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Updated: Dec 12, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Impact of relative systemic hypertension on the heart in sickle cell anaemia
Opeyemi O Oni1, Adewole A Adebiyi2, Akinyemi Aje2
1Department of Medicine, Bowen University Teaching Hospital, Ogbomoso, Oyo State, Nigeria.
Insights
Sickle cell anaemia (SCA) patients with relative systemic hypertension (RSH) show distinct ECG and echocardiography findings. Right atrial area was the primary determinant of RSH in this SCA cohort.
Area of Science:
- Cardiology
- Hematology
- Internal Medicine
Background:
- Sickle cell anaemia (SCA) is linked to macrovascular issues, leading to the concept of relative systemic hypertension (RSH).
- Electrocardiography (ECG) and echocardiography (ECHO) findings in SCA patients with RSH are not well-documented.
Purpose of the Study:
- To investigate the electrocardiography (ECG) and echocardiography (ECHO) characteristics of patients with sickle cell anaemia (SCA) and relative systemic hypertension (RSH).
Main Methods:
- Consecutive recruitment of 83 steady-state SCA patients.
- Data collection included medical history, physical examination, ECG, and ECHO.
- Prevalence of RSH was determined, and patient groups were compared.
Main Results:
- 18.1% of SCA patients (15 individuals) exhibited RSH.
- RSH group showed higher packed cell volume (PCV), longer QTc interval, smaller right atrial area, and lower tricuspid regurgitant velocities.
- Right atrial area was identified as the sole significant predictor of RSH.
Conclusions:
- Relative systemic hypertension (RSH) in sickle cell anaemia (SCA) is associated with specific echocardiographic and ECG findings, including smaller right atrial area and longer QTc.
- Ventricular function remained normal in both RSH and non-RSH groups.
- Further research into sympathetic nervous system activity in SCA patients with RSH is warranted.
Abstract:
Sickle cell anaemia (SCA) is associated with macrovascular complications at relatively normal blood pressures. This has led to the development of the term 'relative systemic hypertension' (RSH). The electrocardiography (ECG) and echocardiography (ECHO) findings in these people has not been well highlighted. Patients with SCA in steady state were consecutively recruited. History, physical examination, ECG and ECHO information were obtained from all participants after informed consent was obtained. Eighty-three people were recruited in all- 15 of which had RSH, giving a prevalence of 18.1%. Those with RSH had higher packed cell volumes (PCV), smaller right atria area, lower tricuspid regurgitant velocities, lower incidence of early satiety, longer QTc and higher frequency of a history of vaso-occlusive crises. The indices of right and left ventricular function were normal in both groups. Right atrial area was the only significant determinant of RSH in this study. RSH is associated with higher PCV, longer QTc and smaller right atrial area in SCA patients. More studies to evaluate sympathetic output in SCA with RSH is required.
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