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Arterial Stiffness Impairment in Sickle Cell Disease Associated With Chronic Vascular Complications: The
Brigitte Ranque1, Aymeric Menet2, Pierre Boutouyrie2
1From Internal Medicine Unit, Hôpital Européen Georges Pompidou, Assistance Publique des Hôpitaux de Paris, France (B.R.); UMR_S970, Inserm, Paris, France (B.R., P.B., S.L.J., X.J.); Faculté de médecine, Université Paris Descartes, France (B.R., P.B., X.J.); Cardiology Unit, groupement des hôpitaux de l'université catholique de Lille, université catholique de Lille, France (A.M.); Physiology Unit, Hôpital Européen Georges Pompidou, Assistance Publique des Hôpitaux de Paris, France (P.B.); Cardiology Unit, CHU de Fann, Dakar, Senegal (I.B.D., G.L.); Cardiology Unit, Hôpital Général, Yaoundé, Cameroon (S.K.); Cardiology Unit, Centre Gynéco-obstétrique, Bamako, Mali (M.D., C.O.D.); Institut de cardiologie, Abidjan, Ivory Coast (R.N'G.); Centre de recherche et Lutte contre la Drépanocytose, Bamako, Mali (D.D., Y.T.); Centre National de Transfusion Sanguine, Dakar, Senegal (S.D., B.F.F., M.S.); Pediatrics Unit, Centre Hopital Albert Royer, CHU de Fann, Dakar, Senegal (I.D., I.D.-L.); Hematology Unit, CHU de Yopougon, Abidjan, Ivory Coast (I.S., A.T., B.K., I.K.); Fondation Mère Enfant Chantal Biya, Yaoundé, Cameroon (D.C.); Pediatrics Unit, Centre Hospitalier d'Essos, Yaoundé, Cameroon (G.W.); CIRMF, Libreville, Gabon (J.P.G., C.A.); Internal Medicine Department, Hôpital Avicenne, Assistance Publique des Hôpitaux de Paris, France (S.L.J.); and Cardiology Department, Hôpital Européen Georges Pompidou, Assistance Publique des Hôpitaux de Paris France (X.J.). brigitte.ranque@egp.aphp.fr.
Insights
Arterial stiffness measures, pulse wave velocity (PWV) and augmentation index (AI), are altered in sickle cell disease (SCD) patients compared to controls. These changes correlate with SCD complications, suggesting prognostic value.
Area of Science:
- Cardiovascular Medicine
- Hematology
- Vascular Biology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder causing chronic vasculopathy and multi-organ damage.
- Arterial stiffness is a key indicator of cardiovascular health and a predictor of vascular complications.
Purpose of the Study:
- To assess arterial stiffness in SCD patients.
- To investigate associations between arterial stiffness and SCD-related vascular complications.
Main Methods:
- The CADRE study prospectively enrolled SCD patients and controls across five African countries.
- Carotid-femoral pulse wave velocity (cf-PWV) and augmentation index (AI) were measured.
- Multivariable regression analysis explored correlates and associations with clinical outcomes.
Main Results:
- Mean cf-PWV was lower in SCD patients than controls (P<0.0001).
- AI increased more rapidly with age in SCD patients and was higher in adult SCD patients.
- cf-PWV and AI were associated with age, sex, blood pressure, hemoglobin levels, and country.
- Both cf-PWV and AI were independently associated with glomerular filtration rate and osteonecrosis.
- AI was linked to stroke, pulmonary hypertension, and priapism; cf-PWV was linked to microalbuminuria.
Conclusions:
- Pulse wave velocity and augmentation index are significantly modified in SCD patients.
- These vascular changes are associated with clinical factors and SCD complications.
- PWV and AI may hold prognostic value for SCD vascular complications, requiring further assessment.
Background:
Although a blood genetic disease, sickle cell disease (SCD) leads to a chronic vasculopathy with multiple organ involvement. We assessed arterial stiffness in SCD patients and looked for associations between arterial stiffness and SCD-related vascular complications.
Methods:
The CADRE (Coeur Artères et Drepanocytose, ie, Heart Arteries and Sickle Cell Disease) study prospectively recruited pediatric and adult SCD patients and healthy controls in Cameroon, Ivory Coast, Gabon, Mali, and Senegal. Patients underwent clinical examination, routine laboratory tests (complete blood count, serum creatinine level), urine albumin/creatinine ratio measure, and a measure of carotid-femoral pulse wave velocity (cf-PWV) and augmentation index (AI) at a steady state. The clinical and biological correlates of cf-PWV and AI were investigated by using a multivariable multilevel linear regression analysis with individuals nested in families further nested in countries.
Results:
Included were 3627 patients with SCD and 943 controls. Mean cf-PWV was lower in SCD patients (7.5±2.0 m/s) than in controls (9.1±2.4 m/s, P<0.0001), and lower in SS-Sβ(0) than in SC-Sβ(+) phenotypes. AI, corrected for heart rate, increased more rapidly with age in SCD patients and was higher in SCD than in control adults. cf-PWV and AI were independently associated with age, sex, height, heart rate, mean blood pressure, hemoglobin level, country, and hemoglobin phenotype. After adjustment for these correlates, cf-PWV and AI were associated with the glomerular filtration rate and osteonecrosis. AI was also associated with stroke, pulmonary hypertension, and priapism, and cf-PWV was associated with microalbuminuria.
Conclusions:
PWV and AI are deeply modified in SCD patients in comparison with healthy controls. These changes are independently associated with a lower blood pressure and a higher heart rate but also with the hemoglobin phenotype. Moreover, PWV and AI are associated with several SCD clinical complications. Their prognostic value will be assessed at follow-up of the patients.
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