title: Diastolic dysfunction is an independent risk factor for death in patients with sickle cell disease.
abstract: OBJECTIVE
  The goal of this study was to characterize left ventricular diastolic function in the sickle cell disease (SCD) population and to relate echocardiographic measures of dysfunction with pulmonary hypertension and mortality.
  
  
  BACKGROUND
  Pulmonary hypertension has been identified as a predictor of death in the adult SCD population. Although diastolic dysfunction is also observed in this population, its prevalence, association with high pulmonary artery systolic pressure, and attributable mortality remain unknown.
  
  
  METHODS
  Diastolic function assessment using tissue Doppler imaging was performed in a group of 141 SCD patients. Conventional echocardiographic parameters of diastolic function were performed in a total of 235 SCD patients.
  
  
  RESULTS
  Diastolic dysfunction was present in 18% of patients. A combination of diastolic dysfunction and pulmonary hypertension was present in 11% of patients, and diastolic dysfunction accounted for only 10% to 20% of the variability in tricuspid regurgitation (TR) jet velocity. Diastolic dysfunction, as reflected by a low E/A ratio, was associated with mortality with a risk ratio of 3.5 (95% confidence interval 1.5 to 8.4, p < 0.001), even after adjustment for tricuspid regurgitation (TR) jet velocity. The presence of both diastolic dysfunction and pulmonary hypertension conferred a risk ratio for death of 12.0 (95% confidence interval 3.8 to 38.1, p < 0.001).
  
  
  CONCLUSIONS
  Diastolic dysfunction and pulmonary hypertension each contribute independently to prospective mortality in patients with SCD. Patients with both risk factors have an extremely poor prognosis. These data support the implementation of echocardiographic screening of adult patients with SCD to identify high-risk individuals for further evaluation.
authors.affiliation: Cardiovascular Branch, Echocardiography Laboratory, National Heart, Lung, and Blood Institute, Bethesda, Maryland 20892-1454, USA. sachdevv@nhlbi.nih.gov
authors.forename: Vandana
authors.initials: V
authors.lastname: Sachdev
authors.forename: Roberto F
authors.initials: RF
authors.lastname: Machado
authors.forename: Yukitaka
authors.initials: Y
authors.lastname: Shizukuda
authors.forename: Yesoda N
authors.initials: YN
authors.lastname: Rao
authors.forename: Stanislav
authors.initials: S
authors.lastname: Sidenko
authors.forename: Inez
authors.initials: I
authors.lastname: Ernst
authors.forename: Marilyn
authors.initials: M
authors.lastname: St Peter
authors.forename: Wynona A
authors.initials: WA
authors.lastname: Coles
authors.forename: Douglas R
authors.initials: DR
authors.lastname: Rosing
authors.forename: William C
authors.initials: WC
authors.lastname: Blackwelder
authors.forename: Oswaldo
authors.initials: O
authors.lastname: Castro
authors.forename: Gregory J
authors.initials: GJ
authors.lastname: Kato
authors.forename: Mark T
authors.initials: MT
authors.lastname: Gladwin
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000755:Anemia, Sickle Cell / Q000150:complications / Q000401:mortality* / Q000503:physiopathology*; D003971:Diastole; D015150:Echocardiography, Doppler; D005260:Female; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D016277:Ventricular Function, Left