TY - JOUR
T1 - Cardiac changes in systemic amyloidosis
T2 - Visualisation by magnetic resonance imaging
AU - Cheng, Adrian S H
AU - Banning, Adrian P.
AU - Mitchell, Andrew R J
AU - Neubauer, Stefan
AU - Selvanayagam, Joseph B.
N1 - Copyright:
Copyright 2008 Elsevier B.V., All rights reserved.
PY - 2006/10/26
Y1 - 2006/10/26
N2 - Cardiac involvement is a significant cause of morbidity and mortality in patients with amyloidosis. A 73-year old Caucasian woman was admitted to a hospital with increasing dyspnoea. Examination revealed a large left-sided pleural effusion, gross peripheral oedema and a pan-systolic murmur. The electrocardiogram showed low voltage QRS complexes with lateral T wave inversion. Recent angiography had demonstrated normal coronaries. Echocardiography revealed left ventricular hypertrophy and an ejection fraction of 43%. There was a restrictive filling pattern with elevated left-sided filling pressures, left atrial enlargement and mild mitral regurgitation. The right heart was normal with normal filling pressures. The patient was referred for cardiovascular magnetic resonance imaging for investigation of restrictive cardiomyopathy. This case provides a striking demonstration of the characteristic features of cardiac amyloidosis by cardiovascular magnetic resonance imaging - impaired biventricular systolic function, thickened atrioventricular valves, bi-atrial enlargement, increased atrial septal thickness and left ventricular mass, pleural and pericardial effusions, and the most impressive finding of widespread subendocardial hyperenhancement of both ventricles, as well as the inter-atrial and inter-ventricular septa, representing infiltration with amyloid protein. Cardiac involvement with systemic amyloidosis was suspected and confirmed on biopsy. Cardiovascular magnetic resonance imaging should be considered early in the diagnostic work-up of suspected cardiac amyloidosis.
AB - Cardiac involvement is a significant cause of morbidity and mortality in patients with amyloidosis. A 73-year old Caucasian woman was admitted to a hospital with increasing dyspnoea. Examination revealed a large left-sided pleural effusion, gross peripheral oedema and a pan-systolic murmur. The electrocardiogram showed low voltage QRS complexes with lateral T wave inversion. Recent angiography had demonstrated normal coronaries. Echocardiography revealed left ventricular hypertrophy and an ejection fraction of 43%. There was a restrictive filling pattern with elevated left-sided filling pressures, left atrial enlargement and mild mitral regurgitation. The right heart was normal with normal filling pressures. The patient was referred for cardiovascular magnetic resonance imaging for investigation of restrictive cardiomyopathy. This case provides a striking demonstration of the characteristic features of cardiac amyloidosis by cardiovascular magnetic resonance imaging - impaired biventricular systolic function, thickened atrioventricular valves, bi-atrial enlargement, increased atrial septal thickness and left ventricular mass, pleural and pericardial effusions, and the most impressive finding of widespread subendocardial hyperenhancement of both ventricles, as well as the inter-atrial and inter-ventricular septa, representing infiltration with amyloid protein. Cardiac involvement with systemic amyloidosis was suspected and confirmed on biopsy. Cardiovascular magnetic resonance imaging should be considered early in the diagnostic work-up of suspected cardiac amyloidosis.
KW - Amyloid
KW - Delayed enhancement
KW - Magnetic resonance imaging
UR - http://www.scopus.com/inward/record.url?scp=33750466562&partnerID=8YFLogxK
U2 - 10.1016/j.ijcard.2006.07.107
DO - 10.1016/j.ijcard.2006.07.107
M3 - Article
C2 - 17049635
AN - SCOPUS:33750466562
SN - 0167-5273
VL - 113
SP - E21-E23
JO - International Journal of Cardiology
JF - International Journal of Cardiology
IS - 1
ER -