Pediatric Cardiology

, Volume 40, Issue 8, pp 1688–1695 | Cite as

Does Restrictive Lung Function Affect the Exercise Capacity in Patients with Repaired Tetralogy of Fallot?

  • Jyothsna Akam-VenkataEmail author
  • Chenni Sriram
  • Michelle French
  • Roxann Smith
  • Sanjeev Aggarwal
Original Article


Patients with repaired Tetralogy of Fallot (rTOF) have decreased exercise capacity (XC) and restrictive lung function (RLF). Our objective was to determine the association between RLF and impaired XC in patients with rTOF. This was a single center retrospective review of patients with rTOF who underwent a cardiopulmonary treadmill exercise testing and spirometry from 2005 to 2015. Patients with a respiratory exchange ratio ≥ 1.05 and peak heart rate > 90% of predicted value were included. Forced vital capacity (FVC) and Forced expiratory volume in 1st second of forceful expiration (FEV1) were used to classify the lung function. Exercise parameters such as peak oxygen uptake (VO2), % of predicted VO2 (%VO2), Metabolic equivalents (METS), and exercise time (ET) were compared between the two groups (i) compared patients with normal lung function (normal FEV1, FVC, and FEV1/FVC > 80%) (ii) RLF (FVC < 80%, normal or increased FEV1/FVC > 80%). In our cohort (n = 151, 52% male, mean age ± SD of 22.3 ± 9.1 years), patients with RLF (n = 73) compared to those with normal lung function (n = 86) had a lower peak VO2 (30.8 ± 8.6 vs. 36.6 ± 9.8 mL/kg/min; p < 0.001) and shorter exercise time (9:23 ± 1:78 vs. 10:23 ± 1:62 min, p < 0.001). On multivariate regression analysis, RLF was independently associated with reduced XC (VO2%) (β-coefficient − 0.182, p < 0.02) after controlling for age and gender. RLF is common in patients with rTOF and is associated with decreased XC. The contribution of RLF to reduced XC in this population should be considered prior to therapeutic decisions.


Repaired Tetralogy of Fallot Restrictive lung function Impaired aerobic capacity Exercise capacity Lung function Exercise time 



Tetralogy of Fallot


Congenital heart disease


Exercise capacity


Volume of oxygen uptake


Pulmonary valve


Right ventricle


Forced vital capacity


Restrictive lung function


Heart rate


Blood pressure


Pulse oximetry


Volume of CO2 elimination


Minute ventilation


Maximum voluntary ventilation


Ventilatory anaerobic threshold


Tidal volume


Metabolic equivalents of task


Exercise time


Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflicts of interest.

Ethical Approval

This article does not contain any studies with human participants or animals performed by any of the authors. The study was approved by the Wayne State University School of Medicine and Detroit Medical Center Institutional Review Board.

Informed Consent

Not applicable. Waiver of consent obtained through Wayne State University School of Medicine and Detroit Medical Center Institutional Review Board.


  1. 1.
    Daliento L (2002) Total correction of tetralogy of Fallot: late clinical follow-up. Ital Heart J 3(1):24–27PubMedGoogle Scholar
  2. 2.
    Rhodes J, Dave A, Pulling MC, Geggel RL, Marx GR, Fulton DR, Hijazi ZM (1998) Effect of pulmonary artery stenoses on the cardiopulmonary response to exercise following repair of tetralogy of Fallot. Am J Cardiol 81(10):1217–1219CrossRefGoogle Scholar
  3. 3.
    Carvalho JS, Shinebourne EA, Busst C, Rigby ML, Redington AN (1992) Exercise capacity after complete repair of tetralogy of Fallot: deleterious effects of residual pulmonary regurgitation. Br Heart J 67(6):470–473CrossRefGoogle Scholar
  4. 4.
    Dallaire F, Wald RM, Marelli A (2017) The role of cardiopulmonary exercise testing for decision making in patients with repaired tetralogy of fallot. Pediatr Cardiol 38(6):1097–1105. CrossRefPubMedGoogle Scholar
  5. 5.
    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. CrossRefPubMedGoogle Scholar
  6. 6.
    Bossers SS, Helbing WA, Duppen N, Kuipers IM, Schokking M, Hazekamp MG, Bogers AJ, Ten Harkel AD, Takken T (2014) Exercise capacity in children after total cavopulmonary connection: lateral tunnel versus extracardiac conduit technique. J Thorac Cardiovasc Surg 148(4):1490–1497. CrossRefPubMedGoogle Scholar
  7. 7.
    Cohen KE, Buelow MW, Dixon J, Brazauskas R, Cohen SB, Earing MG, Ginde S (2017) Forced vital capacity predicts morbidity and mortality in adults with repaired tetralogy of Fallot. Congenit Heart Dis 12(4):435–440. CrossRefPubMedGoogle Scholar
  8. 8.
    Toma N, Bicescu G, Enache R, Dragoi R, Cinteza M (2010) Cardiopulmonary exercise testing in differential diagnosis of dyspnea. Maedica 5(3):214–218PubMedPubMedCentralGoogle Scholar
  9. 9.
    Neder JA, Nery LE, Castle AF, Sachs A, Silva AC, Whipp BJ (1998) Normal values for clinical exercise testing: a prospective and randomized study. Am J Respir Crit Care Med 157:A89CrossRefGoogle Scholar
  10. 10.
    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 (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. Scholar
  11. 11.
    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–H117. CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Alonso-Gonzalez R, Borgia F, Diller GP, Inuzuka R, Kempny A, Martinez-Naharro A, Tutarel O, Marino P, Wustmann K, Charalambides M, Silva M, Swan L, Dimopoulos K, Gatzoulis MA (2013) Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival. Circulation 127(8):882–890. CrossRefPubMedGoogle Scholar
  13. 13.
    Liu WH, Luo Q, Liu ZH, Zhao Q, Xi QY, Xue HF, Zhao ZH (2014) Pulmonary function differences in patients with chronic right heart failure secondary to pulmonary arterial hypertension and chronic left heart failure. Med Sci Monit 20:960–966. CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Guazzi M (2014) Abnormalities in cardiopulmonary exercise testing ventilatory parameters in heart failure: pathophysiology and clinical usefulness. Curr Heart Fail Rep 11(1):80–87. CrossRefPubMedGoogle Scholar
  15. 15.
    Mezzani A, Giordano A, Moussa NB, Micheletti A, Negura D, Saracino A, Canal E, Giannuzzi P, Chessa M, Carminati M (2015) Hemodynamic, not ventilatory, inefficiency is associated with high VE/VCO2 slope in repaired, noncyanotic congenital heart disease. Int J Cardiol 191:132–137. CrossRefPubMedGoogle Scholar
  16. 16.
    Sutton NJ, Peng L, Lock JE, Lang P, Marx GR, Curran TJ, O'Neill JA, Picard ST, Rhodes J (2008) Effect of pulmonary artery angioplasty on exercise function after repair of tetralogy of Fallot. Am Heart J 155(1):182–186. CrossRefPubMedGoogle Scholar
  17. 17.
    Legendre A, Richard R, Pontnau F, Jais JP, Dufour M, Grenier O, Mousseaux E, Ladouceur M, Iserin L, Bonnet D (2016) Usefulness of maximal oxygen pulse in timing of pulmonary valve replacement in patients with isolated pulmonary regurgitation. Cardiol Young 26(7):1310–1318. CrossRefPubMedGoogle Scholar
  18. 18.
    Shafer KM, Opotowsky AR, Rhodes J (2018) Exercise testing and spirometry as predictors of mortality in congenital heart disease: contrasting fontan physiology with repaired tetralogy of Fallot. Congenit Heart Dis 13(6):903–910. CrossRefPubMedGoogle Scholar
  19. 19.
    Hawkins SM, Taylor AL, Sillau SH, Mitchell MB, Rausch CM (2014) Restrictive lung function in pediatric patients with structural congenital heart disease. J Thorac Cardiovasc Surg 148(1):207–211. CrossRefPubMedGoogle Scholar
  20. 20.
    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 32(18):1998–2004. CrossRefPubMedGoogle Scholar
  21. 21.
    Ruchonnet-Metrailler I, Bessieres B, Bonnet D, Vibhushan S, Delacourt C (2014) Pulmonary hypoplasia associated with congenital heart diseases: a fetal study. PLoS ONE 9(4):e93557. CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Geva T (2006) Indications and timing of pulmonary valve replacement after tetralogy of Fallot repair. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. Scholar
  23. 23.
    Geva T (2013) Indications for pulmonary valve replacement in repaired tetralogy of fallot: the quest continues. Circulation 128(17):1855–1857. CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Sabate Rotes A, Johnson JN, Burkhart HM, Eidem BW, Allison TG, Driscoll DJ (2015) Cardiorespiratory response to exercise before and after pulmonary valve replacement in patients with repaired tetralogy of fallot: a retrospective study and systematic review of the literature. Congenit Heart Dis 10(3):263–270. CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Division of Cardiology, Department of PediatricsThe Carman and Ann Adams, Wayne State University School of Medicine, Children’s Hospital of MichiganDetroitUSA

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