Skip to main content

Advertisement

SpringerLink
Log in

Impaired Pulmonary Function is an Additional Potential Mechanism for the Reduction of Functional Capacity in Clinically Stable Fontan Patients

  • Original Article
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

Central factors negatively affect the functional capacity of Fontan patients (FP), but “non-cardiac” factors, such as pulmonary function, may contribute to their exercise intolerance. We studied the pulmonary function in asymptomatic FP and its correlations with their functional capacity. Pulmonary function and cardiopulmonary exercise tests were performed in a prospective study of 27 FP and 27 healthy controls (HC). Cardiovascular magnetic resonance was used to evaluate the Fontan circulation. The mean age at tests, the mean age at surgery, and the median follow-up time of FP were 20(±6), 8(±3), and 11(8–17) years, respectively. Dominant ventricle ejection fraction was within normal range. The mean of peak VO2 expressed in absolute values (L/min), the relative values to body weight (mL/kg/min), and their predicted values were lower in FP compared with HC: 1.69 (±0.56) vs 2.81 (±0.77) L/min; 29.9 (±6.1) vs 41.5 (±9.3) mL/kg/min p < 0.001 and predicted VO2 Peak [71% (±14) vs 100% (±20) p < 0.001]. The absolute and predicted values of the forced vital capacity (FVC), forced expiratory volume in one second (FEV1), inspiratory capacity (IC), total lung capacity (TLC), diffusion capacity of carbon monoxide of the lung (DLCO), maximum inspiratory pressure (MIP), and sniff nasal inspiratory pressure (SNIP) were also significantly lower in the Fontan population compared to HC. An increased risk of restrictive ventilatory pattern was found in patients with postural deviations (OD:10.0, IC:1.02–97.5, p = 0.042). There was a strong correlation between pulmonary function and absolute peak VO2 [FVC (r = 0.86, p < 0.001); FEV1 (r = 0.83, p < 0.001); IC (r = 0.84, p < 0.001); TLC (r = 0.79, p < 0.001); and DLCO (r = 0.72, p < 0.001). The strength of the inspiratory muscles in absolute and predicted values was also reduced in FP [−79(±28) vs −109(±44) cmH2O (p = 0.004) and 67(±26) vs 89(±36) % (p = 0.016)]. Thus, we concluded that the pulmonary function was impaired in clinically stable Fontan patients and the static and dynamic lung volumes were significantly reduced compared with HC. We also demonstrated a strong correlation between absolute Peak VO2 with the FVC, FEV1, TLC, and DLCO measured by complete pulmonary test.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

  1. Caneo LF, Neirotti RA, Turquetto AL, Jatene MB (2016). The Fontan operation is not the end of the road. Arq Bras Cardiol 106:162–165

    PubMed  PubMed Central  Google Scholar 

  2. Gewillig M (2005) The Fontan circulation. Heart 91:839–846

    Article  PubMed  PubMed Central  Google Scholar 

  3. Gewillig M, Brown SC, Eyskens B, Heying R, Ganame J, Budts W et al (2010) The Fontan circulation: who controls cardiac output? Interact Cardiovasc Thorac Surg 10:428–433

    Article  PubMed  Google Scholar 

  4. Ohuchi H (2005) Cardiopulmonary response to exercise in patients with the Fontan circulation. Cardiol Young 15(Suppl 3):39–44

    Article  PubMed  Google Scholar 

  5. Matthews IL, Fredriksen PM, Bjornstad PG, Thaulow E, Gronn M (2006) Reduced pulmonary function in circulation affects their exercise. Cardiol Young 16:261–267

    Article  PubMed  Google Scholar 

  6. Fredriksen PM, Therrien J, Veldtman G, Warsi MA, Liu P, Siu S et al (2001) Lung function and aerobic capacity in adult patients following modified Fontan procedure. Heart 85:295–299

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Opotowsky AR, Landzberg MJ, Earing MG, Wu FM, Triedman JK, Casey A et al (2014) Abnormal spirometry after the Fontan procedure is common and associated with impaired aerobic capacity. Am J Physiol Heart Circ Physiol 307:H110–H117

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Larsson ES, Eriksson BO, Sixt R (2003) Decreased lung function and exercise capacity in Fontan patients. A long-term follow-up. Scand Cardiovasc J 37:58–63

    Article  CAS  PubMed  Google Scholar 

  9. Pereira CA, Sato T, Rodrigues SC (2007) New reference values for forced spirometry in white adults in Brazil. J Bras Pneumol 33:397–406

    Article  PubMed  Google Scholar 

  10. Mallozi MC. Valores de referência para espirometria em crianças e adolescentes, calculadas a partir de uma amostrada cidade de São Paulo. published in: Pereira CAC, Lemle A, Algranti E, Jansen JM, Valença LM, Nery LE, Mallozi M, Gerbasi M, Dias RM, Zim W. I Consenso Brasileiro sobre Espirometria. J Pneumol 1996;22:105–164

  11. Neder JA, Andreoni S, Castelo-Filho A, Nery LE (1999) Reference values for lung function tests. I. Static volumes. Braz J Med Biol Res 32:703–717

    Article  CAS  PubMed  Google Scholar 

  12. Neder JA, Andreoni S, Lerario MC, Nery LE (1999) Reference values for lung function tests. II. Maximal respiratory pressures and voluntary ventilation. Braz J Med Biol Res 32:719–727

    Article  CAS  PubMed  Google Scholar 

  13. Crapo RO, Morris AH (1981) Standardized single breath normal values for carbon monoxide diffusing capacity. Am Rev Respir Dis 123:185–189

    CAS  PubMed  Google Scholar 

  14. Araújo PR, Resqueti VR, Nascimento Junior J, Carvalho LA, Cavalcanti AGL, Silva VC, Silva E, Moreno MA, Andrade AFD, Fregonezi GAF (2012) Reference values for sniff nasal inspiratory pressure in healthy subjects in Brazil: a multicenter study. J Bras Pneumol 38(6):700–707

    Article  PubMed  Google Scholar 

  15. Meneghelo RS, Araujo CGS, Stein R, Mastrocolla LE, Albuquerque PF, Serra SM et al (2010) Sociedade Brasileira de Cardiologia. III Diretrizes da Sociedade de Cardiologia sobre Teste Ergométrico. Arq Bras Cardiol 95(5 supl 1):1–26

    Google Scholar 

  16. Drinkwater BL, Horvath SM, Wells CL (1975) Aerobic power of females, ages 10 to 68. J Gerontol 30:385–394

    Article  CAS  PubMed  Google Scholar 

  17. Morris CK, Myers J, Froelicher VF, Kawaguchi T, Ueshima K, Hideg A (1993) Nomogram based on metabolic equivalents and age for assessing aerobic exercise capacity in men. J Am Coll Cardiol 22:175–182

    Article  CAS  PubMed  Google Scholar 

  18. Skinner JS, McLellan TM (2013) The Transition from Aerobic to Anaerobic Metabolism (vol 51, pg 234, 1980). Res Q Exerc Sport 84:273–73

  19. Wasserman K (1984) The anaerobic threshold measurement to evaluate exercise performanceT. Am Rev Respir Dis 129:S35–S40

    Article  CAS  PubMed  Google Scholar 

  20. Powell AJ, Maier SE, Chung T, Geva T (2000) Phase-velocity cine magnetic resonance imaging measurement of pulsatile blood flow in children and young adults: in vitro and in vivo validation. Pediatr Cardiol 21:104–110

    Article  CAS  PubMed  Google Scholar 

  21. Trojnarska O, Gwizdala A, Katarzynski S, Katarzynska A, Oko-Sarnowska Z, Breborowicz P et al (2010) Evaluation of exercise capacity with cardiopulmonary exercise testing and BNP levels in adult patients with single or systemic right ventricles. Arch Med Sci 6:192–197

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Diller GP, Giardini A, Dimopoulos K, Gargiulo G, Müller J, Derrick G et al (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 31:3073–3083

    Article  PubMed  Google Scholar 

  23. Goldberg DJ, Avitabile CM, McBride MG, Paridon SM (2013) Exercise capacity in the Fontan circulation. Cardiol Young 23:824–830

    Article  PubMed  Google Scholar 

  24. Herrera-Soto JA, Vander Have KL, Barry-Lane P, Myers JL (2007) Retrospective study on the development of spinal deformities following sternotomy for congenital heart disease. Spine (Phila Pa 1976) 32:1998–2004

    Article  PubMed  Google Scholar 

  25. Ohuchi H, Ohashi H, Takasugi H, Yamada O, Yagihara T, Echigo S (2004) Restrictive ventilatory impairment and arterial oxygenation characterize rest and exercise ventilation in patients after Fontan operation. Pediatric Cardiol 25:513–521

    Article  CAS  Google Scholar 

  26. Greutmann M, Thao Lan L, Tobler D, Biaggi P, Oechslin EN, Silversides CK et al (2011) Generalised muscle weakness in young adults with congenital heart disease. Heart 97:1164–1168

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

Our special thanks to Carlos E Negrao, PhD, Director of Unit of Cardiovascular Rehabilitation and Exercise Physiology; Carlos RR Carvalho, MD, PhD, Director of Pulmonary Division; and Cesar Higa Nomura, MD, Director of Unit of Cardiovascular Magnetic Resonance and Tomography for excellent structure of respective laboratories. The authors are grateful to Rodolfo Neirotti Jr. MPP for his help in preparing the different parts of this manuscript.

Funding

This work was supported by the Foundation for Research Support of the State of São Paulo (FAPESP-2012/05252-5).

Author information

Authors and Affiliations

  1. Pediatric Cardiovascular Surgery Unit, Medical School, Heart Institute, University of Sao Paulo, Av. Dr. Eneas de Carvalho Aguiar, 44 – Bloco II - 2° andar – sala 5, São Paulo, SP, CEP: 05403-900, Brazil

    Aída L. R. Turquetto, Luiz F. Canêo, Daniela R. Agostinho, Patrícia A. Oliveira, Maria Isabel C. S. Lopes, Patrícia F. Trevizan, Frederico L. A. Fernandes, Maria A. Binotto, Gabriela Liberato, Glaucia M. P. Tavares & Marcelo B. Jatene

  2. Clinical Professor of Surgery and Pediatrics, Emeritus Michigan State University, 1199 Beacon Street, Unit # 2, Brookline, MA, 02446, USA

    Rodolfo A. Neirotti

Authors
  1. Aída L. R. Turquetto
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Luiz F. Canêo
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Daniela R. Agostinho
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Patrícia A. Oliveira
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Maria Isabel C. S. Lopes
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Patrícia F. Trevizan
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Frederico L. A. Fernandes
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Maria A. Binotto
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Gabriela Liberato
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Glaucia M. P. Tavares
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Rodolfo A. Neirotti
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Marcelo B. Jatene
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Aída L. R. Turquetto.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Turquetto, A.L.R., Canêo, L.F., Agostinho, D.R. et al. Impaired Pulmonary Function is an Additional Potential Mechanism for the Reduction of Functional Capacity in Clinically Stable Fontan Patients. Pediatr Cardiol 38, 981–990 (2017). https://doi.org/10.1007/s00246-017-1606-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-017-1606-9

Keywords

Search

Navigation