Skip to main content
main-content

01.12.2017 | Research | Ausgabe 1/2017 Open Access

Journal of Cardiovascular Magnetic Resonance 1/2017

Post-mortem study of the association between cardiac iron and fibrosis in transfusion dependent anaemia

Zeitschrift:
Journal of Cardiovascular Magnetic Resonance > Ausgabe 1/2017
Autoren:
Paul Kirk, Mary Sheppard, John-Paul Carpenter, Lisa Anderson, Taigang He, Tim St Pierre, Renzo Galanello, Gualtiero Catani, John Wood, Suthat Fucharoen, John B Porter, J Malcolm Walker, Gian Luca Forni, Dudley J Pennell

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 <3%.

Results

The 9 hearts affected by heart failure had severe iron loading with very low T2* of 5.0 ± 2.0 ms (iron concentration 8.5 ± 7.0 mg/g dw) and diffuse granular myocardial iron deposition. In none of the 10 hearts was significant macroscopic replacement fibrosis present. In only 2 hearts was interstitial fibrosis present, but with low CVF: in one patient with no cardiac siderosis (death by stroke, CVF 5.9%) and in a heart failure patient (CVF 2%). In the remaining 8 patients, no interstitial fibrosis was seen despite all having severe cardiac siderosis and heart failure (CVF 1.86% ±0.87%).

Conclusion

Replacement cardiac fibrosis was not seen in the 9 post-mortem hearts from patients with severe cardiac siderosis and heart failure leading to death or transplantation, which contrasts markedly to historical reports. Minor interstitial fibrosis was also unusual and very limited in extent. These findings accord with the potential for reversibility of heart failure seen in iron overload cardiomyopathy.

Trial registration

ClinicalTrials.gov Identifier: NCT00520559
Literatur
Über diesen Artikel

Weitere Artikel der Ausgabe 1/2017

Journal of Cardiovascular Magnetic Resonance 1/2017 Zur Ausgabe

Neu im Fachgebiet Radiologie

Meistgelesene Bücher aus der Radiologie

2016 | Buch

Medizinische Fremdkörper in der Bildgebung

Thorax, Abdomen, Gefäße und Kinder

Dieses einzigartige Buch enthält ca. 1.600 hochwertige radiologische Abbildungen und Fotos iatrogen eingebrachter Fremdmaterialien im Röntgenbild und CT.

Herausgeber:
Dr. med. Daniela Kildal

2011 | Buch

Atlas Klinische Neuroradiologie des Gehirns

Radiologie lebt von Bildern! Der vorliegende Atlas trägt dieser Tatsache Rechnung. Sie finden zu jedem Krankheitsbild des Gehirns Referenzbilder zum Abgleichen mit eigenen Befunden.

Autoren:
Priv.-Doz. Dr. med. Jennifer Linn, Prof. Dr. med. Martin Wiesmann, Prof. Dr. med. Hartmut Brückmann

Mail Icon II Newsletter

Bestellen Sie unseren kostenlosen Newsletter Update Radiologie und bleiben Sie gut informiert – ganz bequem per eMail.

Bildnachweise