Haema 2011; 2(3): 289-296
by Dimitrios Farmakis, Maria Tsironi, Athanasios Aessopos
Attending Cardiologist, University of Athens Medical School, Athens, Greece
Abstract
Cardiac complications represent a leading cause of morbidity and mortality in patients with hemoglobinopathies. Despite the genetic heterogeneity of the disorders, the main pathogenetic mecha-nisms of cardiac injury are common with a different relative degree of contribution in each hemoglobi- nopathy. The high cardiac output state due to chronic anemia, the iron overload resulting from multiple transfusions and increased intestinal iron absorption, the structural and functional vascular disorders, the hypercoagulability, the immune deficiency and a coexistent elastic tissue defect resembling pseu- doxanthoma elasticum are the main mechanisms involved in heart injury. The spectrum of cardiac man- ifestations is broad and includes high output heart failure, iron overload cardiomyopathy, pulmonary hypertension, pericarditis, myocarditis, ischemic heart disease, arrhythmias and valvular abnormalities.