VIEW Open Access Society for Cardiovascular Magnetic Resonance 2019 Case of the Week Series Arun Dahiya1,2, Charles Chao1, John Younger1, Julia Kar3, Bryant M
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Dahiya et al. J Cardiovasc Magn Reson (2021) 23:44 https://doi.org/10.1186/s12968-020-00671-7 REVIEW Open Access Society for Cardiovascular Magnetic Resonance 2019 Case of the Week series Arun Dahiya1,2, Charles Chao1, John Younger1, Julia Kar3, Bryant M. Baldwin3, Michael V. Cohen4, Shane Joseph4, Anam Chowdhry4, Maria S. Figarola5, Christopher Malozzi4, M. Farhan Nasser6, Yassar Nabeel7, Rajiv Shah8, J. Michael Kennen8, Ashish Aneja7, Sameh Khalil9, Sara Ragab9, Omnia Mohammed9, Taher Moustafa9, Ahmed Hamdy9, Shimaa Ahmed9, Ahmed Heny9, Maha Taher9, Madhusudan Ganigara10, Arushi Dhar10, Nilanjana Misra10, Jafar Alzubi6, Kurian Pannikottu6, Ahmad Jabri6, Vinayak Hedge11, Anmar Kanaa’n11, Joseph Lahorra12, Dominique de Waard13, David Horne13, Santokh Dhillon14, Aoife Sweeney15, Christian Hamilton‑Craig16,17, V. S. Katikireddi15, Allan J. Wesley17, Chris Hammet1, Jason N. Johnson18 and Sylvia S. M. Chen16* Abstract The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, educa‑ tion, and clinical application of cardiovascular magnetic resonance (CMR). The SCMR web site (https:// www. scmr. org) hosts a case series designed to present case reports demonstrating the unique attributes of CMR in the diagnosis or management of cardiovascular disease. Each clinical presentation is followed by a brief discussion of the disease and unique role of CMR in disease diagnosis or management guidance. By nature, some of these are somewhat esoteric, but all are instructive. In this publication, we provide a digital archive of the 2019 Case of the Week series as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar search engine. Keywords: Cardiomyopathy, Cardiac tumor, MRI, Eosinophilic granulomatosis Introduction where solitary masses/tumours are rare, was well demon- Firstly, a huge thank you to our wonderful team of associ- strated. Early CMR diagnosis was demonstrated despite ate editors and reviewers for the Society for Cardiovas- seemingly negative testing by other methods including cular Magnetic Resonance (SCMR) website “Case of the tissue biopsy. Te use of a research tool in the clinical Week” series. Tis past year, the cases were predomi- setting was also reported, giving a glimpse of its poten- nantly from the United States, with international cases tial use in early identifcation of a pathological process from Egypt, Canada and Australia. Tere was a mixture and therefore help guide clinical management. Here are of adult and paediatric cases, showcasing the utility of the SCMR Case of the Week series of 2019. Please con- cardiovascular magnetic resonance (CMR) in assessing tinue to submit your instructive cases to: scmr.org/page/ and diagnosing disease. Te importance of CMR in mass SubmitCase. diferentiation, particularly in the paediatric population *Correspondence: [email protected] 16 Department of Cardiology, The Prince Charles Hospital, Brisbane, QLD, Australia Full list of author information is available at the end of the article © The Author(s) 2021. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http:// creat iveco mmons. org/ licen ses/ by/4. 0/. The Creative Commons Public Domain Dedication waiver (http:// creat iveco mmons. org/ publi cdoma in/ zero/1. 0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Dahiya et al. J Cardiovasc Magn Reson (2021) 23:44 Page 2 of 29 Case 1: Early diagnosis of a systemic disease possible cardiac embolic source was unremarkable by CMR prevents complications except for mildly increased left ventricular (LV) apical Clinical history wall thickness (Additional fle 1: Movie S1). Given the A 19-year-old woman presented with a 2-week history abnormal ECG and increased LV apical wall thickness, of painful atraumatic left ankle swelling associated with CMR was requested to assess for possible apical hyper- painful plantar-fexion and petechial rash on the left trophic cardiomyopathy (HCM). foot. Six weeks prior to presentation, she experienced a fu-like illness with headache, fevers, myalgias, and CMR fndings athralgias. On examination, there was a non-blanch- 3 T CMR (Siemens Healthineers, Erlangen Germany) ing petechial rash over the dorsum of the left foot showed normal biventricular size and systolic function with associated edema and warmth, but no erythema. with only minimal increase in the LV apical wall thick- Peripheral pulses were normally palpable with normal ness (Additional fle 2: Movie S2, Additional fle 3: Movie capillary refll. Neurological exam of the foot was nor- S3, Additional fle 4: Movie S4). mal except for 4/5 power on plantar fexion due to pain. Te LV mass index was 64 g/m2, the LV end diastolic Ultrasound of the leg showed a thrombus in the lateral volume index was 73 ml/m2, with a low normal LV ejec- tarsal artery raising a possibility of an embolus. tion fraction (LVEF) of 57%. Te right ventricular (RV) Her past medical history was remarkable for mild size and systolic function were normal with an RV end childhood asthma that was associated with viral infec- diastolic volume index of 74 ml/m2 and RV ejection frac- tions and exercise; and allergic rhinitis. She had no tion (RVEF) of 51%. LV tissue characterization revealed prior hospitalizations. Chest X-ray was unremarkable. subtle sub-endocardial abnormalities. T2-weighted Electrocardiogram (ECG) showed T-wave inversion in (T2w) imaging showed somewhat difuse sub endomyo- most precordial leads (V2–V6) and some inferior leads cardial edema in mid to distal LV segments (Fig. 2). Rest (III and aVF) (Fig. 1). perfusion sequences in short axis and apical 4-chamber Routine blood work was unremarkable except for view showed mid to apical sub-endocardial hypo-perfu- eosinophilia which was attributable to asthma. Infam- sion (Additional fle 5: Movie S5, Additional fle 6: Movie matory markers (erythrocyte sedimentation rate (ESR) S6). 41 mm/h and C-reactive protein (CRP) 22 mg/l were Late gadolinium enhancement (LGE) imaging using mildly elevated. Troponin was negative. Transthoracic T1-weighted (T1w) imaging in short axis showed suben- echocardiogram (TTE) performed to evaluate for a docardial enhancement in the mid to distal LV segments Fig. 1. Case 1. 12 lead electrocardiogram (ECG). Sinus rhythm with T‑wave inversion in most precordial leads (V2 V6) and some inferior limb leads − (III and aVF) Dahiya et al. J Cardiovasc Magn Reson (2021) 23:44 Page 3 of 29 Fig. 2 Case 1. T2‑weighted short tau inversion recovery (STIR) imaging of the left ventricular (LV) short axis from base to apex. Difuse increase in subendocardial signal intensity suggestive of subendocardial edema Fig. 3 Case 1. Short axis stack T1 weighted (high resolution‑magnitude) late gadolinium enhancement (LGE) imaging. Subendocardial enhancement in the mid to distal LV cavity (Fig. 3). Tese fndings correlated with phase sensi- 4-chamber views (Fig. 4) also showing subtle subendo- tive inversion recovery (PSIR) LGE imaging in 3- and cardial enhancement in the mid- and distal-LV segments. Dahiya et al. J Cardiovasc Magn Reson (2021) 23:44 Page 4 of 29 Fig. 4 Case 1. Phase sensitive inversion recovery (PSIR). a Three chamber view with subtle subendocardial LGE of the mid and apical anteroseptal and inferolateral wall segments. b Four chamber view with subtle subendocardial LGE of the mid and apical inferoseptal and anterolateral wall segments Multi-parametric CMR: Pre- and post- contrast (normal ~ 1150–1300 ms) as well as extracellular vol- T1 mapping was performed a using modifed Look- ume (ECV) of 39% (normal ~ 20–30%). In the remote Locker inversion (MOLLI) sequence (Myomaps, myocardium (uninvolved basal inferoseptum) native T1 Siemens Healthineers). Region of interest involv- was in the normal range (1279 ms) and ECV was within ing apical septum had an elevated T1 of 1385 ms normal limits (25%) (Fig. 5). Color maps confrmed Fig. 5 Case 1. Native T1, post‑contrast T1, and extracellular volume (ECV) map in 4‑chamber view. Native T1 (a) and ECV map (b) with abnormal measured native T1 and ECV in region of interest (apical septum) compared to remote region (basal septum). Pre‑contrast T1 (c), post‑contrast T1 (d), and ECV (e) map in 4‑chamber view Dahiya et al. J Cardiovasc Magn Reson (2021) 23:44 Page 5 of 29 subendocardial elevation in pre-contrast T1 and ECV immune suppression. Repeat CMR performed in mid to apical LV segments (Fig. 5). 6 months post treatment revealed signifcant interval Te patient was subsequently investigated for a sys- resolution of abnormal CMR fndings. temic disease such as eosinophilic granulomatosis with polyangitis (EGPA). Chest computed tomography Perspective (CT) identifed non-specifc sub-pleural infamma- EGPA, previously known as Churg-Strauss syndrome, tory changes (Fig. 6). CT guided needle biopsy of this is a multisystem disorder characterized by chronic rhi- lesion demonstrated eosinophilic infltrates consistent nosinusitis, asthma, and prominent peripheral blood with acute eosinophilic pneumonia with some vascu- eosinophilia (≥ 1500 cells/microL and/or > 10% eosino- lar changes. Te patient was subsequently treated with phils on diferential leukocyte count). Te etiology of induction course of intravenous methyl prednisone fol- EGPA remains unknown.