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Background: Williams-Beuren syndrome (WBS) patients have increased risk of sudden death, and these events are frequently associated with general anesthesia. We sought to determine the cardiopulmonary resuscitation (CPR) frequency in WBS patients as well as correlated risk factors associated with CPR need in this population.
Methods: We administered an online survey in collaboration with the Williams Syndrome Association to WBS patient families regarding their family member's experience with anesthesia. Statistical analysis was performed on data from 649 WBS individuals, reflecting 1872 anesthesia events.
Results: CPR was performed during or shortly after 2.1% (40/1872) of WBS anesthesia procedures while 5% (33/649) of WBS individuals had at least one anesthesia-associated CPR event. 97.5% (39/40) of CPR episodes occurred in individuals with supravalvar aortic stenosis (29/40) or peripheral pulmonic stenosis (28/40). Twenty occurred in individuals with both diagnoses. Eleven events occurred in patients with coronary abnormalities, 10 in patients with ventricular septal defects, and 6 in patients with left ventricular hypertrophy. Pulmonary hypertension was present in 4 of 40 cases, while right ventricular hypertrophy was present in seven. Thirty of forty CPR cases were associated with either cardiac catheterization or CT surgical cases. In 21 of 40 episodes, the first CPR event occurred in the OR or cath lab; the remaining episodes occurred within 72 hours of the procedure. Nine individuals reported multiple code events following a single procedure. There were no significant differences between those having and those not having CPR events with respect to the following: gender, pre-procedural hydration duration, arrhythmia or sleep apnea history.
Conclusions: Anesthesia- associated CPR in a multi-center pediatric study was ∼2/30,000. CPR was performed in 2% of our cohort. While elevated compared to the general population, these data suggest WBS anesthesia risk in the current era is less than that reported during prior surgical eras. CPR event risk factors, such as biventricular outflow tract obstruction, may be helpful in identifying WBS patients at highest risk of negative outcome.
Poster Hall, Hall C
Friday, March 17, 2017, 10:00 a.m.-10:45 a.m.
Session Title: Congenital Heart Disease: Progress in Pediatric Heart Surgery
Abstract Category: 10. Congenital Heart Disease: Pediatric
Presentation Number: 1101-020
- 2017 American College of Cardiology Foundation