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
ReviewCurrent understanding and perioperative management of pediatric pulmonary hypertension.
Pediatric pulmonary hypertension is a complex disease with multiple, diverse etiologies affecting the premature neonate to the young adult. Pediatric pulmonary arterial hypertension, whether idiopathic or associated with congenital heart disease, is the most commonly discussed form of pediatric pulmonary hypertension, as it is progressive and lethal. However, neonatal forms of pulmonary hypertension are vastly more frequent, and while most cases are transient, the risk of morbidity and mortality in this group deserves recognition. ⋯ One aspect of pediatric pulmonary hypertension is very clear: anesthetizing the child with pulmonary hypertension is associated with a significantly heightened risk of morbidity and mortality. It is therefore imperative that anesthesiologists who care for children with pulmonary hypertension have a firm understanding of the pathophysiology of the various forms of pediatric pulmonary hypertension, the impact of anesthesia and sedation in the setting of pulmonary hypertension, and anesthesiologists' role as perioperative experts from preoperative planning to postoperative disposition. This review summarizes the current understanding of pediatric pulmonary hypertension physiology, preoperative risk stratification, anesthetic risk, and intraoperative considerations relevant to the underlying pathophysiology of various forms of pediatric pulmonary hypertension.