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Vol. 65. Issue 4.
Pages 234-237 (April 2018)
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Vol. 65. Issue 4.
Pages 234-237 (April 2018)
Case report
Cardiac arrest related to anaesthesia in Williams-Beuren syndrome
Parada cardíaca relacionada con la anestesia en el síndrome Williams-Beuren
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J. Lucena Delgadoa,
Corresponding author
jldfisio@msn.com

Corresponding author.
, P. Sanabria Carreterob, P. Durán la Fuenteb, A. Gónzalez Rocafortc, L. Castro Pargab, F. Reinoso Barberob
a Departamento de Anestesiología, Reanimación y Terapéutica del Dolor, Hospital Universitario La Paz, Madrid, Spain
b Departamento de Anestesiología Pediátrica, Reanimación y Terapéutica del Dolor, Hospital Universitario La Paz, Madrid, Spain
c Departamento de Cirugía Cardíaca Pediátrica, Hospital Universitario La Paz, Madrid, Spain
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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.

Keywords:
Williams syndrome
Cardiac arrest
Anaesthesia
Cardiopathy
Death sudden
Myocardial ischaemia
Resumen

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.

Palabras clave:
Síndrome Williams
Parada cardiaca
Anestesia
Cardiopatía
Muerte súbita
Isquemia miocárdica

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