Rationale and design of a trial on the role of bosentan in Fontan patients: Improvement of exercise capacity?

Contemporary Clinical Trials

Volumn 32, Issue 4, 2011, Pages 586-591

  Schuuring, Mark J ^a,b   Vis, Jeroen C ^a,b   Bouma, Berto J ^a   van Dijk, Arie P J ^c   van Melle, Joost P ^d   Pieper, Petronella G ^d   Vliegen, Hubert W ^e   Sieswerda, Gertjan Tj ^f   Mulder, Barbara J M ^a  

^a ACADEMIC MEDICAL CENTER   (Netherlands)

^b ROYAL NETHERLANDS ACADEMY OF ARTS AND SCIENCES   (Netherlands)

^c RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

^d UNIVERSITY MEDICAL CENTER GRONINGEN   (Netherlands)

^e LEIDEN UNIVERSITY MEDICAL CENTER   (Netherlands)

^f UNIVERSITY MEDICAL CENTER UTRECHT   (Netherlands)

Author keywords

Bosentan; Exercise capacity; Fontan

Indexed keywords

BOSENTAN; ENDOTHELIN 1;

ADULT; ARTICLE; CLINICAL ARTICLE; CONGENITAL HEART DISEASE; DRUG DOSE INCREASE; DRUG EFFICACY; EXERCISE TOLERANCE; FEMALE; FOLLOW UP; FONTAN PROCEDURE; FUNCTIONAL ASSESSMENT; HUMAN; HYPOTHESIS; LUNG VASCULAR RESISTANCE; MALE; METHODOLOGY; MULTICENTER STUDY; OPEN STUDY; OXYGEN CONSUMPTION; PROSPECTIVE STUDY; PROTEIN BLOOD LEVEL; PROTEIN EXPRESSION; PULMONARY HYPERTENSION; RANDOMIZED CONTROLLED TRIAL;

ADULT; ANTIHYPERTENSIVE AGENTS; CLINICAL PROTOCOLS; EXERCISE TEST; EXERCISE TOLERANCE; FEMALE; FONTAN PROCEDURE; HUMANS; MALE; OXYGEN CONSUMPTION; PATIENT SELECTION; PROSPECTIVE STUDIES; RANDOM ALLOCATION; RESEARCH DESIGN; SULFONAMIDES;

EID: 79957469848     PISSN: 15517144     EISSN: 15592030     Source Type: Journal    
DOI: 10.1016/j.cct.2011.04.001     Document Type: Article

References (28)

1. Gewillig M., Brown S.C., Eyskens B. The Fontan circulation: who controls cardiac output?. Interact Cardiovasc Thorac Surg 2010, 10(3):428-433.
2. Aaronson N.K., Muller M., Cohen P.D., Essink-Bot M.L., Fekkes M., Sanderman R., Sprangers M.A., te Velde A., Verrips E. Translation, validation, and norming of the Dutch language version of the SF-36 Health Survey in community and chronic disease populations. J Clin Epidemiol 1998, 11(11):1055-1068.
3. Ovaert C., Thijs D., Dewolf D. The effect of bosentan in patients with a failing Fontan circulation. Cardiol Young 2009, 1-9.
4. Kovacs G., Berghold A., Scheidl S., Olschewski H. Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J 2009, 34(4):888-894.
5. Gewillig M. The Fontan circulation. Heart 2005, 91(6):839-846.
6. Busse R., Fleming I. Pulsatile stretch and shear stress: physical stimuli determining the production of endothelium-derived relaxing factors. J Vasc Res 1998, 35(2):73-84.
7. Humbert M., Sitbon O., Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004, 351(14):1425-1436.
8. McNeill J.R. Role of endothelin in regulation of resistance, fluid-exchange, and capacitance functions of the systemic circulation. Can J Physiol Pharmacol 2003, 81(6):522-532.
9. Inai K., Nakanishi T., Nakazawa M. Clinical correlation and prognostic predictive value of neurohumoral factors in patients late after the Fontan operation. Am Heart J 2005, 150(3):588-594.
10. Galiè N., Begh M., Gatzoulis M. Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigator. Bosentan therapy in patients with Eisenmenger Syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation 2006, 114:48-54.
11. Duffels M.G.J., Vis J.C., van Loon R.L.E. Effect of bosentan on exercise capacity and quality of life in adults with pulmonary arterial hypertension associated with congenital heart disease with and without Down's syndrome. Am J Cardiol 2009, 103(9):1309-1315.
12. Duffels M.G.J., van der Plas M.N., Surie S. Bosentan in pulmonary arterial hypertension: a comparison between congenital heart disease and chronic pulmonary embolism. Neth Heart J 2009, 17(9):334-338.
13. Takahashi K., Mori Y., Yamamura H., Nakanishi T., Nakazawa M. Effect of beraprost sodium on pulmonary vascular resistance in candidates for a Fontan procedure: a preliminary study. Pediatr Int 2003, 45(6):671-675.
14. Khambadkone S., Li J., de Leval M.R. Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation. Circulation 2003, 107(25):3204-3208.
15. Cai J., Su Z., Shi Z. Nitric oxide and milrinone: combined effect on pulmonary circulation after Fontan-type procedure: a prospective, randomized study. Ann Thorac Surg 2008, 86(3):882-88888. discussion 882-8.
16. van der Velde E.T., Vander V.E.T., Vriend J.W.J. CONCOR, an initiative towards a national registry and DNA-bank of patients with congenital heart disease in the Netherlands: rationale, design, and first results. Eur J Epidemiol 2005, 20(6):549-557.
17. Duffels M.G.J., Engelfriet P.M., Berger R.M.F. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol 2007, 120(2):198-204.
18. Lancellotti P., Tribouilloy C., Hagendorff A. European Association of Echocardiography recommendations for the assessment of valvular regurgitation. Part 1: aortic and pulmonary regurgitation (native valve disease). Eur J Echocardiogr 2010, 11(3):223-244.
19. Lancellotti P., Moura L., Pierard L.A. European Association of Echocardiography recommendations for the assessment of valvular regurgitation. Part 2: mitral and tricuspid regurgitation (native valve disease). Eur J Echocardiogr 2010, 11(4):307-332.
20. Ross R.M. ATS/ACCP statement on cardiopulmonary exercise testing. Am J Respir Crit Care Med 2003, 167(10):1451. author reply 1451.
21. Ware J., Snow K., Kosiniski M., Gandek B. SF-36 Health Survey. Manual and Interpretation Guide. SF-36 Health Survey. Man Interpretation Guide 1993.
22. Giardini A., Balducci A., Specchia S. Effect of sildenafil on haemodynamic response to exercise and exercise capacity in Fontan patients. Eur Heart J 2008, 29(13):1681-1687.
23. Engelfriet P., Boersma E., Oechslin E. The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow-up period. The Euro Heart Survey on adult congenital heart disease. Eur Heart J 2005, 26(21):2325-2333.
24. van Nieuwenhuizen R.C., Peters M., Lubbers L.J. Abnormalities in liver function and coagulation profile following the Fontan procedure. Heart 1999, 82(1):40-46.
25. Beghetti M. Fontan and the pulmonary circulation: a potential role for new pulmonary hypertension therapies. Heart 2010, 96(12):911-916.
26. Schuuring M.J., Vis J.C., Duffels M.G., Bouma B.J., Mulder B.J. Adult patients with pulmonary arterial hypertension due to congenital heart disease: a review on advanced medical treatment with bosentan. Ther Clin Risk Manag 2010, 6:359-366.
27. Duffels M.G.J., Hardziyenka M., Surie S. Duration of right ventricular contraction predicts the efficacy of bosentan treatment in patients with pulmonary hypertension. Eur J Echocardiogr 2009, 10(3):433-438.
28. van der Plas M.N., Duffels M.G.J., Ponse D., Mulder B.J.M., Bresser P. Bosentan in mild pulmonary hypertension. Lancet 2008, 372(9651). 1730; author reply 1731.