CT Features of Sickle Cell Disease-Associated Pulmonary Hypertension
- 1Department of Radiology and Medical Imaging, College of Medicine, King Saud University, Riyadh 11451, Saudi Arabia
- 2Department of Medicine, College of Medicine, King Saud University, Riyadh 11451, Saudi Arabia
- 3College of Medicine, King Saud University, Riyadh 11451, Saudi Arabia
Abstract
Background/Objectives: Sickle cell disease (SCD) frequently leads to pulmonary hypertension (PH), affecting 6–11% of patients and increasing mortality. Computed tomography (CT) can assess pulmonary vascular changes, but its role in SCD-associated PH remains unclear. This retrospective study aims to evaluate CT findings in adults with SCD-associated PH and their association with disease severity as assessed by Transthoracic Echocardiography (TTE) and right heart catheterization (RHC). Methods: We conducted a retrospective cohort study at a tertiary center in Riyadh, reviewing CT scans from 2015 to 2024. Adults with confirmed SCD were included if PH was suggested by echocardiography or confirmed by RHC. CT findings, including pulmonary artery diameter and parenchymal abnormalities, were analyzed alongside demographic and clinical data using Fisher’s exact tests with FDR correction for multiple comparisons. Results: Eighteen adults were included (mean age 35.9 ± 10.8 years; 50% male). Of the 18 patients, five (27.8%) had RHC-confirmed PH, whereas the remaining 13 (72.2%) were included based on TTE findings suggestive of PH without RHC confirmation. Echocardiographic probability data were available for 17 patients, among whom intermediate probability was the most frequent category (11/17, 64.7%). CT demonstrated PH signs in 55.6% of patients. Mean pulmonary artery diameter was 2.90 ± 0.60 cm, greatest in patients assessed by both TTE and RHC (3.61 ± 0.58 cm). The most frequent CT findings were cardiomegaly (44.4%) and pleural effusion (38.9%). Using Fisher’s exact test, no individual CT feature was significantly associated with PH severity after correction for multiple comparisons; the presence of any CT sign of PH showed a nominal association with severity (exact p = 0.042) that was no longer significant after correction for multiple comparisons (q = 0.33). CT classified more patients as mild or moderate, but fewer as severe than TTE; neither septal flattening nor RV hypertrophy was significantly associated with severe PH on exact testing (p = 0.10 and p = 0.20, respectively, on CT-based assessment), and these associations should be considered exploratory given the small sample size. Conclusions: CT identifies vascular, cardiac, and parenchymal abnormalities, but does not replace TTE and RHC in evaluating SCD-associated PH.
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BibTeX
@article{molla2026features,
title = {{CT Features of Sickle Cell Disease-Associated Pulmonary Hypertension}},
author = {Nouran Waleed Molla and Sarah Sewaralthahab and Razan Abdulrahman Alotaibi and Abdulaziz Abdulrahman Alymni and Waad Mohammed Abu Nakha and Naif Adeeb Alquwayi and Khalid Abdulrahman Abdulaziz and Noof Abdulaziz Alsaigh},
journal = {Journal of clinical medicine},
year = {2026},
month = oct,
doi = {10.3390/jcm15197611},
url = {https://europepmc.org/article/PMC/PMC13666314},
note = {CC BY},
}
RIS
TY - JOUR
TI - CT Features of Sickle Cell Disease-Associated Pulmonary Hypertension
AU - Nouran Waleed Molla
AU - Sarah Sewaralthahab
AU - Razan Abdulrahman Alotaibi
AU - Abdulaziz Abdulrahman Alymni
AU - Waad Mohammed Abu Nakha
AU - Naif Adeeb Alquwayi
AU - Khalid Abdulrahman Abdulaziz
AU - Noof Abdulaziz Alsaigh
PY - 2026
DA - 2026/10/01
JO - Journal of clinical medicine
DO - 10.3390/jcm15197611
UR - https://europepmc.org/article/PMC/PMC13666314
AB - Background/Objectives: Sickle cell disease (SCD) frequently leads to pulmonary hypertension (PH), affecting 6–11% of patients and increasing mortality. Computed tomography (CT) can assess pulmonary vascular changes, but its role in SCD-associated PH remains unclear. This retrospective study aims to evaluate CT findings in adults with SCD-associated PH and their association with disease severity as assessed by Transthoracic Echocardiography (TTE) and right heart catheterization (RHC). Methods: We conducted a retrospective cohort study at a tertiary center in Riyadh, reviewing CT scans from 2015 to 2024. Adults with confirmed SCD were included if PH was suggested by echocardiography or confirmed by RHC. CT findings, including pulmonary artery diameter and parenchymal abnormalities, were analyzed alongside demographic and clinical data using Fisher’s exact tests with FDR correction for multiple comparisons. Results: Eighteen adults were included (mean age 35.9 ± 10.8 years; 50% male). Of the 18 patients, five (27.8%) had RHC-confirmed PH, whereas the remaining 13 (72.2%) were included based on TTE findings suggestive of PH without RHC confirmation. Echocardiographic probability data were available for 17 patients, among whom intermediate probability was the most frequent category (11/17, 64.7%). CT demonstrated PH signs in 55.6% of patients. Mean pulmonary artery diameter was 2.90 ± 0.60 cm, greatest in patients assessed by both TTE and RHC (3.61 ± 0.58 cm). The most frequent CT findings were cardiomegaly (44.4%) and pleural effusion (38.9%). Using Fisher’s exact test, no individual CT feature was significantly associated with PH severity after correction for multiple comparisons; the presence of any CT sign of PH showed a nominal association with severity (exact p = 0.042) that was no longer significant after correction for multiple comparisons (q = 0.33). CT classified more patients as mild or moderate, but fewer as severe than TTE; neither septal flattening nor RV hypertrophy was significantly associated with severe PH on exact testing (p = 0.10 and p = 0.20, respectively, on CT-based assessment), and these associations should be considered exploratory given the small sample size. Conclusions: CT identifies vascular, cardiac, and parenchymal abnormalities, but does not replace TTE and RHC in evaluating SCD-associated PH.
SN - 2077-0383
ER -