Breath Holding Spells in Children with Long QT Syndrome. (27th April 2015)

• Record Type: Journal Article
• Authors: Robinson, Jeffrey A.; Bos, J. Martijn; Etheridge, Susan P.; Ackerman, Michael J.
• Is Part Of: Congenital heart disease. Volume 10:Number 4(2015)
• Page Start: 354
• Page End: 361
• Publication Date: 2015-04-27
• Subjects: Congenital heart disease -- Periodicals; 616.1204305
• DOI: 10.1111/chd.12262
• Languages: English
• ISSNs: 1747-079X

Abstract

<abstract abstract-type="main"> <title>Abstract</title> <sec id="chd12262-sec-0001" sec-type="section"> <title>Background</title> <p>Long QT syndrome (LQTS) is a genetic heart rhythm disorder that may present with syncope, seizures, or sudden cardiac death. Breath holding spells (BHS) occur in 5% of all children and have been noted in children with LQTS anecdotally. The purpose of this study was to determine the frequency of BHS in children diagnosed with LQTS at ≤5 years of age.</p> </sec> <sec id="chd12262-sec-0002" sec-type="section"> <title>Design</title> <p>A retrospective review was performed to identify children diagnosed with LQTS who were ≤5 years old at initial presentation to our LQTS clinic from August 1999 to November 2013. The mean length of follow‐up was 6.4 ± 2.8 years. The electronic medical records were reviewed for clinical presentation of BHS, as well as LQTS‐associated symptoms, diagnostic tests, and treatment.</p> </sec> <sec id="chd12262-sec-0003" sec-type="section"> <title>Results</title> <p>The study cohort consisted of 115 children with LQTS (58% male; median age at diagnosis, 11 months [range, birth to 5 years]; mean corrected QT interval (QTc), 478 ± 60 milliseconds). At presentation, 80% of patients were asymptomatic. Genetic testing revealed type 1 LQTS (LQT1) in 48%. Overall, 5 of 115 patients (4.3%) had BHS (2 of 5 [40%] male, mean QTc: 492 ± 14 milliseconds, 4 [80%] with family history of LQTS). BHS were the presenting symptom in 1 of 23 symptomatic patients (4.3%). All BHS occurred in patients with LQT1 (<italic>P</italic> = .02).</p> </sec> <sec id="chd12262-sec-0004" sec-type="section"> <title>Conclusions</title> <p>Although BHS among children with LQTS are relatively rare and occur at similar frequency as the general population, they can be the presenting symptom for a heart rhythm disorder. Careful attention to BHS is important to distinguish an innocent BHS from a potential LQTS‐triggered cardiac event so that proper treatment is initiated.</p> </sec> </abstract>