Strategies for the Surgical Management of Highly Aggressive Williams Syndrome Aortopathy: A Three Case Report.

Severe aortopathy in Williams syndrome can sometimes present with an initial ascending aortic pathology, followed in short order by more distal multilevel obstruction and recurrence requiring reintervention. In this series, an early, comprehensive surgical approach using a combination of various access and perfusion strategies yielded excellent long-term results.

Effects of remifentanil anesthesia on cardiac electrophysiologic properties in children undergoing catheter ablation of supraventricular tachycardia.

Remifentanil is commonly used during anesthesia in pediatric electrophysiologic studies (EPS). The purpose of this study is to determine the effects of remifentanil on the cardiac electrophysiologic properties of children undergoing ablation of supraventricular tachycardia (SVT). A prospective study was performed in patients undergoing EPS before ablation of SVT. Each patient received two different anesthetic protocols: protocol 1 = propofol (200 mcg/kg/min) and protocol 2 = propofol (120 mcg/kg/min) plus remifentanil (0.3 mcg/kg/min). EPS data were measured during the steady state of each protocol. Paired Student t test was performed for analysis of continuous data. All p values <0.05 were considered statistically significant. Fifteen patients were enrolled between April 2005 and January 2006. The mean age was 13.3 ± 2.9 years (range 6.7 to 17.7). Seven patients had atrioventricular (AV) nodal re-entry tachycardia; 5 patients had Wolff-Parkinson-White syndrome; 2 patients had a concealed accessory pathway; and 1 patient was not inducible. Of the 14 patients who underwent ablation, 13 (93%) achieved successful. The baseline sinus cycle length extended from 884 ± 141 ms during protocol 1 to 980 ± 165 ms during protocol 2 (p = 0.01), and the Wenckebach cycle length lengthened from 377 ± 96 ms to 406 ± 109 ms (p = 0.01). No other variables measured (atrial-His (AH) and His-ventricular (HV) interval, atrioventricular node (AVN), and atrial, ventricular, and accessory pathway effective refractory periods) changed significantly between the two different protocols. In pediatric patients undergoing EPS before ablation of SVT, remifentanil appears to slow both sinus and AV nodal function. These effects should be taken into consideration when performing EPS.

The effects of propofol, small-dose isoflurane, and nitrous oxide on cortical somatosensory evoked potential and bispectral index monitoring in adolescents undergoing spinal fusion.

In this study we compared the effects of propofol, small-dose isoflurane, and nitrous oxide (N(2)O) on cortical somatosensory evoked potentials (SSEP) and bispectral index (BIS) monitoring in adolescents undergoing spinal fusion. Twelve patients received the following anesthetic maintenance combinations in a randomly determined order: treatment #1: isoflurane 0.4% + N(2)O 70% + O(2) 30%; treatment #2: isoflurane 0.6% + N(2)O 70% + O(2) 30%; treatment #3: isoflurane 0.6% + air + O(2) 30%; treatment #4: propofol 120 microg . kg(-1) . min(-1) + air + O(2) 30%. Cortical SSEP amplitudes measured during anesthesia maintenance with treatment #3 (isoflurane 0.6%/air) were more than those measured during maintenance with treatment #1 (isoflurane 0.4%/N(2)O 70%) (P < 0.0001) and treatment #2 (isoflurane 0.6%/N(2)O 70%) (P < 0.0052). Cortical SSEP amplitudes measured during treatment #4 (propofol 120 microg . kg(-1) . min(-1)/air) were more than treatment #1 (isoflurane 0.4%/N(2)O 70%) (P < 0.0001), treatment #2 (Iso 0.6%/N(2)O 70%) (P < 0.0007), and treatment #3 (isoflurane 0.6%/air) (P < 0.0191). In addition, average BIS values measured during treatments 1, 2, 3 and 4 were 62, 62, 61, and 44 respectively. Only treatment #4 (propofol 120 microg . kg(-1) . min(-1)/air) uniformly maintained BIS values less than 60. Our study demonstrates that propofol better preserves cortical SSEP amplitude measurement and provides a deeper level of hypnosis as measured by BIS values than combinations of small-dose isoflurane/N(2)O or small-dose isoflurane alone.