ABSTRACT
Williams-Beuren syndrome is the clinical manifestation of a congenital genetic disorder in the elastin gene, among others. There is a history of cardiac arrest refractory to resuscitation manoeuvres in anaesthesia. The incidence of myocardial ischaemia is high during anaesthetic induction, but there are patients who do not have this condition yet also have had very serious cardiac events, and issues that are still to be resolved. Case descriptions will enable the common pathophysiological factors to be defined, and decrease morbidity and mortality. We report the case of a 3-year-old boy with cardiac arrest at induction, rescued with circulatory assistance with extracorporeal membrane oxygenation and hypothermia induced for cerebral protection.
Subject(s)
Anesthesia, Inhalation/adverse effects , Anesthetics, Inhalation/adverse effects , Heart Arrest/chemically induced , Intraoperative Complications/chemically induced , Sevoflurane/adverse effects , Williams Syndrome/complications , Aortic Stenosis, Supravalvular/etiology , Aortic Stenosis, Supravalvular/surgery , Arteries/pathology , Bradycardia/etiology , Child, Preschool , Combined Modality Therapy , Disease Susceptibility , Extracorporeal Membrane Oxygenation , Heart Arrest/etiology , Heart Arrest/physiopathology , Heart Valves/pathology , Humans , Hypothermia, Induced , Hypoxia-Ischemia, Brain/etiology , Hypoxia-Ischemia, Brain/prevention & control , Intraoperative Complications/etiology , Male , Muscle Hypotonia/etiology , Paresis/etiology , Postoperative Complications/etiology , Williams Syndrome/pathologyABSTRACT
El síndrome de Williams-Beuren es la manifestación clínica de una alteración genética congénita en el gen de la elastina, entre otros. Existen antecedentes de parada cardíaca refractaria a maniobras de resucitación en contexto anestésico. Es alta la incidencia de isquemia miocárdica durante la inducción anestésica, pero existen pacientes que, sin esta causa, también presentan eventos cardíacos muy graves. Quedan cuestiones aún por resolver. La descripción de casos permitirá definir factores fisiopatológicos comunes y disminuir la morbimortalidad. Presentamos el caso de un niño de 3 años con parada cardíaca en la inducción anestésica, rescatado con asistencia circulatoria con membrana de oxigenación extracorpórea e hipotermia inducida como protección cerebral
Williams-Beuren syndrome is the clinical manifestation of a congenital genetic disorder in the elastin gene, among others. There is a history of cardiac arrest refractory to resuscitation manoeuvres in anaesthesia. The incidence of myocardial ischaemia is high during anaesthetic induction, but there are patients who do not have this condition yet also have had very serious cardiac events, and issues that are still to be resolved. Case descriptions will enable the common pathophysiological factors to be defined, and decrease morbidity and mortality. We report the case of a 3-year-old boy with cardiac arrest at induction, rescued with circulatory assistance with extracorporeal membrane oxygenation and hypothermia induced for cerebral protection