Evaluation of the Prevalence of Pulmonary Hypertension in Adult Patients With Sickle Cell Disease (ETENDARD)

Evaluation of the Characteristics, Prevalence and Prognosis of Pulmonary Hypertension in Adult Patients With Sickle Cell Disease: Study ETENDARD.

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.

Study Overview

• Status: Unknown
• Conditions: Sickle Cell Disease

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 Type: Observational
• Enrollment (Anticipated): 700

Contacts and Locations

Study Locations

• France
  • Clamart, France, 92141
    • Hôpital Antoine Béclère

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Adult Patients With Sickle Cell Disease

Description

Inclusion Criteria:

• Homozygous SS sickle cell disease
• Male or female > 18 years of age
• VOC (Vaso-Occlusive crisis) or ACS (Acute chest syndrome)within 6 weeks of inclusion ("Stable state")
• Signed written Informed consent

Exclusion Criteria:

• Creatinine clearance < 30 ml/mn
• prothrombin ratio < 50%
• Severe pneumopathy and TLC (Total lung capacity) < 70%

Study Plan

How is the study designed?

Collaborators and Investigators

• Sponsor: Assistance Publique - Hôpitaux de Paris
• Investigators: Principal Investigator: Gerald SIMONNEAU, MD, Hopital Antoine Béclère, CLAMART; Principal Investigator: Frederic Galacteros, MD, Hopital Henri Mondor, Creteil; Study Director: Serge ADNOT, MD, Hopital Henri Mondor, Creteil; Study Director: Bernard MAITRE, MD, Hopital Henri Mondor, Creteil; Study Director: Marc HUMBERT, MD, Hopital Antoine Béclère, 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, Hopital Antoine Béclère, CLAMART; Study Director: Xavier Jaïs, MD, Hopital Antoine Béclère, CLAMART; Study Chair: Anoosha Habibi, MD, Hopital Henri Mondor, Creteil; Study Chair: Dora Bachir, MD, Hopital Henri Mondor, Creteil; Study Chair: Laurent SAVALE, MD, Hopital Henri Mondor, Creteil; Study Chair: Saadia Eddahibi, MD, Hopital Henri Mondor, Creteil; Study Chair: Gilles Garcia, MD, Hopital Antoine Béclère, CLAMART

Publications and helpful links

General Publications

• 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. doi: 10.1056/NEJMoa012212. Erratum In: N Engl J Med 2002 Apr 18;346(16):1258.
• Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004 Sep 30;351(14):1425-36. doi: 10.1056/NEJMra040291. No abstract available.
• Simonneau G, Galie 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. doi: 10.1016/j.jacc.2004.02.037.
• 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. doi: 10.1164/rccm.200302-174OC. 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. doi: 10.1378/chest.117.5.1386.
• Parent F, Bachir D, Inamo J, Lionnet F, Driss F, Loko G, Habibi A, Bennani S, Savale L, Adnot S, Maitre B, Yaici A, Hajji L, O'Callaghan DS, Clerson P, Girot R, Galacteros F, Simonneau G. A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med. 2011 Jul 7;365(1):44-53. doi: 10.1056/NEJMoa1005565.

Study record dates

Study Major Dates

• Study Start: February 1, 2007
• Primary Completion (Actual): March 1, 2009
• Study Completion (Anticipated): December 1, 2012

Study Registration Dates

• First Submitted: February 13, 2007
• First Submitted That Met QC Criteria: February 13, 2007
• First Posted (Estimate): February 14, 2007

Study Record Updates

• Last Update Posted (Estimate): December 19, 2012
• Last Update Submitted That Met QC Criteria: December 18, 2012
• Last Verified: November 1, 2012

More Information

Terms related to this study

• Keywords: Pulmonary hypertension; Sickle cell disease; Doppler Echocardiography
• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Respiratory Tract Diseases; Lung Diseases; Hematologic Diseases; Genetic Diseases, Inborn; Anemia; Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Hemoglobinopathies; Hypertension; Anemia, Sickle Cell; Hypertension, Pulmonary
• Other Study ID Numbers: PO51082; AOM-05037