Paediatric anaesthesia
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Williams syndrome affects approximately one in 10 000 people and is caused by the deletion of genes on chromosome 7q11.23 which code for elastin. The phenotypic appearance of people with Williams syndrome is well characterized, but there continues to be new genetic and therapeutic discoveries. Patients with Williams syndrome have increased morbidity and mortality under sedation and anesthesia, largely as a result of cardiovascular abnormalities. This review article focuses on new information about Williams syndrome and outlines a structured approach to patients with Williams syndrome in the perioperative period.
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Paediatric anaesthesia · May 2019
ReviewCurrent perspectives on treatment of perioperative hemodynamic instability and hypotension.
Overall, there are numerous causes of hypotension in the perioperative period. The approach to definitive treatment must be tailored to the child's unique anatomy and physiology, as well as the current factors presumed to be eliciting the hypotensive state. It is imperative to consider both routine and lesion-specific etiologies to the current hypotensive episode. Lastly, when employing pharmacologic therapy for hypotension, there are often multiple combinations of medications that can reasonably be used to achieve the desired hemodynamic effects.
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Paediatric anaesthesia · May 2019
ReviewRecent achievements and future developments in neonatal cardiopulmonary bypass.
A primary goal of improving neonatal cardiopulmonary bypass has been making the circuit smaller and reduce the blood contacting surfaces. As bypass circuit size has decreased, bloodless surgery has become possible even in neonates. Since transfusion guidelines are difficult to construct based on existing literature, these technical advances should be taken advantage of in conjunction with an individualized transfusion scheme, based on monitoring of oxygen availability to the tissues. ⋯ The use of indirect heparin concentration assays and global viscoelastic assays in the operating room is likely to represent the optimal strategy, and requires validation in neonates. Monitoring of global and regional indexes of oxygen availability and consumption on bypass have become possible; however, their use in neonates still has outstanding technical issues which should be addressed and hence needs further validation. Due to the immaturity of the neonatal myocardium, single-shot cold cardioplegia solutions are thought to confer the best myocardial protection; their superiority when compared to more conventional modalities, however, remains to be demonstrated.
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Paediatric anaesthesia · May 2019
ReviewPediatric ventricular assist devices: Bridge to a new era of perioperative care.
Pediatric ventricular assist devices (VADs) are evolving as a standard therapy for end stage heart failure in children. Major recent developments include the increased use of continuous flow (CF) devices in children and increased experience with congenital heart disease (CHD) and outpatient management. ⋯ Successful perioperative management requires an understanding of the interaction between the patient's physiology and the device and a framework to troubleshoot problems. This review focuses on CF devices, VAD support for CHD and perioperative management of pulsatile and CF devices in the pediatric population.
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Paediatric anaesthesia · May 2019
ReviewUse of antifibrinolytics in pediatric cardiac surgery: Where are we now?
Fibrinolytic activation is a major and preventable source of bleeding in neonates and children undergoing cardiac surgery with cardiopulmonary bypass. Based on the existing literature (adult and pediatric; cardiac and noncardiac), prophylactic administration of antifibrinolytic agents can help reduce fibrinolytic activation, and consequently reduces perioperative bleeding and the requirement for blood product transfusion. Due to the increased risk of renal failure and mortality reported in adults undergoing cardiac surgery, aprotinin should not be considered as a safe option in neonates and children. ⋯ Further studies are therefore urgently needed to better define the optimal dose regimen to be used in neonates and children. In the meantime, the dose regimen published in the most recent pharmacokinetic studies can be used. Although no studies have assessed the effect of massive bleeding and transfusion on the plasmatic concentrations of the lysine analogs, additional boluses might be considered in the presence of bleeding and/or when signs of fibrinolytic activations are observed on viscoelastic hemostatic assays.