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The Identification and Impact of Abnormal Spirometry Patterns on Exercise Capacity in Pediatric Patients with Fontan Palliation

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Abstract

Reduced exercise capacity and restrictive lung physiology are common in patients after Fontan palliation (FP). However, there is paucity of data regarding the association between specific spirometry patterns and key exercise parameters in this population. This is a single-center, cross-sectional, study correlating pulmonary function and exercise parameters in children with FP. Patients who were ≤ 18 years of age and underwent a comprehensive cardiopulmonary treadmill exercise stress test (CPT) and spirometry at the same time, were included. Patients were categorized as (i) normal or (ii) abnormal based on the results of spirometry. The abnormal group was subdivided into (a) restrictive, (b) obstructive, and (c) mixed patterns. Demographic and key exercise parameters were compared between groups. Our study included 82 patients who underwent CPT at 13.6 (IQR, 11.3–15.4) years of age. A reduced exercise capacity (%VO2 ≤ 85%) was noted in the majority (n = 50, 61%). Spirometry was abnormal in 47 (57%) patients [restrictive (n = 25, 30%), obstructive (n = 12, 15%), and mixed (n = 10, 12%)]. The abnormal spirometry group had significant lower %VO2 (77% vs. 92%, p = 0.01) and METS (8.4 vs. 9.6, p = 0.02). Subgroup analysis revealed that obstructive (p = 0.04) and mixed (p = 0.02) patterns were associated with a significant decrease in % VO2. Majority of the children demonstrated an abnormal spirometry pattern post-FP. Abnormal pulmonary function was associated with the reduced exercise capacity. Identification and treatment of the abnormal lung function may improve the exercise capacity in these patients and improve the morbidity.

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References

  1. 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

    Article  PubMed  Google Scholar 

  2. Gewillig M, Brown SC (2016) The Fontan circulation after 45 years: update in physiology. Heart 102(14):1081–1086. https://doi.org/10.1136/heartjnl-2015-307467

    Article  PubMed  Google Scholar 

  3. Cunningham JW, Nathan AS, Rhodes J, Shafer K, Landzberg MJ, Opotowsky AR (2017) Decline in peak oxygen consumption over time predicts death or transplantation in adults with a Fontan circulation. Am Heart J 189:184–192. https://doi.org/10.1016/j.ahj.2017.04.009

    Article  PubMed  Google Scholar 

  4. Fernandes SM, Alexander ME, Graham DA, Khairy P, Clair M, Rodriguez E, Pearson DD, Landzberg MJ, Rhodes J (2011) Exercise testing identifies patients at increased risk for morbidity and mortality following Fontan surgery. Congenit Heart Dis 6(4):294–303. https://doi.org/10.1111/j.1747-0803.2011.00500.x

    Article  PubMed  Google Scholar 

  5. 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

    Article  PubMed  Google Scholar 

  6. Goldberg DJ, Avitabile CM, McBride MG, Paridon SM (2013) Exercise capacity in the Fontan circulation. Cardiol Young 23(6):824–830. https://doi.org/10.1017/S1047951113001649

    Article  PubMed  Google Scholar 

  7. Opotowsky AR, Landzberg MJ, Earing MG, Wu FM, Triedman JK, Casey A, Ericson DA, Systrom D, Paridon SM, Rhodes J (2014) Abnormal spirometry after the Fontan procedure is common and associated with impaired aerobic capacity. Am J Physiol Heart Circ Physiol 307(1):H110-117. https://doi.org/10.1152/ajpheart.00184.2014

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Turquetto ALR, Caneo LF, Agostinho DR, Oliveira PA, Lopes M, Trevizan PF, Fernandes FLA, Binotto MA, Liberato G, Tavares GMP, Neirotti RA, Jatene MB (2017) Impaired pulmonary function is an additional potential mechanism for the reduction of functional capacity in clinically stable Fontan patients. Pediatr Cardiol 38(5):981–990. https://doi.org/10.1007/s00246-017-1606-9

    Article  PubMed  Google Scholar 

  9. Callegari A, Neidenbach R, Milanesi O, Castaldi B, Christmann M, Ono M, Muller J, Ewert P, Hager A (2019) A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life. Congenit Heart Dis 14(2):147–155. https://doi.org/10.1111/chd.12694

    Article  PubMed  Google Scholar 

  10. Fredriksen PM, Therrien J, Veldtman G, Warsi MA, Liu P, Siu S, Williams W, Granton J, Webb G (2001) Lung function and aerobic capacity in adult patients following modified Fontan procedure. Heart 85(3):295–299. https://doi.org/10.1136/heart.85.3.295

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Paridon SM, Alpert BS, Boas SR, Cabrera ME, Caldarera LL, Daniels SR, Kimball TR, Knilans TK, Nixon PA, Rhodes J, Yetman AT, American Heart Association Council on Cardiovascular Disease in the Young CoAH, Obesity in Y (2006) Clinical stress testing in the pediatric age group: a statement from the American Heart Association Council on Cardiovascular Disease in the Young, Committee on Atherosclerosis, Hypertension, and Obesity in Youth. Circulation 113(15):1905–1920. https://doi.org/10.1161/CIRCULATIONAHA.106.174375

    Article  PubMed  Google Scholar 

  12. Jat KR (2013) Spirometry in children. Prim Care Respir J 22(2):221–229. https://doi.org/10.4104/pcrj.2013.00042

    Article  PubMed  PubMed Central  Google Scholar 

  13. Johnson JD, Theurer WM (2014) A stepwise approach to the interpretation of pulmonary function tests. Am Fam Physician 89(5):359–366

    PubMed  Google Scholar 

  14. Murray C, Foden P, Lowe L, Durrington H, Custovic A, Simpson A (2017) Diagnosis of asthma in symptomatic children based on measures of lung function: an analysis of data from a population-based birth cohort study. Lancet Child Adolesc Health 1(2):114–123. https://doi.org/10.1016/S2352-4642(17)30008-1

    Article  PubMed  PubMed Central  Google Scholar 

  15. Goldberg DJ, Zak V, McCrindle BW, Ni H, Gongwer R, Rhodes J, Garofano RP, Kaltman JR, Lambert LM, Mahony L, Margossian R, Spector ZZ, Williams RV, Atz AM, Paridon SM, Pediatric Heart Network I (2021) Exercise capacity and predictors of performance after Fontan: results from the pediatric heart network Fontan 3 study. Pediatr Cardiol 42(1):158–168. https://doi.org/10.1007/s00246-020-02465-1

    Article  PubMed  Google Scholar 

  16. Fernandes SM, McElhinney DB, Khairy P, Graham DA, Landzberg MJ, Rhodes J (2010) Serial cardiopulmonary exercise testing in patients with previous Fontan surgery. Pediatr Cardiol 31(2):175–180. https://doi.org/10.1007/s00246-009-9580-5

    Article  PubMed  PubMed Central  Google Scholar 

  17. Giardini A, Hager A, Pace Napoleone C, Picchio FM (2008) Natural history of exercise capacity after the Fontan operation: a longitudinal study. Ann Thorac Surg 85(3):818–821. https://doi.org/10.1016/j.athoracsur.2007.11.009

    Article  PubMed  Google Scholar 

  18. Jenkins PC, Chinnock RE, Jenkins KJ, Mahle WT, Mulla N, Sharkey AM, Flanagan MF (2008) Decreased exercise performance with age in children with hypoplastic left heart syndrome. J Pediatr 152(4):507–512. https://doi.org/10.1016/j.jpeds.2007.09.050

    Article  PubMed  Google Scholar 

  19. Paridon SM, Mitchell PD, Colan SD, Williams RV, Blaufox A, Li JS, Margossian R, Mital S, Russell J, Rhodes J, Pediatric Heart Network I (2008) A cross-sectional study of exercise performance during the first 2 decades of life after the Fontan operation. J Am Coll Cardiol 52(2):99–107. https://doi.org/10.1016/j.jacc.2008.02.081

    Article  PubMed  Google Scholar 

  20. Matthews IL, Fredriksen PM, Bjornstad PG, Thaulow E, Gronn M (2006) Reduced pulmonary function in children with the Fontan circulation affects their exercise capacity. Cardiol Young 16(3):261–267. https://doi.org/10.1017/S1047951106000345

    Article  PubMed  Google Scholar 

  21. Ginde S, Bartz PJ, Hill GD, Danduran MJ, Biller J, Sowinski J, Tweddell JS, Earing MG (2013) Restrictive lung disease is an independent predictor of exercise intolerance in the adult with congenital heart disease. Congenit Heart Dis 8(3):246–254. https://doi.org/10.1111/chd.12010

    Article  PubMed  Google Scholar 

  22. Sharma VJ, Iyengar AJ, Zannino D, Gentles T, Justo R, Celermajer DS, Bullock A, Winlaw D, Wheaton G, Burchill L, Cordina R, d’Udekem Y (2021) Protein-losing enteropathy and plastic bronchitis after the Fontan procedure. J Thorac Cardiovasc Surg 161(6):2158-2165.e2154. https://doi.org/10.1016/j.jtcvs.2020.07.107

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatrics, Children’s Hospital of Michigan, 3901 Beaubien Boulevard, Detroit, MI, 48201, USA

    Daiji Takajo

  2. Division of Pediatric Cardiology, Department of Pediatrics, Children’s Hospital of Michigan, Detroit, MI, USA

    Chenni S. Sriram, Preetha L. Balakrishnan & Sanjeev Aggarwal

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  1. Daiji Takajo
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  2. Chenni S. Sriram
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  3. Preetha L. Balakrishnan
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  4. Sanjeev Aggarwal
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Contributions

Dr. DT and Dr. SA conceptualized and designed the study, designed the data collected instruments, collected data, drafted the initial manuscript, and reviewed and revised the manuscript. Dr. CSS and Dr. PLB critically reviewed the manuscript for important intellectual content. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

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Correspondence to Daiji Takajo.

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The authors declare that they have no conflict of interest.

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This retrospective chart review study involving human participants was in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The Human Investigation Committee (IRB) of Wayne State University approved this study.

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Takajo, D., Sriram, C.S., Balakrishnan, P.L. et al. The Identification and Impact of Abnormal Spirometry Patterns on Exercise Capacity in Pediatric Patients with Fontan Palliation. Pediatr Cardiol 42, 1766–1774 (2021). https://doi.org/10.1007/s00246-021-02661-7

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  • DOI: https://doi.org/10.1007/s00246-021-02661-7

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