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… 
6 Citations

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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.

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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.

Cardiomyopathy With Restrictive Physiology in Sickle Cell Disease.

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.

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Evaluation of 107 patients with sickle cell anemia through tissue Doppler and myocardial performance index.

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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 of

Ventricular diastolic dysfunction in sickle cell anemia is common but not associated with myocardial iron deposition

This work investigated the relationships among transfusional iron burden, myocardial iron deposition, and diastolic ventricular dysfunction by T2*‐MRI and tissue Doppler echocardiography in iron‐overloaded children with SCA.

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.

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.

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