Pulmonary Hypertension in Hemoglobinopathies : A Neglected Entity

Background : Hemoglobinopathies constitute a heterogeneous group of Hereditary Hemoglobin Disorders. Cardiovascular complications are among the leading causes of morbidity and mortality in Hemoglobinopathies. In the wide spectrum of Cardiovascular manifestations, Pulmonary Arterial Hypertension (PAH) holds a prominent place. Screening for Pulmonary Hypertension should be an essential component in assessment of patients with Hemoglobinopathies and may be accomplished by Transthoracic Doppler Echocardiography which is most established screening tool for Pulmonary Hypertension and is widely available and cost-effective. Objective : This study aims to determine the presence of Pulmonary Hypertension in Hemoglobinopathies. Method : Institution/Hospital based, Non-interventional, Observational Descriptive, Cross-sectional study conducted amongst 76 patients of Hemoglobinopathies (>12 years) in North Bengal Medical College and Hospital, Darjeeling. Detailed history and physical examination along with non invasive tests like ECG, Chest X-ray and Echocardiography were performed in all study population to detect PAH. Data was analysed using standard statistical method. Result : 30.26% of patients with Hemoglobinopathy had PAH in Echocardiographic findings, of which 25% had mild and 5.26% cases had Moderate PAH. It was most prevalent in E-β thalassemia, followed by Sickle β and β thalassemia major respectively. The clinical indicators associated with increased risk of PAH in Hemoglobinopathies were presence of Severe Anemia, transfusion >10 U/year, Iron overload state, Splenectomy and their combinations. Conclusion : Echocardiography serves as one of the most useful non-invasive screening tool for the diagnosis of Pulmonary Hypertension. Early detection and management is necessary to decrease the morbidity and mortality