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Toward clinical risk assessment inhypertrophic cardiomyopathy withgadolinium cardiovascular magnetic resonance

James C. C. Moon, MB, BCh^*, William J. McKenna, FACC, FESC, Jane A. McCrohon, FRACP, PhD^*, Perry M. Elliott, MD, MRCP, FACC, Gillian C. Smith, BSc^* and Dudley J. Pennell, MD, FRCP, FESC, FACC^*,*

^* Centre for Advanced Magnetic Resonance in Cardiology (CAMRIC), Royal Brompton Hospital, London, United Kingdom
Department of Cardiological Sciences, St. George’s Hospital Medical School, London, United Kingdom

View larger version (157K): [in a new window]   Figure 1 Pre- and post-contrast images demonstrating hyperenhancement. The pre-contrast images are the diastolic frames of fast imaging with steady-state precession cine loops. In the post-contrast images, normal myocardium appears dark. There is a large area of septal hyperenhancement, with additional papillary muscle hyperenhancement and subendocardial hyperenhancement of the lateral wall. The patient is a 33-year-old female with two risk factors for sudden death. Eight years previously, echocardiography demonstrated marked asymmetric septal hypertrophy, but the ventricle is now thinning and dilating and left bundle branch block has developed. The total extent of hyperenhancement was 25% of the left ventricular (LV) mass. LA = left atrium; RA = right atrial; RV = right ventricle.

View larger version (22K): [in a new window]   Figure 2 Extent of hyperenhancement and presence of progressive disease. Hyperenhancement was associated with the presence of progressive disease (A), even when patients at high risk of sudden death were excluded (B), and appeared more marked in patients >40 years old, regardless of the risk of sudden death (C, D). rfsd = risk factors for sudden death.

View larger version (23K): [in a new window]   Figure 3 Extent of hyperenhancement and clinical risk factors for sudden death. Hyperenhancement was associated with an increased clinical risk of sudden death (A), even when patients with progressive disease were excluded (B), and appeared more marked in patients <40 years old, regardless of the presence of progressive disease (C, D).

View larger version (79K): [in a new window]   Figure 4 (A and B) Patterns of hyperenhancement in hypertrophic cardiomyopathy by cardiovascular magnetic resonance. The patterns of hyperenhancement are grouped into diffuse or confluent types (upper rows: long-axis, vertical, or horizontal; lower rows: short-axis views). These were further subclassified as shown. The different patterns may have clinical or prognostic significance, and this qualitative classification may complement the overall quantification of hyperenhancement. RV = right ventricular.