Case report Cardiac amyloidosis presenting with recurrent ischaemic strokes Suleiman Suleiman,1 John Joseph Coughlan ‍ ‍,2 David Moore1 1 Department of Cardiology, Tallaght University Hospital, Dublin, Ireland 2 Department of Cardiology, University Hospital Limerick, Limerick, Ireland Correspondence to Dr Suleiman Suleiman; ​suleimas@​tcd.i​ e Accepted 6 February 2020 © BMJ Publishing Group Limited 2020. No commercial re-­use. See rights and permissions. Published by BMJ. To cite: Suleiman S, Coughlan JJ, Moore D. BMJ Case Rep 2020;13:e231910. doi:10.1136/bcr-2019231910 SUMMARY A 72-­year-­old man presented to our service with sudden onset right-­sided weakness, aphasia and gaze palsy with diplopia. CT angiogram demonstrated an acute thrombotic occlusion of the distal basilar artery, a basilar infarct and the patient underwent successful thrombectomy. ECG and telemetry demonstrated slow atrial fibrillation (AF). His transthoracic echocardiogram (TTE) showed a reduced ejection fraction of 25% with global hypo-­kinesis, a dilated left ventricle (LV) and LV hypertrophy (LVH). Repeat TTE appeared suspicious for an infiltrative cardiomyopathy with LVH and a speckled appearance to the myocardium. Approximately 10 months later, he suffered another ischaemic stroke post-­ elective cardioversion for AF while on anticoagulation. Cardiac MRI demonstrated areas of delayed gadolinium enhancement consistent with amyloidosis. Fat pad biopsy was positive for amyloidosis. Our patient has made an excellent recovery from the ischaemic strokes and is being managed in our heart failure clinic. that amyloidosis can present initially as stroke and to demonstrate that these patients are at a higher risk of thromboembolic events. Case presentation A 72-­year-­old man with a background of hypertension, osteoarthritis, vertigo and carpal tunnel syndrome, presented with sudden onset right-­sided weakness, aphasia and gaze palsy with diplopia. National Institutes of Health Stroke Scale (NIHSS) was 16. CT of the brain and CT angiogram (CTA) demonstrated an acute thrombotic occlusion of the distal basilar artery. The patient was thrombolysed and immediately transferred to neurosurgery for thrombectomy, which was successful with an NIHSS of 3 day one post-­thrombectomy. MRI of the brain thrombolysis revealed multifocal cerebellar post-­ infarcts involving the right and left cerebellar hemispheres with no haemorrhagic conversion. A work up with ECG and telemetry demonstrated slow atrial fibrillation (AF) and a subsequent TTE highlighted a reduced ejection fraction (EF) of 25% Background with global hypokinesis, a dilated left ventricle (LV) Amyloidoses constitute a group of diseases and LV hypertrophy (LVH). He was initiated on produced by the deposition of amorphous fibrillar appropriate medication and discharged. Ten days proteins in the extracellular spaces of the body’s after his initial presentation, he re-­presented with organs.1 Cardiac amyloidosis (CA) may be a conse- worsening dyspnoea and orthopnoea with a N-­terquence of any of the systemic amyloidosis. The minal pro-­brain natriuretic peptide (NTproBNP) of most commonly encountered systemic amyloidoses 9605. He was treated with an acute decompensated that affect the heart are immunoglobulin light-­ heart failure with intravenous diuresis and medichain (AL) amyloidosis and transthyretin amyloi- cation optimisation with follow-­up with the heart dosis.2 The myocardium is affected in almost 50% efficiency service. A coronary angiogram demonof AL amyloidosis patients.3 4 Involvement limited strated non-­obstructive coronaries. to the heart is rare.5 CA exhibits itself with a wide A repeat TTE 3 months later illustrated focal variety of pathological processes, including conges- severe septal hypertrophy with septal thickness tive heart failure, arrhythmia, heart block and up to 2.9 cm. Myocardial texture was abnormal cardiomyopathy.6 especially in the septum and appeared bright Though not definitive, transthoracic echocardio- and ‘speckled’. LV systolic function was globally gram (TTE) and cardiac MRI (CMR) may contribute severely impaired with biplane left ventricular ejecto additional diagnostic testing and management of tion fraction (LVEF) 20%, suspicious of an infiltrathe pathology.7 Biopsy of the rectum or subcuta- tive cardiomyopathy (figure 1). neous fat with the use of congo red staining is posiHe attended the cardiology unit for an elective tive in 80% of patients with systemic amyloidosis.8 direct current cardioversion (DCCV) for AF 6 Endomyocardial biopsy, however, is the gold stan- months later (10 months from index presentation). dard for diagnosis of CA.9 ECG at the time exhibited AF with low voltage Thromboembolic events are a forgotten compli- QRS waves. cation of CA. The Mayo amyloid autopsy study in Following DCCV, the patient reverted into a sinus 2007 showed that intracardiac thrombosis were bradycardia with a first-­degree heart block. His PR found in over 50% of AL CA patients.10 Investiga- interval was significantly prolonged, with a PR tions for patients presenting with cerebrovascular interval of 300 ms prompting admission. His bisopaccidents may lead to the discovery of CA inciden- rolol was held to prevent any bradycardic episodes. tally.11 The aim of this case report is to highlight Day one post-­ DCCV, the patient suffered from Suleiman S, et al. BMJ Case Rep 2020;13:e231910. doi:10.1136/bcr-2019-231910 1 BMJ Case Rep: first published as 10.1136/bcr-2019-231910 on 23 February 2020. Downloaded from http://casereports.bmj.com/ on February 25, 2020 at Serials Division La Trobe University Library. Protected by copyright. Rare disease Figure 2 The patient’s cardiac MRI highlighting diffuse myocardial delayed enhancement with altered gadolinium kinetics consistent with cardiac amyloidosis. Figure 1 The patient’s repeat transthoracic echocardiogram 3 months later displaying abnormal myocardial texture. The tissue appears brights and echodense suggestive of an infiltrative cardiomyopathy. sudden onset dysarthria and confusion and urgently reviewed by the oncall medical doctor’s emergency response team. On examination, he was confused and dysarthric. There was left-­sided hemianopia and nystagmus present, along with dysdiadochokinesia affecting the left side more than the right. He was treated as a FAST positive stroke and he urgently underwent CT of the brain and CTA. No acute intracranial abnormality was demonstrated with an Alberta stroke programme early CT score (ASPECTS) score of 10. There was no vascular occlusion, thrombosis or significant stenosis of the anterior or posterior circulation. Recanalisation of the previously demonstrated basilar artery occlusion was noted, along with chronic bilateral cerebellar infarcts. The patient made a full recovery with no residual neurological deficit. He was continued on apixaban and underwent MRI of the brain. Grey– white matter differentiation appeared normal. No masses or acute haemorrhage was seen. Stable periventricular deep white matter ischaemia was observable. There was evidence of some old haemorrhage within the right cerebellar hemisphere, at site of the prior infarct, which was more prominent when compared with prior. Vascular flow voids appeared patent. There was evidence of an acute infarct involving the right occipital lobe. The infarct was patchy in nature with three separate areas of infarction. Largest area of infarction measured 1.6 cm in size. He was urgently reviewed by the stroke team and diagnosed with an acute right occipital lobe infarct. During the admission, the patient recalled that he may have missed one dose of his anticoagulation 48 hours prior to his elective admission. Investigations Repeat TTE exhibited a severely dilated LV with an LV end diastolic dimension (LVEDD) of 7.1 cm. There was focal severe septal hypertrophy with septal thickness up to 2.9 cm. Myocardial texture was abnormal especially in the septum and appeared bright and echodense. LV systolic function was severely globally impaired with biplane LVEF 20%. There was no significant improvement compared with previous studies. There was mild right ventricular hypertrophy and reduced right ventricular systolic function with a dilated left atrium. The valves were grossly normal with mild tricuspid regurgitation. Right ventricular systolic pressure was elevated at 30–40 mm Hg. The TTE concluded dilated cardiomyopathy (DCM) with severe 2 LV systolic impairment and marked focal asymmetrical septal hypertrophy. Cardiac strain TTE was undertaken to inspect if the classic bull’s eye pattern of amyloidosis was displayed. This was not visualised due to LV dilation. On contrast-­enhanced TTE, no intra-­cardiac thrombus was demonstrated but swirling of blood was noted in the LV. LV systolic strain was profoundly impaired with a globally reduced strain value of –2.86%. CMR highlighted severe biventricular cardiomyopathy. The left ventricular function was estimated at 14% with swirling of the blood pool in the LV with no definite LV apical thrombus detected. The LVEDD was increased at 7.1 cm and end diastolic volume was 251 mL; global dyskinesia was shown. The right ventricle was less impaired with an EF of 32%. The left septum was again demonstrated to be asymmetrically thickened compared with the lateral wall, anteroseptal segmental thickness measured at 18 mm while the inferalateral segmental thickness was only 6 mm. There was diffuse myocardial delayed enhancement and a suggestion of altered gadollinium kinetics consistent with diffuse myocardial infiltration (figure 2). Findings were non-­specific but reported as potentially consistent with CA. Serum protein electrophoresis was normal, free kappa light chains were elevated; however, the kappa/lambda ratio was normal. Kidney function was normal with no evidence of proteinuria. Fabry’s screen was negative. The patient underwent a fat pad biopsy that was positive for amyloidosis. The histology was sent to the UK for further amyloidosis subtyping. Bone marrow biopsy exhibited no infiltration. Differential diagnosis Our patient had initially presented with sudden onset right-­sided weakness, aphasia and gaze palsy with diplopia. Subsequently, he complained of worsening dyspnoea and orthopnoea with raised NTproBNP, an ECG demonstrating AF and a TTE showing reduced LV function. Our initial differential was ischaemic stroke secondary to AF and de novo non-­ischaemic DCM. His subsequent TTE results raised the possibility of an infiltrative process such as amyloidosis. Other possibilities considered were medication non-­ compliance, Fabry’s disease and multiple myeloma. Treatment Once stable, the patient’s heart efficiency medication were optimised. His bisoprolol was stopped and he was commenced on sacubitril/valsartan. He was continued on apixaban. Outcome and follow-up Our patient was diagnosed with CA. This was based on both TTE and CMR results and confirmed with fat pad biopsy. Suleiman S, et al. BMJ Case Rep 2020;13:e231910. doi:10.1136/bcr-2019-231910 BMJ Case Rep: first published as 10.1136/bcr-2019-231910 on 23 February 2020. Downloaded from http://casereports.bmj.com/ on February 25, 2020 at Serials Division La Trobe University Library. Protected by copyright. Rare disease He made a good recovery and was discharged home, and is continuing with his heart failure therapy. Due to the ventricular dysynchrony on TTE, a CRT defibrillator was implanted. He is currently awaiting a serum amyloid P component scintigraphy scan at the NHS National Amyloidosis Centre in London. Discussion Amyloidosis consists of a group of heterogeneous disorders that result from the extracellular deposition of misfolded low molecular weight proteins in abnormal fibrillar form distorting organ tissues.12 Systemic amyloidoses can affect the renal, nervous, gastrointestinal, hematological and cardiovascular systems.6 13 Cardiac involvement is the critical factor that dictates prognosis.14 Amyloid deposits in the heart produce increased ventricular wall thickness and stiffness, leading to the pathognomonic restrictive cardiomyopathy. AF’s prevalence is increased in patients with CA as the amyloid protein infiltrates the atria. Atrial infiltration causes structural dissociation and increases the likelihood of atrial thrombus formation and thromboembolism, even in normal sinus rhythm.10 15 Deposition of amyloid in and around the arteries can lead to acute coronary syndrome.16 The free light chains can also be directly toxic to the heart by promoting cell death.17 Electrophysiological dysfunction may arise from the misfolded protein deposits, with low voltage ECG, dysrhythmias and heart block.18 19 During our study’s timeline, our patient had both low voltage QRS complexes, AF and first-­degree atrioventricular block. Prognosis of CA is generally poor and varies depending on the specific type of amyloidosis involved.12 20 Based on the Mayo biomarker stage, that utilises serum cardiac troponin T, NTproBNP and serum free light chains levels, AL amyloidosis patient’s median survival rates ranges from 94.1 months for stage I to 5.8 months for stage IV. AL CA patients undergoing stem cell transplantation can have median overall survival surpassing 10 years if treatment is efficacious.21 In contrast, transthyretin amyloidosis (ATTR) is slowly progressive and has an improved prognosis compared with AL CA.14 22 ATTR amyloidosis may be present in the heart of the elderly regardless of what the light chains demonstrate on protein electrophoresis.23 The causative protein must be identified for appropriate therapy as treatments differ. Heart failure management and treatment of conduction disorders is common therapy. The mainstay of AL amyloidosis algorithms include conventional chemotherapy with agents such as cyclophosphamide, bortezomib and dexamethasone; monaclonal antibodies such as daratumumab and novel agents such as venetoclax, a BCL-2 inhibitor if the patients are found to have the translocation t(11;14).23 24 Based on minimal residual disease post-­treatment, AL amyloid patients may be eligible for autologous stem cell transplant.23 24 For ATTR amyloidosis, tafamadis, a TTR binding agent, has shown a 30% reduction in all-­ cause mortality compared with placebo and a slower decline in 6 min walk test and in quality of life.25 Other treatments include diuretics and RNA-­targeted therapies such as patisiran. Heart transplantation in patients with ATTR is rare.25 In the literature, ishcaemic strokes in association with CA have been occasionally noted but not described well. A retrospective study in 2007 reviewed the pathophysiologic relationship between primary systemic amyloidosis and ischaemic cerebrovascular insults. The study included 40 patients with biopsy proven amyloidosis and ischaemic stroke. Thirteen of the patients’ (32.5%) primary presentation was of ischaemic stroke prior to being diagnosed with systemic amyloidosis. Strokes presented 9.6 months prior to diagnosis with primary amyloidosis. Seventy per cent of patients in the study had cardioembolic infarctions. Suleiman S, et al. BMJ Case Rep 2020;13:e231910. doi:10.1136/bcr-2019-231910 Stroke as an initial presentation was a marker for worse prognosis, with median survival of 6.9 months following confirmation of systemic amyloidosis on biopsy. Interestingly, 37% of patients had recurrent ischaemic strokes.26 In this study, this was our patient’s second ischaemic stroke. Diagnosis of CA continues to be difficult. Increased wall thickness and worsening diastolic dysfunction are two crucial characteristics on TTE of CA patients.1 The protein deposition that causes increased LV thickness and subsequent reduced compliance leads to the diastolic failure. These features are not specific to CA and are seen in hypertrophic cardiomyopathy (HCM) and hypertensive heart disease.27 However, strain imaging displays two vital differences. First, a much greater reduction in global longitudinal strain compared with HCM and hypertensive heart disease and second, a unique regional LV longitudinal strain pattern, highlighting vastly reduced basal and mid-­systolic strain with apical sparing, which was shown with our patient’s globally reduced strain value.28 The most characteristic structural findings on echocardiogram are septal and posterior wall thickening at least >12 mm, and with mean thickness of 16 mm at the time of diagnosis congruent with our patient's TTE, which demonstrated septal thickening of 29 mm.19 29 Classically, CA is a restrictive cardiomyopathy; however, ATTR amyloidosis can present with a reduced EF and dilation as shown with our patient.30 CA increases the extracellular volume of the myocardium and in turn leads to the accumulation of gadolinium contrast.31 Delayed gadolinium enhancement on CMR imaging has shown to be effective in distinguishing CA. Varying non-­infarct delayed enhancement patterns, from subendocardial to transmural, have been illustrated.32 CMR is extremely beneficial as an investigation to screen for CA with high specificity and positive predictive values of 80% and 81%, respectively, for diagnosis of AL CA.33 The gadolinium contrast also displays myocardial and blood pool diskinetics deriving a reduced difference between blood and myocardium T1, a key CMR criteria.34 Furthermore, the extent of the delayed enhancement by CMR is in its own a useful prognostic tool as it has been shown to be an independent predictor of mortality in AL CA.35 A high frequency of intra-­cardiac thrombosis are present in CA. A study at the Mayo clinic examining autopsy results of 116 CA (55 of AL and 61 of other subtypes) cases discovered intra-­cardiac thrombus in 33%. Twenty-­three had 1 thrombus, whereas 15 had multiple; for a total of 63 thrombi. The AL subgroup had notably increased intracardiac thrombus found at autopsy (51% vs 16%) and additional fatal embolic events (26% vs 8%) when compared with the other amyloidosis subgroups.10 The authors identified that if AL amyloidosis subtype and AF were simultaneously present, there was a substantially higher risk for thromboembolism.10 In this study, although our patient showed no evidence of intra-­cardiac thrombus on TTE or CMR, he did have both CA and AF increasing his risk for thromboembolism as evident by the recurrent cerebral ischaemic events. Though TTE is a key non-­invasive tool for cardiac imaging, transesophageal echocardiogram is the superior modality for the investigation of many cardiac sources of emboli,36 37 with CMR being more sensitive for left ventricular thrombus detection.38 The mechanisms of thromboembolism in CA are multifactorial. Mural thrombosis may develop due to blood stasis and intracavitary turbulence as seen in our patient’s strain TTE and CMR.39 Deposition of amyloid to endocardial tissue has shown to cause fibrous thickening and impaired wall kinetics contributing to endocardial thrombi.40 Dysrhythmias as stated previously is another well-­documented cause of cerebral ischaemia. The prevalence of AF in CA is approximately 20%. It is theorised 3 BMJ Case Rep: first published as 10.1136/bcr-2019-231910 on 23 February 2020. Downloaded from http://casereports.bmj.com/ on February 25, 2020 at Serials Division La Trobe University Library. Protected by copyright. Rare disease that this is due to left atrial failure secondary to dilation and raised wall stress.40 Using 24 hours hotter monitoring, the incidence of ventricular arrhythmias in amyloidosis was recently evaluated. The study highlighted a significant incidence of arrhythmias, including ventricular ectopies in 72% and complex ventricular arrhythmias in 57% of patients.41 Amyloidosis also creates a hyper-­coagulable state either from damage of the renal system through proteinuria, infiltration of the spleen causing thrombocytosis, the direct effect on the coagulation cascade or increased blood viscosity or procoagulant activity associated to the circulating monoclonal component all also increase the risk for thromboembolism.42 Patient with amyloidosis can also present with carpal tunnel syndrome which may be bilateral. A study of 98 patients undergoing carpal tunnel release surgery, underwent simultaneous Congo red staining of tenosynovial tissue. This subsequently detected amyloid deposits in 10 of the patients (7 ATTR, 2 AL, 1 untyped). Concomitant cardiac evaluation identified two patients with involvement of the myocardium (1 ATTR, 1 AL), modifying therapy.43 allowing for implementation of disease-­ This has led to the possibility of screening patients with carpal tunnel syndrome amyloidosis. Interestingly, our patient also suffered from carpal tunnel syndrome. 1 Banypersad SM, Moon JC, Whelan C, et al. Updates in cardiac amyloidosis: a review. J Am Heart Assoc 2012;1:e000364. 2 Yusuf SW, Solhpour A, Banchs J, et al. Cardiac amyloidosis. Expert Rev Cardiovasc Ther 2014;12:265–77. 3 Dubrey SW, Cha K, Anderson J, et al. The clinical features of immunoglobulin light-­ chain (AL) amyloidosis with heart involvement. QJM 1998;91:141–57. 4 Dubrey SW, Cha K, Simms RW, et al. Electrocardiography and Doppler echocardiography in secondary (AA) amyloidosis. Am J Cardiol 1996;77:313–5. 5 Khalid U, Awar O, Verstovsek G, et al. Case report: isolated cardiac amyloidosis: an enigma unravelled. Methodist Debakey Cardiovasc J 2015;11:53–8. 6 Siddiqi OK, Ruberg FL. Cardiac amyloidosis: an update on pathophysiology, diagnosis, and treatment. Trends Cardiovasc Med 2018;28:10–21. 7 Falk RH, Quarta CC, Dorbala S. How to image cardiac amyloidosis. Circ Cardiovasc Imaging 2014;7:552–62. 8 Quarta CC, Gonzalez-­Lopez E, Gilbertson JA, et al. Diagnostic sensitivity of abdominal fat aspiration in cardiac amyloidosis. Eur Heart J 2017;38:1905–8. 9 Pellikka PA, Holmes DR, Edwards WD, et al. Endomyocardial biopsy in 30 patients with primary amyloidosis and suspected cardiac involvement. Arch Intern Med 1988;148:662–6. 10 Feng D, Edwards WD, Oh JK, et al. Intracardiac thrombosis and embolism in patients with cardiac amyloidosis. Circulation 2007;116:2420–6. 11 Zhang X-­D, Liu Y-­X, Yan X-­W, et al. Cerebral embolism secondary to cardiac amyloidosis: a case report and literature review. Exp Ther Med 2017;14:6077–83. 12 Quarta CC, Riva L, Perlini S. 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Amyloid 2011;18:160–4. 17 Guan J, Mishra S, Qiu Y, et al. Lysosomal dysfunction and impaired autophagy underlie the pathogenesis of amyloidogenic light chain-­mediated cardiotoxicity. EMBO Mol Med 2014;6:1493–507. 18 Ritts AJ, Cornell RF, Swiger K, et al. Current concepts of cardiac amyloidosis: diagnosis, clinical management, and the need for collaboration. Heart Fail Clin 2017;13:409–16. 19 Murtagh B, Hammill SC, Gertz MA, et al. Electrocardiographic findings in primary systemic amyloidosis and biopsy-­proven cardiac involvement. Am J Cardiol 2005;95:535–7. 20 Wechalekar AD, Gillmore JD, Hawkins PN. Systemic amyloidosis. Lancet 2016;387:2641–54. 21 Madan S, Kumar SK, Dispenzieri A, et al. High-­Dose melphalan and peripheral blood stem cell transplantation for light-­chain amyloidosis with cardiac involvement. Blood 2012;119:1117–22. 22 Grogan M, Scott CG, Kyle RA, et al. 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J Am Coll Cardiol 2007;50:2101–10. 28 Phelan D, Collier P, Thavendiranathan P, et al. Relative apical sparing of longitudinal strain using two-­dimensional speckle-­tracking echocardiography is both sensitive and specific for the diagnosis of cardiac amyloidosis. Heart 2012;98:1442–8. 29 Rahman JE, Helou EF, Gelzer-­Bell R, et al. Noninvasive diagnosis of biopsy-­proven cardiac amyloidosis. J Am Coll Cardiol 2004;43:410–5. 30 Feng KY, Loungani RS, Rao VN, et al. Best practices for prognostic evaluation of a patient with transthyretin amyloid cardiomyopathy. JACC CardioOncol 2019;1:273–9. 31 Ruberg FL, Appelbaum E, Davidoff R, et al. Diagnostic and prognostic utility of cardiovascular magnetic resonance imaging in light-­chain cardiac amyloidosis. Am J Cardiol 2009;103:544–9. 32 Syed IS, Glockner JF, Feng D, et al. Role of cardiac magnetic resonance imaging in the detection of cardiac amyloidosis. JACC Cardiovasc Imaging 2010;3:155–64. 33 Bhatti S, Watts E, Syed F, et al. Clinical and prognostic utility of cardiovascular magnetic resonance imaging in myeloma patients with suspected cardiac amyloidosis. Eur Heart J Cardiovasc Imaging 2016;17:970–7. 34 Maceira AM, Joshi J, Prasad SK, et al. Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation 2005;111:186–93. 4 Suleiman S, et al. BMJ Case Rep 2020;13:e231910. doi:10.1136/bcr-2019-231910 Learning points ►► Stroke is a potential complication of systemic amyloidosis, and can also be the initial presentation. Stroke as an initial presentation was a marker for worse prognosis; therefore, early diagnosis is imperative. ►► The diagnosis of cardiac amyloidosis (CA) should be considered in patients with cardiac dysfunction, left ventricular hypertrophy and a speckled appearance of myocardium on transthoracic echocardiogram (TTE). ►► There is an intrinsic tendency towards thromboembolism with amyloidosis. CA patients may have recurrent ischaemic strokes, especially if AF is simultaneously present. It is imperative that strict adherence and compliance to anticoagulation regimes are met, as this may reduce morbidity and mortality. ►► Investigations in these groups of patients suspicious for CA include TTE, cardiac MRI, subcutaneous fat biopsies and if warranted endomyocardial biopsy to aid in early diagnosis and treatment of CA. Twitter John Joseph Coughlan @jjcoughl Acknowledgements I would like to acknowledge the cardiology, haematology and cellular pathology departments at Tallaght University Hospital for their help. Contributors SS: main author; abstract, introduction, literature review and discussion. JJC: coauthor; review of images and review of case report. DM: supervisor. Competing interests None declared. Patient consent for publication Obtained. Provenance and peer review Not commissioned; externally peer reviewed. ORCID iD John Joseph Coughlan http://​orcid.​org/​0000-​0001-​6086-​3279 References BMJ Case Rep: first published as 10.1136/bcr-2019-231910 on 23 February 2020. Downloaded from http://casereports.bmj.com/ on February 25, 2020 at Serials Division La Trobe University Library. Protected by copyright. Rare disease 35 Boynton SJ, Geske JB, Dispenzieri A, et al. LGE Provides Incremental Prognostic Information Over Serum Biomarkers in AL Cardiac Amyloidosis. JACC Cardiovasc Imaging 2016;9:680–6. 36 Pearson AC, Labovitz AJ, Tatineni S, et al. Superiority of transesophageal echocardiography in detecting cardiac source of embolism in patients with cerebral ischemia of uncertain etiology. J Am Coll Cardiol 1991;17:66–72. 37 DeRook FA, Comess KA, Albers GW, et al. Transesophageal echocardiography in the evaluation of stroke. Ann Intern Med 1992;117:922–32. 38 Weinsaft JW, Kim HW, Crowley AL, et al. Lv thrombus detection by routine echocardiography: insights into performance characteristics using delayed enhancement CMR. JACC Cardiovasc Imaging 2011;4:702–12. 39 Bøtker HE, Rasmussen OB. Recurrent cerebral embolism in cardiac amyloidosis. Int J Cardiol 1986;13:81–3. 40 Rice GP, Ebers GC, Newland F, et al. Recurrent cerebral embolism in cardiac amyloidosis. Neurology 1981;31:904. 41 Palladini G, Malamani G, Co F, et al. Holter monitoring in AL amyloidosis: prognostic implications. Pacing Clin Electrophysiol 2001;24:1228–33. 42 Hausfater P, Costedoat-­Chalumeau N, Amoura Z, et al. Al cardiac amyloidosis and arterial thromboembolic events. Scand J Rheumatol 2005;34:315–9. 43 Sperry BW, Reyes BA, Ikram A, et al. Tenosynovial and Cardiac Amyloidosis in Patients Undergoing Carpal Tunnel Release. J Am Coll Cardiol 2018;72:2040–50. Copyright 2020 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit https://www.bmj.com/company/products-services/rights-and-licensing/permissions/ BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ►► Submit as many cases as you like ►► Enjoy fast sympathetic peer review and rapid publication of accepted articles ►► Access all the published articles ►► Re-use any of the published material for personal use and teaching without further permission Customer Service If you have any further queries about your subscription, please contact our customer services team on +44 (0) 207111 1105 or via email at support@bmj.com. Visit casereports.bmj.com for more articles like this and to become a Fellow Suleiman S, et al. BMJ Case Rep 2020;13:e231910. doi:10.1136/bcr-2019-231910 5 BMJ Case Rep: first published as 10.1136/bcr-2019-231910 on 23 February 2020. Downloaded from http://casereports.bmj.com/ on February 25, 2020 at Serials Division La Trobe University Library. Protected by copyright. Rare disease