|Number 10-08: Perfusion Abnormalities in Cardiac Amyloidosis|
Number 10-08: Perfusion Abnormalities in Cardiac Amyloidosis
Case from: AE Grasso, R O'Hanlon, C Bucciarelli-Ducci, F Alpendurada, DJ Pennell
Clinical history: A 68 year-old hypertensive male presented with typical chest pain. The ECG on arrival was ischemic and cardiac TnI was positive. Coronary angiography demonstrated unobstructed epicardial coronary arteries. The echocardiogram showed a significant degree of septal wall thickening, out of proportion with his well-controlled blood pressure profile, suggesting possible hypertrophic cardiomyopathy. A CMR was requested to further assess left ventricular hypertrophy, as well as the origin of the chest pain.
Movie 1a Movie 1b Movie 1c
Standard SSFP cine imaging in the long axis (Movies 1A and 1B) and short axis planes (Movie 1C) confirmed significant left ventricular hypertrophy with a maximum wall thickness of 19mm in the basal inferior septum, and 12 mm for the lateral wall. Total LV mass was 230g (=121g/m2). Biventricular volumes and ejection fraction were normal (LVEF=70%). Pericardial and bilateral pleural effusions were also noted.
Given the presenting symptoms of chest pain, first pass perfusion imaging (Figure 1) using a 3-slice hybrid EPI sequence was performed at stress (top panel, A-C) using intravenous adenosine (140µg/kg/min) and at rest (bottom panel, D-F). A circumferential, sub-endocardial inducible perfusion defect was seen during stress at all levels, suggesting microvascular ischemia (straight arrows).
Corresponding late gadolinium-enhanced sequences (Figure 2 (A) basal, (B) mid, and (C) apical levels) revealed a characteristic dark blood pool (asterisk), with diffuse subendocardial contrast uptake of the LV and RV (curved arrow) sparing the mid wall and epicardium (perpendicular arrows). There was difficulty nulling the myocardium, and gadolinium wash-out was very rapid.
Perspective: In this patient, CMR was a "one shop stop" to1) determine the aetiology of left ventricular hypertrophy, 2) to suggest the diagnosis of cardiac amyloidosis, 3) to determine the nature of the chest pain by showing the presence of inducible perfusion defects, and 4) to identify related findings such as pericardial and pleural effusions.
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