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| ID | Type | Description | Link |
|---|---|---|---|
| AOM-05037 |
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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.
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.
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Inclusion Criteria:
Exclusion Criteria:
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Adult Patients With Sickle Cell Disease
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| Name | Affiliation | Role |
|---|---|---|
| Gerald SIMONNEAU, MD | Hôpital Antoine Béclère, CLAMART | Principal Investigator |
| Frederic Galacteros, MD | Hôpital Henri Mondor, Creteil | Principal Investigator |
| 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 Director |
| Olivier LAMBOTTE, MD | Hôpital Bicêtre, KREMLIN BICETRE | Study Chair |
| Jocelyn INAMO, MD |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hôpital Antoine Béclère | Clamart | 92141 | France |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 15194173 | Background | 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. | |
| 11907289 | Background | 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. |
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| ID | Term |
|---|---|
| D000755 | Anemia, Sickle Cell |
| D006976 | Hypertension, Pulmonary |
| ID | Term |
|---|---|
| D000745 | Anemia, Hemolytic, Congenital |
| D000743 | Anemia, Hemolytic |
| D000740 | Anemia |
| D006402 | Hematologic Diseases |
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| 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 Director |
| 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 Chair |
| 15459304 | Background | 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. |
| 12969866 | Background | 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. |
| 10807826 | Background | 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. |
| 40693500 | Derived | Simon T, Savale L, Grundtvig Skaarup K, Breillat P, Pham LL, Nouraie SM, Dyrby Johansen N, Inamo J, Lionnet F, Loko G, Chantalat C, Pham Hung d'Alexandry d'Orengiani AL, Habibi A, de Luna G, Iles S, Galacteros F, Audureau E, Biering-Sorensen T, Bartolucci P, Derumeaux G, d'Humieres T. Sickle Cell Diastolic Cardiomyopathy and Mortality Risk: A Novel Echocardiographic Framework for Prognostic Stratification. Am J Hematol. 2025 Nov;100(11):1940-1951. doi: 10.1002/ajh.27768. Epub 2025 Jul 22. |
| 21732836 | Derived | 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. |
| D006425 |
| Hemic and Lymphatic Diseases |
| D006453 | Hemoglobinopathies |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D006973 | Hypertension |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |