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

Sickle Cell Disease Clinical Trial

— ETENDARD  

Official title:

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

Clinical Trial Details — Status: Active, not recruiting

Administrative data

• NCT number: NCT00434902
• Other study ID #: PO51082
• Secondary ID: AOM-05037
• Status: Active, not recruiting
• Phase: N/A
• First received: February 13, 2007
• Last updated: December 18, 2012
• Start date: February 2007
• Est. completion date: December 2012

Study information

• Verified date: November 2012
• Source: Assistance Publique - Hôpitaux de Paris
• Contact: n/a
• Is FDA regulated: No
• Health authority: France: Ministry of Health
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Active, not recruiting
• Enrollment: 700
• Est. completion date: December 2012
• Est. primary completion date: March 2009
• Accepts healthy volunteers: No
• Gender: Both
• Age group: 18 Years and older

Eligibility

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 Design

Observational Model: Cohort, Time Perspective: Prospective

Related Conditions & MeSH terms

• Anemia, Sickle Cell
• Hypertension
• Hypertension, Pulmonary
• Sickle Cell Disease

Locations

Country	Name	City	State
France	Hôpital Antoine Béclère	Clamart

Sponsors (1)

Lead Sponsor	Collaborator
Assistance Publique - Hôpitaux de Paris

Country where clinical trial is conducted

France, 

References & Publications (5)

Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004 Sep 30;351(14):1425-36. Review. — View Citation

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. — View Citation

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. — View Citation

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. — View Citation

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. — View Citation