Assessment of pulmonary hypertension in patients with sickle cell disease
Abstract Background Sickle cell anemia is an autosomal recessive Mendelian disorder caused by a point mutation in the beta-globin gene. Pulmonary hypertension (PH) is a progressive vasculopathy complication of sickle cell disease (SCD). The aim of this study was to evaluate the prevalence of pulmona...
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| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
| Published: |
SpringerOpen
2025-06-01
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| Series: | The Egyptian Journal of Bronchology |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s43168-025-00421-2 |
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| Summary: | Abstract Background Sickle cell anemia is an autosomal recessive Mendelian disorder caused by a point mutation in the beta-globin gene. Pulmonary hypertension (PH) is a progressive vasculopathy complication of sickle cell disease (SCD). The aim of this study was to evaluate the prevalence of pulmonary hypertension in patients with SCD and to analyze its correlation with the frequency of vaso-occlusive crises and related parameters. Patients and methods This was an observational, cross-sectional study conducted on 100 patients with SCD attending the outpatient clinic at Mouwasat Hospital Qatif. Patients were either followed up regularly or admitted for vaso-occlusive crises. Data collected included hemoglobin S levels (via electrophoresis), complete blood count (CBC), lactate dehydrogenase (LDH), reticulocyte count, total bilirubin, D-dimer, NT-pro BNP, and echocardiography to evaluate right and left ventricular parameters, mitral and tricuspid regurgitation, and pulmonary artery pressure. Results The median (interquartile range, IQR) left ventricular diastolic diameter was 49 (44–52) mm, while the mean ± SD of the left ventricular systolic diameter was 32.62 ± 4.79 mm. The median (IQR) right ventricular diastolic diameter was 32.5 (30–35) mm. Mitral regurgitation was observed in 22% of cases, while tricuspid regurgitation was present in 84%. Pulmonary hypertension was detected in 80% of cases. Patients with pulmonary hypertension showed significantly higher levels of LDH and total bilirubin compared to those without PH (P = 0.004 and P = 0.03, respectively). D-dimer and NT-pro BNP were also significantly elevated in patients with PH (P = 0.046 and P = 0.008, respectively). No significant differences were observed in other parameters (P > 0.05). A significant negative correlation was observed between pulmonary artery pressure (PAP) and hemoglobin (Hb) (r = -0.325, P = 0.001). Conversely, a significant positive correlation was observed between PAP and LDH (r = 0.321, P = 0.001), left ventricular systolic diameter (r = 0.199, P = 0.048), right ventricular diastolic diameter (r = 0.391, P < 0.001), D-dimer (r = 0.199, P = 0.047), and NT-pro BNP (r = 0.474, P < 0.001). Conclusion Pulmonary hypertension, assessed via Doppler echocardiography, is highly prevalent among adults with SCD. Certain hematological and biochemical parameters are associated with PH, emphasizing the importance of early detection and management. |
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| ISSN: | 2314-8551 |