Protocol
  1. Anatomy (A) and LV function (RV function) module (B/C)
  2. T2* module (L)
Report
  1. Dimensions, mass (corrected for BSA) and function
    • LV: EDV, ESV , SV, EF, longitudinal function, mass
    • RV: EDV, ESV, SV, EF, longitudinal function
  2. T2* values of heart and liver

Key Points
  1. Primary hemochromatosis - autosomal disorder with increased iron absorption and abnormal iron metabolism
    Secondary iron overload - primarily associated with transfusion dependent anemias (myelodysplastic syndrome, thalassemia, sickle cell disease, etc. )
  2. Iron overload cardiomyopathy (IOC)
    • Determinant of survival
    • Potentially reversible cause of heart failure under effective therapy
    • More frequent in secondary iron overload
    • Varied symptoms: from asymptomatic patients early in the disease process to terminal heart failure symptoms
  3. Lab testing
    • Normal serum ferritin, and plasma transferrin saturation excludes iron overload in suspected individuals
  4. Dilated phenotype – majority of patients
    • RV and LV chamber dilation
    • Impaired systolic function
    Restrictive phenotype
    • Non-dilated ventricles
    • Preserved systolic function
    • Diastolic dysfunction
    • Enlarged atria
    • Occasionally pulmonary hypertension with subsequent right ventricular dilatation and systolic impairment
  5. Diagnostic T2* values (ms) - reciprocal of relaxation parameter R2*
    6. Septal myocardiumLiver tissue
    None>20>19
    Mild14-207.3-18
    Moderate10-143.4-7.2
    Severe<10<3.3
  6. Other diagnostic signs
    • Focal signal loss in native T1- and T2-weighted images
    • Abnormally "dark" liver
  7. Risk of developing heart failure
    • T2* > 20 ms: low
    • T2* 10-20 ms: intermediate
    • T2* <10 ms: high
  8. Other cardiac manifestations
    • Conduction system abnormalities
    • Tachyarrhythmias
    • Perimyocarditis
  9. Serial follow-up of iron loading to guide chelation therapy
  10. Isolated cardiac or liver involvement is possible
Tips and Tricks
  1. Assess T2* values in the septum (fewer artefacts) as there is no different iron deposition among the various LV segments
References