prevalence and correlation of pulmonary hypertension in patients with sickle cell disease: a descriptive study

Background: one common outcome for people with chronic hemolytic disorders is the development of pulmonary hypertension (pht). however, there is little knowledge about the correlation between vasculopathy, cardiopulmonary function, contributing variables, and the associated mortality in patients with sickle cell disease (scd). objectives: the purpose of this investigation was to determine the prevalence of pht among patients with sickle cell anemia in bandar abbas city during the years 2022 to 2023.methods: this research was a descriptive cross-sectional investigation carried out on individuals with sickle cell anemia in bandar abbas. the data underwent thorough assessment for reliability and validity using a researcher-developed checklist, which included input from respected professors. this checklist contains essential demographic data, including gender and age, as well as pertinent treatment information related to pht, hemoglobin level, vascular occlusion crisis, high hematocrit level, α-thalassemia, hydroxyurea treatment, reduction of white blood cells, high creatinine level, genotype, and high ferritin level in affected individuals. statistical analysis was performed using spss software, version 22. results: participants had a mean age of 10.50±2.94 years. among the 75 patients examined, 46.7% were female and 53.3% were male. the majority of the patients, specifically 58 individuals (77.3% of the subjects evaluated), did not exhibit pht, whereas 17 individuals (22.7%) were found to have pht. an analysis of platelet data revealed that individuals with pht had lower platelet levels compared to those without pht, and this disparity was statistically significant (p=0.04). there was also a significant correlation between pht and α-thalassemia (p=0.01). in addition, there was no significant correlation between age, genotype, gender, hematocrit, white blood cell count, hemoglobin levels, creatinine levels, ferritin levels, usage of hydroxyurea medication, and the existence of pht (p>0.05). conclusions: according to the study’s findings, pht was present in 22.7% of patients with scd. pht was significantly associated with α-thalassemia and platelet count. additionally, the prevalence of the disease was higher in women than in men. consequently, essential diagnostic and preventative treatments must be implemented for high-risk individuals, given the serious consequences of the disease and the elevated mortality risk among these patients.