Lung function and pulmonary regurgitation limit exercise capacity in postoperative tetralogy of Fallot

Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland 21205.

Fifty-five patients with repair of tetralogy of Fallot were evaluated with treadmill exercise, pulmonary function testing and rest two-dimensional and Doppler echocardiography to determine the relation among cardiopulmonary function, exercise capacity and ventricular arrhythmias. The mean age at repair was 8.1 +/- 2.1 years; age at the time of study ranged from 15 to 37 years (mean follow-up time after repair 18 +/- 5 years). Exercise duration was 92 +/- 17% of predicted. Maximal heart rate was 94 +/- 7% of predicted. No exercise test was stopped because of an arrhythmia. Thirty patients had oxygen consumption and ventilation measured during their final minute of exercise. Peak oxygen consumption was 31 +/- 8 ml/kg per min (86 +/- 18% of predicted). Twenty-five patients (45%) had low vital capacity at rest (less than 80% of predicted). Pulmonary regurgitation was identified in 42 (75%) of the patients and judged to be moderate in 10 (18%). Mild tricuspid valve regurgitation was identified in 64%. Doppler estimated right ventricular outflow gradient was greater than 15 mm Hg in 15% of the patients (mean gradient 24 mm Hg [range 16 to 56]). Age at repair, duration of follow-up and type of repair did not correlate with echocardiographic variables, ventilatory data, exercise performance or arrhythmias. Moderate pulmonary regurgitation was associated with increased right ventricular diastolic area and both were inversely related to exercise duration and vital capacity. Decreased breathing reserve during maximal exercise was associated with moderate pulmonary regurgitation and decreased vital capacity. The results indicate that exercise capacity in these patients is in general good; however, right ventricular volume loading and ventilatory dysfunction may produce exercise limitation.

This article has been cited by other articles:

				C. J. Petit, M. J. Gillespie, M. A. Harris, T. L. Seymour, T. Y. Liu, A. Khan, J. W. Gaynor, and J. J. Rome Relief of branch pulmonary artery stenosis reduces pulmonary valve insufficiency in a swine model. J. Thorac. Cardiovasc. Surg., August 1, 2009; 138(2): 382 - 389. [Abstract] [Full Text] [PDF]

				R. M. Wald, A. N. Redington, A. Pereira, Y. L. Provost, N. S. Paul, E. N. Oechslin, and C. K. Silversides Refining the assessment of pulmonary regurgitation in adults after tetralogy of Fallot repair: should we be measuring regurgitant fraction or regurgitant volume? Eur. Heart J., February 1, 2009; 30(3): 356 - 361. [Abstract] [Full Text] [PDF]

				R. R Chaturvedi and A. N Redington Pulmonary regurgitation in congenital heart disease Heart, July 1, 2007; 93(7): 880 - 889. [Full Text] [PDF]

				M. G. McBride, P. M. Kirshbom, J. W. Gaynor, R. F. Ittenbach, G. Wernovsky, R. R. Clancy, T. B. Flynn, D. M. Hartman, T. L. Spray, R. E. Tanel, et al. Late cardiopulmonary and musculoskeletal exercise performance after repair for total anomalous pulmonary venous connection during infancy J. Thorac. Cardiovasc. Surg., June 1, 2007; 133(6): 1533 - 1539. [Abstract] [Full Text] [PDF]

				M.-T. Wu, Y.-L. Huang, K.-S. Hsieh, J.-T. Huang, N.-J. Peng, J.-Y. Pan, J.-S. Huang, and T.-L. Yang Influence of Pulmonary Regurgitation Inequality on Differential Perfusion of the Lungs in Tetralogy of Fallot After Repair: A Phase-Contrast Magnetic Resonance Imaging and Perfusion Scintigraphy Study J. Am. Coll. Cardiol., May 8, 2007; 49(18): 1880 - 1886. [Abstract] [Full Text] [PDF]

				L. Nolke, A. Azakie, P. V. Anagnostopoulos, N. Alphonso, and T. R. Karl The Lecompte Maneuver for Relief of Airway Compression in Absent Pulmonary Valve Syndrome. Ann. Thorac. Surg., May 1, 2006; 81(5): 1802 - 1807. [Abstract] [Full Text] [PDF]

				J. Rhodes, T. J. Curran, L. Camil, N. Rabideau, D. R. Fulton, N. S. Gauthier, K. Gauvreau, and K. J. Jenkins Impact of Cardiac Rehabilitation on the Exercise Function of Children With Serious Congenital Heart Disease Pediatrics, December 1, 2005; 116(6): 1339 - 1345. [Abstract] [Full Text] [PDF]

				T. Reybrouck and L. Mertens Physical performance and physical activity in grown-up congenital heart disease European Journal of Cardiovascular Prevention & Rehabilitation, October 1, 2005; 12(5): 498 - 502. [Abstract] [Full Text] [PDF]

				M. Ercisli, K. M. Vural, K. N. Gokkaya, F. Koseoglu, O. Tufekcioglu, E. Sener, and O. Tasdemir Does Delayed Correction Interfere With Pulmonary Functions and Exercise Tolerance in Patients With Tetralogy of Fallot? Chest, August 1, 2005; 128(2): 1010 - 1017. [Abstract] [Full Text] [PDF]

				C. A. Warnes The Adult With Congenital Heart Disease: Born To Be Bad? J. Am. Coll. Cardiol., July 5, 2005; 46(1): 1 - 8. [Abstract] [Full Text] [PDF]

				A. van Straten, H. W. Vliegen, M. G. Hazekamp, J. J. Bax, P. H. Schoof, J. Ottenkamp, E. E. van der Wall, and A. de Roos Right Ventricular Function after Pulmonary Valve Replacement in Patients with Tetralogy of Fallot Radiology, December 1, 2004; 233(3): 824 - 829. [Abstract] [Full Text] [PDF]

				K. G. Warner, P. K. H. O'Brien, J. Rhodes, A. Kaur, D. A. Robinson, and D. D. Payne Expanding the indications for pulmonary valve replacement after repair of tetralogy of fallot Ann. Thorac. Surg., October 1, 2003; 76(4): 1066 - 1071. [Abstract] [Full Text] [PDF]

				I-S. Kang, A. N. Redington, L. N. Benson, C. Macgowan, E. R. Valsangiacomo, K. Roman, C. J. Kellenberger, and S.-J. Yoo Differential Regurgitation in Branch Pulmonary Arteries After Repair of Tetralogy of Fallot: A Phase-Contrast Cine Magnetic Resonance Study Circulation, June 17, 2003; 107(23): 2938 - 2943. [Abstract] [Full Text] [PDF]

				A. A. W. Roest, A. de Roos, H. J. Lamb, W. A. Helbing, J. G. van den Aardweg, J. Doornbos, E. E. van der Wall, and P. Kunz Tetralogy of Fallot: Postoperative Delayed Recovery of Left Ventricular Stroke Volume after Physical Exercise--Assessment with Fast MR Imaging Radiology, January 1, 2003; 226(1): 278 - 284. [Abstract] [Full Text] [PDF]

				Y. Mori, R. A. Rusk, M. Jones, X.-N. Li, T. Irvine, A. D. Zetts, and D. J. Sahn A new dynamic three-dimensional digital color doppler method for quantification of pulmonary regurgitation: validation study in an animal model J. Am. Coll. Cardiol., September 18, 2002; 40(6): 1179 - 1185. [Abstract] [Full Text] [PDF]

				C. C. Hew, S. H. Daebritz, D. Zurakowski, P. I. del Nido, J. E. Mayer Jr, and R. A. Jonas Valved homograft replacement of aneurysmal pulmonary arteries for severely symptomatic absent pulmonary valve syndrome Ann. Thorac. Surg., June 1, 2002; 73(6): 1778 - 1785. [Abstract] [Full Text] [PDF]

				T Reybrouck, L Mertens, S Brusselle, M Weymans, B Eyskens, J Defoor, and M Gewillig Oxygen uptake versus exercise intensity: a new concept in assessing cardiovascular exercise function in patients with congenital heart disease Heart, July 1, 2000; 84(1): 46 - 52. [Abstract] [Full Text] [PDF]

				M. A. Norgaard, P. Lauridsen, M. Helvind, and G. Pettersson Twenty-to-thirty-seven-year follow-up after repair for Tetralogy of Fallot Eur J Cardiothorac Surg, August 1, 1999; 16(2): 125 - 130. [Abstract] [Full Text] [PDF]

				P. Munkhammar, S. Cullen, P. Jogi, M. de Leval, M. Elliott, and G. Norgard Early age at repair prevents restrictive right ventricular (RV) physiology after surgery for tetralogy of Fallot (TOF): Diastolic RV function after TOF repair in infancy J. Am. Coll. Cardiol., October 1, 1998; 32(4): 1083 - 1087. [Abstract] [Full Text] [PDF]

				G.-C. Zhang, Z.-W. Wang, R.-F. Zhang, H.-Y. Zhu, and D.-H. Yi Surgical Repair of Patients With Tetralogy of Fallot and Unilateral Absence of Pulmonary Artery Ann. Thorac. Surg., October 1, 1997; 64(4): 1150 - 1153. [Abstract] [Full Text]

				R. R. Chaturvedi, P. J. Kilner, P. A. White, A. Bishop, R. Szwarc, and A. N. Redington Increased Airway Pressure and Simulated Branch Pulmonary Artery Stenosis Increase Pulmonary Regurgitation After Repair of Tetralogy of Fallot: Real-Time Analysis With a Conductance Catheter Technique Circulation, February 4, 1997; 95(3): 643 - 649. [Abstract] [Full Text]

				G. Norgard, M. A. Gatzoulis, F. Moraes, C. Lincoln, D. F. Shore, E. A. Shinebourne, and A. N. Redington Relationship Between Type of Outflow Tract Repair and Postoperative Right Ventricular Diastolic Physiology in Tetralogy of Fallot: Implications for Long-term Outcome Circulation, December 15, 1996; 94(12): 3276 - 3280. [Abstract] [Full Text]

				R. J. Baskett, D. B. Ross, M. A. Nanton, and D. A. Murphy FACTORS IN THE EARLY FAILURE OF CRYOPRESERVED HOMOGRAFT PULMONARY VALVES IN CHILDREN: PRESERVED IMMUNOGENICITY? J. Thorac. Cardiovasc. Surg., November 1, 1996; 112(5): 1170 - 1179. [Abstract] [Full Text]

				D. Mishaly, E. Birk, A. Elami, and B. A. Vidne Autologous Monocusp Pulmonary Valve: Preliminary Results Ann. Thorac. Surg., June 1, 1996; 61(6): 1811 - 1815. [Abstract] [Full Text]

				C. Kondo, M. Nakazawa, K. Kusakabe, and K. Momma Left Ventricular Dysfunction on Exercise Long Term After Total Repair of Tetralogy of Fallot Circulation, November 1, 1995; 92(9): 250 - 255. [Abstract] [Full Text]

				F. Meijboom, A. Szatmari, J. W. Deckers, E. M. W. J. Utens, J. R. T. C. Roelandt, E. Bos, and J. Hess CARDIAC STATUS AND HEALTH-RELATED QUALITY OF LIFE IN THE LONG TERM AFTER SURGICAL REPAIR OF TETRALOGY OF FALLOT IN INFANCY AND CHILDHOOD J. Thorac. Cardiovasc. Surg., October 1, 1995; 110(4): 883 - 891. [Abstract] [Full Text]

				H. Jonsson, T. Ivert, R. Jonasson, H. Wahlgren, A. Holmgren, and V. O. Bjork Pulmonary function thirteen to twenty-six years after repair of tetralogy of Fallot J. Thorac. Cardiovasc. Surg., December 1, 1994; 108(6): 1002 - 1009. [Abstract] [Full Text]

				A. A. W. Roest, W. A. Helbing, P. Kunz, J. G. van den Aardweg, H. J. Lamb, H. W. Vliegen, E. E. van der Wall, and A. de Roos Exercise MR Imaging in the Assessment of Pulmonary Regurgitation and Biventricular Function in Patients after Tetralogy of Fallot Repair Radiology, April 1, 2002; 223(1): 204 - 211. [Abstract] [Full Text] [PDF]