Pulmonary arterial hypertension associated with fenfluramine exposure: Report of 109 cases

European Respiratory Journal

Volumn 31, Issue 2, 2008, Pages 343-348

  Souza, R ^a   Humbert, M ^a,c   Sztrymf, B ^a   Jais X ^a   Yaici A ^a   Le Pavec, J ^a   Parent, F ^a   Herve P ^a   Soubrier, F ^b   Sitbon, O ^a   Simonneau, G ^a  

^a HÔPITAL ANTOINE BÉCLÈRE   (France)

^b PITIÉ SALPÊTRIÈRE HOSPITAL   (France)

^c UNIVERSITÉ PARIS SACLAY   (France)

Author keywords

Bone morphogenetic protein receptor type 2; Fenfluramine derivatives; Idiopathic pulmonary arterial hypertension; Pulmonary arterial hypertension; Survival

Indexed keywords

BONE MORPHOGENETIC PROTEIN RECEPTOR 2; FENFLURAMINE; ANOREXIGENIC AGENT;

ADULT; ARTICLE; CONTROLLED STUDY; DRUG EXPOSURE; FEMALE; GENE MUTATION; HEART INDEX; HEMODYNAMICS; HUMAN; MALE; PRIORITY JOURNAL; PULMONARY HYPERTENSION; SURVIVAL TIME; AGE DISTRIBUTION; ANALYSIS OF VARIANCE; CHEMICALLY INDUCED DISORDER; CONFIDENCE INTERVAL; FOLLOW UP; FRANCE; HOSPITALIZATION; INCIDENCE; MIDDLE AGED; MULTIVARIATE ANALYSIS; OBESITY; PATHOPHYSIOLOGY; PROPORTIONAL HAZARDS MODEL; RISK ASSESSMENT; SEX RATIO; SURVIVAL RATE;

ADULT; AGE DISTRIBUTION; ANALYSIS OF VARIANCE; APPETITE DEPRESSANTS; CONFIDENCE INTERVALS; FEMALE; FENFLURAMINE; FOLLOW-UP STUDIES; FRANCE; HUMANS; HYPERTENSION, PULMONARY; INCIDENCE; MALE; MIDDLE AGED; MULTIVARIATE ANALYSIS; OBESITY; PROPORTIONAL HAZARDS MODELS; RISK ASSESSMENT; SEVERITY OF ILLNESS INDEX; SEX DISTRIBUTION; SURVIVAL RATE;

EID: 40649100110     PISSN: 09031936     EISSN: 13993003     Source Type: Journal    
DOI: 10.1183/09031936.00104807     Document Type: Article

References (35)

1. Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004; 351: 1425-1436.
2. Eddahibi S, Morrell N, d'Ortho MP, Naeije R, Adnot S. Pathobiology of pulmonary arterial hypertension. Eur Respir J 2002; 20: 1559-1572.
3. Perros F, Dorfmüller P, Humbert M. Current insights on the pathogenesis of pulmonary arterial hypertension. Semin Respir Crit Care Med 2005; 26: 355-364.
4. Trembath RC, Harrison R. Insights into the genetic and molecular basis of primary pulmonary hypertension. Pediatr Res 2003; 53: 883-888.
5. Gurtner HP. [Chronic pulmonary hypertension of vascular origin, plexogenic pulmonary arteriopathy and the appetite depressant aminorex: addenda to an epidemic.]. Schweiz Med Wochenschr 1985; 115: 782-789.
6. Gurtner HP. Aminorex and pulmonary hypertension. A review. Cor Vasa 1985; 27: 160-171.
7. Loogen F, Worth H, Schwan G, Goeckenjan G, Lösse B, Horstkotte D. Long-term follow-up of pulmonary hypertension in patients with and without anorectic drug intake. Cor Vasa 1985; 27: 111-124.
8. Douglas JG, Munro JF, Kitchin AH, Muir AL, Proudfoot AT. Pulmonary hypertension and fenfluramine. Br Med J (Clin Res Ed) 1981; 283: 881-883.
9. Brenot F, Herve P, Petitpretz P, Parent F, Duroux P, Simonneau G. Primary pulmonary hypertension and fenfluramine use. Br Heart J 1993; 70: 537-541.
10. Abenhaim L, Moride Y, Brenot F, et al. Appetite-suppressant drugs and the risk of primary pulmonary hypertension. International Primary Pulmonary Hypertension Study Group. N Engl J Med 1996; 335: 609-616.
11. Eddahibi S, Adnot S. Anorexigen-induced pulmonary hypertension and the serotonin (5-HT) hypothesis: lessons for the future in pathogenesis. Respir Res 2002; 3: 9.
12. Hervé P, Launay JM, Scrobohaci ML, et al. Increased plasma serotonin in primary pulmonary hypertension. Am J Med 1995; 99: 249-254.
13. Eddahibi S, Humbert M, Fadel E, et al. Hyperplasia of pulmonary artery smooth muscle cells is causally related to overexpression of the serotonin transporter in primary pulmonary hypertension. Chest 2002; 121: Suppl. 3, 97S-98S.
14. Eddahibi S, Humbert M, Fadel E, et al. Serotonin transporter overexpression is responsible for pulmonary artery smooth muscle hyperplasia in primary pulmonary hypertension. J Clin Invest 2001; 108: 1141-1150.
15. Rich S, Rubin L, Walker AM, Schneeweiss S, Abenhaim L. Anorexigens and pulmonary hypertension in the United States: results from the surveillance of North American pulmonary hypertension. Chest 2000; 117: 870-874.
16. Rich S, Shillington A, McLaughlin V. Comparison of survival in patients with pulmonary hypertension associated with fenfluramine to patients with primary pulmonary hypertension. Am J Cardiol 2003; 92: 1366-1368.
17. Simonneau G, Galiè N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol 2004; 43: 5S-12S.
18. Guyatt GH, Sullivan MJ, Thompson PJ, et al. The 6-minute walk: a new measure of exercise capacity in patients with chronic heart failure. Can Med Assoc J 1985; 132: 919-923.
19. Sitbon O, Humbert M, Jaïs X, et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 111: 3105-3111.
20. Deng Z, Morse JH, Slager SL, et al. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 2000; 67: 737-744.
21. Humbert M, Deng Z, Simonneau G, et al. BMPR2 germline mutations in pulmonary hypertension associated with fenfluramine derivatives. Eur Respir J 2002; 20: 518-523.
22. Sitbon O, Humbert M, Nunes H, et al. Long-term intravenous epoprostenol infusion in primary pulmonary hypertension: prognostic factors and survival. J Am Coll Cardiol 2002; 40: 780-788.
23. McLaughlin VV, Shillington A, Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002; 106: 1477-1482.
24. McLaughlin VV, Sitbon O, Badesch DB, et al. Survival with first-line bosentan in patients with primary pulmonary hypertension. Eur Respir J 2005; 25: 244-249.
25. Souza R, Bogossian HB, Humbert M, et al. N-terminal-pro-brain natriuretic peptide as a haemodynamic marker in idiopathic pulmonary arterial hypertension. Eur Respir J 2005; 25: 509-513.
26. Costa EL, Jardim C, Bogossian HB, Amato MB, Carvalho CR, Souza R. Acute vasodilator test in pulmonary arterial hypertension: evaluation of two response criteria. Vascul Pharmacol 2005; 43: 143-147.
27. Thomson JR, Machado RD, Pauciulo MW, et al. Sporadic primary pulmonary hypertension is associated with germline mutations of the gene encoding BMPR-II, a receptor member of the TGF-β family. J Med Genet 2000; 37: 741-745.
28. Humbert M, Sitbon O, Chaouat A, et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006; 173: 1023-1030.
29. McLaughlin VV, Oudiz RJ, Frost A, et al. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med 2006; 174: 1257-1263.
30. Patel MR, Donahue M, Wilson PW, Califf RM. Clinical trial issues in weight-loss therapy. Am Heart J 2006; 151: 633-642.
31. Simonneau G, Fartoukh M, Sitbon O, Humbert M, Jagot JL, Hervé P. Primary pulmonary hypertension associated with the use of fenfluramine derivatives. Chest 1998; 114: 195S-199S.
32. Humbert M, Trembath RC. Genetics of pulmonary hypertension: from bench to bedside. Eur Respir J 2002; 20: 741-749.
33. Yuan JX, Rubin LJ. Pathogenesis of pulmonary arterial hypertension: the need for multiple hits. Circulation 2005; 111: 534-538.
34. D'Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991; 115: 343-349.
35. Jing Z, Sitbon O, Souza R, Jaïs X, Humbert M, Simonneau G. Survival of patients with pulmonary arterial hypertension (PAH) diagnosed at 3 different time periods. Proc Am Thorac Soc 2006; 3: A729.