Which cardiac pathologies can lead to increased native T1?
Many, anything that can lead to fibrosis, amyloidosis, myocarditis, myocardial infarction, Tako-tsubo cardiomyopathy etc.
Which findings suggest the diagnosis in this non-contrast cardiac MRI?
Diffuse left and right ventricular hypertrophy including thickening of the atrial walls in conjunction with the abnormalities seen on the mapping sequences, especially the diffusely increased T1 values. In addition, the cine sequences show impaired ventricular filling and decreased mitral annular plane systolic excursion suggesting a restrictive cardiomyopathy.
What would be good additional tests for the work-up of this case?
Bone scintigraphy to see whether this could be ATTR amyloidosis, serum electrophoresis to look for light chains in case AL amyloidosis is suspected as well as tissue sampling.
Heart rate: ~90 bpm
Image quality: good, occasional artifact
The patient refused contrast media due to renal impairment.
Morphology and functional analysis (endo-volume):
LV-EDVI: 104 mL/m²
LV-ESVI: 65 mL/m²
LV-SVI: 39 mL/m²
LV-EF: 38%
cardiac output: 6.8 L/min
cardiac index: 3.5 L/min/m²
LV-ED wall mass index (without papillary muscle): 108 g/m²
Septum thickness: 18 mm
Findings:
Global hypokinesia. Signs of mild intraventricular asynchrony.
Impaired longitudinal contraction and diastolic filling.
Thickened left and right ventricular myocardium as well as atrial septum and right atrial wall.
Mitral prolapse with mild mitral insufficiency. Mild tricuspid insufficiency.
Pericardial effusion especially inferiorly and at the left lateral free wall.
Large right pleural effusion (~48 mm)
Myocardial tissue properties
STIR: diffuse high signal of the whole myocardium indicating myocardial edema
T1 mapping native: 1240-1300 ms [940-1060 ms*] - (z-score: 8-10)
T2 mapping: 57-72 ms [44-56 ms*] - (z-score: 3-7)
*reference range based on local data
Impression:
non-obstructive left ventricular hypertrophy with moderate systolic dysfunction
the high values in native T1 (z-score: 8-10) and signs of myocardial edema in conjunction with the cardiac hypertrophy raise the suspicion of cardiac amyloidosis
Exam courtesy: Kirsten Fleckstein (radiographer)