Heart failure in beta-thalassemia.

Heart failure remains the main cause of death in beta-thalassemia despite the progress that has been made. Myocardial iron deposition alone does not affect left ventricular relaxation but directly causes left ventricular myocardial restriction with considerably elevated pulmonary pressure. This leads to symptoms and signs of predominantly right-sided heart failure, which is usually observed in elderly and severely hemosiderotic populations. Left ventricular systolic dysfunction and failure, which occurs in younger, less hemosiderotic populations, seems to be multifactorial in etiology. Apart from iron loading, immunogenetic risk factors trigger the mechanisms of left-sided heart failure development in the context of dilated-type cardiomyopathy. (c)2001 CHF, Inc.