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OBJECTIVES: Our goal was to evaluate early and mid-term outcomes of physician-modified endografting for pararenal and thoraco-abdominal aortic aneurysms from 10 Japanese aortic centres. METHODS: From January 2012 to March 2022, a total of 121 consecutive adult patients who underwent physician-modified endografting for pararenal and thoraco-abdominal aortic aneurysms were enrolled. We analysed early and mid-term postoperative outcomes, including postoperative complications and mortality. RESULTS: The pararenal and thoraco-abdominal aortic aneurysm groups included 62 (51.2%) and 59 (48.8%) patients, respectively. The overall in-hospital mortality rate was 5.8% (n = 7), with mortality rates of 3.2% (n = 2) and 8.5% (n = 5) in pararenal and thoraco-abdominal aortic aneurysm groups, respectively (P = 0.225). Type IIIc endoleaks occurred postoperatively in 18 patients (14.9%), with a significantly higher incidence (P = 0.033) in the thoraco-abdominal aortic aneurysm group (22.0%, n = 13) than in the other group (8.1%, n = 5). Major adverse events occurred in 7 (11.3%) and 14 (23.7%) patients in pararenal and thoraco-abdominal aortic aneurysm groups (P = 0.074), respectively. The mean follow-up period was 24.2 months. At the 3-year mark, both groups differed significantly in freedom from all-cause mortality (83.3% and 54.1%, P = 0.004), target aneurysm-related mortality (96.8% and 82.7%, P = 0.013) and any reintervention (89.3% and 65.6%, P = 0.002). Univariate and multivariate regression analyses demonstrated that ruptures, thoraco-abdominal aortic aneurysms and postoperative type IIIc endoleaks were associated with an increased risk of all-cause mortality. CONCLUSIONS: The mid-term outcomes of physician-modified endografting for pararenal and thoraco-abdominal aortic aneurysms were clinically acceptable and comparable with those in other recently published studies. Notably, pararenal and thoraco-abdominal aortic aneurysms represent distinct pathological entities with different postoperative outcomes.
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BACKGROUND: We investigated the safety and efficacy of physician-modified thoracic endovascular aortic repair using a low-profile device for aortic arch lesions. METHODS: A total of 42 consecutive patients (mean age 67.2 ± 12.7 years; 32 men) with aortic arch lesions were treated by physician-modified thoracic endovascular aortic repair using a low-profile device (Zenith Alpha Thoracic Endovascular Graft) with four scallops or 13 fenestrations for the common carotid artery and 38 fenestrations or 30 branches for the left subclavian artery. The aortic repair indications were acute type B aortic dissection (n = 17, 40.5%), degenerative aneurysm (n = 14, 33.3%), chronic dissection aneurysmal degeneration (n = 4, 9.5%), and ulcer-like projection (n = 2, 4.8%). The mean iliac artery diameter was 7.6 ± 1.1â mm. RESULTS: There were no branches covered unintentionally or patients who died and suffered from severe spinal cord ischemia perioperatively. One patient (2.4%) experienced a postoperative minor stroke with full neurological recovery. The mean follow-up time was 18 ± 11 months, with 28 patients (66.7%) having at least 12 months. One access-related complication (2.4%) occurred. Two residual Ia (4.8%) and three residual IIIa (7.1%) endoleaks were treated by reintervention. There were no open repair conversions, ruptures, or other aortic complications. CONCLUSION: Physician-modified thoracic endovascular aortic repair using the low-profile device may be a safe, feasible, and time-saving method for preserving the cervical artery and has high reproducibility and anatomical reconstruction. However, its durability requires long-term follow-up.
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Aneurisma da Aorta Torácica , Implante de Prótese Vascular , Procedimentos Endovasculares , Masculino , Humanos , Pessoa de Meia-Idade , Idoso , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Prótese Vascular/efeitos adversos , Aneurisma da Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/cirurgia , Aneurisma da Aorta Torácica/complicações , Stents/efeitos adversos , Correção Endovascular de Aneurisma , Implante de Prótese Vascular/efeitos adversos , Reprodutibilidade dos Testes , Resultado do Tratamento , Complicações Pós-Operatórias/etiologia , Fatores de Tempo , Estudos RetrospectivosRESUMO
Objective: Emergency surgical repair is the standard treatment for acute aortic dissection type A. However, the surgical risk of total arch replacement remains high. The Viabahn Open Revascularization TEChnique has been used for supra-aortic reconstruction during total arch replacement. This Cleveland Clinic technique is called "branched stented anastomosis frozen elephant trunk repair." Our total arch replacement with reconstructed extended branched stented anastomosis frozen elephant trunk repair requires no unnecessary cervical artery exposure. We compared the outcomes of extended branched stented anastomosis frozen elephant trunk repair and conventional total arch replacement in acute aortic dissection type A. Methods: We compared the clinical course of patients undergoing total arch replacement using sutureless direct branch vessel stent grafting with frozen elephant trunk (extended branched stented anastomosis frozen elephant trunk repair) for acute aortic dissection type A with patients undergoing conventional total arch replacement. For the procedure, the aortic arch was transected circumferentially distal to the brachiocephalic artery origin. Frozen elephant trunk was fenestrated by heating with a cautery, and the self-expandable stent graft was delivered into the branch vessels through the fenestration. Results: Of 58 cases, 21 and 37 were classified in the extended branched stented anastomosis frozen elephant trunk repair and conventional total arch replacement groups, respectively. The times (minutes) of selective antegrade cerebral perfusion (75 ± 24, 118 ± 47), total operation (313 ± 83, 470 ± 151), and cardiopulmonary bypass (195 ± 46, 277 ± 96) were significantly better in the extended branched stented anastomosis frozen elephant trunk repair group (P < .001). Six surgical deaths occurred: 2 (9%) in the extended branched stented anastomosis frozen elephant trunk repair group and 4 (10%) in the conventional total arch replacement group. In all cases, only 1 patient (2%) in the conventional total arch replacement group had a branch artery-related complication during the postoperative follow-up period. In the extended branched stented anastomosis frozen elephant trunk repair group, blood product use significantly decreased (P < .05). Conclusions: Extended branched stented anastomosis frozen elephant trunk repair has shown comparable safety and efficacy to conventional total arch replacement and can be used for acute aortic dissection type A emergency repair. It optimizes true lumen perfusion and facilitates supra-aortic artery remodeling.
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BACKGROUND: The effect of the surgical sealant AQUABRID on outcomes after acute aortic dissection repair has not been evaluated. The objective of this study was to examine whether the use of AQUABRID affects the volume of intraoperative blood transfusion or operative time in patients undergoing emergency surgery to repair acute aortic dissection. METHODS: A multicenter retrospective cohort study from January 2007 to December 2021. A total of 399 patients underwent emergency acute aortic dissection repair. Propensity score matching was used to adjust for the type of surgery and other patient characteristics. RESULTS: A total of 387 of the eligible 399 patients were included in this study and propensity score matching yielded 94 patients for whom characteristics were not significantly different between the two groups. The type of surgery was exactly matched (ascending aorta replacement: 19 [40%]; partial arch replacement: 13 [28%]; total arch replacement: 15 [32%] in each group). Within the matched cohort, there was a statistically significant difference in the volume of intraoperative blood transfusion. (AQUABRID vs. control: 34 [26-38] vs. 50 [38-60] U in Japan, p = .03). Operating time was significantly shorter in the AQUABRID group (total operation: mean ± SD, 343 ± 92 vs. 402 ± 161 min, p = .03; reconstruction for arch vessels: 29 ± 17 vs. 56 ± 22, p < .01). The rate of postoperative complications was comparable in the two groups. Failure to use AQUABRID was a significant predictor of the need for massive transfusion (>40 U) (odds ratio: 7.20; 95% confidence interval: 2.56-20.23; p < .01). CONCLUSIONS: The use of AQUABRID during emergency acute aortic dissection repair significantly decreased the volume of intraoperative blood transfusion and the duration of surgery.
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Aneurisma da Aorta Torácica , Dissecção Aórtica , Implante de Prótese Vascular , Humanos , Estudos Retrospectivos , Duração da Cirurgia , Dissecção Aórtica/cirurgia , Aorta/cirurgia , Transfusão de Sangue , Complicações Pós-Operatórias/cirurgia , Aneurisma da Aorta Torácica/cirurgia , Doença Aguda , Aorta Torácica/cirurgiaRESUMO
In the future, when fossil fuels are exhausted, alternative energy sources will be essential for everyday needs. Hydrogen-based energy can play a vital role in this aspect. This energy is green, clean, and renewable. Electrochemical hydrogen devices have been used extensively in nuclear power plants to manage hydrogen-based renewable fuel. Doped zirconate materials are commonly used as an electrolyte in these electrochemical devices. These materials have excellent physical stability and high proton transport numbers, which make them suitable for multiple applications. Doping enhances the physical and electronic properties of zirconate materials and makes them ideal for practical applications. This review highlights the applications of zirconate-based proton-conducting materials in electrochemical cells, particularly in tritium monitors, tritium recovery, hydrogen sensors, and hydrogen pump systems. The central section of this review summarizes recent investigations and provides a comprehensive insight into the various doping schemes, experimental setup, instrumentation, optimum operating conditions, morphology, composition, and performance of zirconate electrolyte materials. In addition, different challenges that are hindering zirconate materials from achieving their full potential in electrochemical hydrogen devices are discussed. Finally, this paper lays out a few pathways for aspirants who wish to undertake research in this field.
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We have investigated the current-voltage (I-V) characteristics of Josephson junctions made by a low-temperature superconductor of vanadium (V) with a small amount of hydrogen (H) and deuterium (D) impurities using a mechanically controllable break junction (MCBJ) technique. Below the superconducting transition temperature TC, the differential conductance dI/dV spectra show distinct peaks within the superconducting gap, known as the subgap structure, which result from multiple Andreev reflections in pure V nanocontacts. Additionally, the H and D impurities in V nanocontacts induce several new conductance peaks outside the gap, referred to as an over-the-gap structures (OGSs). We found that the OGS peaks exhibit strong temperature dependence until the temperature is close to TC and follow the gap function predicted by BCS theory. When the contact diameter is changed at low temperature using the MCBJ technique, the OGS anomalies change almost linearly with the inverse of the contact diameter. In addition, the gap anomalies are significantly shifted outward by changing the positions of the H atoms through application of a high bias voltage between the contacts. The above features suggest that the OGS peaks are caused by the superconducting quasiparticle interference induced by H or D impurities and/or inhomogeneous structures in the nanocontacts.
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BACKGROUND: Acute aortic dissection (AAD) with concurrent ST-segment elevation myocardial infarction (STEMI) is relatively rare and sometimes overlooked. As D-dimer testing has been reported to have high sensitivity to diagnose AAD in a clinical scale, Aortic Dissection Detection Risk Score (ADD-RS), a point-of-care D-dimer analyzer capable of measuring in 10 min would be useful to deny AAD with concurrent STEMI. However, an optimal cut-off value of D-dimer in such population remains unclear. Therefore, the aim of this study was to elucidate the optimal D-dimer threshold in patients clinically diagnosed with STEMI. METHODS: This retrospective cohort study was conducted at two tertiary care centers between 2014 and 2019. Patients clinically diagnosed with STEMI who underwent serum D-dimer measurement on hospital arrival were included. The primary outcome was the diagnosis of AAD. The area under the receiver operating characteristic curve (AUROC) for D-dimer values to diagnose AAD was evaluated, particularly in patients with low to moderate risks of AAD (1 of ADD-RS). The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated with several cut-off values. RESULTS: A total of 322 patients were included, and 28 were diagnosed with AAD. The AUROC for D-dimer to diagnose AAD was 0.970 (95% confidence interval: 0.948-0.993) in 262 patients with 1 of ADD-RS. If D-dimer ≥750 ng/mL was used as a cut-off value, sensitivity, specificity, PPV and NPV were 100%, 86.4%, 37.7%, and 100%, respectively. AAD could be denied in 209 (79.8%) patients using the cut-off value (D-dimer <750 ng/mL). CONCLUSIONS: Serum D-dimer ≥750 ng/mL exhibited high sensitivity and NPV to diagnose AAD with concurrent STEMI, while the ADD-RS originally utilized ≥500 ng/mL as a cut-off for any suspected AAD. A point-of-care D-dimer measurement with the new cut-off would be useful to rule-out AAD among patients with STEMI.
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Aneurisma Aórtico , Dissecção Aórtica , Infarto do Miocárdio com Supradesnível do Segmento ST , Dissecção Aórtica/complicações , Dissecção Aórtica/diagnóstico , Aneurisma Aórtico/diagnóstico , Biomarcadores , Produtos de Degradação da Fibrina e do Fibrinogênio , Humanos , Estudos Retrospectivos , Infarto do Miocárdio com Supradesnível do Segmento ST/diagnósticoRESUMO
Prosthetic valve endocarditis, especially when complicated by an aortic root abscess and a left ventricular outflow tract rupture, is a life-threatening condition. We present a case of infective prosthetic aortic valve endocarditis with a ruptured left ventricular outflow tract successfully treated with a root-commando operation using a secure anastomosis for reconstruction of the damaged aortic annulus.
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Endocardite Bacteriana , Endocardite , Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Endocardite/cirurgia , Endocardite Bacteriana/cirurgia , Próteses Valvulares Cardíacas/efeitos adversos , Implante de Prótese de Valva Cardíaca/efeitos adversos , HumanosAssuntos
Estenose da Valva Aórtica , Oclusão Coronária , Substituição da Valva Aórtica Transcateter , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Estenose da Valva Aórtica/diagnóstico por imagem , Estenose da Valva Aórtica/cirurgia , Oclusão Coronária/diagnóstico por imagem , Oclusão Coronária/etiologia , Oclusão Coronária/cirurgia , Vasos Coronários , Humanos , Substituição da Valva Aórtica Transcateter/efeitos adversos , Resultado do TratamentoRESUMO
OBJECTIVES: The open-style stent graft technique has been changing the strategy for true distal arch aneurysms extending to the descending aorta. Our mid-term results of surgical repair using a J-graft open stent graft are presented. METHODS: Between May 2015 and June 2020, 69 patients with a distal arch aneurysm (53 males, median age 74 years) underwent total arch replacement combined with J-graft open stent deployment. All 59 surviving patients were followed for a median follow-up period of 1.8 (0.6-3.6) years. RESULTS: Antegrade deployment was successfully performed in all patients without any difficulties. The deployed device was securely fixed at the target area, and it initiated thrombus formation. The diameter of the excluded aneurysm was decreased in 54 patients (91.5%) during the follow-up period. There were no type I endoleaks, but there were 3 type II endoleaks; 2 of the 3 type II endoleaks disappeared during the follow-up period. Additional endovascular operations were performed in 3 patients. There were 10 in-hospital deaths (14.5%), and the incidences of stroke, spinal cord injury and distal embolism were 11.6%, 5.8% and 2.9%, respectively. The 1- and 3-year survival rates were 84.8% and 79.4%, respectively, and the 1- and 3-year freedom from reintervention rates were 97.2% and 81.3%, respectively. CONCLUSIONS: The J-graft open stent graft was easy to deploy, and it could shift the distal anastomosis to a more proximal side. The mid-term performance of this device was good. It has the potential to provide one-stage repair.
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Aneurisma da Aorta Torácica , Dissecção Aórtica , Implante de Prótese Vascular , Procedimentos Endovasculares , Idoso , Dissecção Aórtica/cirurgia , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Aneurisma da Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/cirurgia , Prótese Vascular , Implante de Prótese Vascular/efeitos adversos , Procedimentos Endovasculares/efeitos adversos , Feminino , Humanos , Japão , Masculino , Stents , Resultado do TratamentoRESUMO
OBJECTIVE: This study evaluated the efficacy of the provisional extension to induce complete attachment (PETTICOAT) technique for type B and postoperative residual type B aortic dissections compared with the conventional thoracic endovascular aortic repair (TEVAR) technique. METHODS: In this retrospective study, we compared sequential aortic morphologic changes in consecutive patients with type B and postoperative residual type B aortic dissections treated with the PETTICOAT technique between January 2016 and December 2017 with patients treated with the conventional TEVAR between January 2013 and December 2015. Outcomes included aortic remodeling and aorta-related adverse events for 2 years postoperatively. RESULTS: Forty-eight patients were included in this study (24 in the PETTICOAT group, 24 patients in the conventional TEVAR group). Although both groups showed aortic remodeling in the descending thoracic aorta, the PETTICOAT group developed significantly better aortic remodeling in the abdominal aorta compared with the conventional TEVAR group during the observation period. The PETTICOAT group had significantly fewer aorta-related adverse events compared with the conventional TEVAR group (8% vs 54%; P < .001). Aorta-related adverse events more commonly occurred in the poor remodeling group compared with in the good remodeling group (P = .001; hazard ratio, 8.32; 95% confidence interval, 2.26-30.64). CONCLUSIONS: This study suggests that the PETTICOAT technique for aortic dissection may promote aortic remodeling and decrease the incidence of aorta-related adverse events. Additional studies are required to confirm these preliminary findings.
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Aorta Abdominal/fisiopatologia , Aorta Torácica/cirurgia , Aneurisma da Aorta Torácica/cirurgia , Dissecção Aórtica/cirurgia , Implante de Prótese Vascular , Procedimentos Endovasculares , Remodelação Vascular , Idoso , Dissecção Aórtica/diagnóstico por imagem , Dissecção Aórtica/fisiopatologia , Aorta Abdominal/diagnóstico por imagem , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/fisiopatologia , Aneurisma da Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/fisiopatologia , Aortografia , Prótese Vascular , Implante de Prótese Vascular/efeitos adversos , Implante de Prótese Vascular/instrumentação , Angiografia por Tomografia Computadorizada , Procedimentos Endovasculares/efeitos adversos , Procedimentos Endovasculares/instrumentação , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/fisiopatologia , Complicações Pós-Operatórias/prevenção & controle , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Stents , Fatores de Tempo , Resultado do TratamentoRESUMO
OBJECTIVE: The provisional extension to induce complete attachment (PETTICOAT) technique is a unique thoracic endovascular aortic repair (TEVAR) for aortic dissection, which consists of proximal descending aortic endografting plus distal bare-metal stenting. This study aimed to investigate the efficacy of the PETTICOAT technique in patients with acute-sub-acute complicated type B aortic dissections. In particular, we compared the remodeling effect of full PETTICOAT covering down to the abdominal aorta with that of simple entry closure. METHODS: In this retrospective pre-post study, we compared the clinical course of consecutive patients undergoing TEVAR with the PETTICOAT technique in which proximal entry tear was excluded with a covered stent, and extension bare stents were placed down to the abdominal segment for acute-sub-acute complicated type B aortic dissections, between 2015 and 2017, with a control group treated with TEVAR with entry closure between 2011 and 2015. Outcomes included the aortic remodeling rate and the aortic diameter up to 1 year after surgery. RESULTS: Subjects consisted of 47 patients (21 in full PETTICOAT group, 26 in the simple entry closure group). The remodeling rate of the abdominal aorta in the full PETTICOAT group was significantly higher than in the simple entry closure group (p < 0.05), while that of the thoracic aorta was comparable between the two groups. CONCLUSIONS: This study suggests that the full PETTICOAT technique achieves better aortic remodeling compared to entry closure alone, and might lead to less reintervention.
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Aneurisma da Aorta Torácica , Dissecção Aórtica , Implante de Prótese Vascular , Procedimentos Endovasculares , Dissecção Aórtica/diagnóstico por imagem , Dissecção Aórtica/cirurgia , Aneurisma da Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/cirurgia , Prótese Vascular , Humanos , Estudos Retrospectivos , Stents , Resultado do TratamentoRESUMO
Mediastinitis is an unusual but potentially life-threatening complication of cardiac surgery. Open drainage is one of the standard therapies, but there could sometimes be potential complications. We had a patient who underwent open drainage surgery for postoperative mediastinitis, and right ventricular rupture occurred subsequently to extubation in an operation room. Retrospectively reviewed, computed tomography showed strong adhesions between the right ventricle and the posterior margin of sternum, pulling his right ventricle to the right side of his sternum. We should have noticed the risk of leaving the sternum open and performed adhesiolysis of the right ventricle and the posterior margin of sternum to prevent the devastating complication. This case illustrates the importance of recognizing the rare computed tomography sign of ventricular pulling-a predictor for right ventricular rupture after open drainage for mediastinitis.
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A 73-year-old man with an acute myocardial infarction experienced severe cardiogenic shock due to an inferior ventricular septal rupture with a massive left-to-right shunt. Emergency surgery was considered a too high mortality risk. The patient was implanted with an extracorporeal membrane oxygenation system as a bridge to surgery. On the seventh day after admission, the ventricular septal defect was successfully repaired. Our case study demonstrates that extracorporeal membrane oxygenation could be an option in cases of ventricular septal rupture as a bridge for stabilizing patients.
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Oxigenação por Membrana Extracorpórea/métodos , Cuidados Pré-Operatórios/métodos , Choque Cardiogênico/terapia , Ruptura do Septo Ventricular/cirurgia , Idoso , Procedimentos Cirúrgicos Cardíacos , Ecocardiografia , Humanos , Masculino , Choque Cardiogênico/etiologia , Ruptura do Septo Ventricular/complicações , Ruptura do Septo Ventricular/diagnósticoRESUMO
BACKGROUND: The aims of this study were to evaluate the on-pump beating-heart technique of coronary artery bypass in patients with acute myocardial infarction and left main disease, and to retrospectively compare the early postoperative results with those of conventional on-pump arrested-heart coronary surgery. METHODS: Eighty-five patients with acute myocardial infarction caused by left main disease, who underwent emergency surgery between January 1998 and April 2017 at Saiseikai Utsunomiya Hospital, were enrolled in this study. Of these patients, 56 were evaluated using propensity-matched analysis. The patients were divided into two groups according to the surgical procedure: group A ( n = 28) had on-pump surgery on the arrested heart, and group B ( n = 28) had on-pump surgery on the beating heart. Early postoperative results were compared between the two groups. RESULTS: Preoperative and intraoperative characteristics showed no significant differences between the two groups. The peak creatine kinase myocardial band level was significantly lower in group B (group A 151 vs. group B 91 IU·L-1, p = 0.01). The early mortality rate was higher in group A than group B, but the difference was not significant (group A 28.6% vs. group B 17.9%, p = 0.53). CONCLUSIONS: There was no significant advantage based on surgical procedure between on-pump beating-heart surgery and on-pump surgery on the arrested heart. On-pump beating-heart coronary artery bypass grafting significantly reduced the peak creatine kinase myocardial band level, but there were no significant differences in the early postoperative data, including the mortality rate and left ventricular function.
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Ponte Cardiopulmonar , Ponte de Artéria Coronária/métodos , Doença da Artéria Coronariana/cirurgia , Parada Cardíaca Induzida , Infarto do Miocárdio/cirurgia , Idoso , Biomarcadores/sangue , Ponte Cardiopulmonar/efeitos adversos , Ponte Cardiopulmonar/métodos , Ponte de Artéria Coronária/efeitos adversos , Ponte de Artéria Coronária/mortalidade , Doença da Artéria Coronariana/diagnóstico por imagem , Doença da Artéria Coronariana/mortalidade , Doença da Artéria Coronariana/fisiopatologia , Creatina Quinase Forma MB/sangue , Emergências , Feminino , Parada Cardíaca Induzida/efeitos adversos , Parada Cardíaca Induzida/mortalidade , Humanos , Masculino , Pessoa de Meia-Idade , Infarto do Miocárdio/diagnóstico por imagem , Infarto do Miocárdio/fisiopatologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Função Ventricular EsquerdaRESUMO
Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder (CTD) caused by mutations in the gene encoding transforming growth factor-ß receptors â and â ¡. Patients with LDS manifest spontaneous aneurysms and dissections of the aorta and peripheral artery. We report a successful treatment with a hybrid endovascular repair for a rapidly expanding thoracoabdominal aneurysm in a 41-year-old woman affected by LDS. To overcome the difficulties of anatomical and surgical repair, we applied an original strategy using surgeon-modified fenestrated endografts.