascular aortic repair between 2007 and 2017. Of these, 71 patients for whom balloon protection of the proximal left subclavian artery was used were assigned to the balloon protection of the proximal left subclavian artery group (58 men; age: 78 ± 6.7 years), and 86 patients were assigned to the control group (66 men; age: 78 ± 8.9 years). A total of 51 patients from each group were matched by their propensity scores to adjust for differences in the patients' characteristics., Results: Perioperative stroke was significantly lower in the balloon protection of the proximal left subclavian artery group than in the control group (0%: 0/71 vs 7.9%: 7/86, P = .014). Freedom from all causes of mortality at 2 and 4 years was significantly higher in the balloon protection of the proximal left subclavian artery group compared with the control group (93%/76% vs 77%/59%, P = .015). Freedom from aortic death at 2 and 4 years was similar in both groups (97%/97% vs 91%/86%, P = .094). Propensity score matching yielded similar results of better freedom from all causes of mortality in the balloon protection of the proximal left subclavian artery group (93%/93% vs 81%/63%, P = .017) and equivalent aortic death in both groups (95%/95% vs 92%/88%, P = .30)., Conclusions: Debranching thoracic endovascular aortic repair using balloon protection of the proximal left subclavian artery demonstrated more appropriate early and late outcomes. Evaluation using propensity score matching enhanced the efficacy of balloon protection of the proximal left subclavian artery., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Anastomosis in dissected aorta management remains challenging. The patent false lumen of remnant aorta influences the growth of the residual aorta after hemiarch replacement. We evaluated the beneficial effect of adventitial inversion technique for distal anastomotic reinforcement of hemiarch replacement in acute type A aortic dissection., Methods: From 2006 to 2014, 90 patients with DeBakey type I aortic dissection who underwent hemiarch replacement for acute type A aortic dissection management at the National Cerebral and Cardiovascular Center were retrospectively analyzed. Patients were divided according to the technique used: the adventitial inversion technique in group A and the original Sandwich method with Teflon felt in group S. Surgical variables and aortic morphology from distal aortic anastomosis were evaluated by computed tomography after surgery., Results: The mean follow-up time was 2.2 ± 2.1 years with a follow-up rate of 91.1%. Cardiopulmonary bypass time was 208.2 ± 93.9 minutes in group A and 220.6 ± 93.9 minutes in group S; lower body circulatory arrest time was 51.6 ± 10.2 minutes in group A and 54.5 ± 17.8 minutes in group S. No significant differences were observed between groups. The overall hospital mortality rate was 10.0%. Postoperative false lumen thrombosis rate at proximal descending aorta on enhanced delayed phase computed tomography was significantly higher in group A than in group S., Conclusions: The adventitial inversion technique may facilitate thrombotic closure of the distal false lumen in acute type A aortic dissection management by hemiarch replacement., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Twenty-four patients with myasthenia gravis of Osserman's generalized type underwent extended thymectomy through a sternal-splitting approach. Their clinical responses to thymectomy and postoperative changes in anti-acetylcholine receptor antibody titers were evaluated. The follow-up time ranged from 1 month to 7 years and 7 months (average, 36 months). Six patients (25%) had remissions and 17 patients (71%) were improved after operation. The preoperative anti-acetylcholine receptor antibody titers dropped significantly after operation (p less than 0.001). The postoperative reduction in these titers correlated with the time course after operation (p less than 0.05). Their postoperative reduction was significantly greater in the six patients having remissions than in the 15 having marked (p less than 0.02) and the six having moderate improvement (p less than 0.005). This study has revealed that anti-acetylcholine receptor antibody titer in plasma declines progressively after thymectomy, and the postoperative reduction of this titer correlates with the clinical effect of thymectomy.