A reappraisal of the mechanisms underlying the cardiac complications of sickle cell anemia

  title={A reappraisal of the mechanisms underlying the cardiac complications of sickle cell anemia},
  author={Parul Rai and Omar Niss and Punam Malik},
  journal={Pediatric Blood \& Cancer},
Anemia, hemolysis‐driven vasculopathy, and intrinsic myocardial injury have been proposed as predisposing factors to cardiac disease in sickle cell anemia (SCA). The individual impact of these mechanisms on the cardiac features of SCA and the way they influence complications such as sudden death and dysrhythmias have been unclear. Recent findings of an acquired restrictive SCA‐related cardiomyopathy, driven by myocardial fibrosis, may explain some of these cardiac features. Given the complexity… Expand
4 Citations
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Treatment exposures stratify need for echocardiographic screening in asymptomatic long-term survivors of hematopoietic stem cell transplantation
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Although sickle cardiomyopathy is associated with myocardial fibrosis and diastolic dysfunction, the molecular basis for the cardiac pathology is unclear and detailed and well-designed mechanistic studies that elucidates these pathways are needed. Expand


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Awareness for this complication is increased and prospective studies to look at treatment strategies for myocardial infarction in SCD are Prompted. Expand
Cardiomyopathy With Restrictive Physiology in Sickle Cell Disease.
Patients with SCD have a unique form of cardiomyopathy with restrictive physiology that is superimposed on hyperdynamic physiology and is characterized by diastolic dysfunction, left atrial dilation, and normal systolic function, which results in mild, secondary, pulmonary venous hypertension and elevated TRV. Expand
Sickle cell anemia mice develop a unique cardiomyopathy with restrictive physiology
It is shown that in SCA mice, anemia-induced hyperdynamic physiology was gradually superimposed with restrictive physiology, characterized by progressive left atrial enlargement and diastolic dysfunction with preserved systolic function, which may provide a unifying cardiac pathophysiology that explains the reported cardiac abnormalities and sudden death seen in humans with SCA. Expand
Cardiovascular abnormalities in sickle cell disease.
Both PH and diastolic dysfunction are associated with marked abnormalities in exercise capacity in patients with sickle cell disease, and further cardiac investigations are necessary to recognize and treat high-risk patients. Expand
Evaluation of 107 patients with sickle cell anemia through tissue Doppler and myocardial performance index.
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Myocardial tissue characterization and the role of chronic anemia in sickle cell cardiomyopathy
To use cardiovascular magnetic resonance (CMR) techniques to examine possible causes for the left ventricular (LV) dilatation that occurs in sickle cell disease (SCD), including the effects ofExpand
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Echocardiographic findings in patients with sickle cell disease
It is confirmed that elevated TRV is common among hospital-based adults with sickle cell disease, and significant, independent associations were found with both elevated LDH concentration and degree of anemia, suggesting that hemolytic and other mechanisms contribute to pulmonary hypertension in patients with sicke cell disease. Expand
Pulmonary hypertension in sickle cell disease.
Recommendations include intensification of sickle cell-directed therapies, treatment of causal factors or associated diseases, general supportive measures, and use of PH-specific pharmacologic agents to improve survival. Expand
Doppler‐defined pulmonary hypertension and the risk of death in children with sickle cell disease followed for a mean of three years
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