title: Left ventricular non-compaction in identical twins with thalassaemia and cardiac iron overload.
abstract: Cardiac disease in patients with transfusion-dependent beta-thalassaemia major is well described. Cardiac manifestations may include left ventricular wall thickening and both systolic and diastolic dysfunctions. We describe a group of family members, including a pair of identical twins, each of whom suffered from thalassaemia major requiring multiple transfusions. Cardiac magnetic resonance demonstrated myocardial iron overload, and impairment of systolic function. Echocardiography confirmed both significant left ventricular systolic and diastolic impairment, along with features consistent with left ventricular non-compaction. This finding has not been noted in association with thalassaemia-related cardiac disease before. We then review the cardiac manifestations which occur in association with thalassaemia major.
authors.affiliation: Manchester Heart Centre, Manchester Royal Infirmary, Oxford Road, Manchester M13 9WL, UK. mluckie@doctors.net.uk
authors.forename: Matthew
authors.initials: M
authors.lastname: Luckie
authors.forename: Bruce
authors.initials: B
authors.lastname: Irwin
authors.forename: Satheesh
authors.initials: S
authors.lastname: Nair
authors.forename: John
authors.initials: J
authors.lastname: Greenwood
authors.forename: Rajdeep
authors.initials: R
authors.lastname: Khattar
mesh_terms: D003676:Deferoxamine / Q000627:therapeutic use; D004200:Diseases in Twins / Q000000981:diagnostic imaging*; D004452:Echocardiography; D005260:Female; D005293:Ferritins / Q000097:blood; D020022:Genetic Predisposition to Disease; D006331:Heart Diseases / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use; D019190:Iron Overload / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D009206:Myocardium / Q000473:pathology; D035781:Siblings; D065227:Transfusion Reaction; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging*; D055815:Young Adult; D017086:beta-Thalassemia / Q000150:complications* / Q000000981:diagnostic imaging / Q000628:therapy
chemical_list: D007502:Iron Chelating Agents; D005293:Ferritins; D003676:Deferoxamine