Bosentan improves exercise capacity in adolescents and adults after fontan operation: The TEMPO (treatment with endothelin receptor antagonist in fontan patients, a randomized, placebo-controlled, double-blind study measuring peak oxygen consumption) study

Circulation

Volumn 130, Issue 23, 2014, Pages 2021-2030

  Hebert, Anders ^a   Mikkelsen, Ulla R ^b   Thilen, Ulf ^c   Idorn, Lars ^a   Jensen, Annette S ^a   Nagy, Edit ^d   Hanseus, Katarina ^c   Sørensen, Keld E ^e   Søndergaard, Lars ^a  

^a Mental Health Center Glostrup   (Denmark)

^b UNIVERSITY OF COPENHAGEN   (Denmark)

^c LUND UNIVERSITY HOSPITAL   (Sweden)

^d KAROLINSKA UNIVERSITY HOSPITAL   (Sweden)

^e AARHUS UNIVERSITY HOSPITAL   (Denmark)

Author keywords

Bosentan; Congenital; Exercise test; Fontan procedure; Heart defects; Vasodilation

Indexed keywords

AMINOTRANSFERASE; BOSENTAN; BRAIN NATRIURETIC PEPTIDE; OXYGEN; PLACEBO; ENDOTHELIN A RECEPTOR; ENDOTHELIN RECEPTOR ANTAGONIST; SULFONAMIDE;

ADULT; AMINOTRANSFERASE BLOOD LEVEL; ANAEROBIC THRESHOLD; ARTHRALGIA; ARTICLE; BACKACHE; CARDIOPULMONARY EXERCISE TEST; CONTROLLED STUDY; DEPRESSED BLOOD PRESSURE; DOUBLE BLIND PROCEDURE; DRUG EFFECT; DRUG EFFICACY; DRUG SAFETY; DRUG WITHDRAWAL; DYSPEPSIA; EDEMA; EXERCISE TOLERANCE; FAINTNESS; FATIGUE; FEMALE; FLUSHING; FONTAN PROCEDURE; FUNCTIONAL ASSESSMENT; HEART MUSCLE OXYGEN CONSUMPTION; HEART PALPITATION; HUMAN; MAJOR CLINICAL STUDY; MALE; NAUSEA; NEW YORK HEART ASSOCIATION CLASS; OUTCOME ASSESSMENT; PROTEIN BLOOD LEVEL; PROTEIN LOSING GASTROENTEROPATHY; PRURITUS; QUALITY OF LIFE; RANDOMIZED CONTROLLED TRIAL; RECURRENT DISEASE; SHORT FORM 36; SIDE EFFECT; SYSTOLIC BLOOD PRESSURE; THORAX PAIN; ADOLESCENT; BLOOD; CHILD; DRUG EFFECTS; HEMODYNAMICS; INFANT; NONPARAMETRIC TEST; OXYGEN CONSUMPTION; POSTOPERATIVE COMPLICATIONS; PRESCHOOL CHILD; TREATMENT OUTCOME; YOUNG ADULT;

ADOLESCENT; ADULT; CHILD; CHILD, PRESCHOOL; DOUBLE-BLIND METHOD; ENDOTHELIN RECEPTOR ANTAGONISTS; EXERCISE TOLERANCE; FEMALE; FONTAN PROCEDURE; HEMODYNAMICS; HUMANS; INFANT; MALE; OXYGEN CONSUMPTION; PLACEBOS; POSTOPERATIVE COMPLICATIONS; RECEPTOR, ENDOTHELIN A; STATISTICS, NONPARAMETRIC; SULFONAMIDES; TREATMENT OUTCOME; YOUNG ADULT;

EID: 84916631939     PISSN: 00097322     EISSN: 15244539     Source Type: Journal    
DOI: 10.1161/CIRCULATIONAHA.113.008441     Document Type: Article

References (32)

1. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26: 240-248.
2. Samánek M. Children with congenital heart disease: Probability of natural survival. Pediatr Cardiol. 1992;13: 152-158.
3. de Leval MR. The Fontan circulation: A challenge to William Harvey? Nat Clin Pract Cardiovasc Med. 2005;2: 202-208.
4. Gewillig M. The Fontan circulation. Heart. 2005;91: 839-846.
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 Cardiothorac 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.
7. 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. 2013;167: 1311-1316.
8. Idorn L, Juul K, Jensen AS, Hanel B, Nielsen KG, Andersen H, Reimers JI, Sørensen KE, Søndergaard L. Arrhythmia and exercise intolerance in Fontan patients: Current status and future burden. Int J Cardiol. 2013;168: 1458-1465.
9. Diller GP, 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. La Gerche A, Gewillig M. What limits cardiac performance during exercise in normal subjects and in healthy Fontan patients? Int J Pediatr. 2010;2010. pii: 791291.
11. Gewillig M, Brown SC, Eyskens B, Heying R, Ganame J, Budts W, La Gerche A, Gorenflo M. The Fontan circulation: Who controls cardiac output? Interact Cardiovasc Thorac Surg. 2010;10: 428-433.
12. 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. Thorac Cardiovasc Surg. 2010;58: 468-472.
13. 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.
14. 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 Thorac Surg. 2009;88: 1961-1969.
15. 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.
16. Bowater SE, Weaver RA, Thorne SA, Clift PF. The safety and effects of bosentan in patients with a Fontan circulation. Congenit Heart Dis. 2012;7: 243-249.
17. Schuuring MJ, Vis JC, van Dijk AP, van Melle JP, Vliegen HW, Pieper PG, Sieswerda GT, de Bruin-Bon RH, Mulder BJ, Bouma BJ. Impact of bosentan on exercise capacity in adults after the Fontan procedure: A randomized controlled trial. Eur J Heart Fail. 2013;15: 690-698.
18. 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.
19. Thomas V, Costes F, Chatagnon M, Pouilly JP, Busso T. A comparison of lactate indices during ramp exercise using modelling techniques and conventional methods. J Sports Sci. 2008;26: 1387-1395.
20. 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.
21. Giardini A, Hager A, Pace Napoleone C, Picchio FM. Natural history of exercise capacity after the Fontan operation: A longitudinal study. Ann Thorac Surg. 2008;85: 818-821.
22. 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.
23. Rhodes J, Ubeda-Tikkanen A, Clair M, Fernandes SM, Graham DA, Milliren CE, Daly KP, Mullen MP, Landzberg MJ. Effect of inhaled iloprost on the exercise function of Fontan patients: A demonstration of concept. Int J Cardiol. 2013;168: 2435-2440.
24. Galiè N, Manes A, Negro L, Palazzini M, Bacchi-Reggiani ML, Branzi A. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J. 2009;30: 394-403.
25. Kuwana M, Watanabe H, Matsuoka N, Sugiyama N. Pulmonary arterial hypertension associated with connective tissue disease: Meta-analysis of clinical trials. BMJ Open. 2013;3: E003113.
26. Liu C, Chen J, Gao Y, Deng B, Liu K. Endothelin receptor antagonists for pulmonary arterial hypertension. Cochrane Database Syst Rev. 2013;2: CD004434.
27. Galiè N, Beghetti M, Gatzoulis MA, Granton J, Berger RM, Lauer A, Chiossi E, Landzberg M; Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators. Bosentan therapy in patients with Eisenmenger syndrome: A multicenter, double-blind, randomized, placebo-controlled study. Circulation. 2006;114: 48-54.
28. Boston: Little, Brown. Diseases of the Heart and Blood Vessels: Nomenclature and Criteria for Diagnosis. Boston, MA: Criteria Committee, New York Heart Association; 1964: 114.
29. Overgaard D, King C, Christensen RF, Schrader AM, Adamsen L. Living with half a heart: Experiences of young adults with single ventricle physiology: A qualitative study. J Cardiovasc Nurs. 2013;28: 187-196.
30. Drugs.com. Tracleer Official FDA information, side effects and uses. http://www.drugs.com/pro/tracleer.html. Accessed December 10, 2013.
31. Camposilvan S, Milanesi O, Stellin G, Pettenazzo A, Zancan L, D'Antiga L. Liver and cardiac function in the long term after Fontan operation. Ann Thorac Surg. 2008;86: 177-182.
32. Humbert M, Barst RJ, Robbins IM, Channick RN, Galiè N, Boonstra A, Rubin LJ, Horn EM, Manes A, Simonneau G. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J. 2004;24: 353-359.