Cardiopulmonary exercise tests in rare cardiovascular heart diseases

Natalia Dluzniewska, Klaudia Knap, Lidia Tomkiewicz‑Pająk, Maria Olszowska, Piotr Podolec

Full Text:



Cardiopulmonary exercise testing (CPET) is commonly used in clinica practice for both functional and diagnostic assessments of patients with cardiovascular and pulmonary disease. It provides assessment of the integrative exercise responses involving the pulmonary, cardiovascular and skeletal muscle systems, which are not adequately reflected through the measurement of individual organ system function. In a group of patients with a congenital heart disease or pulmonary hypertension assessment of exercise capacity and exercise tolerance can be a long term evaluation of treatment efficacy. It is also an objective diagnostic and prognostic tool of exercise capacity that allows to evaluate full actual physical condition of this population. JRCD 2015; 2 (5): 139–143


orphan diseases; exercise capacity; oxygen uptake; congenital heart diseases


Podolec P, Tomkiewicz L, Szcześniak J, Tracz W. Exercise tests and respiratory gas analysis in cardiology. Testing procedure and selected parameters needed for evaluation of cardiopulmonary system function. Przegl Lek. 1998; 55: 57–63.

Tomkiewicz-Pajak L, Podolec P, Kostkiewicz M, Tracz W. Lung function and exercise tolerance in patients with heart failure. Acta Cardiol. 2002; 57: 80–81.

Albouaini K, Egred M, Alahmar A, Wright D.J. Cardiopulmonary exercise testing and its application. Heart. 2007; 93: 1285–1292.

Balady GJ, Arena R, Sietsema K. et al. Clinician’s Guied to Cardiopulmonary Exercise Testing in Adults: A scientific statement from the American Heart Association; Circulation. 2010; 122: 191–225.

Arena R, Siestsema K. Cardiopulmonary Exercise Testing in the Clinical Evaluation of Patients with heart and lung disease. Circulation. 2011; 123: 668–680.

Podolec P, Jankowska EA, Ponikowski P, Banasiak W. Przewlekła niewydolność serca. Medycyna Praktyczna. 2009; 299–310.

ATS/ACCP statement on cardiopulmonary exercise testing. Am J Respir Care Med. 2003; 167: 211–277.

Fredriksen PM, Veldtman G, Hechter S. et all. Aerobic capacity in adults with various congenital heart diseases. Am J Cardiol. 2001; 87: 310–314.

Suchoń E, Tracz W, Podolec P, Tomkiewicz-Pajak L. Cardiopulmonary capacity and hemodynamic changes in adults with atrial septal defect Przegl Lek. 2008; 65: 177–179.

Giardini A, Donti A, Specchia S. et all. Long-term impact of transcatheter atrial septal defect closure in adults on cardiac function and exercise capacity. Int J Cardiol. 2008; 124: 179–182.

Meadows J, Lang P, Marx G. et al. Fontan fenestration closure has no acute effect on exercise capacity but improves ventilatory response to exercise. J Am Coll Cardiol. 2008; 52: 108–113

Trojnarska O, Gwizdała A, Katarzyński S, Katarzyńska A, Szyszka A, Lanocha M, Grajek S, Kramer L. Evaluation of exercise capacity with cardiopulmonary exercise test and B-type natriuretic peptide in adults with congenital heart disease. Cardiol J. 2009; 16: 133–141.

Gierat−Haponiuk K, Haponiuk I, Chojnicki M. et al. Exercise capacity and the quality of life late after surgical correction of congenital heart defects.; Kardiologia Polska. 2011; 69: 810–815.

Rhodes J. Cardiopulmonary Exercise Testing in Adults with Congenital Heart Disease: Can We Prognosticate and Improve Prognosis? Circulation. 2012; 125: 210–211.

Diller GP, Dimopoulos K, Okonko D. et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005; 112: 828–835.

Inuzuka R, Diller GP, Borgia F. et al. Comprehensive Use of Cardiopulmonary Exercise Testing Identifies Adults With Congenital Heart Disease at Increased Mortality Risk in the Medium Term. Circulation. 2012; 125: 250–259.

Dimopoulos K, Okonko DO, Diller GP. et al. Abnormal ventilatory response to exercise in adults with congenital heart disease relates to cyanosis and predicts survival. Circulation. 2006; 113: 2796–2802.

Gierat-Haponiuk K, Haponiuk I, Chojnicki M, et al. Exercise capacity and the quality of life late after surgical correction of congenital heart defects. Kardiol Pol. 2011; 69: 810–815.

Arena R, Lavie CJ, Milanie RV, Myers J, Guazzi M. Cardiopulmonary exercise testing in patients with pulmonary hypertension: an evidence-based review. J Heart Lung Transplant. 2010; 29: 159–173.

Janicki JS, Weber KT, Likoff MJ, Fishman AP. Exercise testing to evaluate patients with pulmonary vascular disease. Am Rev Respir Dis. 1984; 129: 93–95.

Sun XG, Hansen JE, Oudiz RJ, Wasserman K. Gas exchange detection of exercise-induced right-to-left shunt in patients with primary pulmonary hypertension. Circulation. 2002; 105: 54–60.

Holverda S, Bogaard HJ, Groepenhoff H. et al. Cardiopulmonary exercise test characteristics in patients with chronic obstructive pulmonary disease and associated pulmonary hypertension. Respiration. 2008; 76: 160–167.

Ting H, Sun XG, Chuang ML. et al. A noninvasive assessment of pulmonary perfusion abnormality in patients with primary pulmonary hypertension. Chest. 2001; 119: 824–832.

Ponikowski P, Francis DP, Piepoli MF. et al. Enhanced ventilatory response to exercise in patients with chronic heart failure and preserved exercise tolerance: marker of abnormal cardiorespiratory reflex control and predictor of poor prognosis. Circulation. 2001; 103: 967–972.

Francis DP, Shamim W, Davies LC. Et al. Cardiopulmonar exercise testing for prognosis in chronic heart failure: continuous and independent prognostic value from VE/VCO(2)slope and peak VO(2). Eur Heart J. 2000; 21: 154–161.

Tomkiewicz-Pajak L, Podolec P, Kostkiewicz M. i wsp. Prognostic assessment of patients with heart failure-based on the non-invasive procedures. Acta Cardiol. 2002; 57: 81–82.

Witte KK, Cleland JG, Clark AL. Chronic heart failure, chronotropic incompetence, and the effects of beta blockade. Heart. 2006; 92: 481–486.

Arena R, Guazzi M, Myers J, Peberdy MA. Prognostic value of heart rate recovery in patients with heart failure. Am Heart J. 2006; 151: 7–13.

Brubaker PH, Marburger CT, Morgan TM, Fray B, Kitzman DW. Exercise responses of elderly patients with diastolic versus systolic heart failure. Med Sci Sports Exerc. 2003; 35: 1477–1485.

Farr MJ, Lang CC, Lamanca JJ, Zile MR, Francis G, Tavazzi L, Gaasch WH, St John Sutton M, Itoh H, Mancini D; MCC-135 GO1 Investigators. Cardiopulmonary exercise variables in diastolic versus systolic heart failure. Am J Cardiol. 2008; 102: 203–206.

Guazzi M, Myers J, Arena R. Cardiopulmonary exercise testing in the clinical and prognostic assessment of diastolic heart failure. J Am Coll Cardiol. 2005; 46: 1883–1890.

Guazzi M, Myers J, Peberdy MA, Bensimhon D, Chase P, Arena R. Exercise oscillatory breathing in diastolic heart failure: prevalence and prognostic insights. Eur Heart J. 2008; 29: 2751–2759.

Guazzi M, Myers J, Peberdy MA. et al. Cardiopulmonary exercise testing variables reflect the degree of diastolic dysfunction in patients with heart failure-normal ejection fraction. J Cardiopulm Rehabil Prev. 2010; 30: 165–172.

Płazak W, Heród P, Drapisz S, et al. Influence of disease-related heart pathology on peak oxygen uptake and ventilation/carbon dioxide output ratio in systemic sclerosis and systemic lupus terythematosus patients (RCD code: I-3C). J Rare Cardiovasc Dis 2013; 1: 96–102.

Hunt SA, Abraham WT, Chin MH. et al. ACC/AHA 2005 guideline update for the diagnosis and management of chronic heart failure in the adult: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Update the 2001 Guidelines for the Evaluation and Management of Heart Failure): developed in collaboration with the American College of Chest Physicians and the International Society for Heart and Lung Transplantation: endorsed by the Heart Rhythm Society. Circulation. 2005; 112: 154–235.

Arena R, Myers J, Abella J. et al. Development of a ventilatory classification system in patients with heart failure. Circulation. 2007; 115: 2410–2417.

Weber KT, Janicki JS, McElroy PA. Determination of aerobic capacity and the severity of chronic cardiac and circulatory failure. Circulation. 1987; 76(suppl): VI-40–VI-45.

Arena R, Myers J, Guazzi M. The clinical and research applications of aerobic capacity and ventilatory efficiency in heart failure: an evidence-based review. Heart Fail Rev. 2008; 13: 245–269.

Guazzi M,Arena R. The impact of pharmacotherapy on the cardiopulmonary exercise test response in patients with heart failure: a mini review. Curr Vasc Pharmacol. 2009; 7: 557–569.

Mehra MR, Kobashigawa J, Starling R. et al. Listing criteria for heart transplantation: International Society for Heart and Lung Transplantation guidelines for the care of cardiac transplant candidates-2006. J Heart Lung Transplant. 2006; 25: 1024–1042.

Rywik TM, Janas J, Klisiewicz A, et al. Prognostic value of novel biomarkers compared with detailed biochemical evaluation in patients with heart failure. Pol Arch Med Wewn. 2015; 125: 434–442.



  • There are currently no refbacks.
Journal of Rare Cardiovascular Diseases (JRCD)
John Paul II Hospital in Kraków, 80 Prądnicka Str., 31-202 Kraków, Poland
Phone: +48 (12) 614 33 99, +48 (12) 614 34 88 Fax: +48 (12) 614 34 88
Published by SoftQ sp. z o.o.
ul. Oleandry 2, 30-063 Kraków, Poland
Phone: +48 (12) 444 1650 Fax: +48 (12) 444 1659