P2A100: Sleep-disordered Breathing in Pediatric Patients with Single Ventricle Physiology: A Systematic Review

Background: The reported prevalence of sleep-disordered breathing (SDB) in the general pediatric population is estimated to be between 2-11%. However, patients with underlying comorbidities may be at greater risk for SDB. While it is commonly accepted there is an increased prevalence of SDB in adults with congenital heart disease (CHD) and single ventricle (SV) physiology, research regarding SDB in pediatric patients with CHD has been sparse. The aim of this systematic review was to assess the prevalence, risk factors, and outcomes associated with SDB in pediatric patients with SV physiology.

Methods: A systematic search of PubMed, Web of Science, Embase, the Cochrane Library, and ClinicalTrials.gov was performed to screen for studies related to SDB, CHD, and SV physiology. Data sources were last queried on 03/31/2023. All peer-reviewed publications written in the English language reporting SDB among pediatric subjects (ages 0-21 years) with SV physiology were analyzed. The primary author assessed study quality and extracted study data including year of publication, sample size, subject ages, underlying cardiac lesion with stage of cardiac palliative surgery, screening and testing measures for SDB, and patient outcomes related to SDB.

Results: Thirty unique publications were identified that met inclusion criteria. Of these, twenty-four publications were excluded (e.g. conference abstracts, case reports, population with underlying genetic comorbidity; see PRISMA diagram for full details). Six publications were included in the final analysis. No other genetic disorders or high-risk qualities, such as obesity or craniofacial abnormalities, were noted within the analyzed publications. The reported prevalence of SDB in pediatric patients with SV physiology ranged from 15-100% in all study participants, but ranged from 15-47% in non-neonates. Four studies utilized either polysomnography or at-home sleep tests to diagnose SDB. One study reported hemodynamic or cardiopulmonary concerns and showed improvements in overall respiratory rates and work of breathing when SDB was treated with continuous positive airway pressure (CPAP) therapy. Four studies reported neuropsychological concerns potentially associated with SDB including lower overall intellectual quotients, greater prevalence of mood disorders such as depression and anxiety, and lower scores on Quality-of-Life questionnaires. Of note, zero current or upcoming clinical trials focusing on SDB in pediatric patients with CHD or SV physiology were identified.

Conclusion: This is the first systematic review of the published literature describing SDB in pediatric patients with SV physiology. These studies suggest the prevalence of SDB in this cohort may be greater than the prevalence in the general pediatric population. Moreover, they provide insight into the hemodynamic and neuropsychological consequences SDB can have in this unique population. While this review is limited by a small number of studies with small sample sizes, these publications suggest a greater need for prospective research on the consequences of SDB in pediatric patients with SV physiology.