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Number 10-08: Perfusion Abnormalities in Cardiac Amyloidosis
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Number 10-08: Perfusion Abnormalities in Cardiac Amyloidosis 

Case from: AE Grasso, R O'Hanlon, C Bucciarelli-Ducci, F Alpendurada, DJ Pennell
Institution: CMR Unit, Royal Brompton Hospital, London, UK

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.



Figure 1


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).



Figure 2



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. 

These CMR features were highly suggestive of cardiac amyloidosis. 


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.

Cardiac amyloidosis is an infiltrative disease of the heart with deposition of insoluble amyloid fibrils leading to organ failure and death with a median survival of 20 months1. The interstitial deposition of amyloid fibrils within the ventricle leads to left ventricular hypertrophy with a restrictive cardiomyopathic phenotype, conduction disturbances and microvascular dysfunction2.

Evaluation of microvascular dysfunction using CMR in patients with cardiac amyloidosis has infrequently been studied, and the exact origin of the perfusion defect in this case is still debatable (myocardial amyloid infiltration? amyloid angiopathy?). Histopathological studies have shown that symptoms of angina, myocardial infarction and progressive heart failure in amyloidosis are the consequence of occlusion of small intramural coronary arteries by amyloid fibril deposition within the wall. In some cases, the occlusion of these small vessels can also lead to conduction disturbances and may represent an important risk factor for sudden death in this cohort.


1. Gertz MA, Kyle RA. Amyloidosis: prognosis and treatment. Semin Arthritis Rheum. 1994; 24: 124-38.

2. Neben-Wittich MA, Wittich CM, Muller PS. Obstructive intramural coronary amyloidosys and myocardial ischaemia are common in primary amyloidosis. Am J Med. 2005; 118: 1287.e1-1287.e7

3. Maceira AM, Joshi J, Prasad SK, Moon JC, Perugini E, Harding I, Sheppard MN, Poole-Wilson PA, Hawkins PN, Pennell DJ.Cardiovascular magnetic resonance in cardiac amyloidosis.  Circulation. 2005;111:186-93.

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COTW handling editor: Monica Deac

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