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Antoine AbdelMassih, Pediatric Cardiology unit, Department of Pediatrics, Kasr Al Ainy School of Medicine, Cairo University, Cairo, EgyptFollow
Mervat Haroun, Department of Pediatrics, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Rasha AbdelRaouf AbdelAziz Afifi, Pediatric Hematology unit, Department of Pediatrics, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Gehan Hussein, Pediatric Cardiology unit, Department of Pediatrics, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Manal AbdelHameed, Pediatric Cardiology unit, Department of Pediatrics, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Marina George Asaad, Department of Pediatrics, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Heba Tarabeh, Pediatric Cardiology unit, Department of Pediatrics, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Nourhan Essam El Din Taha, Department of Pediatrics, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
Nourine Diab, Residency program, Faculty of Medicine, New Giza University, New Giza, Egypt
Noura Shebl, Residency program, Faculty of Medicine, New Giza University, New Giza, Egypt
Raghda Fouda, Hematology, Faculty of Medicine, Cairo University, Cairo, Egypt
Marianne Edward Yassa, Clinical and Chemical Pathology Department, Faculty of Medicine, Cairo University, Cairo, Egypt
Mohamed Ghobashy, Radiology department, Faculty of Medicine, Cairo University, Cairo, Egypt
Hala Agha, Pediatric Cardiology unit, Department of Pediatrics, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt

Abstract

Background

Sickle Cell Disease (SCD) is not a hematologic disease that occurs in isolation; it results in multi-organ complications. There is growing evidence of vascular stiffness as its underlying cause.

This study aimed to investigate the relationship between endothelial stiffness and LV dysfunction in SCD patients and to explore its pathophysiology, particularly regarding the depletion of vasodilators such as Nitric Oxide (NO).

Methodology

32 patients with established criteria for SCD and 40 healthy control subjects were selected for this case-control study. Comprehensive clinical assessment and assessment of endothelial function using Brachial Flow-mediated dilation (FMD) were performed, along with serum NO measurement, which was followed by diagnosis and echocardiographic assessment using 3D speckle tracking echocardiography (STE) and tissue Doppler imaging (TDI).

Results

Collected SCD cases showed echocardiographic features of Systo-diastolic dysfunction with reduced FMD compared to controls, denoting endothelial dysfunction in those patients.

LDH showed a marked elevation, while serum NO showed a significant reduction in cases compared with controls. We also noted a positive correlation between FMD on the one hand and measures of ventricular dysfunction and level of serum NO on the other hand, the latter proving that reduction of NO is responsible for reduced endothelial function.

Conclusion

We present the first report to date to outline the role of vascular stiffness as measured by brachial FMD in the induction of left ventricular dysfunction in SCD. We recommend that more research be conducted regarding possible strategies to replenish serum NO stores to delay microvascular injury and, in turn, ventricular dysfunction in SCD.

Creative Commons License

Creative Commons Attribution-Noncommercial-No Derivative Works 4.0 License
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 4.0 License.

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