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high IMR and low CFR are associated with the worst prognosis&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">5</span></a> Our case will present the long-term prognosis of a patient with such characteristics&#44; with the additional finding of a systemic disease with cardiac involvement&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Case description</span><p id="par0010" class="elsevierStylePara elsevierViewall">A 64-year-old male presented with exertional retrosternal pain&#46; He had a past medical history of hypertension&#44; dyslipidemia&#44; obesity&#44; bilateral carpal tunnel syndrome and underwent bilateral surgical release and cervical laminectomy&#46; The resting electrocardiogram revealed an incomplete left bundle branch block&#44; while the resting echocardiogram was completely unremarkable&#46; He underwent a stress echocardiogram with dobutamine&#59; at peak stress&#44; the left ventricle developed regional wall motion abnormalities including hypokinesis of the inferior wall&#44; associated with angina and soon followed by ventricular tachycardia&#44; reversed with propranolol&#46; The patient was hospitalized and underwent a coronary angiography which revealed slow flow&#44; dominant right coronary artery with non-obstructive atherosclerosis and a left anterior descending artery &#40;LAD&#41; with intermediate lesions in mid and distal segments &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figures 1 and 2</a>&#41;&#46; A functional evaluation revealed a non-significant fractional flow reserve of 0&#46;86 in the LAD&#44; but a pathological CFR of 1&#46;8 and an IMR of 61&#46; Given the patient&#39;s symptoms&#44; risk factors and diagnostic tests&#44; he was considered to have microvascular angina and was treated with antiplatelet therapy&#44; a statin&#44; a calcium channel blocker and a transdermal nitrate&#44; with symptomatic relief&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Three years later he developed complete atrioventricular block and presented in the emergency department with sudden hemodynamic collapse&#46; A dual chamber pacemaker was implanted&#44; and he was discharged asymptomatic&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Shortly after&#44; the patient developed progressive symmetrical tetraparesis&#44; associated with marked muscle atrophy&#44; hand numbness&#44; orthostatic hypotension and dysphagia&#46; The neurology workup led to the diagnosis of familial amyloidotic polyneuropathy &#40;FAP&#41;&#44; and the <span class="elsevierStyleItalic">Val30Met</span> mutation in the transthyretin &#40;TTR&#41; gene was detected&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The following year&#44; he developed NYHA class II to III congestive heart failure &#40;HF&#41;&#44; requiring uptitration of diuretics&#46; The repeat echocardiogram showed moderate concentric left ventricular hypertrophy&#44; with preserved ejection fraction but an abnormal global longitudinal strain and elevated filling pressures &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#41;&#46; He repeated the coronary angiography which excluded CAD progression&#46; He underwent a 99mTc-DPD scintigraphy that revealed significant myocardial tracer uptake&#44; diagnosing TTR amyloid infiltration &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>&#41;&#46; He was stabilized with medical therapy and kept under close follow-up&#44; requiring antianginal therapy uptitration for exertional microvascular angina&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Discussion</span><p id="par0030" class="elsevierStylePara elsevierViewall">Transthyretin FAP with the <span class="elsevierStyleItalic">Val30Met</span> mutation in the transthyretin gene affects primarily the peripheral nervous system&#46; Still&#44; it can be associated with clinically significant myocardial infiltration<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">6</span></a> in up to 15&#37; of the patients with late onset disease&#46;<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">7</span></a> Cardiac presentation includes conduction block&#44; arrhythmia and cardiac hypertrophy&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">In our clinical case&#44; the patient presented with microvascular angina&#44; a rare symptom of cardiac infiltration&#46; The only findings from his medical history that were in keeping with the suspicion of an infiltrative disease were the bilateral carpal tunnel syndrome and the atrioventricular block&#46;<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">8</span></a> The echocardiographic findings suggestive of myocardial infiltration were present only when the patient developed HF&#44; after 4 years of medical follow-up&#46; At that time&#44; the 99mTc-DPD scintigraphy was consistent with TTR infiltration&#46; This technique has been shown to be specific for cardiac involvement in TTR amyloidosis&#44; with mild or no tracer uptake in patients without cardiac involvement<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">9</span></a> or in patients with other types of systemic amyloidosis&#46;<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">10</span></a> As a consequence&#44; 99mTc-DPD scintigraphy is recommended as a diagnostic test for TTR cardiac amyloidosis in the European guidelines&#46;<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">11</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">An alternative&#44; well validated imaging technique for the diagnosis of cardiac amyloidosis would be cardiovascular magnetic resonance&#44; which often reveals a pattern of global&#44; sub-endocardial or segmental late gadolinium enhancement&#46;<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">12</span></a> However&#44; this exam was contraindicated in this case&#44; as the patient had a conventional pacemaker implanted before developing HF&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Therapeutic options are limited for patients with FAP&#46; Liver transplantation is a treatment option that prolongs life&#44; particularly in patients with the <span class="elsevierStyleItalic">Val30Met</span> mutation&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">13</span></a> Tafamidis is a TTR protein stabilizer approved for the treatment of this condition&#46; In a recently published trial&#44; tafamidis reduced mortality and cardiovascular hospitalization when compared with placebo&#44; in patients with wild type TTR amyloidosis and FAP&#46;<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">14</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">An important learning point of this case is that in patients with exertional angina and microvascular disease&#44; a specific etiology should be sought&#44; as this may occur in the context of myocardial disease&#44; due to vascular infiltration or adverse remodeling&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">15</span></a></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Conflicts of interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Clinical case</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A 64-year-old male&#44; with cardiovascular risk factors and previous history of bilateral carpal tunnel syndrome&#44; presented with exertional retrosternal pain&#46; The resting echocardiogram was unremarkable&#46; A stress echocardiogram with dobutamine revealed hypokinesis of the inferior wall&#44; associated with angina&#44; followed by ventricular tachycardia&#46; The coronary angiography revealed slow flow&#44; a dominant right coronary artery with non-obstructive atherosclerosis and a left anterior descending artery with intermediate lesions in mid and distal segments&#46; The invasive functional evaluation&#44; including fractional flow reserve&#44; thermodilution coronary flow reserve and index of microvascular resistance&#44; led to the diagnosis of microvascular angina&#44; treated with calcium channel blockers and transdermal nitrate&#44; giving symptom relief&#46;</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Evolution</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Three years later he developed complete atrioventricular block and a dual chamber pacemaker was implanted&#46; Shortly after&#44; the patient developed progressive symmetrical tetraparesis&#44; associated with marked muscle atrophy&#44; hand numbness&#44; orthostatic hypotension and dysphagia&#46; The neurology workup led to the diagnosis of familial amyloidotic polyneuropathy&#44; with the Val30Met mutation in the transthyretin gene&#46; The following year he developed congestive heart failure&#46; The echocardiogram showed moderate concentric left ventricular hypertrophy with preserved ejection fraction&#46; A 99mTc-DPD Scintigraphy showed significant myocardial tracer uptake&#44; leading to a diagnosis of TTR amyloid infiltration&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Discussion</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Patients with exertional angina and microvascular disease should be kept under close surveillance&#44; as they may have systemic disease with cardiac involvement&#46; Carpal tunnel syndrome&#44; in the context of undiagnosed cardiac disease&#44; should trigger suspicion of cardiac amyloidosis&#46;</p></span>"
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        "resumen" => "<span id="abst0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Caso cl&#237;nico</span><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Um homem de 64 anos&#44; com fatores de risco cardiovascular e antecedentes de s&#237;ndrome do canal c&#225;rpico bilateral&#44; apresentou-se com queixas de angina de esfor&#231;o&#46; O ecocardiograma em repouso era normal&#46; O ecocardiograma de sobrecarga com dobutamina revelou hipocin&#233;sia da parede inferior&#44; acompanhada de angina e taquicardia ventricular autolimitada&#46; A coronariografia revelou art&#233;rias com fluxo lento&#44; coron&#225;ria direita dominante com aterosclerose n&#227;o obstrutiva e descendente anterior com les&#245;es interm&#233;dias nos segmentos m&#233;dio e distal&#46; A avalia&#231;&#227;o invasiva do significado funcional das les&#245;es incluiu <span class="elsevierStyleItalic">fractional flow reserve</span>&#44; <span class="elsevierStyleItalic">coronary flow reserve</span> e <span class="elsevierStyleItalic">index of microvascular resistance</span> e resultou no diagn&#243;stico de angina microvascular&#44; tratada com bloqueador dos canais de c&#225;lcio e nitratos&#44; com al&#237;vio sintom&#225;tico&#46;</p></span> <span id="abst0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Evolu&#231;&#227;o</span><p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Tr&#234;s anos depois&#44; o doente desenvolveu bloqueio auriculoventricular completo&#44; tendo sido implantado um <span class="elsevierStyleItalic">pacemaker</span> de dupla c&#226;mara&#46; Concomitantemente&#44; evoluiu com tetrapar&#233;sia sim&#233;trica&#44; atrofia muscular marcada&#44; dorm&#234;ncia das m&#227;os&#44; hipotens&#227;o ortost&#225;tica e disfagia&#46; A investiga&#231;&#227;o do quadro neurol&#243;gico resultou no diagn&#243;stico de Polineuropatia Amiloid&#243;tica Familiar&#44; com muta&#231;&#227;o Val30Met no gene da transtirretina&#46; No ano seguinte&#44; desenvolveu insufici&#234;ncia card&#237;aca congestiva&#46; O ecocardiograma apresentava agora hipertrofia ventricular esquerda conc&#234;ntrica&#44; com fun&#231;&#227;o sist&#243;lica preservada&#46; Realizou uma cintigrafia com 99mTc-DPD que mostrou intensidade de sinal compat&#237;vel com dep&#243;sito intramioc&#225;rdico de subst&#226;ncia amiloide&#46;</p></span> <span id="abst0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Discuss&#227;o</span><p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">Os doentes com angina de esfor&#231;o e doen&#231;a microvascular devem manter seguimento cl&#237;nico regular&#44; uma vez que poder&#227;o ter uma doen&#231;a sist&#233;mica com envolvimento card&#237;aco&#46; A s&#237;ndrome do canal c&#225;rpico&#44; no contexto de doen&#231;a card&#237;aca n&#227;o esclarecida&#44; deve levantar a suspeita de amiloidose card&#237;aca&#46;</p></span>"
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Case report
Exertional chest pain is sometimes more than just coronary atherosclerosis
Angina de esforço: por vezes não é apenas aterosclerose coronária
G.J. Morgado
Autor para correspondência
gjmorgado@outlook.com

Corresponding author.
, A.C. Gomes, I.R. Cruz, S. Carmona, P. Fazendas, I. João, A.I. Santos, L.R. Lopes, H. Pereira
Hospital Garcia de Orta, Almada, Portugal
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Coronary artery disease &#40;CAD&#41; is a common cause of exertional retrosternal pain in middle aged men&#46; In patients with non-obstructive CAD&#44; other diagnoses&#44; including microvascular angina &#40;and microvascular disease in the setting of other specific cardiac diseases&#41;&#44; should be considered&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">1</span></a> Microcirculatory dysfunction can be diagnosed invasively with thermodilution coronary flow reserve &#40;CFR&#41; and the index of microvascular resistance &#40;IMR&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">2</span></a> CFR interrogates coronary circulation as a whole&#44; identifying whether global flow supply is normal&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">3</span></a> IMR is considered a surrogate for microvascular damage and has been shown to have predictive value for myocardial recovery in patients with acute myocardial infarction&#46;<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">4</span></a> When IMR and CFR evaluations are combined in patients with intermediate coronary stenosis&#44; high IMR and low CFR are associated with the worst prognosis&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">5</span></a> Our case will present the long-term prognosis of a patient with such characteristics&#44; with the additional finding of a systemic disease with cardiac involvement&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Case description</span><p id="par0010" class="elsevierStylePara elsevierViewall">A 64-year-old male presented with exertional retrosternal pain&#46; He had a past medical history of hypertension&#44; dyslipidemia&#44; obesity&#44; bilateral carpal tunnel syndrome and underwent bilateral surgical release and cervical laminectomy&#46; The resting electrocardiogram revealed an incomplete left bundle branch block&#44; while the resting echocardiogram was completely unremarkable&#46; He underwent a stress echocardiogram with dobutamine&#59; at peak stress&#44; the left ventricle developed regional wall motion abnormalities including hypokinesis of the inferior wall&#44; associated with angina and soon followed by ventricular tachycardia&#44; reversed with propranolol&#46; The patient was hospitalized and underwent a coronary angiography which revealed slow flow&#44; dominant right coronary artery with non-obstructive atherosclerosis and a left anterior descending artery &#40;LAD&#41; with intermediate lesions in mid and distal segments &#40;<a class="elsevierStyleCrossRefs" href="#fig0005">Figures 1 and 2</a>&#41;&#46; A functional evaluation revealed a non-significant fractional flow reserve of 0&#46;86 in the LAD&#44; but a pathological CFR of 1&#46;8 and an IMR of 61&#46; Given the patient&#39;s symptoms&#44; risk factors and diagnostic tests&#44; he was considered to have microvascular angina and was treated with antiplatelet therapy&#44; a statin&#44; a calcium channel blocker and a transdermal nitrate&#44; with symptomatic relief&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Three years later he developed complete atrioventricular block and presented in the emergency department with sudden hemodynamic collapse&#46; A dual chamber pacemaker was implanted&#44; and he was discharged asymptomatic&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Shortly after&#44; the patient developed progressive symmetrical tetraparesis&#44; associated with marked muscle atrophy&#44; hand numbness&#44; orthostatic hypotension and dysphagia&#46; The neurology workup led to the diagnosis of familial amyloidotic polyneuropathy &#40;FAP&#41;&#44; and the <span class="elsevierStyleItalic">Val30Met</span> mutation in the transthyretin &#40;TTR&#41; gene was detected&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">The following year&#44; he developed NYHA class II to III congestive heart failure &#40;HF&#41;&#44; requiring uptitration of diuretics&#46; The repeat echocardiogram showed moderate concentric left ventricular hypertrophy&#44; with preserved ejection fraction but an abnormal global longitudinal strain and elevated filling pressures &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#41;&#46; He repeated the coronary angiography which excluded CAD progression&#46; He underwent a 99mTc-DPD scintigraphy that revealed significant myocardial tracer uptake&#44; diagnosing TTR amyloid infiltration &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Figure 4</a>&#41;&#46; He was stabilized with medical therapy and kept under close follow-up&#44; requiring antianginal therapy uptitration for exertional microvascular angina&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Discussion</span><p id="par0030" class="elsevierStylePara elsevierViewall">Transthyretin FAP with the <span class="elsevierStyleItalic">Val30Met</span> mutation in the transthyretin gene affects primarily the peripheral nervous system&#46; Still&#44; it can be associated with clinically significant myocardial infiltration<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">6</span></a> in up to 15&#37; of the patients with late onset disease&#46;<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">7</span></a> Cardiac presentation includes conduction block&#44; arrhythmia and cardiac hypertrophy&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">In our clinical case&#44; the patient presented with microvascular angina&#44; a rare symptom of cardiac infiltration&#46; The only findings from his medical history that were in keeping with the suspicion of an infiltrative disease were the bilateral carpal tunnel syndrome and the atrioventricular block&#46;<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">8</span></a> The echocardiographic findings suggestive of myocardial infiltration were present only when the patient developed HF&#44; after 4 years of medical follow-up&#46; At that time&#44; the 99mTc-DPD scintigraphy was consistent with TTR infiltration&#46; This technique has been shown to be specific for cardiac involvement in TTR amyloidosis&#44; with mild or no tracer uptake in patients without cardiac involvement<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">9</span></a> or in patients with other types of systemic amyloidosis&#46;<a class="elsevierStyleCrossRef" href="#bib0125"><span class="elsevierStyleSup">10</span></a> As a consequence&#44; 99mTc-DPD scintigraphy is recommended as a diagnostic test for TTR cardiac amyloidosis in the European guidelines&#46;<a class="elsevierStyleCrossRef" href="#bib0130"><span class="elsevierStyleSup">11</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">An alternative&#44; well validated imaging technique for the diagnosis of cardiac amyloidosis would be cardiovascular magnetic resonance&#44; which often reveals a pattern of global&#44; sub-endocardial or segmental late gadolinium enhancement&#46;<a class="elsevierStyleCrossRef" href="#bib0135"><span class="elsevierStyleSup">12</span></a> However&#44; this exam was contraindicated in this case&#44; as the patient had a conventional pacemaker implanted before developing HF&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Therapeutic options are limited for patients with FAP&#46; Liver transplantation is a treatment option that prolongs life&#44; particularly in patients with the <span class="elsevierStyleItalic">Val30Met</span> mutation&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">13</span></a> Tafamidis is a TTR protein stabilizer approved for the treatment of this condition&#46; In a recently published trial&#44; tafamidis reduced mortality and cardiovascular hospitalization when compared with placebo&#44; in patients with wild type TTR amyloidosis and FAP&#46;<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">14</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">An important learning point of this case is that in patients with exertional angina and microvascular disease&#44; a specific etiology should be sought&#44; as this may occur in the context of myocardial disease&#44; due to vascular infiltration or adverse remodeling&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">15</span></a></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Conflicts of interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare&#46;</p></span></span>"
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        "resumen" => "<span id="abst0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Clinical case</span><p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A 64-year-old male&#44; with cardiovascular risk factors and previous history of bilateral carpal tunnel syndrome&#44; presented with exertional retrosternal pain&#46; The resting echocardiogram was unremarkable&#46; A stress echocardiogram with dobutamine revealed hypokinesis of the inferior wall&#44; associated with angina&#44; followed by ventricular tachycardia&#46; The coronary angiography revealed slow flow&#44; a dominant right coronary artery with non-obstructive atherosclerosis and a left anterior descending artery with intermediate lesions in mid and distal segments&#46; The invasive functional evaluation&#44; including fractional flow reserve&#44; thermodilution coronary flow reserve and index of microvascular resistance&#44; led to the diagnosis of microvascular angina&#44; treated with calcium channel blockers and transdermal nitrate&#44; giving symptom relief&#46;</p></span> <span id="abst0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Evolution</span><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Three years later he developed complete atrioventricular block and a dual chamber pacemaker was implanted&#46; Shortly after&#44; the patient developed progressive symmetrical tetraparesis&#44; associated with marked muscle atrophy&#44; hand numbness&#44; orthostatic hypotension and dysphagia&#46; The neurology workup led to the diagnosis of familial amyloidotic polyneuropathy&#44; with the Val30Met mutation in the transthyretin gene&#46; The following year he developed congestive heart failure&#46; The echocardiogram showed moderate concentric left ventricular hypertrophy with preserved ejection fraction&#46; A 99mTc-DPD Scintigraphy showed significant myocardial tracer uptake&#44; leading to a diagnosis of TTR amyloid infiltration&#46;</p></span> <span id="abst0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Discussion</span><p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Patients with exertional angina and microvascular disease should be kept under close surveillance&#44; as they may have systemic disease with cardiac involvement&#46; Carpal tunnel syndrome&#44; in the context of undiagnosed cardiac disease&#44; should trigger suspicion of cardiac amyloidosis&#46;</p></span>"
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ISSN: 08702551
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