Der Anaesthesist
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Malignant hyperthermia (MH) is a life-threatening, acute pharmacogenetic disorder mostly due to heterozygous mutations in the ryanodin receptor 1 (RYR1) gene. Diagnosis is generally confirmed by the in vitro contracture test (IVCT). In this study the genotype-phenotype correlation was analyzed and the presumed prevalence of MH is discussed. ⋯ In the two patients described the presence of two mutated RYR1 alleles seemed to have an additive effect on the functional restriction of the (RYR1 receptor and to lead to a stronger response both in the IVCT and with regard to clinical signs. The patients with no detected RYR1 mutations possibly have a RYR1 mutation with smaller effects outside the hot spot regions tested and/or false positive IVCT results. The data from a small patient group indicate a substantially higher prevalence of MH with a correspondingly lower penetrance in the German population than previously assumed.
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Due to the technical advances in pumps, oxygenators and cannulas, veno-arterial extracorporeal membrane oxygenation (va-ECMO) or extracorporeal life support (ECLS) has been widely used in emergency medicine and intensive care medicine for several years. An accepted indication is peri-interventional cardiac failure in cardiac surgery (postcardiotomy low cardiac output syndrome). Furthermore, especially the use of va-ECMO for other indications in critical care medicine, such as in patients with severe sepsis with septic cardiomyopathy or in cardiopulmonary resuscitation has tremendously increased. ⋯ This review gives an overview of the indications, complications and contraindications for va-ECMO. It discusses its advantages in organ transplantation and transport of critically ill patients. The reader will learn the differences between peripheral and central cannulation and how to monitor and manage va-ECMO.
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Intraoperative transesophageal echocardiography (TEE) is an established diagnostic tool and has to be regarded as the standard of care for intraoperative monitoring and cardiac surgical decision-making. Furthermore, intraoperative TEE is also used for monitoring and assessment of hemodynamic changes and the detection of previously unknown pathologies. ⋯ Especially during mitral valve surgery, 3D-TEE has proven its superiority in the diagnosis of the underlying pathology as demonstrated by a large number of studies in this field. This review presents the available data about the role of intraoperative 3D-TEE echocardiography and introduces practical fields of application.
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The administration of blood products is strictly regulated. Several weeks before the operation the preparation for transfusion begins with optimizing the patient's hematological and hemostaseological situation. In elective surgery blood group testing and antibody screening are performed soon after admission of the patient. ⋯ On the day before the operation a further blood sample is necessary for cross-matching if red blood cells are to be transfused. Usually blood products are issued for immediate administration. Before transfusion begins the blood product has to be checked, the identity of the patient must be controlled and in the case of red blood cell transfusions the AB0 bedside test has to be performed.