Pulmonary hypertension in beta thalassemia: a prospective cohort study on treatment outcomes and cardiopulmonary function

BACKGROUND: Beta thalassaemia, an inherited blood disorder, exhibits a broad range of phenotypes from asymptomatic to severe anaemia. Pulmonary hypertension (PH) is a major complication in thalassaemia patients, significantly impacting morbidity and mortality. This study aimed to assess the clinical condition of thalassaemia patients with PH over the course of one year while receiving treatment in accordance with the guidelines of the European Society of Cardiology (ESC). METHODS: A prospective cohort study was carried out at Rohani Hospital in Iran from May 2020 to May 2021. The study included patients diagnosed with beta thalassaemia intermedia who also had PH confirmed by echocardiography. Treatment was administered according to ESC guidelines, incorporating vasodilators and other supportive medications. Clinical and laboratory data were gathered, and statistical analyses were conducted. RESULTS: = 0.01), suggesting enhanced right ventricular (RV) function. However, there were no significant changes in pulmonary artery pressure (PAP) or RV size. Additionally, repeated blood transfusions were linked to RV dysfunction. CONCLUSIONS: Repeated blood transfusions correlate with RV dysfunction in thalassaemia patients with PH. Vasodilatory treatment improves 6MWT, proBNP levels, and E/e' ratio, indicating RV function enhancement. This study underscores the importance of comprehensive management strategies for thalassaemia patients with PH to mitigate associated complications and improve outcomes.