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Myocardial infarction in sickle cell disease
C R Martin1, C S Johnson, C Cobb
1Department of Medicine, LAC+USC Medical Center 90033, USA.
Insights
Sickle cell disease patients can experience myocardial infarction (heart attack) without typical atherosclerosis. This suggests a need to consider ischemic heart disease in these patients presenting with chest pain.
Area of Science:
- Cardiology
- Hematology
- Pathology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder.
- Cardiac complications are increasingly recognized in SCD.
- Myocardial infarction (MI) is not commonly associated with SCD.
Purpose of the Study:
- To investigate the prevalence and characteristics of myocardial infarction in patients with sickle cell disease.
- To explore potential underlying mechanisms of MI in this population.
Main Methods:
- Autopsy study of 72 consecutive hearts from patients with sickle cell disease.
- Analysis of gross and microscopic findings for evidence of myocardial infarction.
- Review of clinical data including chest pain and ECG findings.
Main Results:
- Myocardial infarction (acute or healed) was found in 9.7% (7/72) of patients.
- No obstructive atherosclerotic lesions were observed in the affected patients.
- Microthrombi were present in the arterioles of infarcted tissue in two patients.
- Chest pain was reported by 6/7 patients; ECG changes were noted in 2/7.
- One patient experienced sudden death.
Conclusions:
- Ischemic heart disease, including myocardial infarction, may be a significant but underrecognized complication in sickle cell disease.
- Pathophysiological mechanisms may involve anemia, platelet thrombi, vasospasm, and abnormal sickle cell rheology, rather than atherosclerosis.
- Chest pain in SCD patients warrants consideration of ischemic heart disease, irrespective of crisis status.
Abstract:
Gross and microscopic findings consistent with acute (three patients) and healed (four patients) myocardial infarction were found in seven (9.7%) of 72 consecutive hearts from patients with sickle cell disease studied after autopsy between 1950 and 1982. Gross obstructive and atherosclerotic lesions were absent in all seven patients, while microthrombi were present in the arterioles of infarcted tissue in two patients. Pathophysiological mechanisms responsible for the infarction are unclear, but anemia, platelet thrombi, coronary vasospasm, and abnormal rheology related to sickle cells may all be important. Chest pain occurred clinically in six of the seven patients and ECG findings typical of infarction were found in two patients. One patient died suddenly. These findings suggest that ischemic heart disease may be present in a significant number of patients with sickle cell disease and should be considered in all patients who complain of chest pain, whether or not the patient is in crisis.