title: Haematological determinants of cardiac involvement in adults with sickle cell disease.
abstract: OBJECTIVE
  Cardiac involvement is common in sickle cell disease (SCD). Studies are needed to establish haematological determinants of this involvement and prognostic markers. The aim of the study was to identify haematological factors associated with cardiac involvement in SCD and their impact on prognosis.
  
  
  RESULTS
  This longitudinal observational study was performed on 1780 SCD patients with SS or S-β(0)-thalassemia referred to our centre. Six hundred fifty-six met our inclusion criteria (availability of a blood-workup and echocardiogram obtained <1 year apart, no heart valve surgery and no current pregnancy). Median age was 31 (interquartile range, 25-40) years, and median haemoglobin (Hb) was 87 (80-95)g/L. Left ventricular (LV) dilation, left atrial dilation, cardiac index (CI) >4 L/min/m(2), LV ejection fraction <55%, and tricuspid regurgitant velocity (TRV) ≥2.5 m/s were found in 35, 78, 23, 8.5, and 17% of patients, respectively. Compared with other patients, those in the fourth quartiles (Q4) of LV end-diastolic dimension index (LVEDDind) and left atrial dimension index (LADind) and those with high CI had significantly lower Hb, % foetal Hb (HbF), and red blood cell (RBC) counts; and significantly higher lactate dehydrogenase, bilirubin, and %dense RBCs. Independent haematologic determinants of Q4 LVEDDind and LADind were low RBC count and %HbF; high %dense RBCs were associated with LADind. Low %HbF and RBC count were associated with high CI. High %dense RBCs or no α-thalassemia gene deletion was associated with greater severity of anaemia and cardiac dilation and with higher CI. During the median follow-up of 48 (32-59) months, 50 (7.6%) patients died. Tricuspid regurgitant velocity ≥ 2.5 m/s was a predictor of mortality. The risk of death increased four-fold when left ventricular ejection fraction <55% was present also (P = 0.0001).
  
  
  CONCLUSIONS
  Cardiac dilation and CI elevation in patients with SCD are associated with haematologic variables reflecting haemolysis, RBC rigidity, and blood viscosity. Tricuspid regurgitant velocity ≥ 2.5 and LV dysfunction (even mild) predict mortality.
authors.affiliation: AP-HP, Department of Cardiology, Henri Mondor Teaching Hospital, 51 Avenue Maréchal de Lattre de Tassigny, Creteil F-94000, France.|School of Medicine, Paris-Est University (UPEC), 61 avenue du Général de Gaulle, Créteil F-94000, France.|IMRB INSERM U955, GRC Amyloidosis Research Institute, Paris-Est University (UPEC), 8 rue du Général Sarrail, Créteil 94000, France.|DHU ATVB, Henri Mondor Teaching Hospital, Creteil F-94000, France.|INSERM Clinical Investigation Centre 1430, Créteil F-94000, France.|Mondor Amyloidosis Network, Créteil F-94000, France.
authors.forename: Thibaud
authors.initials: T
authors.lastname: Damy
authors.affiliation: AP-HP, Department of Cardiology, Henri Mondor Teaching Hospital, 51 Avenue Maréchal de Lattre de Tassigny, Creteil F-94000, France.|School of Medicine, Paris-Est University (UPEC), 61 avenue du Général de Gaulle, Créteil F-94000, France.|IMRB INSERM U955, GRC Amyloidosis Research Institute, Paris-Est University (UPEC), 8 rue du Général Sarrail, Créteil 94000, France.|DHU ATVB, Henri Mondor Teaching Hospital, Creteil F-94000, France.|INSERM Clinical Investigation Centre 1430, Créteil F-94000, France.|Mondor Amyloidosis Network, Créteil F-94000, France.
authors.forename: Diane
authors.initials: D
authors.lastname: Bodez
authors.affiliation: DHU ATVB, Henri Mondor Teaching Hospital, Creteil F-94000, France.|AP-HP, UMGGR, Henri Mondor Teaching Hospital, Creteil F-94000, France.
authors.forename: Anoosha
authors.initials: A
authors.lastname: Habibi
authors.affiliation: AP-HP, Department of Cardiology, Henri Mondor Teaching Hospital, 51 Avenue Maréchal de Lattre de Tassigny, Creteil F-94000, France.|School of Medicine, Paris-Est University (UPEC), 61 avenue du Général de Gaulle, Créteil F-94000, France.|IMRB INSERM U955, GRC Amyloidosis Research Institute, Paris-Est University (UPEC), 8 rue du Général Sarrail, Créteil 94000, France.|DHU ATVB, Henri Mondor Teaching Hospital, Creteil F-94000, France.|INSERM Clinical Investigation Centre 1430, Créteil F-94000, France.|Mondor Amyloidosis Network, Créteil F-94000, France.
authors.forename: Aziz
authors.initials: A
authors.lastname: Guellich
authors.affiliation: AP-HP, Department of Cardiology, Henri Mondor Teaching Hospital, 51 Avenue Maréchal de Lattre de Tassigny, Creteil F-94000, France.|School of Medicine, Paris-Est University (UPEC), 61 avenue du Général de Gaulle, Créteil F-94000, France.|IMRB INSERM U955, GRC Amyloidosis Research Institute, Paris-Est University (UPEC), 8 rue du Général Sarrail, Créteil 94000, France.|DHU ATVB, Henri Mondor Teaching Hospital, Creteil F-94000, France.|INSERM Clinical Investigation Centre 1430, Créteil F-94000, France.|Mondor Amyloidosis Network, Créteil F-94000, France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Rappeneau
authors.affiliation: Department of Cardiology, Martinique Teaching Hospital, Fort-de-France 97200, France.
authors.forename: Jocelyn
authors.initials: J
authors.lastname: Inamo
authors.affiliation: AP-HP, Department of Cardiology, Henri Mondor Teaching Hospital, 51 Avenue Maréchal de Lattre de Tassigny, Creteil F-94000, France.|School of Medicine, Paris-Est University (UPEC), 61 avenue du Général de Gaulle, Créteil F-94000, France.|IMRB INSERM U955, GRC Amyloidosis Research Institute, Paris-Est University (UPEC), 8 rue du Général Sarrail, Créteil 94000, France.|DHU ATVB, Henri Mondor Teaching Hospital, Creteil F-94000, France.|Mondor Amyloidosis Network, Créteil F-94000, France.
authors.forename: Soulef
authors.initials: S
authors.lastname: Guendouz
authors.affiliation: AP-HP, UMGGR, Henri Mondor Teaching Hospital, Creteil F-94000, France.
authors.forename: Justine
authors.initials: J
authors.lastname: Gellen-Dautremer
authors.affiliation: AP-HP, Department of Genetics, Henri Mondor Teaching Hospital, Creteil F-94000, France.
authors.forename: Serge
authors.initials: S
authors.lastname: Pissard
authors.affiliation: AP-HP, Department of Biochemistry, Henri Mondor Teaching Hospital, Creteil F-94000, France.
authors.forename: Sylvain
authors.initials: S
authors.lastname: Loric
authors.affiliation: AP-HP, Department of Biological Hematology and Immunology, Henri Mondor Teaching Hospital, Creteil F-94000, France.
authors.forename: Orianne
authors.initials: O
authors.lastname: Wagner-Ballon
authors.affiliation: School of Medicine, Paris-Est University (UPEC), 61 avenue du Général de Gaulle, Créteil F-94000, France.|AP-HP, Department of Internal Medicine, Henri Mondor Teaching Hospital, Creteil F-94000, France.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Godeau
authors.affiliation: AP-HP, Department of Physiology, Henri Mondor Teaching Hospital, Creteil F-94000, France.|IMRB INSERM U955, Team 8, Paris-Est University, UPEC, France.
authors.forename: Serge
authors.initials: S
authors.lastname: Adnot
authors.affiliation: AP-HP, Department of Cardiology, Henri Mondor Teaching Hospital, 51 Avenue Maréchal de Lattre de Tassigny, Creteil F-94000, France.|School of Medicine, Paris-Est University (UPEC), 61 avenue du Général de Gaulle, Créteil F-94000, France.|IMRB INSERM U955, GRC Amyloidosis Research Institute, Paris-Est University (UPEC), 8 rue du Général Sarrail, Créteil 94000, France.|DHU ATVB, Henri Mondor Teaching Hospital, Creteil F-94000, France.
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Dubois-Randé
authors.affiliation: AP-HP, Department of Cardiology, Henri Mondor Teaching Hospital, 51 Avenue Maréchal de Lattre de Tassigny, Creteil F-94000, France.|School of Medicine, Paris-Est University (UPEC), 61 avenue du Général de Gaulle, Créteil F-94000, France.|IMRB INSERM U955, GRC Amyloidosis Research Institute, Paris-Est University (UPEC), 8 rue du Général Sarrail, Créteil 94000, France.|DHU ATVB, Henri Mondor Teaching Hospital, Creteil F-94000, France.
authors.forename: Luc
authors.initials: L
authors.lastname: Hittinger
authors.affiliation: School of Medicine, Paris-Est University (UPEC), 61 avenue du Général de Gaulle, Créteil F-94000, France.|DHU ATVB, Henri Mondor Teaching Hospital, Creteil F-94000, France.|AP-HP, UMGGR, Henri Mondor Teaching Hospital, Creteil F-94000, France.|AP-HP, Department of Internal Medicine, Henri Mondor Teaching Hospital, Creteil F-94000, France.|IMRB INSERM U955, Team 2, Paris Est University, UPEC, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Galactéros
authors.affiliation: School of Medicine, Paris-Est University (UPEC), 61 avenue du Général de Gaulle, Créteil F-94000, France.|DHU ATVB, Henri Mondor Teaching Hospital, Creteil F-94000, France.|AP-HP, UMGGR, Henri Mondor Teaching Hospital, Creteil F-94000, France.|AP-HP, Department of Internal Medicine, Henri Mondor Teaching Hospital, Creteil F-94000, France.|IMRB INSERM U955, Team 2, Paris Est University, UPEC, France.
authors.forename: Pablo
authors.initials: P
authors.lastname: Bartolucci
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications*; D002311:Cardiomyopathy, Dilated / Q000097:blood / Q000209:etiology; D004452:Echocardiography; D004906:Erythrocyte Count; D004912:Erythrocytes / Q000502:physiology; D005260:Female; D006331:Heart Diseases / Q000097:blood / Q000209:etiology*; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D012307:Risk Factors; D013318:Stroke Volume / Q000502:physiology; D014262:Tricuspid Valve Insufficiency / Q000097:blood / Q000209:etiology; D018487:Ventricular Dysfunction, Left / Q000097:blood / Q000209:etiology; D020257:Ventricular Remodeling / Q000502:physiology; D017086:beta-Thalassemia / Q000150:complications
keywords: Anaemia; Cardiac remodelling; Haemolysis; Prognosis; Sickle cell disease; Tricuspid regurgitant velocity