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1.
J Artif Organs ; 2023 Jun 08.
Artigo em Inglês | MEDLINE | ID: mdl-37291209

RESUMO

The St. Jude Medical Epic Supra valve is a porcine bioprosthesis designed for complete supraannular implantation. No report has shown the hemodynamic performance and clinical outcomes of aortic valve replacement with the Epic Supra valve for severe aortic stenosis in a Japanese cohort. We retrospectively evaluated 65 patients who underwent aortic valve replacement using the Epic Supra valve for aortic stenosis at our department between May, 2011 and October, 2016. The mean follow-up period was 68.7 ± 32.7 months, and the follow-up rate was 89.2%. The mean age was 76.8 ± 5.3 years. The 1-, 5-, and 8-year survival rates were 96.9%, 79.4%, and 60.3%, respectively. The rates of freedom from valve-related events were 96.6% and 81.9% at 5 and 8 years, respectively. Four patients were diagnosed with structural valve deterioration (SVD), and reintervention was performed in two patients. The rates of freedom from SVD were 98.2% and 83.3% at 5 and 8 years, respectively, and the mean time to diagnosis of SVD was 72.5 ± 25.3 months. The mean pressure gradient (MPG) was 16.8 ± 6.0 mmHg postoperatively, 17.5 ± 9.4 mmHg at 5 years, and 21.2 ± 12.4 mmHg at 8 years (p = 0.08). The effective orifice area index (EOAI) was 0.95 ± 0.2 cm2/m2 immediately after surgery, 0.96 ± 0.27 cm2/m2 at 5 years, and 0.84 ± 0.2 cm2/m2 at 8 years (p = 0.10). An increase in MPG and decrease in EOAI were also observed, which may be associated with SVD. Follow-up after 5 years is important to determine if there is an increase.

2.
Cardiol Young ; 33(4): 660-662, 2023 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35912591

RESUMO

We experienced a case of a 1-year-old female with congenital tricuspid valve regurgitation caused by tricuspid valve dysplasia. The anterior and septal leaflets were particularly dysplastic, and leaflet tethering was observed. The anterior papillary muscle was approximated to the interventricular septum, and a commissural edge-to-edge suture was inserted on the anteroseptal commissure. Tricuspid valve regurgitation improved to be trivial after surgery and has not worsened 1 year later. Papillary muscle approximation could be one of the feasible reparative techniques for congenital tricuspid valve regurgitation.


Assuntos
Cardiopatias Congênitas , Insuficiência da Valva Tricúspide , Feminino , Humanos , Lactente , Valva Tricúspide/diagnóstico por imagem , Valva Tricúspide/cirurgia , Valva Tricúspide/anormalidades , Insuficiência da Valva Tricúspide/etiologia , Insuficiência da Valva Tricúspide/cirurgia , Músculos Papilares/cirurgia , Cardiopatias Congênitas/cirurgia
3.
Cardiol Young ; 33(9): 1772-1774, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-37038834

RESUMO

We performed coronary artery bypass grafting with the internal thoracic artery in three infants. Grafts were patent in all cases. One patient was lost due to chronic heart failure. Coronary artery bypass grafting can be performed even in infancy, and early surgical intervention may be necessary when myocardial ischaemia is recognised.


Assuntos
Doença da Artéria Coronariana , Insuficiência Cardíaca , Isquemia Miocárdica , Humanos , Criança , Ponte de Artéria Coronária , Insuficiência Cardíaca/cirurgia
4.
Kyobu Geka ; 76(8): 638-641, 2023 Aug.
Artigo em Japonês | MEDLINE | ID: mdl-37500553

RESUMO

A 64-year-old man was transferred to our hospital due to a diagnosis of Stanford type A acute aortic dissection complicated by cardiac tamponade. He was in shock status as well. Careful inspection of contrast- enhanced computed tomography revealed Kommerell's diverticulum with the aberrant right subclavian artery running behind the esophagus. The artery connected to the right axillary artery. The left vertebral artery was separately branched from the aortic arch. Primary entry was not detected on the preoperative computed tomography( CT). Left ventricular function was preserved by transthoracic echocardiography. At emergency surgery, total aortic arch replacement with reconstruction of the right axillary artery, both carotid arteries, and the left subclavian artery along with the left vertebral artery was successfully performed. The aberrant right subclavian artery was ligated at the origin. Kommerell' s diverticulum was completely excluded. Postoperative contrast-enhanced CT showed the patency of all reconstructed arteries. Although he suffered from acute cholecystitis and persistent bilateral pleural effusion, he was discharged in a good condition.


Assuntos
Dissecção Aórtica , Divertículo , Masculino , Humanos , Pessoa de Meia-Idade , Artéria Subclávia/diagnóstico por imagem , Artéria Subclávia/cirurgia , Dissecção Aórtica/complicações , Dissecção Aórtica/diagnóstico por imagem , Dissecção Aórtica/cirurgia , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Divertículo/complicações , Divertículo/diagnóstico por imagem , Divertículo/cirurgia
5.
Kyobu Geka ; 76(6): 419-421, 2023 Jun.
Artigo em Japonês | MEDLINE | ID: mdl-37258017

RESUMO

Whereas cerebral aneurysm is a well-known consequence of autosomal dominant polycystic kidney disease (ADPKD), acute aortic dissection has been rarely reported. A patient was a 44-year-old male with a diagnosis of ADPKD, who had previously undergone transcatheter arterial embolization for a renal cyst hemorrhage. He presented with sudden onset of back pain, which got worse at emergency service. Contrast-enhanced computed tomography (CT) revealed Stanford type A acute aortic dissection. The patient subsequently underwent partial aortic arch replacement with a vascular graft under circulatory arrest. His postoperative course was complicated by pneumonia and required ventilation support for a week. Peak creatinine level was 3.28 mg/dl, but hemodialysis was not required. Patients with ADPKD should be considered a high-risk cohort of aortic dissection.


Assuntos
Aneurisma da Aorta Torácica , Dissecção Aórtica , Cistos , Parada Cardíaca , Doenças Renais Policísticas , Rim Policístico Autossômico Dominante , Masculino , Humanos , Adulto , Rim Policístico Autossômico Dominante/complicações , Rim Policístico Autossômico Dominante/diagnóstico por imagem , Rim Policístico Autossômico Dominante/cirurgia , Dissecção Aórtica/complicações , Dissecção Aórtica/diagnóstico por imagem , Doenças Renais Policísticas/complicações , Doenças Renais Policísticas/diagnóstico por imagem , Doenças Renais Policísticas/cirurgia , Dor nas Costas , Parada Cardíaca/complicações , Aneurisma da Aorta Torácica/complicações , Aneurisma da Aorta Torácica/diagnóstico por imagem , Doença Aguda
6.
Kyobu Geka ; 76(6): 477-480, 2023 Jun.
Artigo em Japonês | MEDLINE | ID: mdl-37258029

RESUMO

Ascending aorta thrombosis unaccompanied by an aneurysm or a primary hypercoagulable state is rare. We report a surgical case of ascending aorta thrombosis with multiple emboli. A 44-year-old woman visited the hospital for evaluation of dysarthria and was diagnosed with multiple cerebral infarcts. Contrast-enhanced computed tomography (CT) revealed a mass in the ascending aorta and the brachiocephalic artery. We performed emergency removal of the masses and endarterectomy with cardiopulmonary bypass under hypothermic circulatory arrest. Histopathological examination of the resected specimen showed thrombi. The patient had an uneventful recovery and was discharged 12 days postoperatively. No recurrent thrombus or hypercoagulable state was observed for 3 years postoperatively.


Assuntos
Doenças da Aorta , Embolia , Trombose , Feminino , Humanos , Adulto , Trombose/complicações , Trombose/diagnóstico por imagem , Doenças da Aorta/complicações , Doenças da Aorta/diagnóstico por imagem , Doenças da Aorta/cirurgia , Embolia/diagnóstico por imagem , Embolia/etiologia , Embolia/cirurgia , Aorta/diagnóstico por imagem , Aorta/cirurgia , Ponte Cardiopulmonar
7.
J Artif Organs ; 25(4): 323-328, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35129732

RESUMO

The INSPIRIS RESILIA aortic bioprosthesis (Edwards Lifesciences LLC, Irvine, USA) was fixed in novel tissue preservation technology to ensure long-term durability. Hemodynamic performance after aortic valve replacement (AVR) for severe aortic stenosis (AS) has not been published in the Japanese cohort. Twenty-nine patients underwent AVR with INSPIRIS RESILIA bioprosthesis for severe AS between November 1, 2018 and December 31, 2020. The mean age was 75.1 ± 4.5 years with 19 female patients. Body surface area was 1.58 ± 0.19 m2 and New York Heart Association functional class was 2.2 ± 0.5. Hemodynamic performance was assessed using follow-up transthoracic echocardiographic data collected at 3-6 months, 1 year and 2 years. The mean follow-up time was 19.2 ± 7.2 months, with a 100% follow-up rate. One patient died of postoperative heart failure. The preoperative mean and peak transvalvular pressure gradients (PGs) were 51.9 ± 18.4 mmHg and 89.3 ± 34.9 mmHg, respectively, and effective orifice area 0.72 ± 0.26 cm2. They improved at 10.2 ± 3.5 mmHg (p < 0.0001), 19.3 ± 6.6 mmHg (p < 0.0001) and 1.73 ± 0.47cm2 (p < 0.0001) at discharge. The mean transvalvular PG at 3-6 months (n = 24), 1 year (n = 25) and 2 years (n = 15) was 11.2 ± 3.8 mmHg (p < 0.0001), 11.1 ± 3.2 mmHg (p < 0.0001) and 11.2 ± 3.3 mmHg (p < 0.0001), respectively. Left ventricular mass index decreased from 123.0 ± 35.0 g/m2 before surgery to 113.4 ± 35.0 g/m2 (p = 0.0133) at discharge. It has dropped to 88.0 ± 25.0 g/m2 (p = 0.0007) at 2 years. Constrictive pericarditis caused heart failure in one patient. INSPIRIS RESILIA bioprosthesis showed improved hemodynamic performance in the early postoperative phase. There were fewer valve-related events observed.


Assuntos
Estenose da Valva Aórtica , Bioprótese , Insuficiência Cardíaca , Implante de Prótese de Valva Cardíaca , Próteses Valvulares Cardíacas , Humanos , Feminino , Idoso , Estenose da Valva Aórtica/cirurgia , Seguimentos , Japão , Desenho de Prótese , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Hemodinâmica , Insuficiência Cardíaca/cirurgia , Resultado do Tratamento
8.
Kyobu Geka ; 75(6): 467-471, 2022 Jun.
Artigo em Japonês | MEDLINE | ID: mdl-35618694

RESUMO

Guide wire fracture is a rare, yet potentially life-threatening complication of percutaneous coronary intervention. A 65-years old man underwent emergent coronary angioplasty for myocardial ischemia. Percutaneous coronary intervention for the residual lesions of left anterior descending artery (LAD) was planned. The protection guide wire for left circumflex artery( LCx) was entangled in the stent in LAD. Despite many attempts, the wire could not be retrieved. Finally, the wire was fractured and the stent in LAD was deformed. The patient was sent to our service to remove the fractured wire. We performed urgent removal of the guide wire and coronary artery bypass grafting( CABG). The postoperative course was uneventful. The timing of surgical removal of the guide wire and the indication for coronary artery reconstruction should be discussed by heart team.


Assuntos
Angioplastia Coronária com Balão , Doença da Artéria Coronariana , Idoso , Ponte de Artéria Coronária , Humanos , Masculino , Revascularização Miocárdica
9.
Kyobu Geka ; 75(3): 217-220, 2022 Mar.
Artigo em Japonês | MEDLINE | ID: mdl-35249957

RESUMO

A 48-year-old female patient was transferred with dysarthria and left-sided hemiplegia. Contrast-enhanced computed tomography revealed occlusion of the first branch of the right middle cerebral artery, for which an emergency thrombectomy was successfully performed within 2 hours of patient's initial symptoms. Postoperatively, transthoracic echocardiography revealed a massive mobile left atrial mass, measuring approximately 65×30 mm, a part of which moved in and out of the mitral valve without significant mitral regurgitation. Embolisms to the kidneys and the spleen were demonstrated. Another emergency cardiac surgery was performed, 4 hours after the thrombectomy, to resect the mass from the fossa oval with the atrial septum;the defect was closed using autologous pericardium. The histopathological findings of the specimen were consistent with a myxoma. The patient completely recovered and was discharged when ambulatory. We have discussed the importance of the timing of surgical intervention in the context of patients undergoing cardiac surgery after a cerebral embolism.


Assuntos
Neoplasias Cardíacas , Embolia Intracraniana , Mixoma , Feminino , Átrios do Coração/cirurgia , Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/cirurgia , Humanos , Embolia Intracraniana/diagnóstico por imagem , Embolia Intracraniana/etiologia , Embolia Intracraniana/cirurgia , Pessoa de Meia-Idade , Mixoma/complicações , Mixoma/diagnóstico por imagem , Mixoma/cirurgia , Trombectomia
10.
Kyobu Geka ; 75(13): 1074-1077, 2022 Dec.
Artigo em Japonês | MEDLINE | ID: mdl-36539221

RESUMO

Mycotic aneurysm of the aortic arch is a rare, but critical entity. We reviewed our surgical experience of mycotic aneurysm of the aortic arch. Between January 2007 and December 2015, we operated on six patients who had mycotic aneurysm of the aortic arch. The mean age was 72 years old, and four males were included. Preoperative white blood cell count was 18,266/µl and C-reactive peptide was 18.5 µg/dl, respectively. The initial presentations included fever( n=2), hoarseness( n=2), weakness of a leg( n=1), dyspnea (n=1) and hemoptysis (n=1). Preoperative blood cultures were positive in three patients. All patients underwent a total aortic arch repair with a four-branched vascular tube, and five received pedicled omental grafting. One patient who did not receive pedicled omental grafting died of recurrence of infection on postoperative day 21, and the other died of multi-organ failure on postoperative day 77. We experienced tracheostomy( n=1), minor stroke( n=1), and atrial fibrillation( n=1). During the follow-up period, no recurrence of infection was observed in four survivors. Our surgical strategy is satisfactory to achieve good clinical outcomes.


Assuntos
Aneurisma Infectado , Aneurisma da Aorta Torácica , Implante de Prótese Vascular , Procedimentos Endovasculares , Masculino , Humanos , Idoso , Aneurisma da Aorta Torácica/cirurgia , Aneurisma Infectado/diagnóstico por imagem , Aneurisma Infectado/cirurgia , Resultado do Tratamento , Estudos Retrospectivos , Stents
11.
Kyobu Geka ; 75(9): 663-666, 2022 Sep.
Artigo em Japonês | MEDLINE | ID: mdl-36156513

RESUMO

A 73-year-old female who underwent aortic valve replacement with a biological valve, coronary artery bypass, and left atrial appendage closure had sudden onset of nausea and abdominal pain 43 days after surgery. She had a history of nonocclusive mesenteric ischemia on 4th postoperative day, for which conservative management was successfully carried out. A contrast-enhanced computed tomography(CT) was performed because a recurrence of nonocclusive mesenteric ischemia was suspected. It revealed a whirl sign in the small intestine, suggestive of small intestine volvulus. At the subsequent emergency laparotomy, volvulus caused severe congestion in the small intestine, aproximately 40 cm from the cecum. However, there was no evidence of transmural necrosis, and reduction of torsion notably improved blood supply to the small intestine. Her regular diet was resumed on 4th postoperative day, and her postoperative course was uneventful. Volvulus should be considered as a differential diagnosis in the setting of acute abdominal pain after open-heart surgery.


Assuntos
Volvo Intestinal , Isquemia Mesentérica , Dor Abdominal/diagnóstico , Dor Abdominal/etiologia , Idoso , Ponte de Artéria Coronária/efeitos adversos , Feminino , Humanos , Volvo Intestinal/diagnóstico por imagem , Volvo Intestinal/etiologia , Intestino Delgado/cirurgia , Isquemia Mesentérica/diagnóstico por imagem , Isquemia Mesentérica/etiologia
12.
Kyobu Geka ; 74(5): 375-378, 2021 May.
Artigo em Japonês | MEDLINE | ID: mdl-33980799

RESUMO

Valve sparing aortic root replacement( VSRR) is currently an established option for patients with annuloaortic ectasia( AAE). A newly designed Valsalva graft, the J Graft Shield Neo Valsalva, was used for VSRR in three cases. All operations were successful and postoperative courses were uneventful. Graft designs closer in shape to the native Valsalva may contribute to the improvement of late outcomes in VSRR.


Assuntos
Aneurisma da Aorta Torácica , Insuficiência da Valva Aórtica , Implante de Prótese Vascular , Aorta/cirurgia , Aneurisma da Aorta Torácica/cirurgia , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/cirurgia , Humanos
13.
Cardiol Young ; 25(3): 560-2, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24666924

RESUMO

A Japanese girl first pointed out the mass in the left atrial appendage by the echocardiography on the 3rd day of life. At 30th day of life, the mass in the left atrial appendage was excised en bloc successfully under cardiopulmonary bypass because it appeared mobile. Its pathologic finding revealed an old organised thrombus. She is doing well with no episode of thrombus formation or thromboembolism, 2 years postoperatively.


Assuntos
Apêndice Atrial/patologia , Apêndice Atrial/cirurgia , Ponte Cardiopulmonar/métodos , Ecocardiografia , Trombose/patologia , Apêndice Atrial/diagnóstico por imagem , Feminino , Humanos , Recém-Nascido , Tomografia Computadorizada por Raios X , Resultado do Tratamento
14.
J Med Invest ; 71(1.2): 158-161, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38735713

RESUMO

We investigated impact of persistent malperfusion syndrome (MPS) following central repair of acute type A aortic dissection (ATAAD) on outcomes. Thirty patients who underwent central repair for ATAAD with MPS were included. Patients were divided into two groups:23 patients without MPS following central repair (No-MPS group) and 7 with MPS (Persistent-MPS group). The mean age was 66.8±9.6 and 59.4±13.4 years in the No-MPS and Persistent-MPS groups, respectively (P=0.176). Preoperative MPS included the left coronary artery (n=3), brain (n=3), abdomen (n=7), and extremities (n=11) in the No-MPS group. In the Persistent-MPS group, the right coronary (n=1), brain (n=2), abdomen (n=3), and extremities (n=5) were observed. In the No-MPS group, one patient died of extensive cerebral infarction (4.3%). In the Persistent-MPS group, 2 patients died of sepsis and multi-organ failure, respectively (28.6%) (P=0.061). The Persistent-MPS group had more patients requiring hemodialysis than the No-MPS group (P=0.009). Three patients underwent intestinal resection due to persistent MPS (P<0.001). Persistent MPS following central repair for ATAAD significantly contributed to outcomes. J. Med. Invest. 71 : 158-161, February, 2024.


Assuntos
Dissecção Aórtica , Humanos , Dissecção Aórtica/cirurgia , Masculino , Pessoa de Meia-Idade , Feminino , Idoso , Doença Aguda , Complicações Pós-Operatórias/etiologia , Resultado do Tratamento , Síndrome , Estudos Retrospectivos , Aneurisma Aórtico/cirurgia
15.
Gen Thorac Cardiovasc Surg ; 72(4): 216-224, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-37542572

RESUMO

OBJECTIVES: This study investigated early and late outcomes between ascending aorta/partial arch replacement and total arch replacement with entry resection in DeBakey type I acute aortic dissection (DIAAD) repair. METHODS AND RESULTS: This study included 98 patients who underwent DIAAD repair from January 2005 to December 2020. Seventy-four patients underwent ascending aorta and partial arch replacement with entry resection (Non-TAR group), and 24 underwent total arch replacement with entry resection (TAR group). The mean follow-up period was 4.8 ± 3.2 years. The follow-up rate was 92.3%. The mean age in the Non-TAR and TAR groups was 68.8 ± 10.4 years and 61.6 ± 13.7 years, respectively (P = 0.046). No difference in preoperative shock and malperfusion syndrome was observed between the groups. Hospital death was observed in 5.4% and 12.5% of the Non-TAR and TAR groups, respectively (P = 0.241). Postoperative permanent neurologic deficits and temporary hemodialysis were more frequently seen in the TAR compared to the Non-TAR group (P = 0.03 and 0.003, respectively). The 5-year survival rates were 95.1% ± 3.4% and 89.2% ± 7.2% in the Non-TAR and TAR groups, respectively (Log-rank P = 0.603). Freedom from downstream aorta-related reinterventions at 5 years was 87.8% ± 4.5% and 64.1% ± 11.0% in the Non-TAR and TAR groups, respectively (Log-rank P = 0.007). Three patients in each group underwent thoracic endovascular aortic repair for residual aortic dissection. CONCLUSIONS: Early and late outcomes in the Non-TAR group were satisfactory compared to those in the TAR group. Entry resection with graft replacement remains a standard approach in DIAAD repair.


Assuntos
Aneurisma da Aorta Torácica , Dissecção Aórtica , Implante de Prótese Vascular , Humanos , Pessoa de Meia-Idade , Idoso , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Aneurisma da Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/cirurgia , Implante de Prótese Vascular/efeitos adversos , Implante de Prótese Vascular/métodos , Resultado do Tratamento , Complicações Pós-Operatórias/cirurgia , Estudos Retrospectivos , Aorta/cirurgia , Dissecção Aórtica/diagnóstico por imagem , Dissecção Aórtica/cirurgia
16.
J Cardiothorac Surg ; 19(1): 130, 2024 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-38491553

RESUMO

GRAND FIX mesh-type plates and pins (Gunze, Kyoto, Japan) are thin, bioabsorbable fixation devices made of poly-L-lactide (PLLA) composite. These devices are new, and how best to use them in clinical practice remains to be determined. Thus, we describe our approach to rib fixation after thoracotomy in an aortic surgery using GRAND FIX mesh-type plates and pins. The advantage of our approach is that it is easy to perform, but careful attention should be paid to fix the ribs appropriately.


Assuntos
Costelas , Toracotomia , Humanos , Costelas/cirurgia , Pinos Ortopédicos , Poliésteres , Implantes Absorvíveis
17.
Asian Cardiovasc Thorac Ann ; 31(6): 512-514, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-37438909

RESUMO

Various surgical techniques have been reported for Scimitar syndrome, because of the heterogenous anatomy of the disease. We developed a novel surgical method to repair Scimitar syndrome, in which, a new pulmonary venous route is constructed behind the inferior vena cava using autologous flaps of the inferior vena cava and the interatrial septum. An adult case of Scimitar syndrome was repaired by this method with good results.


Assuntos
Septo Interatrial , Veias Pulmonares , Síndrome de Cimitarra , Adulto , Humanos , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/cirurgia , Síndrome de Cimitarra/diagnóstico por imagem , Síndrome de Cimitarra/cirurgia , Veia Cava Inferior/diagnóstico por imagem , Veia Cava Inferior/cirurgia , Feminino
18.
Asian Cardiovasc Thorac Ann ; 31(4): 357-359, 2023 May.
Artigo em Inglês | MEDLINE | ID: mdl-37093739

RESUMO

Implantable cardioverter defibrillators are implanted in children to prevent sudden death due to life-threatening ventricular arrhythmias. Transvenous electrode placement is difficult for children because of the small vessel size. Especially, defibrillator electrode placement requires other innovative techniques. We successfully implanted a defibrillator electrode in the pleural cavity using a thoracoscopic approach for a 4-year-old child with sodium channelopathy.


Assuntos
Morte Súbita Cardíaca , Desfibriladores Implantáveis , Humanos , Pré-Escolar , Morte Súbita Cardíaca/prevenção & controle , Cavidade Pleural , Arritmias Cardíacas
19.
Cureus ; 15(12): e51324, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38288180

RESUMO

Vascular Ehlers-Danlos syndrome is a fatal disease caused by a type III collagen mutation that can result in the rupture of blood vessels, the intestinal tract, and/or the uterus. Despite being the most severe form of Ehlers-Danlos syndrome, it is not well known in the pediatric context because it rarely presents serious complications in childhood. In this case, the patient experienced a subclavian artery rupture triggered by sneezing, which was initially managed with an endovascular stent. However, the descending aorta subsequently ruptured, and the patient died. Traditionally, surgical or endovascular treatments have been avoided due to the inherent fragility of blood vessels. Nevertheless, favorable outcomes have been documented with a wait-and-see surgical approach or endovascular treatment, especially when the diagnosis precedes the onset of vascular complications. Notably, celiprolol, a partial ß2-agonist and ß1-blocker, has demonstrated efficacy in preventing vascular complications. Therefore, early diagnosis plays a pivotal role. Raising awareness about this syndrome, along with its management and prophylaxis, holds the potential to enhance the survival rate.

20.
Asian Cardiovasc Thorac Ann ; 30(7): 834-836, 2022 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34672213

RESUMO

A retroesophageal aberrant brachiocephalic artery is a very rare congenital aortic arch anomaly. We herein presented a 29-year-old man with right aortic arch, retroesophageal aberrant left brachiocephalic artery, left ligamentum arteriosus, and absent left internal carotid artery. Graft replacement of the descending aorta and anatomical reconstruction of left brachiocephalic artery was successfully performed using a midline sternotomy approach without blood transfusion. We discuss the surgical management for Kommerell's diverticulum.


Assuntos
Divertículo , Cardiopatias Congênitas , Adulto , Aorta Torácica/anormalidades , Aorta Torácica/diagnóstico por imagem , Aorta Torácica/cirurgia , Tronco Braquiocefálico/diagnóstico por imagem , Tronco Braquiocefálico/cirurgia , Divertículo/complicações , Divertículo/diagnóstico por imagem , Divertículo/cirurgia , Humanos , Masculino , Artéria Subclávia/anormalidades , Artéria Subclávia/diagnóstico por imagem , Artéria Subclávia/cirurgia , Resultado do Tratamento
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