The effect of Bosentan on exercise capacity in Fontan patients; rationale and design for the TEMPO study

BMC Cardiovascular Disorders

Volumn 13, Issue , 2013, Pages

  Hebert, Anders ^a   Jensen, Annette S ^a   Idorn, Lars ^a   Sørensen, Keld E ^b   Søndergaard, Lars ^a  

^a Rigshospitalet   (Denmark)

^b AARHUS UNIVERSITY HOSPITAL   (Denmark)

Author keywords

Endothelin receptor antagonist; Exercise capacity; Fontan; Randomized placebo controlled trial; Univentricular heart

Indexed keywords

ASPARTATE AMINOTRANSFERASE; BILIRUBIN; BOSENTAN; BRAIN NATRIURETIC PEPTIDE; ENDOTHELIN RECEPTOR ANTAGONIST; HEMOGLOBIN; PLACEBO;

ADOLESCENT; AMINOTRANSFERASE BLOOD LEVEL; ARTICLE; BICYCLE ERGOMETRY; BILIRUBIN BLOOD LEVEL; CHILD; CONTROLLED STUDY; DOUBLE BLIND PROCEDURE; DRUG SAFETY; DRUG WITHDRAWAL; EXERCISE; FEMALE; FONTAN PROCEDURE; HEART SINGLE VENTRICLE; HEMATOCRIT; HEMOGLOBIN BLOOD LEVEL; HUMAN; LUNG BLOOD FLOW; MAJOR CLINICAL STUDY; MALE; MULTICENTER STUDY; OXYGEN CONSUMPTION; OXYGEN SATURATION; PRIORITY JOURNAL; PROTEIN ANALYSIS; QUALITY OF LIFE; RANDOMIZED CONTROLLED TRIAL; SCHOOL CHILD; SIDE EFFECT; SURGICAL PATIENT; SYSTOLIC BLOOD PRESSURE; THROMBOCYTE COUNT; TOTAL CAVOPULMONARY CONNECTION; TREATMENT OUTCOME;

CHILD; CHILD, PRESCHOOL; CYCLIC N-OXIDES; DOUBLE-BLIND METHOD; EXERCISE TEST; FEMALE; FONTAN PROCEDURE; HEART DEFECTS, CONGENITAL; HUMANS; MALE; RECEPTORS, ENDOTHELIN; SULFONAMIDES; TREATMENT OUTCOME;

EID: 84877260982     PISSN: None     EISSN: 14712261     Source Type: Journal    
DOI: 10.1186/1471-2261-13-36     Document Type: Article

References (30)

1. Samánek M. Children with congenital heart disease: probability of natural survival. Pediatr Cardiol 1992, 13:152-158.
2. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971, 26:240-248. 10.1136/thx.26.3.240, 1019078, 5089489.
3. De Leval MR. The Fontan circulation: a challenge to William Harvey?. Nat Clin Pract Cardiovasc Med 2005, 2:202-208. 10.1038/ncpcardio0157, 16265484.
4. Gewillig M. The Fontan circulation. Heart 2005, 91:839-846. 10.1136/hrt.2004.051789, 1768934, 15894794.
5. Hosein RBM, Clarke AJB, McGuirk SP, Griselli M, Stumper O, De Giovanni JV, Barron DJ, Brawn WJ. Factors influencing early and late outcome following the Fontan procedure in the current era. The [']Two Commandments'?. Eur J Cardio-Thoracic Surg 2007, 31:344-353.
6. Ono M, Boethig D, Goerler H, Lange M, Westhoff-Bleck M, Breymann T. Clinical outcome of patients 20 years after Fontan operation-effect of fenestration on late morbidity. Eur J Cardiothorac Surg 2006, 30:923-929. 10.1016/j.ejcts.2006.08.025, 17074498.
7. McGuirk SP, Winlaw DS, Langley SM, Stumper OF, De Giovanni JV, Wright JG, Brawn WJ, Barron DJ. The impact of ventricular morphology on midterm outcome following completion total cavopulmonary connection. Eur J Cardiothorac Surg 2003, 24:37-46. 10.1016/S1010-7940(03)00186-6, 12853043.
8. Idorn L, Olsen M, Jensen AS, Juul K, Reimers JI, Sørensen K, Johnsen SP, Søndergaard L. Univentricular hearts in Denmark 1977 to 2009: Incidence and survival. Int J Cardiol 2012, [Epub].
9. Ostrow AM, Freeze H, Rychik J. Protein-losing enteropathy after fontan operation: investigations into possible pathophysiologic mechanisms. Ann Thorac Surg 2006, 82:695-700. 10.1016/j.athoracsur.2006.02.048, 16863787.
10. Diller G-P, Dimopoulos K, Okonko D, Li W, Babu-Narayan SV, Broberg CS, Johansson B, Bouzas B, Mullen MJ, Poole-Wilson PA, Francis DP, Gatzoulis MA. Exercise Intolerance in Adult Congenital Heart Disease: Comparative Severity, Correlates, and Prognostic Implication. Circulation 2005, 112:828-835. 10.1161/CIRCULATIONAHA.104.529800, 16061735.
11. D'Udekem Y, Cheung MMH, Setyapranata S, Iyengar AJ, Kelly P, Buckland N, Grigg LE, Weintraub RG, Vance A, Brizard CP, Penny DJ. How Good Is a Good Fontan? Quality of Life and Exercise Capacity of Fontans Without Arrhythmias. Ann Thoracic Surg 2009, 88:1961-1969.
12. Paridon SM, Mitchell PD, Colan SD, Williams RV, Blaufox A, Li JS, Margossian R, Mital S, Russell J, Rhodes J. A Cross-Sectional Study of Exercise Performance During the First 2 Decades of Life After the Fontan Operation. J Am Coll Cardiol 2008, 52:99-107. 10.1016/j.jacc.2008.02.081, 18598887.
13. La Gerche A, Gewillig M. What Limits Cardiac Performance during Exercise in Normal Subjects and in Healthy Fontan Patients?. Int J Pediatr 2010, 2010:1-8.
14. Gewillig M, Brown SC, Eyskens B, Heying R, Ganame J, Budts W, Gerche AL, Gorenflo M. The Fontan circulation: who controls cardiac output?. Interact Cardiovasc Thoracic Surg 2009, 10:428-433.
15. Zongtao Y, Huishan W, Zengwei W, Hongyu Z, Minhua F, Xinmin L, Nanbin Z, Hongguang H. Experimental Study of Nonpulsatile Flow Perfusion and Structural Remodeling of Pulmonary Microcirculation Vessels. Thoracic Cardiovascul Surg 2010, 58:468-472.
16. Yamagishi M, Kurosawa H, Hashimoto K, Nomura K, Kitamura N. The role of plasma endothelin in the Fontan circulation. J Cardiovasc Surg (Torino) 2002, 43:793-797.
17. Natarajan S, Heiss C, Yeghiazarians Y, Fineman JR, Teitel DF, Tacy TA. Peripheral arterial function in infants and young children with one-ventricle physiology and hypoxemia. Am J Cardiol 2009, 103:862-866. 10.1016/j.amjcard.2008.11.059, 19268746.
18. Giardini A, Balducci A, Specchia S, Gargiulo G, Bonvicini M, Picchio FM. Effect of sildenafil on haemodynamic response to exercise and exercise capacity in Fontan patients. Eur Heart J 2008, 29:1681-1687. 10.1093/eurheartj/ehn215, 18534975.
19. Ovaert C, Thijs D, Dewolf D, Ottenkamp J, Dessy H, Moons P, Gewillig M, Mertens L. The effect of bosentan in patients with a failing Fontan circulation. Cardiol Young 2009, 1-9.
20. Beghetti M. Fontan and the pulmonary circulation: a potential role for new pulmonary hypertension therapies. Heart 2010, 96:911-916. 10.1136/hrt.2010.193912, 20538665.
21. Uzun O, Wong JK, Bhole V, Stumper O. Resolution of protein-losing enteropathy and normalization of mesenteric Doppler flow with sildenafil after Fontan. Ann Thorac Surg 2006, 82:e39-40. 10.1016/j.athoracsur.2006.08.043, 17126088.
22. Stringer WW, Hansen JE, Wasserman K. Cardiac output estimated noninvasively from oxygen uptake during exercise. J Appl Physiol 1997, 82:908-912.
23. Thomas V, Costes F, Chatagnon M, Pouilly J-P, Busso T. A comparison of lactate indices during ramp exercise using modelling techniques and conventional methods. J Sports Sci 2008, 26:1387-1395. 10.1080/02640410802104920, 18923955.
24. Klausen SH, Mikkelsen UR, Hirth A, Wetterslev J, Kjærgaard H, Søndergaard L, Andersen LL. Design and rationale for the PREVAIL study: Effect of e-Health individually tailored encouragements to physical exercise on aerobic fitness among adolescents with congenital heart disease-a randomized clinical trial. Am Heart J 2012, 163:549-556. 10.1016/j.ahj.2012.01.021, 22520519.
25. McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, Mathier MA, McGoon MD, Park MH, Rosenson RS, Rubin LJ, Tapson VF, Varga J. ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension: A Report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association Developed in Collaboration With the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol 2009, 53:1573-1619. 10.1016/j.jacc.2009.01.004, 19389575.
26. Goldberg DJ, French B, McBride MG, Marino BS, Mirarchi N, Hanna BD, Wernovsky G, Paridon SM, Rychik J. Impact of Oral Sildenafil on Exercise Performance in Children and Young Adults After the Fontan Operation: A Randomized, Double-Blind, Placebo-Controlled, Crossover Trial. Circulation 2011, 123:1185-1193. 10.1161/CIRCULATIONAHA.110.981746, 3073351, 21382896.
27. Schuuring MJ, Vis JC, Van Dijk APJ, Van Melle JP, Vliegen HW, Pieper PG, Sieswerda GT, De Bruin-Bon RHACM, Mulder BJM, Bouma BJ. Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial. Eur J Heart Fail 2013, [Epub].
28. Bowater SE, Weaver RA, Thorne SA, Clift PF. The Safety and Effects of Bosentan in Patients with a Fontan Circulation. Congenital Heart Disease 2012, 7:243-249. 10.1111/j.1747-0803.2012.00635.x, 22348734.
29. Sullivan MJ, Knight JD, Higginbotham MB, Cobb FR. Relation between central and peripheral hemodynamics during exercise in patients with chronic heart failure. Muscle blood flow is reduced with maintenance of arterial perfusion pressure. Circulation 1989, 80:769-781. 10.1161/01.CIR.80.4.769, 2791242.
30. Weber KT, Janicki JS. Cardiopulmonary exercise testing for evaluation of chronic cardiac failure. Am J Cardiol 1985, 55:22A-31A. 10.1016/0002-9149(85)90792-1, 3966407.