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

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

UNKNOWN

Total Enrollment

700 participants

Study Classification

OBSERVATIONAL

Study Start Date

2007-02-28

Study Completion Date

2012-12-31

Brief Summary

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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.

Detailed Description

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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.

Conditions

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Sickle Cell Disease

Keywords

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Doppler Echocardiography Pulmonary hypertension Sickle cell disease

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Eligibility Criteria

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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%

Minimum Eligible Age

18 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Responsible Party

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Responsibility Role SPONSOR

Role: STUDY_CHAIR

Hopital Antoine Béclère, CLAMART

Locations

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Hôpital Antoine Béclère

Clamart, , France

Site Status

Countries

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France

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AOM-05037

Identifier Type: -

Identifier Source: secondary_id

PO51082

Identifier Type: -

Identifier Source: org_study_id

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

Study Results

Basic Information

Brief Summary

Detailed Description

Conditions

Keywords

Study Design

Eligibility Criteria

Inclusion Criteria

Exclusion Criteria

Sponsors

Assistance Publique - Hôpitaux de Paris

Responsible Party

Principal Investigators

Gerald SIMONNEAU, MD

Frederic Galacteros, MD

Serge ADNOT, MD

Bernard MAITRE, MD

Marc HUMBERT, MD

Robert GIROT, MD

François LIONNET, MD

Françoise DRISS, MD

Olivier LAMBOTTE, MD

Jocelyn INAMO, MD

Gylna LOKO, MD

Olivier SITBON, MD

Xavier Jaïs, MD

Anoosha Habibi, MD

Dora Bachir, MD

Laurent SAVALE, MD

Saadia Eddahibi, MD

Gilles Garcia, MD

Locations

Hôpital Antoine Béclère

Countries

References

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.

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.

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.

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.

Other Identifiers

AOM-05037

PO51082