Related Experiment Video
Updated: Jan 25, 2026
Evaluation of Right Ventricular Function in Experimental Models of Pulmonary Arterial Hypertension
Published on: June 27, 2025
Pulmonary hypertension and right ventricular function in Nigerian children with sickle cell anaemia
Igoche D Peter1, Mustafa O Asani2, Shehu U Abdullahi2
1Department of Paediatrics, Aminu Kano Teaching Hospital, Kano, Nigeria.
Insights
Pulmonary hypertension (PH) affects 22% of children with sickle cell anaemia (SCA). This condition is linked to more frequent hospitalizations and a history of blood transfusions in pediatric SCA patients.
Area of Science:
- Pediatric Cardiology
- Hematology
- Pulmonology
Background:
- Pulmonary hypertension (PH) is a significant complication of sickle cell anaemia (SCA), contributing to substantial patient morbidity.
- Understanding the prevalence and associations of PH in pediatric SCA is crucial for improving patient outcomes.
Purpose of the Study:
- To determine the prevalence of echocardiography-suggested PH in children with SCA.
- To identify clinical and laboratory associations of PH in this pediatric population.
Main Methods:
- A cross-sectional comparative study was conducted on 100 children (ages 3-14) with SCA in steady state, matched with healthy controls.
- Echocardiography data, including tricuspid regurgitation velocity (TRV), mean pulmonary arterial pressure (mPAP), and tricuspid annular plane systolic excursion (TAPSE), were collected.
- Statistical analyses were performed to identify significant correlations and associations.
Main Results:
- Twenty-two percent (22%) of SCA subjects exhibited echocardiographic findings suggestive of PH, compared to none in the control group.
- Lower TAPSE values were observed in SCA patients with PH, indicating poorer right ventricular function.
- PH was significantly associated with increased frequency of hospitalizations for vaso-occlusive crises and a history of blood transfusions.
Conclusions:
- Echocardiography-suggested PH is prevalent in children with SCA.
- PH in pediatric SCA is associated with impaired right ventricular function, frequent vaso-occlusive crises, and a history of blood transfusions.
Background:
Pulmonary hypertension (PH), a complication of sickle cell anaemia (SCA), results in considerable morbidity. This study aims to determine the prevalence and associations of echocardiography-suggested PH in children with SCA.
Methods:
We performed a cross-sectional comparative study involving 100 systematically sampled SCA subjects 3-14 y of age in their steady state with matched haemoglobin AA phenotype controls. Clinical, laboratory and echocardiography data (including tricuspid regurgitation velocity [TRV], mean pulmonary arterial pressure [mPAP] and tricuspid annular plane systolic excursion [TAPSE]) were obtained from all patients. Statistical analyses were performed using SPSS version 22 (IBM, Armonk, NY, USA). A p-value <0.05 was considered statistically significant.
Results:
Of the 100 SCA subjects studied, 22 (22%) had echocardiographic findings suggestive of PH compared with none in the controls. The median TAPSE was significantly lower in the PH group (2.55 cm [interquartile range {IQR} 2.2-2.8]) compared with the no PH group (2.77 cm [IQR 2.4-3.2]) (p=0.03). No significant correlation existed between mPAP and age, nor any laboratory parameters studied. The odds ratio (OR) suggested PH significantly increased with an increase in the frequency of hospitalizations for vaso-occlusive crises within a 12-month period (OR 15.15 [95% CI 1.57 to 146.35], p=0.02) and a lifetime history of blood transfusion (OR 5.44 [95% CI 1.09 to 27.24], p=0.04).
Conclusions:
Echocardiography-suggested PH is common in children with SCA and is associated with poorer right ventricular function, frequent vaso-occlusive crises and blood transfusions.
More Related Videos
09:22Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
07:02A Large Animal Model for Pulmonary Hypertension and Right Ventricular Failure: Left Pulmonary Artery Ligation and Progressive Main Pulmonary Artery Banding in Sheep
Published on: July 15, 2021
Related Concept Videos
01:30Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
01:25Pulmonary Function Tests
Pulmonary Function Tests are crucial diagnostic tools for assessing respiratory function, particularly in patients with chronic respiratory disorders. They comprehensively evaluate lung volumes, ventilatory function, breathing mechanics, diffusion, and gas exchange. These tests help diagnose pulmonary diseases and play a significant role in monitoring disease progression, evaluating disability, and assessing response to therapy.
PFTs involve using a spirometer, a...
01:28Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors
Among the PDE5 inhibitors, sildenafil (Revatio) stands out as a competitive and selective inhibitor. It operates by elevating cellular levels of cGMP and augmenting signaling through the cGMP-PKG pathway, promoting vasodilation. Upon oral...
01:18Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
01:23Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
01:16Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...