Abstract
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.
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Abbreviations
- TOF:
-
Tetralogy of Fallot
- CHD:
-
Congenital heart disease
- XC:
-
Exercise capacity
- VO2 :
-
Volume of oxygen uptake
- PV:
-
Pulmonary valve
- RV:
-
Right ventricle
- FVC:
-
Forced vital capacity
- RLF:
-
Restrictive lung function
- HR:
-
Heart rate
- BP:
-
Blood pressure
- SpO2 :
-
Pulse oximetry
- VCO2 :
-
Volume of CO2 elimination
- VE:
-
Minute ventilation
- MVV:
-
Maximum voluntary ventilation
- VAT:
-
Ventilatory anaerobic threshold
- TV:
-
Tidal volume
- METS:
-
Metabolic equivalents of task
- ET:
-
Exercise time
References
Daliento L (2002) Total correction of tetralogy of Fallot: late clinical follow-up. Ital Heart J 3(1):24–27
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):1217–1219
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):470–473
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. https://doi.org/10.1007/s00246-017-1656-z
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
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. https://doi.org/10.1016/j.jtcvs.2013.12.046
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. https://doi.org/10.1111/chd.12470
Toma N, Bicescu G, Enache R, Dragoi R, Cinteza M (2010) Cardiopulmonary exercise testing in differential diagnosis of dyspnea. Maedica 5(3):214–218
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:A89
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. https://doi.org/10.1161/CIRCULATIONAHA.106.174375.
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. https://doi.org/10.1152/ajpheart.00184.2014
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. https://doi.org/10.1161/CIRCULATIONAHA.112.126755
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. https://doi.org/10.12659/MSM.890409
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. https://doi.org/10.1007/s11897-013-0183-3
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. https://doi.org/10.1016/j.ijcard.2015.04.285
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. https://doi.org/10.1016/j.ahj.2007.08.019
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. https://doi.org/10.1017/S1047951115002504
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. https://doi.org/10.1111/chd.12661
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. https://doi.org/10.1016/j.jtcvs.2013.07.080
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. https://doi.org/10.1097/BRS.0b013e318131b225
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. https://doi.org/10.1371/journal.pone.0093557
Geva T (2006) Indications and timing of pulmonary valve replacement after tetralogy of Fallot repair. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. https://doi.org/10.1053/j.pcsu.2006.02.009.
Geva T (2013) Indications for pulmonary valve replacement in repaired tetralogy of fallot: the quest continues. Circulation 128(17):1855–1857. https://doi.org/10.1161/CIRCULATIONAHA.113.005878
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. https://doi.org/10.1111/chd.12207
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Akam-Venkata, J., Sriram, C., French, M. et al. Does Restrictive Lung Function Affect the Exercise Capacity in Patients with Repaired Tetralogy of Fallot?. Pediatr Cardiol 40, 1688–1695 (2019). https://doi.org/10.1007/s00246-019-02205-0
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DOI: https://doi.org/10.1007/s00246-019-02205-0