ETENDARD: Evaluation of the Prevalence of Pulmonary Hypertension in Adult Patients With Sickle Cell Disease
Study Details
Study Description
Brief Summary
Recent data show that pulmonary hypertension (PH), defined by a tricuspid regurgitation jet (TRJ) velocity > or equal at 2.5m/s on Doppler echocardiography, is present in about 30% of adults with sickle cell disease (SCD) and is associated with poor prognosis. However in SCD the occurrence of PH (defined by mean pulmonary arterial pressure (mPAP)> or equal at 25 mmHg) is related to at least 3 mechanisms: PH due to hyperkinetic state with high cardiac output (CO) but normal pulmonary vascular resistance (PVR <160 dynes), or postcapillary PH (pulmonary capillary wedge pressure PCWP >15 mmHg), or precapillary pulmonary arterial hypertension (PAH) defined by mPAP > or equal at 25 mmHg, PCWP< or equal at 15 mmHg and PVR > or equal at 160 dynes.The aim of this study is to evaluate in a French population of adults with sickle cell disease the characteristics, prevalence and prognosis of pulmonary hypertension.
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Detailed Description
Consecutive adult patients with sickle cell disease (SCD) had a Doppler echocardiography to evaluate if they had a suspected pulmonary hypertension (PH) on the basis of a tricuspid regurgitation jet (TRJ) velocity > or equal at 2.5m/s. In this case, a right heart catheterization was performed to confirm or not this diagnosis and its mechanisms. Each included patient was followed every year for 3 years: during each visit, a clinical evaluation was obtained and a Doppler echocardiography. In case of emergence of a suspected PH, a right heart catheterization was performed to confirm or not this diagnosis and its mechanisms.
Three groups of patients were defined: no PH, precapillary PH, and a third group including post-capillary PH and hyperkinetic state. These groups were well defined on the basis of the results of th Doppler echocardiography and right heart catheterisation.
Characteristics of patients and their prognosis were evaluated in each group.
In the same, way, biological study is planned to evaluate some biological markers of the mechanism of PH, and prognostic factors.
Study Design
Outcome Measures
Primary Outcome Measures
Eligibility Criteria
Criteria
Inclusion Criteria:
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Homozygous SS sickle cell disease
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Male or female > 18 years of age
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VOC (Vaso-Occlusive crisis) or ACS (Acute chest syndrome)within 6 weeks of inclusion ("Stable state")
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Signed written Informed consent
Exclusion Criteria:
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Creatinine clearance < 30 ml/mn
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prothrombin ratio < 50%
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Severe pneumopathy and TLC (Total lung capacity) < 70%
Contacts and Locations
Locations
| Site | City | State | Country | Postal Code | |
|---|---|---|---|---|---|
| 1 | Hôpital Antoine Béclère | Clamart | France | 92141 |
Sponsors and Collaborators
- Assistance Publique - Hôpitaux de Paris
Investigators
- Principal Investigator: Gerald SIMONNEAU, MD, Hôpital Antoine Béclère, CLAMART
- Principal Investigator: Frederic Galacteros, MD, Hôpital Henri Mondor, Creteil
- Study Director: Serge ADNOT, MD, Hôpital Henri Mondor, CRETEIL
- Study Director: Bernard MAITRE, MD, Hopital Henri Mondor, CRETEIL
- Study Director: Marc HUMBERT, MD, Hôpîtal Antoine Béclere, CLAMART
- Study Director: Robert GIROT, MD, Hôpital Tenon, PARIS
- Study Director: François LIONNET, MD, Hôpital TENON, PARIS
- Study Director: Françoise DRISS, MD, Hôpital Bicêtre, KREMLIN BICETRE
- Study Chair: Olivier LAMBOTTE, MD, Hôpital Bicêtre, KREMLIN BICETRE
- Study Director: Jocelyn INAMO, MD, CHU Fort de France
- Study Director: Gylna LOKO, MD, CHU Fort de France
- Study Director: Olivier SITBON, MD, Hôpital Antoine Béclère, CLAMART
- Study Director: Xavier Jaïs, MD, Hôpital Antoine Béclère, CLAMART
- Study Chair: Anoosha Habibi, MD, Hôpital Henri Mondor, CRETEIL
- Study Chair: Dora Bachir, MD, Hôpital Henri Mondor, CRETEIL
- Study Chair: Laurent SAVALE, MD, Hopital Henri Mondor, CRETEIL
- Study Chair: Saadia Eddahibi, MD, Hôpital Henri Mondor, CRETEIL
- Study Chair: Gilles Garcia, MD, Hopital Antoine Béclère, CLAMART
Study Documents (Full-Text)
None provided.More Information
Publications
- Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004 Sep 30;351(14):1425-36. Review.
- Lechapt E, Habibi A, Bachir D, Galacteros F, Schaeffer A, Desvaux D, Brochard L, Housset B, Godeau B, Maitre B. Induced sputum versus bronchoalveolar lavage during acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med. 2003 Dec 1;168(11):1373-7. Epub 2003 Sep 11.
- Maitre B, Habibi A, Roudot-Thoraval F, Bachir D, Belghiti DD, Galacteros F, Godeau B. Acute chest syndrome in adults with sickle cell disease. Chest. 2000 May;117(5):1386-92.
- Rubin LJ, Badesch DB, Barst RJ, Galie N, Black CM, Keogh A, Pulido T, Frost A, Roux S, Leconte I, Landzberg M, Simonneau G. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002 Mar 21;346(12):896-903. Erratum in: N Engl J Med 2002 Apr 18;346(16):1258.
- Simonneau G, Galiè N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, Gibbs S, Lebrec D, Speich R, Beghetti M, Rich S, Fishman A. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl S):5S-12S. Review.
- PO51082
- AOM-05037