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Abnormal diastolic function and Global longitudinal strain in patients with Thalassemia Major on long term chelation

Sunil K Nadar1, Shahina Daar2,3, Wael A Abdelmottaleb4

  • 1Department of Medicine, Sultan Qaboos University Hospital, POBox 38, Muscat, 123, Oman. sunilnadar@gmail.com.

The International Journal of Cardiovascular Imaging
|September 23, 2020
PubMed

Insights

Cardiac T2* MRI assesses iron in thalassemia major (TM) patients. Echocardiography revealed diastolic dysfunction in TM patients, even with normal iron levels, highlighting the need for ongoing cardiac monitoring.

Area of Science:

  • Cardiology
  • Hematology
  • Medical Imaging

Background:

  • Cardiac complications are a primary cause of mortality in Thalassemia major (TM) patients.
  • Cardiac T2* MRI is the established method for quantifying myocardial iron concentration.
  • Assessing cardiac function through echocardiography is crucial for managing TM patients on chelation therapy.

Purpose of the Study:

  • To investigate correlations between echocardiographic parameters and cardiac iron levels (measured by T2* MRI) in Thalassemia major patients.
  • To identify potential echocardiographic markers for early diastolic dysfunction in TM.
  • To compare cardiac function in TM patients with Thalassemia intermedia (TI) and healthy controls.

Main Methods:

  • Prospective study involving TM patients undergoing cardiac MRI and echocardiography within a two-month interval.
  • Echocardiographic parameters assessed included global longitudinal strain (GLS) and diastolic function (e.g., E/E', pulmonary vein flow).
  • Comparison groups included patients with Thalassemia intermedia (TI) and normal controls.

Main Results:

  • 84 TM patients (mean age 26.3 years) showed normal left ventricular ejection fraction; only 8 had T2* < 10 ms.
  • TM patients exhibited significantly higher E/E' and lower pulmonary vein s/dd ratio compared to TI and controls, indicating diastolic dysfunction.
  • Diastolic dysfunction was present in 28 TM patients despite normal MRI T2* values; GLS was significantly lower in TM patients.

Conclusions:

  • Echocardiographic findings of diastolic dysfunction persist in Thalassemia major patients even with normal myocardial iron levels (T2* MRI > 20 ms) and normal global longitudinal strain.
  • There was no significant correlation found between echocardiographic parameters and cardiac T2* MRI values in TM patients.
  • Continuous cardiac monitoring, including assessment of diastolic function, is essential for TM patients, irrespective of myocardial iron levels.

Keywords:
Diastolic functionEchocardiogramGlobal longitudinal strainThalassemia intermediaThalassemia major

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