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Diastolic dysfunction is an independent risk factor for death in patients with sickle cell disease

Vandana Sachdev1, Roberto F Machado, Yukitaka Shizukuda

  • 1Cardiovascular Branch, Echocardiography Laboratory, National Heart, Lung, and Blood Institute, Bethesda, Maryland 20892-1454, USA. sachdevv@nhlbi.nih.gov

Journal of the American College of Cardiology
|January 30, 2007
PubMed

Insights

Diastolic dysfunction and pulmonary hypertension independently increase mortality risk in sickle cell disease (SCD) patients. Combining both conditions results in an extremely poor prognosis, highlighting the need for echocardiographic screening in adult SCD populations.

Area of Science:

  • Cardiology
  • Hematology
  • Pulmonology

Background:

  • Pulmonary hypertension is a known mortality predictor in adult sickle cell disease (SCD).
  • Diastolic dysfunction is observed in SCD, but its prevalence, association with pulmonary hypertension, and mortality impact are unclear.

Purpose of the Study:

  • To characterize left ventricular diastolic function in SCD patients.
  • To correlate echocardiographic measures of diastolic dysfunction with pulmonary hypertension and mortality.

Main Methods:

  • Tissue Doppler imaging and conventional echocardiography assessed diastolic function in 141 and 235 SCD patients, respectively.
  • Echocardiographic parameters were analyzed for associations with pulmonary hypertension and mortality.

Main Results:

  • Diastolic dysfunction was found in 18% of patients; 11% had both dysfunction and pulmonary hypertension.
  • Diastolic dysfunction (low E/A ratio) independently predicted mortality (RR 3.5).
  • The combination of diastolic dysfunction and pulmonary hypertension significantly increased mortality risk (RR 12.0).

Conclusions:

  • Both diastolic dysfunction and pulmonary hypertension are independent contributors to mortality in SCD.
  • Patients with both conditions face an extremely poor prognosis.
  • Echocardiographic screening is recommended for adult SCD patients to identify high-risk individuals.
Abstract

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