ECHOCARDIOGRAPHIC PARAMETERS OF PULMONARY HYPERTENSION IN PATIENTS WITH SICKLE CELL ANEMIA: A SYSTEMATIC REVIEW
- 1Universidade de Fortaleza (UNIFOR), Fortaleza, CE, Brazil
Abstract
Introduction: Pulmonary hypertension (PH) in sickle cell disease (SCD) is associated with increased mortality, though its prevalence is lower when confirmed by right heart catheterization. Echocardiographic screening, particularly tricuspid regurgitation velocity (TRV) and estimated pulmonary artery systolic pressure (PASP), has variable accuracy. Elevated TRV is linked to worse outcomes even without confirmed PH, and variability in measurement and cutoff values limits comparability. Comparative analysis of echocardiographic parameters in SCD with and without PH is needed to clarify their diagnostic value. Objectives: This review aims to evaluate the role of tricuspid regurgitation velocity (TRV) and echocardiographically estimated pulmonary artery pressure in patients with sickle cell disease, including their diagnostic value for pulmonary hypertension and their association with clinical outcomes, as well as differences in measurements across studies. Methods: This systematic review was conducted in accordance with PRISMA guidelines and registered in PROSPERO (CRD1359910). The study aimed to assess echocardiographic parameters in patients with sickle cell disease, with and without pulmonary hypertension. A literature search was performed in the Cochrane Library, Embase, and PubMed databases, covering studies published up to February 2026. MeSH and DeCS terms such as “Sickle cell disease”, “Pulmonary Hypertension”, “tricuspid valve insufficiency”, and “Echocardiography” were combined using AND/OR. Results/Conclusion: Overall, 9 studies were included in this review and the selection process was summarized in the PRISMA flow diagram. The studies were predominantly retrospective cohort studies. Across the 9 studies, 927 patients with SCD were included (mean age 18.4–39 years), with a female predominance. The prevalence of PH suspicion ranged from 26% to 50.6%, with elevated PASP reaching 59% when considering only the elevation of PASP. Additionally, PH was consistently associated with lower hemoglobin levels. Tricuspid Regurgitant Jet Velocity (TRV) proved to be a sensitive marker, patients with suspected PH exhibited a mean TRV of 2.9 m/s, significantly higher than those without the condition (2.3 m/s). Notably, one longitudinal study identified a TRV ≥ 3.0 m/s as a strong predictor of mortality (OR = 5.8). with progression linked to significant cardiac remodeling. These findings highlight that, beyond diagnosis, certain echocardiographic markers serve as prognostic indicators of progression and survival in SCD.
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BibTeX
@article{nobre2026echocardiographic,
title = {{ECHOCARDIOGRAPHIC PARAMETERS OF PULMONARY HYPERTENSION IN PATIENTS WITH SICKLE CELL ANEMIA: A SYSTEMATIC REVIEW}},
author = {Clara SIMÕES NOBRE and Lara DAMASCENO DUARTE and Maria Eduarda SANTOS DE OLIVEIRA and Clarissa FREIRE COUTINHO and Arthur BARROS MIRANDA CILIÃO LUCENA and Thaís FACÓ DE CASTRO and Fernanda DE CARVALHO SOARES and Beatriz BARSI CUNHA and Letícia MATOS LEANDRO and Maria Beatriz MAGALHÃES PINHO and Lucas CARTAXO TAVARES},
journal = {Hematology, Transfusion and Cell Therapy},
year = {2026},
month = oct,
doi = {10.1016/j.htct.2026.108474},
url = {https://doi.org/10.1016/j.htct.2026.108474},
note = {CC BY},
}
RIS
TY - JOUR
TI - ECHOCARDIOGRAPHIC PARAMETERS OF PULMONARY HYPERTENSION IN PATIENTS WITH SICKLE CELL ANEMIA: A SYSTEMATIC REVIEW
AU - Clara SIMÕES NOBRE
AU - Lara DAMASCENO DUARTE
AU - Maria Eduarda SANTOS DE OLIVEIRA
AU - Clarissa FREIRE COUTINHO
AU - Arthur BARROS MIRANDA CILIÃO LUCENA
AU - Thaís FACÓ DE CASTRO
AU - Fernanda DE CARVALHO SOARES
AU - Beatriz BARSI CUNHA
AU - Letícia MATOS LEANDRO
AU - Maria Beatriz MAGALHÃES PINHO
AU - Lucas CARTAXO TAVARES
PY - 2026
DA - 2026/10/23
JO - Hematology, Transfusion and Cell Therapy
DO - 10.1016/j.htct.2026.108474
UR - https://doi.org/10.1016/j.htct.2026.108474
AB - Introduction: Pulmonary hypertension (PH) in sickle cell disease (SCD) is associated with increased mortality, though its prevalence is lower when confirmed by right heart catheterization. Echocardiographic screening, particularly tricuspid regurgitation velocity (TRV) and estimated pulmonary artery systolic pressure (PASP), has variable accuracy. Elevated TRV is linked to worse outcomes even without confirmed PH, and variability in measurement and cutoff values limits comparability. Comparative analysis of echocardiographic parameters in SCD with and without PH is needed to clarify their diagnostic value. Objectives: This review aims to evaluate the role of tricuspid regurgitation velocity (TRV) and echocardiographically estimated pulmonary artery pressure in patients with sickle cell disease, including their diagnostic value for pulmonary hypertension and their association with clinical outcomes, as well as differences in measurements across studies. Methods: This systematic review was conducted in accordance with PRISMA guidelines and registered in PROSPERO (CRD1359910). The study aimed to assess echocardiographic parameters in patients with sickle cell disease, with and without pulmonary hypertension. A literature search was performed in the Cochrane Library, Embase, and PubMed databases, covering studies published up to February 2026. MeSH and DeCS terms such as “Sickle cell disease”, “Pulmonary Hypertension”, “tricuspid valve insufficiency”, and “Echocardiography” were combined using AND/OR. Results/Conclusion: Overall, 9 studies were included in this review and the selection process was summarized in the PRISMA flow diagram. The studies were predominantly retrospective cohort studies. Across the 9 studies, 927 patients with SCD were included (mean age 18.4–39 years), with a female predominance. The prevalence of PH suspicion ranged from 26% to 50.6%, with elevated PASP reaching 59% when considering only the elevation of PASP. Additionally, PH was consistently associated with lower hemoglobin levels. Tricuspid Regurgitant Jet Velocity (TRV) proved to be a sensitive marker, patients with suspected PH exhibited a mean TRV of 2.9 m/s, significantly higher than those without the condition (2.3 m/s). Notably, one longitudinal study identified a TRV ≥ 3.0 m/s as a strong predictor of mortality (OR = 5.8). with progression linked to significant cardiac remodeling. These findings highlight that, beyond diagnosis, certain echocardiographic markers serve as prognostic indicators of progression and survival in SCD.
SN - 2531-1379
SN - 2531-1387
ER -