Congenital Supravalvular Aortic Stenosis and Sudden Death Associated with Anesthesia: What’s the Mystery?

Abstract

Patients with congenital supravalvular aortic stenosis and associated peripheral pulmonary artery stenoses, the majority of whom have Williams-Beuren syndrome, are inherently at risk for development of myocardial ischemia. This is particularly true in the setting of procedural sedation and anesthesia. The biventricular hypertrophy that accompanies these lesions increases myocardial oxygen consumption and compromises oxygen delivery. In addition, these patients often have direct, multifactorial compromise of coronary blood flow. In this article, we review both the pathophysiology of congenital supravalvular aortic stenosis and the literature regarding sudden death in association with sedation and anesthesia. Recommendations as to preoperative assessment and management of these patients are made based on the best available evidence.

Case report of sudden death in a child with Williams syndrome following administration of anaesthesia

“…The biventricular hypertrophy associated with SVAS will lead to an increase myocardial oxygen consumption and compromised oxygen delivery, which can progress to myocardial ischaemia. 20 Therefore, the induction of anaesthesia with sevoflurane in this patient was not the optimal choice. Likewise, the addition of propofol depressed cardiac output even more.…”

“…5,19,20 Wessel et al made the distinction in their study and excluded the deaths during anaesthesia from the sudden-death group. 17 In the study by Bird et al pathological findings in two of the seven patients showed anatomic abnormalities that predisposed the individuals to sudden death (coronary artery stenosis and biventricular outflow tract obstruction).…”

Cardiovascular Disease in Williams Syndrome

“…A large number of cardiovascular collapses and deaths in patients with WS have been in the periprocedural and perianesthetic setting. 80 The risk of sudden cardiovascular collapse appears to be greater in the presence of bilateral outflow tract obstruction, 43 especially with coronary arterial stenosis. 44 However, sudden death has been reported in the absence of autopsy evidence of outflow tract or coronary obstruction.…”

“…Those authors also recommended against the use of succinylcholine because of a theoretical risk of a hyperkalemic response and recommended titrating neuromuscular blockade with intraoperative monitoring of train of four. Burch and colleagues 80 have published anesthetic goals in patients with congenital SVAS, including patients with WS, that can be summarized as follows: maintain sinus rhythm at an age-appropriate heart rate, ensure an adequate preload while avoiding rapid shifts in intravascular volume, avoid anesthetic strategies associated with negative inotropic effects and decreased systemic vascular resistance (ie, propofol, Sodium Pentothal, sevoflurane, isoflurane, and desflurane), and treat hypotension aggressively (phenylephrine, ephedrine, or low-dose epinephrine may be appropriate, depending on the status of the patient).…”

Sporadic supravalvular stenosis in a young man

“…Cardiac catheterization with coronary and aortic angiography remains the "gold standard" for delineation of aortic leaflet tethering and assessment of coronary artery lumen calibre (1).…”