Published Online
on January 3, 2005

A more recent version of this article appeared on January 18, 2005

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Submitted on April 30, 2004
Revised on October 20, 2004
Accepted on October 25, 2004

Cardiovascular Magnetic Resonance in Cardiac Amyloidosis

Alicia Maria Maceira MD, Jayshree Joshi BSc, Sanjay Kumar Prasad MD, MRCP, James Charles Moon MB, MRCP, Enrica Perugini MD, Idris Harding BSc, Mary Noelle Sheppard MD, FRCPath, Philip Alexander Poole-Wilson MD, FRCP, Philip Nigel Hawkins PhD, FRCP, and Dudley John Pennell MD, FRCP^*

From the Cardiovascular Magnetic Resonance Unit (A.M.M., S.K.P., J.C.M., D.J.P.) and Department of Pathology (I.H., M.N.S.), Royal Brompton Hospital, London, UK; National Amyloidosis Centre, Royal Free Hospital, London, UK (J.J., P.N.H.); Istituto di Cardiologia, Hospital Sant Orsola, Bologna, Italy (E.P.); and Department of Cardiac Medicine, NHLI, Imperial College, London, UK (P.A.P.-W.).

^* To whom correspondence should be addressed. E-mail: d.pennell{at}ic.ac.uk.

Background--Cardiac amyloidosis can be diagnostically challenging. Cardiovascular magnetic resonance (CMR) can assess abnormal myocardial interstitium.

Methods and Results--Late gadolinium enhancement CMR was performed in 30 patients with cardiac amyloidosis. In 22 of these, myocardial gadolinium kinetics with T₁ mapping was compared with that in 16 hypertensive controls. One patient had CMR and autopsy only. Subendocardial T₁ in amyloid patients was shorter than in controls (at 4 minutes: 427±73 versus 579±75 ms; P<0.01), was shorter than subepicardium T₁ for the first 8 minutes (P0.01), and was correlated with markers of increased myocardial amyloid load, as follows: left ventricular (LV) mass (r=-0.51, P=0.013); wall thickness (r=-0.54 to -0.63, P<0.04); interatrial septal thickness (r=-0.52, P=0.001); and diastolic function (r=-0.42, P=0.025). Global subendocardial late gadolinium enhancement was found in 20 amyloid patients (69%); these patients had greater LV mass (126±30 versus 93±25 g/m²; P=0.009) than unenhanced patients. Histological quantification showed substantial interstitial expansion with amyloid (30.5%) but only minor fibrosis (1.3%). Amyloid was dominantly subendocardial (42%) compared with midwall (29%) and subepicardium (18%). There was 97% concordance in diagnosis of cardiac amyloid by combining the presence of late gadolinium enhancement and an optimized T₁ threshold (191 ms at 4 minutes) between myocardium and blood.

Conclusions--In cardiac amyloidosis, CMR shows a characteristic pattern of global subendocardial late enhancement coupled with abnormal myocardial and blood-pool gadolinium kinetics. The findings agree with the transmural histological distribution of amyloid protein and the cardiac amyloid load and may prove to have value in diagnosis and treatment follow-up.

Key words: amyloid • gadolinium • magnetic resonance imaging • heart diseases • cardiomyopathy

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