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

Locations

1 France site

Age

> 18 Years

Genotypes

SS

Study type

Observational

Gender

All

Compensation

Unknown

About the study

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 15 mmHg), or precapillary pulmonary arterial
hypertension (PAH) defined by mPAP > or equal at 25 mmHg, PCWP
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.

participation requirements

– 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

participation restrictions

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

Locations

  • Clamart, France, Hôpital Antoine Béclère, 92141