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Published on: May 28, 2019
Myocardial infarction in patients with severe beta thalassaemia: a case series
Anuja Premawardhena1, Shamila De Silva2, Megha Rajapaksha3
1Department of Medicine: Faculty of Medicine, University of Kelaniya, Kelaniya, Sri Lanka. premawa@kln.ac.lk.
Insights
Cardiac disease is a major concern in thalassemia patients. Acute coronary syndromes, including myocardial infarction, are rarely reported but occurred in three patients, with unclear causes.
Area of Science:
- Cardiology
- Hematology
- Internal Medicine
Background:
- Cardiac disease is a leading cause of morbidity and mortality in thalassemia patients, especially those with thalassemia major.
- Myocardial infarction and coronary artery disease are seldom reported in this population.
- This study examines rare cases of acute coronary syndrome in patients with thalassemia syndromes.
Purpose of the Study:
- To investigate the presentation and potential etiology of acute coronary syndrome (ACS) in patients with thalassemia.
- To highlight the diagnostic and therapeutic challenges in managing ACS in this unique patient group.
Main Methods:
- Case series of three adult patients with distinct thalassemia syndromes presenting with ACS.
- Detailed review of clinical presentation, transfusion history, cardiac investigations including coronary angiography, and management.
- Analysis of etiological factors, distinguishing between atherosclerotic and non-atherosclerotic causes.
Main Results:
- Two heavily transfused patients experienced ST-elevation myocardial infarctions (STEMI); one minimally transfused patient had unstable angina.
- Coronary angiography revealed normal findings in two patients; one STEMI patient had a 50% plaque.
- All patients were managed with standard ACS protocols, despite a presumed non-atherogenic etiology.
Conclusions:
- The precise cause of ACS in these thalassemia patients remains undetermined.
- The optimal use of thrombolytic therapy, primary percutaneous coronary intervention, antiplatelet agents, and high-dose statins in this subgroup requires further clarification.
Background:
Cardiac disease remains a dominant if not the most important cause of morbidity and mortality in patients with thalassaemia, particularly in those with thalassaemia major. Myocardial infarction and coronary artery disease however are rarely reported.
Case Presentations:
Three older patients with three distinct thalassaemia syndromes presented with acute coronary syndrome. Two were heavily transfused whilst the other was a minimally transfused patient. Both heavily transfused patients had ST-elevation myocardial infarctions (STEMI) while the minimally transfused patient had unstable angina. Coronary angiogram (CA) was normal in two patients. One patient who developed a STEMI had a 50% plaque. All three were managed as standard ACS, although the aetiology appeared non-atherogenic.
Conclusions:
The exact etiology of the presentation, remains a mystery and therefore the rational use of thrombolytic therapy, carrying out angiogram in the primary setting, using and continuing antiplatelet and high dose statins all remains unclear in this sub group of patients.
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