European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery
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Eur J Cardiothorac Surg · Feb 2001
Perioperative determinants and outcome of cardiopulmonary arrest in children after heart surgery.
To identify perioperative factors associated with postoperative cardiopulmonary arrest (CA) in the pediatric intensive care unit (PICU) in children undergoing cardiovascular surgery, and to report the outcome of cardiopulmonary resuscitation (CPR) in these patients. ⋯ CA after pediatric cardiac surgery is associated with repair of complex congenital heart anomalies in patients who require preoperative mechanical ventilation and vasoactive agents, prolonged aortic cross-clamp, circulatory arrest; and heavy postoperative inotropic support.
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The underlying cause of congenital supravalvular aortic stenosis (SVAS) has recently been identified as a loss-of function mutation of the elastin gene on chromosome 7q11.23, resulting in an obstructive arteriopathy of varying severity, which is most prominent at the aortic sinutubular junction. The generalized nature of the disease explains the frequent association with stenoses of systemic and pulmonary arteries. Furthermore, localization of the supravalvular stenosis at the level of the commissures of the aortic valve has important implications for both aortic valve function and coronary circulation. This review summarizes the recent advances with regard to the pathogenesis of SVAS and describes the multitude of clinically relevant pathologic features other that the mere 'supra-aortic' narrowing that have important implications for surgical therapy.