Alpendurada, Francisco

Malley, Tamir

Mathew, George Lathra

Symmonds, Karen

Vassiliou, Vassilios S.

Oxford Medical Case Reports

PubMed

University of East Anglia digital repository

C L IN I CA L IMAGEAn unexpected cause of hypertrophic myocardiumGeorge Lathra Mathew1, Vassilios S. Vassiliou1,2,*, Tamir Malley1,Karen Symmonds1, and Francisco Alpendurada11CMR Unit, Royal Brompton Hospital, London, UK and 2National Heart and Lung Institute, Imperial College,London, UK*Correspondence address. CMR Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK. Tel: +44-207-352-8121; Fax: +44-0207-351-8718;E-mail: v.vassiliou@rbht.nhs.ukA 60-year-oldmanwith known atrial ﬁbrillation (AF)was referredfor routine cardiovascular magnetic resonance imaging (CMR)prior to AF ablation. He was normotensive and had no other pre-vious medical conditions. A transthoracic echocardiogram hadshown good systolic function with moderate left ventricularhypertrophy and mildly dilated atria (Fig. 1).Initial CMR imaging for anatomy and function demonstratednormal ventricular size and mildly impaired systolic function(ejection fraction = 50%, normal >55%) with no signiﬁcant valvu-lar pathology. Therewas asymmetrical septal hypertrophymeas-uring 16 mm (normal <13 mm) in the basal septum versus 9 mmin the lateral wall at the same level with signiﬁcantly elevated leftventricular mass at 241 g (Fig. 2a). There was no systolic anteriormotion of the mitral valve or left ventricular outﬂow tract ob-struction at rest. Both atria were slightly enlarged, the left atriummeasuring 35 cm2 and the right atrium measuring 36 cm2.To identify the etiology of hypertrophy, conventional late gado-linium enhancement (Fig. 2b) was undertaken alongside novelnative T1 mapping sequences (Fig. 3 and Supplementary Videos1 and 2). Acquisition of late gadolinium images was technicallychallenging, giving the impression of diffuse enhancementassociated with a relatively dark blood pool, both of which aresuspicious of amyloid inﬁltration of the heart [1]. The native T1maps showed an elevated septal T1 relaxation time (1148 ms,normal <1050 ms), supporting the initial impression of cardiacamyloid and further suggesting that this was most likely due tolight chain deposition (AL amyloid) [2]. AL amyloid was subse-quently conﬁrmed by serum free light chain immunoassay andbone marrow trephine biopsy.A combination of late gadolinium enhancement and novel T1mapping sequences have the ability to correctly determine theunderlying pathology in patients with hypertrophicmyocardiumand potentially help in the identiﬁcation of the amyloid class.SUPPLEMENTARY MATERIALSupplementary material is available at Oxford Medical CaseReports online.Figure 1: Four-chamber transthoracic echocardiography demonstratingmoderatemyocardial hypertrophy and mildly dilated atria.Received: December 30, 2014. Revised: February 17, 2015. Accepted:March 16, 2015© The Author 2015. Published by Oxford University Press.This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited.For commercial re-use, please contact journals.permissions@oup.comOxford Medical Case Reports, 2015 , 276–277doi: 10.1093/omcr/omv029Clinical Image276; 4CONFLICT OF INTEREST STATEMENTThis work was supported by NIHR Cardiovascular BiomedicalResearch Unit of Royal Brompton & Hareﬁeld NHS FoundationTrust and Imperial College London.REFERENCES1. Maceira AM, Joshi J, Prasad SK, Moon JC, Perugini E, Harding I,et al. Cardiovascular magnetic resonance in cardiac amyloid-osis. Circulation 2005;111:186–93.2. Karamitsos TD, Piechnik SK, Banypersad SM, Fontana M,Ntusi NB, Ferreira VM, et al. Noncontrast T1 mapping for thediagnosis of cardiac amyloidosis. JACC Cardiovasc Imaging2013;6:488–97.Figure 2: (a) A hypertrophic myocardium with mildly dilated atria. (b) Diffuse enhancement associated with a relatively dark blood pool (difﬁcult ‘nulling’) of themyocardium, typical of cardiac amyloid.Figure 3: T1 map following motion correction and co-registration to enablecalculation of native T1 values. In this case, the septal value was elevated at1148 ms suggestive of light chain amyloid deposition (AL amyloid) anddemonstrating how new techniques can be utilized to aid the diagnosis of rarepathology.An unexpected cause of hypertrophic myocardium | 277

An unexpected cause of hypertrophic myocardium

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