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Physiology and Pathophysiology of Iron Cardiomyopathy in Thalassemia
Author(s) -
WOOD JOHN C.,
ENRIQUEZ CATHLEEN,
GHUGRE NILESH,
OTTODUESSEL MAYA,
AGUILAR MICHELLE,
NELSON MARVIN D.,
MOATS REX,
COATES THOMAS D.
Publication year - 2005
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1196/annals.1345.047
Subject(s) - asymptomatic , medicine , thalassemia , cardiology , pathophysiology , cardiomyopathy , cardiac function curve , anemia , magnetic resonance imaging , hemodynamics , cardiac magnetic resonance imaging , heart failure , physiology , radiology
A bstract : Iron cardiomyopathy remains the leading cause of death in patients with thalassemia major. Magnetic resonance imaging (MRI) is ideally suited for monitoring thalassemia patients because it can detect cardiac and liver iron burdens as well as accurately measure left ventricular dimensions and function. However, patients with thalassemia have unique physiology that alters their normative data. In this article, we review the physiology and pathophysiology of thalassemic heart disease as well as the use of MRI to monitor it. Despite regular transfusions, thalassemia major patients have larger ventricular volumes, higher cardiac outputs, and lower total vascular resistances than published data for healthy control subjects; these hemodynamic findings are consistent with chronic anemia. Cardiac iron overload increases the relative risk of further dilation, arrhythmias, and decreased systolic function. However, many patients are asymptomatic despite heavy cardiac burdens. We explore possible mechanisms behind cardiac iron‐function relationships and relate these mechanisms to clinical observations.