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PURPOSE: To demonstrate the efficacy of a combination of Amplatzer vascular plug (AVP2) and Endologix AFX for a visceral entry closure. CASE REPORT: A 70-year-old woman with treated chronic type B aortic dissection was seen to have sac enlargement. An isolated residual tear was observed at the origin of the celiac artery. A 12 mm AVP2 was successfully deployed for the entry closure. Angiography after the plug deployment still demonstrated residual flow to the false lumen. Hence, an Endologix AFX VELA proximal endograft was deployed, covering the AVP membrane and leaving no endoleak. CONCLUSION: Deployment of a vascular plug with Endologix AFX lining was efficacious for securing complete closure of an entry tear at the origin of the celiac artery in our patient. CLINICAL IMPACT: Vascular plugs are reported to be effective for closing small entries, although their effectiveness is limited when the entry site is not located on the flat intima, or in a high-flow situation. In the presented case, an isolated entry tear at the origin of the celiac artery was successfully closed with a vascular plug following Endologix AFX stent-graft lining of the true lumen.
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INTRODUCTION: Neurologic complications of open thoracic aortic surgery are devastating problems in patients with severely diseased aortas. This study aimed to clarify whether directing the aortic cannula tip toward the aortic root affects the postoperative cardiac function in patients undergoing open thoracic aortic surgery. METHODS: A total of 16 patients who underwent total or partial arch replacement between January 2014 and April 2019 were enrolled and divided into two groups. Ascending aorta perfusion was performed by placing the cannula tip toward the aortic root (reversed direction group, seven patients) or toward the aortic arch (standard direction group, nine patients). Intraoperative and perioperative data, including mortality, morbidity, and postoperative cardiac function, were compared between the groups. RESULTS: There were no hospital deaths or stroke events in either group. The aortic cross-clamping time was 102.4 ± 20.3 minutes in the reversed direction group and 87.1 ± 9.9 minutes in the standard direction group (p = 0.049). Furthermore, the intubation time was 28.4 ± 12.9 hours in the reversed direction group and 12.4 ± 6.8 hours in the standard direction group (p = 0.022). Both times were significantly longer in the reverse direction group. Postoperative serum creatine kinase-MB levels were significantly lower in the reversed direction group (6.2 ± 3.3 U/L vs 13.3 ± 4.8 U/L, respectively, p = 0.006). The cardiac output and cardiac index did not significantly differ. CONCLUSIONS: Directing the aortic cannula tip toward the aortic root does not adversely affect the postoperative cardiac function after aortic arch surgery.
Assuntos
Aorta Torácica , Cânula , Aorta/cirurgia , Aorta Torácica/cirurgia , Humanos , Perfusão , Período Pós-OperatórioRESUMO
A 63-year-old man was admitted to our hospital owing to the shortness of breath. He had undergone ascending aortic repair for acute type-A aortic dissection 14 years ago. In the previous surgery, the primary entry of the ascending aorta had been resected using direct end-to-end anastomosis after transecting the ascending aorta at the level of the entry and gluing the false lumen using a gelatin-resorcin-formalin glue. The anastomosis site on the ascending aorta had been reinforced using Teflon felt strips. The patient developed heart failure owing to severe aortic regurgitation caused by aortic root dilatation. Since the aortic arch was also dilated, he underwent aortic arch and root replacement. The distinctive difficulties experienced during surgery owing to the prior ascending aortic direct repair have been highlighted in this report.
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Aneurisma da Aorta Torácica , Dissecção Aórtica , Dissecção Aórtica/cirurgia , Aorta/cirurgia , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Aneurisma da Aorta Torácica/cirurgia , Formaldeído , Gelatina , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
A 74-year-old woman developed inferior myocardial infarction due to right coronary artery occlusion and underwent percutaneous coronary intervention. Two days later, echocardiography revealed ventricular septal rupture, and Impella CP was inserted to avoid emergency surgery. However, the patient's hemodynamics deteriorated rapidly, necessitating additional venoarterial extracorporeal membranous oxygenation support with concomitant Impella support (ECPELLA). The ventricular septal rupture was surgically repaired using the extended sandwich technique via a right ventricular approach; the ascending aorta was clamped with the clampable portion of the Impella. The patient was successfully weaned from the Impella 3 days postsurgery. This case suggests that urgent surgery with ECPELLA support could be a useful option for patients with ventricular septal rupture, even in severe cases wherein emergency surgery is unavoidable.
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Oxigenação por Membrana Extracorpórea , Ruptura do Septo Ventricular , Idoso , Ecocardiografia , Feminino , Ventrículos do Coração , Hemodinâmica , Humanos , Ruptura do Septo Ventricular/etiologia , Ruptura do Septo Ventricular/cirurgiaRESUMO
A 73-year-old woman with Valsalva aneurysm and mitral regurgitation was introduced to our department. We performed combined operation including aortic root partial repair and mitral valve repair. After wenning from cardiopulmonary bypass, intraoperative aortic dissection was confirmed by transesophageal echocardiography from the ascending aorta to the descending aorta, entry was near to proximal anastomosis line of the ascending aorta. To avoid heart failure, the ascending aorta wrapping by prothesis graft was performed to protect from urgent rupture postoperative at first stage. According to contrast computed tomography (CT) findings, location of entry was correspond with aortic clamping. We performed partial aortic replacement including innominate artery reconstruction for her at fourth day postoperative for treating aortic dissection at second stage. Postoperative course was uneventful.
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Dissecção Aórtica , Insuficiência da Valva Mitral , Idoso , Dissecção Aórtica/diagnóstico por imagem , Dissecção Aórtica/cirurgia , Aorta/diagnóstico por imagem , Aorta/cirurgia , Tronco Braquiocefálico , Ecocardiografia Transesofagiana , Feminino , Humanos , Insuficiência da Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/cirurgiaRESUMO
Coral reef Aortic Syndrome can result in significant visceral and lower limb ischemia. We present a 72-year-old male with postprandial abdominal pain and intermittent claudication. Computed tomography demonstrated a calcified plaque occluding the thoracoabdominal aorta. Additionally, the celiac axis was stenotic, and the superior mesenteric artery was completely occluded. The origin of the inferior mesenteric artery was aneurysmal. Aortic rerouting from the ascending to the infrarenal aorta was performed. The superior mesenteric artery was reconstructed with a saphenous vein, and the inferior mesenteric artery was divided and anastomosed directly to the aortic bypass. The procedure resulted in complete relief from the ischemic symptoms.
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Aorta/cirurgia , Doenças da Aorta/cirurgia , Aterosclerose/cirurgia , Implante de Prótese Vascular , Artéria Mesentérica Inferior/cirurgia , Artéria Mesentérica Superior/cirurgia , Veia Safena/transplante , Calcificação Vascular/cirurgia , Idoso , Anastomose Cirúrgica , Aorta/diagnóstico por imagem , Doenças da Aorta/complicações , Doenças da Aorta/diagnóstico por imagem , Aterosclerose/complicações , Aterosclerose/diagnóstico por imagem , Prótese Vascular , Implante de Prótese Vascular/instrumentação , Humanos , Masculino , Artéria Mesentérica Inferior/diagnóstico por imagem , Artéria Mesentérica Superior/diagnóstico por imagem , Síndrome , Resultado do Tratamento , Calcificação Vascular/complicações , Calcificação Vascular/diagnóstico por imagemRESUMO
BACKGROUND: Acute pulmonary embolism (PE) is a major threat to the health and lives of hospitalized patients. This study was conducted to clarify the real-world outcomes of pulmonary embolectomy.MethodsâandâResults:Retrospective investigation of 355 patients who underwent pulmonary embolectomy for acute PE was conducted using the Japanese Cardiovascular Surgery Database. Risk factors for operative death within 30 days after pulmonary embolectomy and major adverse cardiovascular events (MACE), including operative death, postoperative stroke and postoperative coma, were analyzed. Cardiopulmonary resuscitation (CPR) was required preoperatively in 27.6%, and preoperative veno-arterial extracorporeal membrane oxygenation was performed in 26.5%. Urgent or emergency operation was performed in 93% of patients. Operative mortality rate was 73/355 (20.6%). Incidence of MACE was 97/355 (27.3%). In univariate analysis, preoperative predictors of death were obesity, renal dysfunction, chronic obstructive pulmonary disease, liver injury, recent myocardial infarction, shock, refractory shock, CPR, heart failure, inotrope use, poor left ventricular function, preoperative arrhythmia and tricuspid regurgitation. In multivariate analysis, independent risk factors for operative death were heart failure (P=0.013), poor left ventricular function (P=0.007), and respiratory failure (P=0.001). Poor left ventricular function (P=0.033), preoperative CPR (P=0.002) and respiratory failure (P=0.007) were independent risk factors for MACE. CONCLUSIONS: The outcomes of pulmonary embolectomy were acceptable, considering the urgency and preoperative comorbidities of patients. Early triage of patients with hemodynamically unstable PE is important.
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Embolectomia/métodos , Pulmão/cirurgia , Embolia Pulmonar/cirurgia , Doença Aguda , Idoso , Reanimação Cardiopulmonar , Bases de Dados Factuais , Embolectomia/efeitos adversos , Embolectomia/mortalidade , Oxigenação por Membrana Extracorpórea , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Embolia Pulmonar/mortalidade , Estudos Retrospectivos , Fatores de Risco , Análise de Sobrevida , Resultado do Tratamento , TriagemRESUMO
Cardiac surgery on a patient with cold agglutinin disease is high risk for thromboembolism due to hypothermia perioperative. A 75-year-old woman with cold agglutinin disease underwent aortic valve replacement for severe aortic stenosis. Cold antibody was detected by preoperative screening test for blood transfusion. In order to prevent thromboembolic event during the operation, we maintained rectal temperature at around 36 degrees centigrade during the cardiopulmonary bypass by warming blood in the bypass circuit. Furthermore, antegrade warm blood cardioplegia was injected intermittently for keeping cardiac arrest. There was no thromboembolic event perioperatively.
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Anemia Hemolítica Autoimune/complicações , Estenose da Valva Aórtica/cirurgia , Valva Aórtica/cirurgia , Idoso , Estenose da Valva Aórtica/complicações , Temperatura Corporal , Feminino , Implante de Prótese de Valva Cardíaca , Humanos , Reto/fisiologia , Resultado do TratamentoRESUMO
A 73-year-old man was admitted for cardiac tamponade by oozing type left ventricular free wall rupture associated with acute myocardial infarction. Transthoracic echocardiography demonstrated moderate pericardial effusion and the presence of pseudoaneurysm within posterior wall. He went into shock with a systolic blood pressure of 60 mmHg. After introducing percutaneous cardiopulmonary support and intraaortic balloon pump, the sutureless repair was performed immediately. After having rehabilitation for right-sided hemiparesis, an elective pseudoaneurysm repair was planned. While a waiting an operation, 7 weeks later, he went into shock again with chest pain. Echocardiography and computed tomography demonstrated much amount of pericardial bloody effusion on the posterior aspect and 1.5 cm defect on the pseudoaneurysm wall. Emergently a patch closure with a bovine pericardium was performed using cardiopulmonary bypass under ventricular fibrillation through a left thoracotomy. Postoperative course was uneventful.
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Ruptura Cardíaca Pós-Infarto/cirurgia , Ruptura Cardíaca/cirurgia , Infarto do Miocárdio/cirurgia , Idoso , Ruptura Cardíaca/diagnóstico por imagem , Ruptura Cardíaca/etiologia , Ruptura Cardíaca Pós-Infarto/diagnóstico por imagem , Ruptura Cardíaca Pós-Infarto/etiologia , Humanos , Masculino , Imagem Multimodal , Infarto do Miocárdio/complicações , ReoperaçãoRESUMO
A 71-year-old man underwent total arch replacement for the true aortic arch aneurysm. On the post-operative day (POD) 10, right hemiplegia and motor aphasia occurred, and it was revealed that there were multiple cerebral infarction in brain computer tomography scan and magnetic resonanse imaging. Furthermore, platelet count has declined significantly from POD 15, so we suspected that heparin-induced thrombocytopenia might occurred. Then, we stopped continuous injection of heparin and administered argatroban and warfarin. In blood examinations, anti-platelet factor 4(PF4)/ heparin antibody measured by latex turbidimetry significantly increased at 5.2 U/ml, and specific immunoglobulin G for PF4/ heparin was also significantly high( optical density 2.334, cut off 0.400). Measurement of platelet derived microparticles produced by stimulation using various dose of heparin( functional assay) indicated typical pattern observed in heparin-induced thrombocytopenia. Thereafter, platelet count recovered and the patient recovered without another thromboembolic event.
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Aorta Torácica/cirurgia , Heparina/efeitos adversos , Trombocitopenia/induzido quimicamente , Idoso , Aneurisma Aórtico/cirurgia , Humanos , Masculino , Complicações Pós-OperatóriasRESUMO
BACKGROUND: Surgical pulmonary artery thrombectomy is a well-established emergency treatment for massive pulmonary embolism (PE) in which fibrinolysis or thrombolysis are not effective. However, surgery for massive PE that requires peripheral pulmonary artery thrombus removal remains challenging. We established a simple and secure pulmonary artery thrombectomy method using cardiopulmonary bypass and cardiac arrest. In this procedure, the surgical assistant arm, typically used for coronary artery bypass grafting, is used to obtain a feasible working space during thrombectomy. CASE PRESENTATION: We present seven consecutive massive PE cases that were treated with the present surgical method and successfully weaned from cardiopulmonary bypass or extracorporeal membrane oxygenation postoperatively. CONCLUSIONS: This procedure can be used to prevent right ventricular failure after surgery as surgeons can remove the peripheral thrombus with clear vision up to the second branch of the pulmonary artery.
Assuntos
Parada Cardíaca , Embolia Pulmonar , Trombose , Humanos , Resultado do Tratamento , Trombectomia/métodos , Embolia Pulmonar/cirurgia , Parada Cardíaca/etiologia , Parada Cardíaca/cirurgia , Artéria Pulmonar/cirurgia , Trombose/cirurgiaRESUMO
BACKGROUND: During the coronavirus disease (COVID-19) pandemic, medical resources have often been limited to emergency surgeries. This study aimed to evaluate our experience with delayed surgery for acute type A aortic dissections (ATAADs). METHODS: A retrospective study was conducted on 33 patients who underwent surgery for ATAADs between January 2020 and December 2021. The patients were divided into two groups: patients treated within 12 h of arrival (E group; N = 21) and those treated > 12 h after arrival (D group; N = 12) with strict antihypertensive therapy until surgery. RESULTS: The plasma fibrinogen levels on arrival were lower in the D group than in the E group (174.3 ± 109.1 vs 293.4 ± 165.4, p = 0.038). The time to surgery from symptom onset was longer in the D group than in the E group (4 ± 1 h vs. 86 ± 108 h, p < 0.001). There was one case (3%) of mortality and seven cases (21%) of cerebral infarctions in the E group. There was no significant difference in the intraoperative data and quantity of blood transfused between the two groups. CONCLUSION: Thus, delayed surgery for ATAAD with appropriate preoperative management may be an alternative surgical strategy in the COVID-19 era.
Assuntos
Dissecção Aórtica , COVID-19 , Humanos , Estudos Retrospectivos , Dissecção Aórtica/cirurgia , Testes de Coagulação SanguíneaRESUMO
BACKGROUND: Late gadolinium enhanced (LGE) cardiovascular magnetic resonance (CMR) is frequently used to evaluate myocardial viability, estimate total infarct size and transmurality, but is not always straightforward is and contraindicated in patients with renal failure because of the risk of nephrogenic systemic fibrosis. T2- and T1-weighted CMR alone is however relatively insensitive to chronic myocardial infarction (MI) in the absence of a contrast agent. The objective of this manuscript is to explore T1ρ-weighted rotating frame CMR techniques for infarct characterization without contrast agents. We hypothesize that T1ρ CMR accurately measures infarct size in chronic MI on account of a large change in T1ρ relaxation time between scar and myocardium. METHODS: 7Yorkshire swine underwent CMR at 8 weeks post-surgical induction of apical or posterolateral myocardial infarction. Late gadolinium enhanced and T1ρ CMR were performed at high resolution to visualize MI. T1ρ-weighted imaging was performed with a B1 = 500 Hz spin lock pulse on a 3 T clinical MR scanner. Following sacrifice, the heart was excised and infarct size was calculated by optical planimetry. Infarct size was calculated for all three methods (LGE, T1ρ and planimetry) and statistical analysis was performed. T1ρ relaxation time maps were computed from multiple T1ρ-weighted images at varying spin lock duration. RESULTS: Mean infarct contrast-to-noise ratio (CNR) in LGE and T1ρ CMR was 2.8 ± 0.1 and 2.7 ± 0.1. The variation in signal intensity of tissues was found to be, in order of decreasing signal intensity, LV blood, fat and edema, infarct and healthy myocardium. Infarct size measured by T1ρ CMR (21.1% ± 1.4%) was not significantly different from LGE CMR (22.2% ± 1.5%) or planimetry (21.1% ± 2.7%; p < 0.05).T1ρ relaxation times were T1ρinfarct = 91.7 ms in the infarct and T1ρremote = 47.2 ms in the remote myocardium. CONCLUSIONS: T1ρ-weighted imaging using long spin locking pulses enables high discrimination between infarct and myocardium. T1ρ CMR may be useful to visualizing MI without the need for exogenous contrast agents for a wide range of clinical cardiac applications such as to distinguish edema and scar tissue and tissue characterization of myocarditis and ventricular fibrosis.
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Ventrículos do Coração/patologia , Imagem Cinética por Ressonância Magnética/métodos , Contração Miocárdica/fisiologia , Infarto do Miocárdio/patologia , Miocárdio/patologia , Animais , Doença Crônica , Meios de Contraste/administração & dosagem , Modelos Animais de Doenças , Seguimentos , Ventrículos do Coração/fisiopatologia , Injeções Intravenosas , Imagem Cinética por Ressonância Magnética/efeitos adversos , Meglumina/administração & dosagem , Infarto do Miocárdio/fisiopatologia , Reprodutibilidade dos Testes , SuínosRESUMO
A 70-year-old man was admitted to our hospital with a thoracic aortic aneurysm. He underwent elective thoracic endovascular aneurysm repair with left common carotid artery-left subclavian artery bypass via the left supraclavicular approach. During the bypass, the thoracic duct was injured and ligated. On postoperative day 3, the patient complained of dyspnea. Chest radiography revealed a massive right-sided pleural effusion. On postoperative day 5, he was diagnosed with right-sided chylothorax and underwent chest tube insertion. The next day, a left-sided chylothorax was noted, and chest tube drainage was performed. Conservative management, including nil per os and subcutaneous octreotide (300 µg/day) injection, was carried out for 2 weeks; subsequently, the chylothorax improved. This report highlights the diagnostic challenge of right-sided chylothorax after debranching thoracic endovascular aneurysm repair.
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Aneurisma da Aorta Abdominal , Aneurisma da Aorta Torácica , Implante de Prótese Vascular , Quilotórax , Procedimentos Endovasculares , Idoso , Aneurisma da Aorta Abdominal/cirurgia , Aneurisma da Aorta Torácica/complicações , Aneurisma da Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/cirurgia , Implante de Prótese Vascular/efeitos adversos , Quilotórax/diagnóstico por imagem , Quilotórax/etiologia , Quilotórax/terapia , Procedimentos Endovasculares/efeitos adversos , Humanos , Masculino , Resultado do TratamentoRESUMO
We have presented the case of a 76-year-old patient with a type V thoracoabdominal aortic aneurysm treated by retrograde in situ branched stent grafting to the superior mesenteric artery (SMA). Via a 9-cm, median laparotomy, a percutaneous transhepatic gallbladder drainage needle was inserted from the distal part of the first jejunal artery to the origin of the SMA. After stent graft placement into the aorta, the graft was retrogradely punctured using the percutaneous transhepatic gallbladder drainage needle. The SMA ischemic time was 6 minutes. The puncture site was dilated, and a small, covered stent was deployed. Postoperative computed tomography scanning showed no endoleak with sac regression.
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Increased myocardial wall stress after myocardial infarction (MI) initiates the process of adverse left ventricular (LV) remodeling that is manifest as progressive LV dilatation, loss of global contractile function, and symptomatic heart failure, and recent work has shown that reduction in wall stress through injectable bulking agents attenuates these outcomes. In this study, hyaluronic acid (HA) was functionalized to exhibit controlled and tunable mechanics and degradation once cross-linked, in an attempt to assess the temporal dependency of mechanical stabilization in LV remodeling. Specifically, two hydrolytically degrading (low and high HeMA-HA, degrading in ~3 and 10 weeks, respectively) and two stable (low and high MeHA, little mass loss even after 8 weeks) hydrogels with similar initial mechanics (low: ~7 kPa; high: ~35-40 kPa) were evaluated in an ovine model of MI. Generally, the more stable hydrogels maintained myocardial wall thickness in the apical and basilar regions more efficiently (low MeHA: apical: 6.5 mm, basilar: 7 mm, high MeHA: apical: 7.0 mm basilar: 7.2 mm) than the hydrolytically degrading hydrogels (low HeMA-HA: apical: 3.5 mm, basilar: 6.0 mm, high HeMA-HA: apical: 4.1 mm, basilar: 6.1 mm); however, all hydrogel groups were improved compared to infarct controls (IC) (apical: 2.2 mm, basilar: 4.6 mm). Histological analysis at 8 weeks demonstrated that although both degradable hydrogels resulted in increased inflammation, all treatments resulted in increased vessel formation compared to IC. Further evaluation revealed that while high HeMA-HA and high MeHA maintained reduced LV volumes at 2 weeks, high MeHA was more effective at 8 weeks, implying that longer wall stabilization is needed for volume maintenance. All hydrogel groups resulted in better cardiac output (CO) values than IC.
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Ácido Hialurônico/análogos & derivados , Ácido Hialurônico/uso terapêutico , Hidrogéis/uso terapêutico , Infarto do Miocárdio/terapia , Remodelação Ventricular , Implantes Absorvíveis , Animais , Débito Cardíaco , Volume Cardíaco , Vasos Coronários/patologia , Reagentes de Ligações Cruzadas/química , Hidrogéis/síntese química , Inflamação , Injeções , Masculino , Metilmetacrilatos/química , Infarto do Miocárdio/patologia , Infarto do Miocárdio/fisiopatologia , Miocárdio/patologia , Neovascularização Fisiológica , Ovinos , ViscosidadeRESUMO
A 24-year-old Japanese woman underwent ilioaxillary bypass with an expanded polytetrafluoroethylene graft and axillocarotid bypass with an autologous saphenous vein graft for severe brain ischemia due to Takayasu arteritis. A method that involved wrapping strips of the graft around the artery was used to prevent stretching of the anastomotic site. Her general condition and symptoms improved remarkably. She became pregnant three times and delivered the infants without any complications caused by the operation. The present case contributes to proof of patency, effectiveness, and durability of these bypass grafts.
Assuntos
Isquemia Encefálica/cirurgia , Revascularização Cerebral , Complicações Cardiovasculares na Gravidez/cirurgia , Arterite de Takayasu/complicações , Adulto , Anastomose Cirúrgica/métodos , Artéria Axilar/cirurgia , Implante de Prótese Vascular , Isquemia Encefálica/etiologia , Artéria Carótida Primitiva/cirurgia , Infarto Cerebral/etiologia , Infarto Cerebral/cirurgia , Parto Obstétrico , Feminino , Humanos , Artéria Ilíaca/cirurgia , Recém-Nascido , Politetrafluoretileno , Gravidez , Veia Safena/transplante , Grau de Desobstrução VascularRESUMO
Using autologous common femoral artery and external oblique muscle fascia is a simple and reliable option for repairing infected aortic pseudoaneurysms. Reoperation of infected pseudoaneurysms is challenging and requires secure aortic repair with complete infection eradication. Here, we report two cases of infected pseudoaneurysms in the ascending aorta cannulation site after cardiac surgeries. Common femoral arteries and fascia were harvested in the same lesion as repair materials. The aortic pseudoaneurysms were repaired under deep hypothermic circulatory arrest. Femoral arterial patches were reinforced with circumferential aponeurosis strips. There was no infection recurrence or repair site dilatation in the long-term follow up of both patients.
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Falso Aneurisma , Artéria Femoral , Falso Aneurisma/diagnóstico por imagem , Falso Aneurisma/etiologia , Falso Aneurisma/cirurgia , Fáscia , Artéria Femoral/diagnóstico por imagem , Artéria Femoral/cirurgia , Humanos , Reoperação , Procedimentos Cirúrgicos VascularesRESUMO
We report a case of periaortic lymphoma mimicking Stanford type B acute aortic dissection treated for impending rupture by thoracic endovascular aortic repair. Although no endoleak was detected, the aneurysm enlarged continuously. Repeat computed tomography scans showed that an aortic aneurysm-like structure around the stent graft had enlarged irregularly. Histopathological examination revealed diffuse large B-cell malignant lymphoma. Post-chemotherapy, the aneurysm-like structure disappeared without any fistula or rupture. In open surgery, differentiating between aneurysms and malignancy is easy under direct vision; however, in the endovascular surgery era, this is a pitfall because no surgical specimen of the lesion can be obtained.
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Approximately 20% of aortic aneurysm and/or dissection (AAD) cases result from inherited disorders, including several systemic and syndromatic connective-tissue disorders, such as Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome, which are caused by mutations in the FBN1, COL3A1, and TGFBR1 and TGFBR2 genes, respectively. Nonsyndromatic AAD also has a familial background, and mutations of the ACTA2 gene were recently shown to cause familial AAD. In the present study, we conducted sequence analyses of the ACTA2 gene in 14 unrelated Japanese patients with familial thoracic AAD (TAAD), and in 26 with sporadic and young-onset TAAD. Our results identified three mutations of ACTA2, two novel [p.G152_T205del (c.616+1G>T), p.R212Q] and one reported (p.R149C), in the 14 patients with familial TAAD, and a novel mutation (p.Y145C) of ACTA2 in the 26 sporadic and young-onset TAAD patients, each of which are considered to be causative for TAAD. Some of the clinical features of these patients were the same as previously reported, whereas others were different. These findings confirm that ACTA2 mutations are important in familial TAAD, while the first sporadic and young-onset TAAD case with an ACTA2 mutation was also identified.