Abnormal myocardial flow reserve in sickle cell disease: a myocardial contrast echocardiography study

Ana G Almeida1, Francisco Araújo, Fernanda Rego

  • 1Cardiology Department, University Hospital Santa Maria, Av. Prof. Egas Moniz, 1649-035 Lisbon Portugal. anagalmeida@hotmail.com

Insights

Myocardial contrast echocardiography (MCE) reveals reduced myocardial perfusion reserve in sickle cell disease (SCD) patients. This reduced perfusion is linked to impaired left ventricle systolic function, indicating a role for perfusion in SCD-related heart dysfunction.

Area of Science:

  • Cardiology
  • Echocardiography
  • Hematology

Background:

  • Sickle cell disease (SCD) involves microvascular obstruction, leading to ischemia and necrosis.
  • Cardiac involvement is a significant concern in SCD patients.
  • The relationship between myocardial perfusion and left ventricle (LV) function in SCD requires further investigation.

Purpose of the Study:

  • To determine if myocardial contrast echocardiography (MCE) can detect myocardial perfusion abnormalities in SCD patients.
  • To assess the correlation between MCE-derived perfusion indices and LV systolic function in SCD.

Main Methods:

  • A study involving 25 SCD patients and 19 healthy controls.
  • Myocardial perfusion reserve indices (A, beta, A x beta) were measured using MCE before and after dipyridamole-induced hyperemia.
  • Left ventricle (LV) systolic function was assessed using ejection fraction (EF), index of myocardial performance (IMP), E/Ea ratio, Sa, and S%.

Main Results:

  • SCD patients exhibited significantly lower myocardial velocity (beta) and myocardial blood flow (A x beta) reserves compared to controls (P < 0.001).
  • In SCD patients, beta reserve correlated with EF, IMP, Sa, E/Ea, and S%.
  • A x beta reserve showed a correlation with Sa in SCD patients.

Conclusions:

  • MCE effectively detects abnormal myocardial perfusion reserve in individuals with SCD.
  • The detected perfusion abnormalities are significantly correlated with indices of systolic function.
  • These findings suggest that impaired myocardial perfusion contributes to ventricular dysfunction in SCD.
Abstract