Contribution of Cardiac Magnetic Resonance to Hematochromatosis
Downloads
Hemochromatosis is a disease characterized by the progressive accumulation of iron in the body. It can be primitive or secondary. It affects several organs including the heart, liver, pancreas and pituitary gland.
The preponderant cardiac involvement is myocardial, related to the iron myocyte overload, causing a decrease in left ventricular distensibility.
Echocardiography is the first-line examination, showing an abnormality of left ventricular filling and, later, cavitary dilatation with left ventricular systolic dysfunction.
Cardiac MRI is the gold standard for measuring excess iron in the myocardium in the context of hemochromatosis using the T2 * technique. It plays a major role for the diagnosis of cardiac involvement and for monitoring the chelation treatment.
In this study, we report three cases of patients with hemochromatosis whose cardiac localization was confirmed or eliminated using cardiac MRI.
Cardiovascular magnetic resonance has been used to assess myocardial iron deposition using the relaxation parameters T2*. Heart T2* falls with increasing iron loading.
One patient had a myocardial T2* below 20 ms indicating myocardial siderosis.
The others patients had a T2 *> 20 ms. The late enhancement was absent in all our patients.
This study aims at detecting the myocardial iron overload using cardiac MRI.
The treatment is based on bleeding in primary hemochromatosis while in secondary hemochromatosis, it is based on excretion of iron by chemical chelation.
Vinay Gulati, MD, Prakash Harikrishnan, MD, Chandrasekar Palaniswamy, MD, Wilbert S. Aronow, MD,Diwakar Jain, MD and William H. Frishman, Cardiac Involvement in Hemochromatosis . MD. Cardiology in Review 2014;22: 56–68 .
wood Jc. Magnetic resonance imaging measurement of iron overload. Curr Opin Hematol. 2007;14:183–190.
Mavrogeni SI, Gotsis ED, Markussis V, et al. T2 relaxation time study of iron overload in b-thalassemia. MAGMA 1998 ; 6 : 7–12.
Westwood MA, Sheppard MN, Awogbade M, Ellis G, Stephens AD, Pennell DJ. Myocardial biopsy and T2* magnetic resonance in heart failure due to thalassaemia. Br J Haematol 2005 ;128 :2.
Ghugre NR, Enriquez CM, Gonzalez I, Nelson MD Jr, Coates TD, Wood JC. MRI detects myocardial iron in the human heart. Magn Reson Med 2006;56 : 681–686.
Management of Cardiac hematochromatosis. Wilbert S. Aronow. Cardiology Division, Department of Medicine, Westchester Medical Center/New York Medical College, Valhalla, NY, USA. Arch Med Sci 2018; 14, 3: 560–568.
Barrera Portillo Mc, Uranga Uranga M, Sánchez González J, et al. liver and heart t2* measurement in secondary haemochromatosis. Radiologia. 2013;55:331–339.
Westwood M, Anderson LJ, Firmin DN, Gatehouse PD, Charrier CC, Wonke B, Pennell DJ. A single breath-hold multiecho T2* cardiovascular magnetic resonance technique for diagnosis of myocardial iron overload. J Magn Reson Imaging 2003;18:33-9.
Pepe A, Positano V, Santarelli MF, Sorrentino F, Cracolici E, De Marchi D, Maggio A, Midiri M, Landini L, Lombardi M. Multislice multiecho T2* cardiovascular magnetic resonance for detection of the heterogeneous distribution of myocardial iron overload. J Magn Reson Imaging 2006;23:662-8.
Baksi AJ, Pennell DJ. T2* imaging of the heart: methods, applications, and outcomes. Top Magn Reson Imaging 2014;23:13-20.
Anderson LJ, Holden S, Davis B, Prescott E, Charrier CC, Bunce NH, Firmin DN, Wonke B, Porter J, Walker JM, Pennell DJ. Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload. Eur Heart J 2001;22:2171-9.
Taigang.He Cardiovascular magnetic resonance T2* for tissue iron assessment in the heart. Quant Imaging Med Surg 2014 ;4(5) :407-412.
Pepe A, Positano V, Santarelli MF, et al. Multislice mul- tiecho T2* cardiovascular magnetic resonance for de- tection of the heterogeneous distribution of myocardial iron overload. J MagnReson Imaging 2006; 23: 662-8.
Mavrogeni S, Markousis-Mavrogenis G, Markussis V, Kolovou G. The emerging role of cardiovascular magnetic resonance imaging in the evaluation of metabolic cardiomyopathies. Horm Metab Res 2015; 47: 623-32.
Messroghli DR, Radjenovic A, Kozerke S, Higgins DM, Sivananthan MU, Ridgway JP. Modified Look-Locker inversion recovery (MOLLI) for high-resolution T1 mapping of the heart. Magn Reson Med2004;52:141-6.
Feng Y, He T, Carpenter JP, Jabbour A, Alam MH, Gatehouse PD, Greiser A, Messroghli D, Firmin DN, Pennell DJ. In vivo comparison of myocardial T1 with T2 and T2* in thalassaemia major. J Magn Reson Imaging 2013;38:588-93.
Jensen JH, Tang H, Tosti CL, Swaminathan SV, Nunez A, Hultman K, Szulc KU, Wu EX, Kim D, Sheth S, Brown TR, Brittenham GM. Separate MRI quantification of dispersed (ferritin-like) and aggregated (hemosiderin-like) storage iron. Magn Reson Med 2010;63:1201-9.
All Content should be original and unpublished.