Sleep-disordered breathing (SDB) is described in patients with acquired heart failure but its prevalence in adults with congenital heart disease is not well documented. It is likely that single-ventricle patients who have undergone Fontan palliation poorly tolerate the additional stress of SDB on their fragile cardiac physiology. This study sought to determine the prevalence of SDB in a population of adult Fontan patients. We performed a retrospective chart review of adult Fontan patients followed in our program (n = 55). All charts were evaluated for documentation of polysomnography (PSG). SDB was characterized as nocturnal hypoxemia without apnea or obstructive sleep apnea (OSA). Baseline demographics and clinical characteristics were documented, as were PSG findings including Apnea Hypopnea Index (AHI, events/hour) and desaturation events of > 5% from baseline saturation. Twenty-two of our 55 patients (40%) had undergone PSG (45% male, mean age 29 years). Of these patients, 17 had a PSG positive for SDB (77%). Nine patients (41%) had nocturnal hypoxemia without apnea, while 8 patients (36%) had a PSG positive for OSA. Lower baseline oxygen saturations, but not BMI or age, were statistically significantly correlated with higher AHI. SDB was present in the majority of our Fontan cohort who had undergone PSG, occurring at a higher prevalence than what is described in the general population, suggesting that screening should be considered for all Fontan patients. However, further study is needed to confirm this prevalence in a larger population, and if therapy has an impact on clinical outcomes.
This is a preview of subscription content, log in to check access.
Buy single article
Instant access to the full article PDF.
Price includes VAT for USA
Subscribe to journal
Immediate online access to all issues from 2019. Subscription will auto renew annually.
This is the net price. Taxes to be calculated in checkout.
Data available on request from the authors.
Fontan F, Baudet E (1971) Surgical repair of tricuspid atresia. Thorax 26(3):240–248. https://doi.org/10.1136/thx.26.3.240
Diller GP, Giardini A, Dimopoulos K, Gargiulo G, Muller J, Derrick G, Giannakoulas G, Khambadkone S, Lammers AE, Picchio FM, Gatzoulis MA, Hager A (2010) Predictors of morbidity and mortality in contemporary Fontan patients: results from a multicenter study including cardiopulmonary exercise testing in 321 patients. Eur Heart J 31(24):3073–3083. https://doi.org/10.1093/eurheartj/ehq356
Pundi KN, Johnson JN, Dearani JA, Pundi KN, Li Z, Hinck CA, Dahl SH, Cannon BC, O'Leary PW, Driscoll DJ, Cetta F (2015) 40-Year Follow-Up After the Fontan Operation: Long-Term Outcomes of 1,052 Patients. J Am Coll Cardiol 66(15):1700–1710. https://doi.org/10.1016/j.jacc.2015.07.065
Schwartz I, McCracken CE, Petit CJ, Sachdeva R (2018) Late outcomes after the Fontan procedure in patients with single ventricle: a meta-analysis. Heart 104(18):1508–1514. https://doi.org/10.1136/heartjnl-2017-312807
Driscoll DJ (2007) Long-term results of the Fontan operation. Pediatr Cardiol 28(6):438–442. https://doi.org/10.1007/s00246-007-9003-4
Egbe AC, Reddy YNV, Khan AR, Al-Otaibi M, Akintoye E, Obokata M, Borlaug BA (2018) Venous congestion and pulmonary vascular function in Fontan circulation: Implications for prognosis and treatment. Int J Cardiol 271:312–316. https://doi.org/10.1016/j.ijcard.2018.05.039
Kimura H, Ota H, Kimura Y, Takasawa S (2019) Effects of Intermittent Hypoxia on Pulmonary Vascular and Systemic Diseases. Int J Environ Res Public Health. https://doi.org/10.3390/ijerph16173101
Niijima M, Kimura H, Edo H, Shinozaki T, Kang J, Masuyama S, Tatsumi K, Kuriyama T (1999) Manifestation of pulmonary hypertension during REM sleep in obstructive sleep apnea syndrome. Am J Respir Crit Care Med 159(6):1766–1772. https://doi.org/10.1164/ajrccm.159.6.9808064
Osman AM, Carter SG, Carberry JC, Eckert DJ (2018) Obstructive sleep apnea: current perspectives. Nat Sci Sleep 10:21–34. https://doi.org/10.2147/NSS.S124657
Ramakrishnan S, Juneja R, Bardolei N, Sharma A, Shukla G, Bhatia M, Kalaivani M, Kothari SS, Saxena A, Bahl VK, Guleria R (2013) Nocturnal hypoxaemia in patients with Eisenmenger syndrome: a cohort study. BMJ Open. https://doi.org/10.1136/bmjopen-2012-002039
Cotts T, Smith KR, Lu J, Dorfman AL, Norris MD (2014) Risk for sleep-disordered breathing in adults after atrial switch repairs for d-looped transposition of the great arteries. Pediatr Cardiol 35(5):888–892. https://doi.org/10.1007/s00246-014-0873-y
Herold SE, Young TW, Ge D, Snieder H, Lovrekovic GZ (2006) Sleep disordered breathing in pediatric patients with tetralogy of Fallot. Pediatr Cardiol 27(2):243–249. https://doi.org/10.1007/s00246-005-1168-0
Duran J, Esnaola S, Rubio R, Iztueta A (2001) Obstructive sleep apnea-hypopnea and related clinical features in a population-based sample of subjects aged 30 to 70 yr. Am J Respir Crit Care Med 163(3 Pt 1):685–689. https://doi.org/10.1164/ajrccm.163.3.2005065
Franklin KA, Lindberg E (2015) Obstructive sleep apnea is a common disorder in the population-a review on the epidemiology of sleep apnea. J Thorac Dis 7(8):1311–1322. https://doi.org/10.3978/j.issn.2072-1439.2015.06.11
Kasai T (2012) Sleep apnea and heart failure. J Cardiol 60(2):78–85. https://doi.org/10.1016/j.jjcc.2012.05.013
Kasai T, Bradley TD (2011) Obstructive sleep apnea and heart failure: pathophysiologic and therapeutic implications. J Am Coll Cardiol 57(2):119–127. https://doi.org/10.1016/j.jacc.2010.08.627
Egbe AC, Connolly HM, Miranda WR, Ammash NM, Hagler DJ, Veldtman GR, Borlaug BA (2017) Hemodynamics of Fontan Failure: The Role of Pulmonary Vascular Disease. Circ Heart Fail. https://doi.org/10.1161/CIRCHEARTFAILURE.117.004515
Hetzel M, Kochs M, Marx N, Woehrle H, Mobarak I, Hombach V, Hetzel J (2003) Pulmonary hemodynamics in obstructive sleep apnea: frequency and causes of pulmonary hypertension. Lung 181(3):157–166. https://doi.org/10.1007/s00408-003-1017-y
Tan HL, Gozal D, Kheirandish-Gozal L (2013) Obstructive sleep apnea in children: a critical update. Nat Sci Sleep 5:109–123. https://doi.org/10.2147/NSS.S51907
Redline S, Tishler PV, Schluchter M, Aylor J, Clark K, Graham G (1999) Risk factors for sleep-disordered breathing in children. Associations with obesity, race, and respiratory problems. Am J Respir Crit Care Med 159(5):1527–1532. https://doi.org/10.1164/ajrccm.159.5.9809079
Sanchez-Armengol A, Fuentes-Pradera MA, Capote-Gil F, Garcia-Diaz E, Cano-Gomez S, Carmona-Bernal C, Castillo-Gomez J (2001) Sleep-related breathing disorders in adolescents aged 12 to 16 years : clinical and polygraphic findings. Chest 119(5):1393–1400. https://doi.org/10.1378/chest.119.5.1393
Ferry PC (1990) Neurologic sequelae of open-heart surgery in children An irritating question. Am J Dis Child 144(3):369–373. https://doi.org/10.1001/archpedi.1990.02150270119040
Idorn L, Jensen AS, Juul K, Overgaard D, Nielsen NP, Sorensen K, Reimers JI, Sondergaard L (2013) Quality of life and cognitive function in Fontan patients, a population-based study. Int J Cardiol 168(4):3230–3235. https://doi.org/10.1016/j.ijcard.2013.04.008
Marelli A, Miller SP, Marino BS, Jefferson AL, Newburger JW (2016) Brain in congenital heart disease across the lifespan: the cumulative burden of injury. Circulation 133(20):1951–1962. https://doi.org/10.1161/CIRCULATIONAHA.115.019881
Ilardi D, Ono KE, McCartney R, Book W, Stringer AY (2017) Neurocognitive functioning in adults with congenital heart disease. Congenit Heart Dis 12(2):166–173. https://doi.org/10.1111/chd.12434
McCrindle BW, Williams RV, Mital S, Clark BJ, Russell JL, Klein G, Eisenmann JC (2007) Physical activity levels in children and adolescents are reduced after the Fontan procedure, independent of exercise capacity, and are associated with lower perceived general health. Arch Dis Child 92(6):509–514. https://doi.org/10.1136/adc.2006.105239
Guilleminault C, Korobkin R, Winkle R (1981) A review of 50 children with obstructive sleep apnea syndrome. Lung 159(5):275–287. https://doi.org/10.1007/bf02713925
Rosen CL (2004) Obstructive sleep apnea syndrome in children: controversies in diagnosis and treatment. Pediatr Clin North Am 51(1):153–167. https://doi.org/10.1016/s0031-3955(03)00183-4
Gozal D, Pope DW Jr (2001) Snoring during early childhood and academic performance at ages thirteen to fourteen years. Pediatrics 107(6):1394–1399. https://doi.org/10.1542/peds.107.6.1394
Williams DB, Kiernan PD, Metke MP, Marsh HM, Danielson GK (1984) Hemodynamic response to positive end-expiratory pressure following right atrium-pulmonary artery bypass (Fontan procedure). J Thorac Cardiovasc Surg 87(6):856–861
Watson NF, Bushnell T, Jones TK, Stout K (2009) A novel method for the evaluation and treatment of obstructive sleep apnea in four adults with complex congenital heart disease and Fontan repairs. Sleep Breath 13(4):421–424. https://doi.org/10.1007/s11325-009-0260-8
We thank Dominik Wiktor, MD for his dedication and service to our program and patients. He was unable to participate in the final development of this manuscript secondary to his untimely death, but he helped care for many of these included patients during his time as a fellow and faculty in our program.
Not applicable, as the study was not funded.
Conflict of interest
The authors declare that they have no conflict of interest.
This research study was conducted retrospectively from data obtained for clinical purposes. The Colorado Multiple Institutions Review Board determined that our study did not need ethical approval. An IRB official waiver of ethical approval was granted from the IRB.
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
About this article
Cite this article
CG, S., RM, J., AD, K. et al. Prevalence of Sleep-Disordered Breathing in a Cohort of Adult Fontan Patients. Pediatr Cardiol (2020). https://doi.org/10.1007/s00246-020-02403-1
- Single ventricle
- Sleep-disordered breathing
- Congenital heart defect