1. Post-mortem study of the association between cardiac iron and fibrosis in transfusion dependent anaemia
- Author
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Gualtiero Catani, J Malcolm Walker, Gian Luca Forni, Taigang He, Suthat Fucharoen, John B. Porter, John C. Wood, Lisa J. Anderson, Mary N. Sheppard, Timothy G. St. Pierre, Paul Kirk, Renzo Galanello, Dudley J. Pennell, John-Paul Carpenter, National Institutes of Health, British Heart Foundation, and Royal Brompton & Harefield NHS Foundation Trust
- Subjects
Male ,lcsh:Diseases of the circulatory (Cardiovascular) system ,Cardiac & Cardiovascular Systems ,CHELATION-THERAPY ,Cardiac fibrosis ,medicine.medical_treatment ,Cardiomyopathy ,030204 cardiovascular system & hematology ,Severity of Illness Index ,030218 nuclear medicine & medical imaging ,0302 clinical medicine ,Cause of Death ,Child ,Coloring Agents ,Heart transplantation ,Medicine(all) ,Radiological and Ultrasound Technology ,HYPERTROPHIC CARDIOMYOPATHY ,T2-ASTERISK ,Radiology, Nuclear Medicine & Medical Imaging ,Hypertrophic cardiomyopathy ,OVERLOAD ,Heart ,Middle Aged ,Hypertensive heart disease ,3. Good health ,Nuclear Medicine & Medical Imaging ,Thalassaemia ,CARDIOVASCULAR MAGNETIC-RESONANCE ,Cardiology ,Female ,Autopsy ,Siderosis ,Cardiology and Cardiovascular Medicine ,Cardiomyopathies ,Life Sciences & Biomedicine ,Adult ,medicine.medical_specialty ,Hemosiderosis ,Adolescent ,MYOCARDIAL FIBROSIS ,Iron ,Histopathology ,1102 Cardiovascular Medicine And Haematology ,03 medical and health sciences ,Young Adult ,Cardiac siderosis ,Internal medicine ,medicine ,Humans ,Radiology, Nuclear Medicine and imaging ,Blood Transfusion ,Cardiac MR ,Heart Failure ,Science & Technology ,Staining and Labeling ,business.industry ,Research ,Myocardium ,Spectrophotometry, Atomic ,BETA-THALASSEMIA MAJOR ,beta-Thalassemia ,QUANTIFICATION ,medicine.disease ,HYPERTENSIVE HEART-DISEASE ,Fibrosis ,COLLAGEN ,lcsh:RC666-701 ,Heart failure ,Cardiovascular System & Cardiology ,Heart Transplantation ,Myocardial fibrosis ,business ,Azo Compounds - Abstract
Background Heart failure related to cardiac siderosis remains a major cause of death in transfusion dependent anaemias. Replacement fibrosis has been reported as causative of heart failure in siderotic cardiomyopathy in historical reports, but these findings do not accord with the reversible nature of siderotic heart failure achievable with intensive iron chelation. Methods Ten whole human hearts (9 beta-thalassemia major, 1 sideroblastic anaemia) were examined for iron loading and fibrosis (replacement and interstitial). Five had died from heart failure, 4 had cardiac transplantation for heart failure, and 1 had no heart failure (death from a stroke). Heart samples iron content was measured using atomic emission spectroscopy. Interstitial fibrosis was quantified by computer using picrosirius red (PSR) staining and expressed as collagen volume fraction (CVF) with normal value for left ventricle
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