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Pulmonary Hypertension in Thalassemia: Association with Hemolysis ...

Source: www.phassociation.org
Topic: Thalassemia
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Sort Desciption: The thalassemia syndromes are a heterogeneous group of inherited hemoglobin disorders resulting from impaired production of either the alpha or beta globin chain subunits of the hemoglobin tetramer. ...

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The thalassemia syndromes are a heterogeneous group of inherited hemoglobin disorders resulting from impaired production of either the alpha or beta globin chain subunits of the hemoglobin tetramer. The clinical spectrum is a consequence of chronic hemolytic anemia and imbalanced globin chain accumulation. 1 Depending on clinical severity, two forms of beta-thalassemia have been classified: thalassemia major (TM) and thalassemia intermedia (TI). TM is characterized by severe anemia starting during the first year of life and requiring lifelong transfusion therapy for survival, while TI has a later clinical onset with a milder anemia, permitting survival without regular transfusions, and a longer life expectancy. 2 Heart failure is the most common cause of death in both forms of the disease. 3 Thalassemia heart disease involves mainly left ventricular (LV) dysfunction caused by transfusion-induced iron overload. However, recent studies suggest that both TM and TI patients have a unique hemodynamic pattern consistent with right ventricular (RV) cardiomyopathy, and pulmonary hypertension (PH) in addition to the LV abnormalities. 4 PH in beta-thalassemia represents a common, yet less well-explored complication in the cardiopulmonary spectrum of the disease, and is the focus of this review. Earlier studies in both TI and TM demonstrate that adults frequently have undetected PH, with a reported prevalence of 60% to 75%, 5-9 and there is a growing body of literature that suggests asymptomatic PH is a leading factor in heart failure and death in ...

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