Anaesthetic Approach for Patient with Hereditary Angioedema.

Hereditary Angioedema (HEA), a disease caused by a mutation in the gene that encodes for the production of the fraction C1 in the complement (C1-INH), is a rare pathology (1/50.000) that causes swelling of the skin and submucosa in various organs, either naturally triggered or provoked by physical and psychological traumas, infections, or by the use of nonsteroidal anti-inflammatory drugs (NSAIDs) and angiotensin-converting enzyme inhibitors (ACEIs). Surgical trauma may spur the HEA crisis, leading to complications such as the swelling of the respiratory tracts and hemodynamic instability. Thus, the pre-surgical approach to HEA patients requires a specific plan that ensures short term prophylaxis, careful intra-operative management, rescue therapy and intensive post-surgery care. We present a report on a video-laparoscopic cholecystectomy approach for a 28-year-old woman diagnosed with asthma and HEA with symptomatic choledocholithiasis. We opted for short-term prophylaxis and immunology with the intravenous application of C1-INH. Ultrasonography imaging showed arterial wall oedema, which could correspond to a manifestation of C1-INH deficiency in the wall of the manipulated arteries during ultrasonography-guided puncture. Once the patient recovered consciousness, she was transferred to the intensive care unit and was discharged on the 6th day of hospitalisation.

Tromboelastógrafo em cirurgia cardíaca: estado atual

JUSTIFICATIVA E OBJETIVOS: O manuseio da hemostasia do paciente submetido à circulação extracorpórea (CEC) permanece como um grande desafio. Novos métodos de monitorização, novas drogas hemostáticas e inibidores da função plaquetária vêm sendo incorporados no pré, intra e pós-operatório. A natureza multifatorial dos distúrbios da hemostasia causados pela circulação extracorpórea exige conhecimento da fisiopatologia desses processos e avaliação acurada da hemostasia para anticoagulação eficaz durante a CEC e manutenção de hemostasia adequada após a cirurgia. Tempo de coagulação ativado (TCA) e coagulograma não bastam para esse manuseio. É necessária avaliação mais ampla através de monitores capazes de medir a função plaquetária e a dinâmica do processo hemostático como um todo. CONTEUDO: A hemostasia é resultado do equilíbrio entre os componentes dos sistemas de coagulação, anticoagulação e fibrinólise. Esse equilíbrio sofre ruptura durante a CEC, tornando o paciente susceptível a sangramento microvascular. A CEC induz alteração no crescimento da força plaquetária e nas propriedades elásticas do coágulo, de etiologia multifatorial. O uso de hemoderivados é constante e surge a necessidade de protocolos para orientar decisões para terapia transfusional. É importante determinar a função plaquetária, através de monitores que medem as propriedades visco-elásticas do coágulo, como tromboelastógrafo (TEG) e Sonoclot. CONCLUSÕES: O tromboelastógrafo é um importante monitor da hemostasia na abordagem dos pacientes submetidos a CEC. Tem sido incorporado com bons resultados nos protocolos de avaliação dos distúrbios hemostáticos e de terapêutica transfusional.

[Thromboelastograph in cardiac surgery: state of the art.]. / Tromboelastógrafo em cirurgia cardíaca: estado atual.

BACKGROUND AND OBJECTIVES: Management of hemostasis of cardiopulmonary bypass (CPB) patients is still a major challenge. New monitoring methods, new hemostatic drugs and new platelet function inhibitors are being added to the pre, intra and postoperative periods. The multifactorial nature of CBP-induced hemostasis disorders requires the understanding of their pathophysiology and the accurate hemostasis evaluation for effective coagulation during CPB, in addition to the maintenance of adequate postoperative hemostasis. Activated clotting time (ACT) and coagulogram are not enough for this management. A broader evaluation is needed with monitors able to measure platelet function and hemostatic process dynamics as a whole. CONTENTS: Hemostasis is the result of the balance of coagulation, anticoagulation and fibrinolysis systems components. This balance is disrupted during CPB making patients susceptible to microvascular bleeding. CPB induces multifactorial changes in platelet force growth and clot elastic properties. Blood products are often used and there is the need for protocols to guide transfusion decisions. It is important to determine platelet function with monitors measuring clot visco-elastic properties, such as thromboleastograph (TEG) and Sonoclot. CONCLUSIONS: Thromboelastograph is an important hemostasis monitor for patients submitted to CPB. It has been incorporated to hemostatic disorders evaluation protocols and transfusion therapy, with good results.