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Echocardiographic abnormalities in sickle cell disease.

Shahid Ahmed1, Anita K Siddiqui, Adnan Sadiq

  • 1Royal University Hospital, University of Saskatchewan, Saskatoon, Saskatchewan, Canada. shahidahmed00@yahoo.com

American Journal of Hematology
|June 30, 2004
PubMed
Summary

Pulmonary hypertension is common in sickle cell disease (SCD), affecting 58% of patients. Older age and acute chest syndrome history are linked to higher prevalence, with lower hemoglobin levels also increasing pulmonary arterial systolic pressure (PASP).

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Area of Science:

  • Cardiology
  • Hematology
  • Pulmonology

Background:

  • Sickle cell disease (SCD) is a genetic blood disorder associated with various cardiovascular complications.
  • Echocardiography is a key tool for assessing cardiac structure and function in patients with SCD.

Purpose of the Study:

  • To determine the prevalence of echocardiographic abnormalities in hospitalized adults with SCD.
  • To estimate pulmonary arterial systolic pressure (PASP) and identify factors associated with pulmonary hypertension.

Main Methods:

  • Retrospective review of clinical data and echocardiograms from 38 adult SCD patients.
  • Fisher's exact test used to correlate pulmonary hypertension with clinical variables.
  • Estimation of PASP using echocardiographic measurements.

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Main Results:

  • Pulmonary hypertension was the most common abnormality, found in 58% of patients (mean PASP 37.5 mmHg).
  • Older age, prior acute chest syndrome, and hemoglobin levels <8 g/dL were significantly correlated with pulmonary hypertension.
  • Other abnormalities included left and right heart chamber dilation, hypertrophy, and dysfunction; no structural valve disease was observed.

Conclusions:

  • A majority of SCD patients exhibit echocardiographic evidence of pulmonary hypertension.
  • Pulmonary hypertension in SCD is associated with older age, acute chest syndrome, and lower hemoglobin levels.
  • While other cardiac abnormalities exist, pulmonary hypertension is the most prevalent echocardiographic finding in this population.