Pulmonary Hypertension in Hemoglobinopathies : a mini literature review

Résumé

Blood transfusion is considered the cornerstone of treatment for patients suffering from haemoglobinopathies such as thalassemia, sickle cell disease, and anemia. It can occur in either acute care or long-term transfusion settings. Major transfusion complications have been reported in patients with hemoglobinopathies, including pulmonary hypertension, which has become a significant cause of mortality and morbidity in these patients. Although the true pathophysiology of pulmonary hypertension in patients with haemoglobinopathies is unknown, severe hemolysis is thought to be the primary cause. Dyspnea, chronic liver disease, iron overload, sleep apnea, and thromboembolism are all symptoms of pulmonary hypertension in patients with haemoglobinopathies following transfusion. There are no specific treatment guidelines for patients with hemoglobinopathy-related pulmonary hypertension. It is critical to maximize primary hemoglobinopathy treatment, correct hypoxia with chronic oxygen use, and treat associated complications like cardiopulmonary conditions. There are two aspects to management in these patients: hemoglobinopathy-specific treatment and pulmonary hypertension-specific treatment. Hemoglobinopathy is treated with blood transfusions, iron chelation, and hydroxyurea, whereas PH is treated with anticoagulants, diuretics, digoxin, oxygen, and pulmonary hypertension specific vasodilator agents.