Purpose: To examine the efficacy of preoperative electrocardiogram (EKG) screening for Timothy syndrome, a rare and fatal condition characterized by prolonged QT, in children referred for syndactyly release.
Methods: We reviewed the records of nonsyndromic syndactyly patients seen by a hand surgeon at our institution between 2007 and 2013. All underwent a preoperative screening EKG for Timothy syndrome. We reviewed the medical records for demographics, presentation, EKG results, and operative findings, and calculated median age at the time of EKG and surgery and frequency distributions for sex, side affected, EKG result, and clinical finding. The mean patient charge for EKG and interpretation was calculated.
Results: We identified 128 syndactyly patients, 72% of which were boys. Median age at the time of EKG testing and syndactyly release was 1 year. A total of 92% of patients had normal EKG results; one patient exhibited a prolonged QT. Ten patients (8%) had further cardiac evaluation because of the EKG result and were found to be normal on repeat testing. No patient met QT threshold for Timothy syndrome and all patients were cleared for surgery. The minimum patient charge for EKG testing was $183.
Conclusions: To improve patient safety, some have advocated preoperative EKG testing for all children undergoing syndactyly release to rule out Timothy syndrome. Analysis of our experience failed to yield an instance of Timothy syndrome over a 7-year period. Although EKG charges were relatively low, costs resulting from additional testing, cardiology consultation, and provider and parent time should be considered. Our study does not support routine EKG testing for children referred for syndactyly release, and we have abandoned this practice.
Type Of Study/level Of Evidence: Prognostic IV.
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View Article and Find Full Text PDFA Natural History Study of Timothy Syndrome.
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Laboratory of Biochemistry and Genetics, National Institute of Diabetes, Digestive, and Kidney Diseases, National Institute of Health, Bethesda, MD, USA.
Article Synopsis
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Department of Cardiovascular Medicine, The Second Xiangya Hospital, Central South University, 139 Renmin Road, Furong District, Changsha, 410011, Hunan Province, People's Republic of China.
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Cardiovascular Research Institute, Weill Cornell Medicine, 413 E. 69th St., New York, NY, 10021, USA.
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