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

Study Overview
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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.

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

Conditions
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Sickle Cell Disease

Participation Criteria
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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%

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Doppler Echocardiography, Pulmonary hypertension, Sickle cell disease