Presentation
Arrhythmia, dyspnea, known pleural effusion, known back pain. No known malignancy. Impaired renal function.
Patient Data
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)
Rectal biopsy
In addition to inconspicuous colon mucosa, parts of the submucosa with several incisions of vessels are shown. Pale eosinophilic deposits in the vessel wall. These are positive in Congo red staining showing apple-green birefringence under polarized light corresponding to amyloidosis.
Serum immunofixation electrophoresis
Indicative of monoclonal gammopathy of free light chains (lambda type).
Genotyping
No TTR gene mutation.
Case Discussion
A case of cardiac amyloidosis on a non-contrast cardiac MRI in a patient with moderately reduced systolic function.
Bone scintigraphy revealed a Perugini score of 0. Due to the findings of cardiac MRI, especially the increased interventricular septum thickness and considerably diffusely increased native T1 values (z-score: 8-10) also suggested the possibility of AL amyloidosis. Testing including a rectal biopsy confirmed the diagnosis of amyloidosis and the detection of immunoglobulin light chains in serum and the absence of TTR gene mutation and helped to confirm the diagnosis of AL amyloidosis.
The cardiac MRI in this patient did not only help in the diagnosis but also provides important information with respect to the prognosis of this patient by the demonstration of myocardial edema which unfortunately is associated with a poorer outcome 2.
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