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• W3132299792 abstract "CARDIAC AMYLOIDOSIS (CA) is a form of infiltrative cardiomyopathy caused by the extracellular deposition of misfolded fibrillar proteins (amyloid) within the myocardium.1Martinez-Naharro A Hawkins PN Fontana M. Cardiac amyloidosis.Clin Med (Lond). 2018; 18: s30-s35Crossref PubMed Scopus (34) Google Scholar, 2Falk RH Alexander KM Liao R et al.AL (light-chain) cardiac amyloidosis: A review of diagnosis and therapy.J Am Coll Cardiol. 2016; 68: 1323-1341Crossref PubMed Scopus (212) Google Scholar, 3Yamamoto H Yokochi T. Transthyretin cardiac amyloidosis: An update on diagnosis and treatment.ESC Heart Fail. 2019; 6: 1128-1139Crossref PubMed Scopus (24) Google Scholar CA is one of the most rapidly progressive forms of heart disease and, in its most severe form, carries a median survival from diagnosis of fewer than six months if untreated.4Merlini G Palladini G. Light chain amyloidosis: The heart of the problem.Haematologica. 2013; 98: 1492-1495Crossref PubMed Scopus (51) Google Scholar However, during the past decade, major advances in diagnosis and treatment have made this once dire disease a treatable and potentially curable condition.2Falk RH Alexander KM Liao R et al.AL (light-chain) cardiac amyloidosis: A review of diagnosis and therapy.J Am Coll Cardiol. 2016; 68: 1323-1341Crossref PubMed Scopus (212) Google Scholar Because there are numerous emerging therapeutic options for this disease, noninvasive imaging strategies to obtain early diagnosis and appropriate monitoring of response to treatment have become increasingly important. Furthermore, CA now also is understood to be a more common condition than previously estimated.2Falk RH Alexander KM Liao R et al.AL (light-chain) cardiac amyloidosis: A review of diagnosis and therapy.J Am Coll Cardiol. 2016; 68: 1323-1341Crossref PubMed Scopus (212) Google Scholar Accordingly, as this disease becomes more frequently recognized and better understood, it becomes essential for the cardiothoracic anesthesiologist, tasked with both perioperative management and imaging of the cardiac patient, to be familiar with CA in order provide optimal care of these high-risk patients. In this editorial, the authors highlight the recent state-of-the art review by Dorbala et al., which provided a practical approach to diagnostic imaging in patients with known or suspected CA.5Dorbala S Cuddy S Falk RH. How to image cardiac amyloidosis: A practical approach.JACC Cardiovasc Imaging. 2020; 13: 1368-1383Crossref PubMed Scopus (42) Google Scholar In addition, this editorial discusses treatment options and clinical implications of CA relevant to the perioperative period. Amyloidosis represents a group of systemic disorders in which one or more organs are infiltrated by proteinaceous amyloid deposits.2Falk RH Alexander KM Liao R et al.AL (light-chain) cardiac amyloidosis: A review of diagnosis and therapy.J Am Coll Cardiol. 2016; 68: 1323-1341Crossref PubMed Scopus (212) Google Scholar More than 30 different amyloid precursor proteins have been identified, with prognosis related to the type of amyloid and specific organs affected. Cardiac involvement represents the most significant cause of morbidity and mortality.6Fontana M Banypersad SM Treibel TA et al.Differential myocyte responses in patients with cardiac transthyretin amyloidosis and light-chain amyloidosis: A cardiac MR imaging study.Radiology. 2015; 277: 388-397Crossref PubMed Scopus (83) Google Scholar The two most common forms of CA are light chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis. AL amyloidosis is a hematologic disorder in which clonal plasma cells proliferate to produce abnormal immunoglobulin light chains that form fibrils and deposit throughout the body. The kidney is the most commonly affected organ in AL amyloidosis, and the heart is the next most commonly affected organ system. AL is the most severe type of CA, with median survival from the onset of heart failure of fewer than six months if untreated.4Merlini G Palladini G. Light chain amyloidosis: The heart of the problem.Haematologica. 2013; 98: 1492-1495Crossref PubMed Scopus (51) Google Scholar The particularly poor prognosis of AL amyloidosis is believed to be a result of the amyloid light chains possessing both infiltrative and toxic components.7Liao R Jain M Teller P et al.Infusion of light chains from patients with cardiac amyloidosis causes diastolic dysfunction in isolated mouse hearts.Circulation. 2001; 104: 1594-1597Crossref PubMed Scopus (185) Google Scholar ATTR amyloidosis results when one of two distinct types of the liver-derived transthyretin proteins (wild type or variant) becomes unstable, leading to misfolded amyloid fibrils.3Yamamoto H Yokochi T. Transthyretin cardiac amyloidosis: An update on diagnosis and treatment.ESC Heart Fail. 2019; 6: 1128-1139Crossref PubMed Scopus (24) Google Scholar This most often occurs because of age-related misfolding of the wild-type protein (primarily in males) and less commonly as a result of misfolding of the variant protein in patients with an autosomal dominant transthyretin gene mutation.3Yamamoto H Yokochi T. Transthyretin cardiac amyloidosis: An update on diagnosis and treatment.ESC Heart Fail. 2019; 6: 1128-1139Crossref PubMed Scopus (24) Google Scholar,5Dorbala S Cuddy S Falk RH. How to image cardiac amyloidosis: A practical approach.JACC Cardiovasc Imaging. 2020; 13: 1368-1383Crossref PubMed Scopus (42) Google Scholar Cardiac involvement in ATTR amyloidosis (ATTR CA) increasingly has been recognized as an etiology of heart failure with preserved ejection fraction (HFpEF) in elderly patients.3Yamamoto H Yokochi T. Transthyretin cardiac amyloidosis: An update on diagnosis and treatment.ESC Heart Fail. 2019; 6: 1128-1139Crossref PubMed Scopus (24) Google Scholar A high degree of clinical suspicion is required for the diagnosis of CA because many of the signs and symptoms of this disease (eg, HFpEF, left ventricular hypertrophy [LVH], atrial fibrillation, diastolic dysfunction) are common cardiac manifestations of other conditions. CA also demonstrates heterogeneity in clinical course and symptom presentation, further complicating identification. Diagnostic testing focused on identifying CA may be limited as a result of perceived disease rarity, poor prognosis, and lack of treatment options.8Siddiqi OK Ruberg FL. Cardiac amyloidosis: An update on pathophysiology, diagnosis, and treatment.Trends Cardiovasc Med. 2018; 28: 10-21Crossref PubMed Scopus (93) Google Scholar Because no existing diagnostic tools individually can provide the information needed to diagnose CA, a multimodal cardiac imaging and laboratory testing approach is required. Despite the multitude of imaging options, CA remains largely underrecognized or delayed in diagnosis.9Alexander KM Orav J Singh A et al.Geographic disparities in reported US amyloidosis mortality from 1979 to 2015: Potential underdetection of cardiac amyloidosis.JAMA Cardiol. 2018; 3: 865-870Crossref PubMed Scopus (32) Google Scholar Cardiac involvement in patients with AL amyloid is a major predictor of adverse cardiac outcomes and, if untreated, has a median survival from diagnosis of fewer than six months.4Merlini G Palladini G. Light chain amyloidosis: The heart of the problem.Haematologica. 2013; 98: 1492-1495Crossref PubMed Scopus (51) Google Scholar With earlier diagnosis, however, treatment may be initiated before terminal systemic damage ensues. Although the survival after diagnosis for ATTR CA is longer, at three-to-five years, emergence of therapeutic options that interfere with TTR (transthyretin) synthesis, stabilize TTR proteins, and disrupt fibril deposition may allow patients to experience longer survival periods. As CA becomes a more recognized and understood condition, it becomes increasingly important for the cardiac anesthesiologist to understand disease manifestations and diagnostic workup. Amyloidosis is a systemic disorder with fibril deposition in a range of tissues. Because of its systemic nature, biopsies can be obtained from several sites, including the heart, abdominal fat pad, bone marrow, or kidney.10Sipe JD Benson MD Buxbaum JN et al.Amyloid fibril proteins and amyloidosis: Chemical identification and clinical classification International Society of Amyloidosis 2016 nomenclature guidelines.Amyloid. 2016; 23: 209-213Crossref PubMed Scopus (331) Google Scholar Congo red staining of amyloid deposits identifies the presence of amyloid in tissue, and mass spectrometry provides typing (>98% sensitivity).11Vrana JA Gamez JD Madden BJ et al.Classification of amyloidosis by laser microdissection and mass spectrometry-based proteomic analysis in clinical biopsy specimens.Blood. 2009; 114: 4957-4959Crossref PubMed Scopus (554) Google Scholar Fat pad biopsy is the most accessible site, but the diagnostic yield is less in identifying ATTR amyloid (15%-45%) compared with AL amyloid (84%), and a degree of expertise is required to find limited amyloid deposits.12Quarta CC Gonzalez-Lopez E Gilbertson JA et al.Diagnostic sensitivity of abdominal fat aspiration in cardiac amyloidosis.Eur Heart J. 2017; 38: 1905-1908Crossref PubMed Scopus (80) Google Scholar Historically, CA has been diagnosed only with a positive endomyocardial biopsy (EMB) or a positive extracardiac biopsy, combined with characteristic LVH of >12 mm on transthoracic echocardiography (TTE), unexplained by other causes. Accumulating literature supports the use of technetium-99m (Tc-99m) pyrophosphate (PYP) in the diagnosis of CA. Tc-99m PYP scanning almost definitely can diagnose ATTR CA, obviating the need for specialized, invasive EMB. Workup typically begins with TTE imaging, which provides quantification of cardiac structure and function, including assessment of chamber size, wall thickness, and mass. A number of nonspecific TTE findings are helpful in the evaluation and diagnosis of CA (Table 1).4Merlini G Palladini G. Light chain amyloidosis: The heart of the problem.Haematologica. 2013; 98: 1492-1495Crossref PubMed Scopus (51) Google Scholar,13Quarta CC Solomon SD Uraizee I et al.Left ventricular structure and function in transthyretin-related versus light-chain cardiac amyloidosis.Circulation. 2014; 129: 1840-1849Crossref PubMed Scopus (178) Google Scholar, 14Brenner DA Jain M Pimentel DR et al.Human amyloidogenic light chains directly impair cardiomyocyte function through an increase in cellular oxidant stress.Circ Res. 2004; 94: 1008-1010Crossref PubMed Scopus (256) Google Scholar, 15Bhandari AK Nanda NC. Myocardial texture characterization by two-dimensional echocardiography.Am J Cardiol. 1983; 51: 817-825Abstract Full Text PDF PubMed Scopus (99) Google Scholar, 16Falk RH Plehn JF Deering T et al.Sensitivity and specificity of the echocardiographic features of cardiac amyloidosis.Am J Cardiol. 1987; 59: 418-422Abstract Full Text PDF PubMed Scopus (170) Google Scholar, 17Klein AL Hatle LK Burstow DJ et al.Doppler characterization of left ventricular diastolic function in cardiac amyloidosis.J Am Coll Cardiol. 1989; 13: 1017-1026Crossref PubMed Scopus (276) Google Scholar These cardiac changes are most prominent in advanced disease. Nevertheless, TTE provides exclusive insight into diastolic function and left ventricular (LV) filling pressures. Early CA is marked by low E-wave and high transmitral A-wave velocity, with a decreased E/A ratio and normal deceleration time. Advanced CA is suggested by a normal E-wave, small A-wave, high E/A ratio, and restrictive pattern of LV filling characterized by rapid mitral inflow deceleration time.17Klein AL Hatle LK Burstow DJ et al.Doppler characterization of left ventricular diastolic function in cardiac amyloidosis.J Am Coll Cardiol. 1989; 13: 1017-1026Crossref PubMed Scopus (276) Google Scholar Tissue Doppler analysis of the mitral and tricuspid annular e’ velocities is reduced, with a high E/e’ ratio indicating high ventricular filling pressures.18Koyama J Ray-Sequin PA Davidoff R et al.Usefulness of pulsed tissue Doppler imaging for evaluating systolic and diastolic left ventricular function in patients with AL (primary) amyloidosis.Am J Cardiol. 2002; 89: 1067-1071Abstract Full Text Full Text PDF PubMed Scopus (104) Google Scholar These high filling pressures can lead to atrial enlargement, atrial arrhythmias, and atrial thrombus formation.Table 1Echocardiographic “Red Flags” in Cardiac AmyloidosisUnexplained left ventricular hypertrophy (>12 mm)Low-voltage ECG in the setting of ventricular hypertrophyRight ventricular hypertrophyThickened cardiac valvesThickening of the interatrial septumPericardial effusionAtrial fibrillationSmall left ventricular cavity sizeDiminished stroke volumeParadoxical low-flow, low-gradient aortic stenosisRestrictive left ventricular filling patternReduction in longitudinal contractionGlobal longitudinal strain analysis demonstrating reduced strain in the mid and basal ventricle with apical sparing, “cherry on top” patternAbnormal atrial strain pattern demonstrating severely reduced contractile function of the atrial myocardiumAbbreviation: ECG, electrocardiogram. Open table in a new tab Abbreviation: ECG, electrocardiogram. Changes in myocardial deformation measured with two-dimensional speckle tracking may be seen early in the disease. Specifically, this includes a reduction in longitudinal LV contraction and a characteristic global longitudinal strain (GLS) pattern of relative apical sparing with impairment of strain at the mid and basal segments (Fig 1).19Phelan 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: 1442-1448Crossref PubMed Scopus (412) Google Scholar This strain pattern may prove helpful in distinguishing CA from ventricular thickening in the setting of LVH, hypertrophic obstructive cardiomyopathy, and aortic stenosis. Patients with these other causes of LVH typically show reduced LV GLS in the regions of maximal hypertrophy.19Phelan 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: 1442-1448Crossref PubMed Scopus (412) Google Scholar,20Liu D Hu K Niemann M et al.Effect of combined systolic and diastolic functional parameter assessment for differentiation of cardiac amyloidosis from other causes of concentric left ventricular hypertrophy.Circ Cardiovasc Imaging. 2013; 6: 1066-1072Crossref PubMed Scopus (90) Google Scholar Although an apical-sparing GLS pattern is not diagnostic, this information provides some support for the diagnosis of CA and has been shown to be useful in patients with known CA. Abnormal GLS is an independent predictor of poor survival in AL and ATTR CA.13Quarta CC Solomon SD Uraizee I et al.Left ventricular structure and function in transthyretin-related versus light-chain cardiac amyloidosis.Circulation. 2014; 129: 1840-1849Crossref PubMed Scopus (178) Google Scholar,21Koyama J Falk RH. Prognostic significance of strain Doppler imaging in light-chain amyloidosis.JACC Cardiovasc Imaging. 2010; 3: 333-342Crossref PubMed Scopus (165) Google Scholar,22Senapati A Sperry BW Grodin JL et al.Prognostic implication of relative regional strain ratio in cardiac amyloidosis.Heart. 2016; 102: 748-754Crossref PubMed Scopus (67) Google Scholar Cardiac magnetic resonance (CMR) imaging provides noninvasive, high-definition structural images and tissue characterization, adding incremental value to the information obtained with TTE. CMR allows for measurement of the intrinsic signal of the myocardium using T1/T2 imaging sequences with pre- and post-contrast T1 mapping. CMR allows for the assessment of late gadolinium enhancement (LGE) and extracellular volume (ECV). LGE is the cornerstone technique for the diagnosis of CA but requires administration of gadolinium, which may be not possible in patients with renal dysfunction. Features of CA seen on LGE include global subendocardial enhancement, transmural LGE, and focal patchy LGE.23Syed IS Glockner JF Feng D et al.Role of cardiac magnetic resonance imaging in the detection of cardiac amyloidosis.JACC Cardiovasc Imaging. 2010; 3: 155-164Crossref PubMed Scopus (316) Google Scholar,24Fontana M Pica S Reant P et al.Prognostic value of late gadolinium enhancement cardiovascular magnetic resonance in cardiac amyloidosis.Circulation. 2015; 132: 1570-1579Crossref PubMed Scopus (262) Google Scholar LGE is more common in ATTR than in AL CA but cannot be used to distinguish between the two types. Meta-analysis based on seven published studies estimated a sensitivity of 85% and specificity of 92% for a CMR-based LGE diagnosis of CA.25Zhao L Tian Z Fang Q. Diagnostic accuracy of cardiovascular magnetic resonance for patients with suspected cardiac amyloidosis: A systematic review and meta-analysis.BMC Cardiovasc Disord. 2016; 16: 129Crossref PubMed Scopus (48) Google Scholar Because LGE imaging is a qualitative measure, it is difficult to track disease progression over time. T1 mapping offers a quantitative advantage over LGE imaging. T1 mapping measures the T1 signal of each pixel in an image. T1 values measured before gadolinium contrast are greater in areas of amyloid deposition and diffuse fibrosis and provide a combined signal from myocyte and ECV. With deposition of amyloid fibrils in the extracellular space, native T1 values are elevated markedly in CA and correlate well with markers of systolic and diastolic dysfunction.26Fontana M Banypersad SM Treibel TA et al.Native T1 mapping in transthyretin amyloidosis.JACC Cardiovasc Imaging. 2014; 7: 157-165Crossref PubMed Scopus (223) Google Scholar,27Karamitsos TD Piechnik SK Banypersad SM et al.Noncontrast T1 mapping for the diagnosis of cardiac amyloidosis.JACC Cardiovasc Imaging. 2013; 6: 488-497Crossref PubMed Scopus (373) Google Scholar T1 imaging is able to detect CA with a 92% sensitivity and 91% specificity, but cannot be used to distinguish between the subtypes. T1 imaging may be performed without contrast. In patients who can tolerate contrast administration, T1 mapping after contrast and ECV estimation are performed, with ECV being more reproducible than absolute T1 values.28Fontana M White SK Banypersad SM et al.Comparison of T1 mapping techniques for ECV quantification. Histological validation and reproducibility of ShMOLLI versus multibreath-hold T1 quantification equilibrium contrast CMR.J Cardiovasc Magn Reson. 2012; 14: 88Crossref PubMed Scopus (164) Google Scholar ECV is elevated in CA and correlates with other markers of CA severity, but there are no characteristic ECV values or features to distinguish between subtypes of CA.29Banypersad SM Sado DM Flett AS et al.Quantification of myocardial extracellular volume fraction in systemic AL amyloidosis: An equilibrium contrast cardiovascular magnetic resonance study.Circ Cardiovasc Imaging. 2013; 6: 34-39Crossref PubMed Scopus (192) Google Scholar Because EVC is quantifiable, the measurement offers potential for tracking disease burden and response to therapy.30Martinez-Naharro A Abdel-Gadir A Treibel TA et al.CMR-verified regression of cardiac AL amyloid after chemotherapy.JACC Cardiovasc Imaging. 2018; 11: 152-154Crossref PubMed Scopus (45) Google Scholar Uptake of Tc-99m–labeled bone-avid radiotracers in the myocardium of patients with CA has been recognized for some time. Initial diagnostic work did not distinguish the results of Tc-99m scanning for patients with AL versus ATTR CA, leading to variable diagnostic accuracy for detection of CA. Demonstration of the high specificity of Tc–99m-labeled tracers (most commonly PYP) for ATTR CA led to widespread clinical adoption of this scanning technique.31Perugini E Guidalotti PL Salvi F et al.Noninvasive etiologic diagnosis of cardiac amyloidosis using 99mTc-3.3-diphosphono-1.2-propanodicarboxylic acid scintigraphy.J Am Coll Cardiol. 2005; 46: 1076-1084Crossref PubMed Scopus (398) Google Scholar Bone-avid radiotracer scanning provides incremental value to TTE and CMR by distinguishing ATTR CA from other causes of LV thickening.32Gillmore JD Maurer MS Falk RH et al.Nonbiopsy diagnosis of cardiac transthyretin amyloidosis.Circulation. 2016; 133: 2404-2412Crossref PubMed Scopus (648) Google Scholar However, TTE and/or CMR imaging still are used to provide structural and hemodynamic information that is lacking with PYP scanning. PYP images are graded using visual grading and semiquantitative metrics. Visual grading compares myocardial uptake with rib uptake and is demonstrated in Figure 2 and categorized in Table 2. Semiquantitative evaluation compares the tracer uptake in the heart with an area of similar size in the contralateral lung (H/CL ratio). The H/CL ratio can be measured at one hour or three hours depending on institutional protocols. An H/CL ratio >1.5 at one hour accurately distinguishes ATTR from AL CA, with 97% sensitivity and 100% specificity.33Castano A Haq M Narotsky DL et al.Multicenter study of planar technetium 99m pyrophosphate cardiac imaging: Predicting survival for patients with ATTR cardiac amyloidosis.JAMA Cardiol. 2016; 1: 880-889Crossref PubMed Scopus (160) Google Scholar An H/CL ratio >1.3 at three hours is proposed as diagnostic of ATTR CA.Table 2Visual Grading Schemes for Technetium-99m Pyrophosphate ScanningGrade 0: No heart uptakeGrade 1: Heart uptake is less than rib uptake (equivocal finding)Grade 2: Heart uptake is equal to rib uptake (strongly suggestive)Grade 3: Heart uptake is more than rib uptake (positive finding) Open table in a new tab Multicenter assessment of the diagnostic accuracy of the bone-avid tracers that showed any myocardial radiotracer uptake (grades 1-3) was >99% sensitive and 86% specific for detecting ATTR CA. False positives were primarily in patients with AL amyloidosis.32Gillmore JD Maurer MS Falk RH et al.Nonbiopsy diagnosis of cardiac transthyretin amyloidosis.Circulation. 2016; 133: 2404-2412Crossref PubMed Scopus (648) Google Scholar That study confirmed that it is possible to diagnose CA using PYP scanning, particularly the ATTR type, but highlighted that AL CA also can demonstrate uptake on PYP scanning. Laboratory analysis to rule out AL amyloid is of critical importance. In the absence of detectable monoclonal proteins despite appropriate fixation, heart failure on TTE or CMR and positive PYP scanning (grade 2 or 3 uptake) can be used to diagnose ATTR CA without histology provided with EMB. Once a diagnosis of ATTR CA is suspected, the patient should undergo genetic testing to distinguish hereditary from wild type ATTR. Molecular imaging using amyloid-binding positron emission tomography (PET) tracers allows for direct detection of amyloid fibrils. These PET tracers offer the opportunity to specifically image and quantify global and regional amyloid burden in the heart and detect changes in fibril structure as an early response to therapy. Although not currently used in clinical practice, these amyloid PET radiotracers may offer potential for early detection, quantification, and assessment of response to CA therapy. AL CA is a disease of the plasma cells treated with chemotherapy to destroy the clonal cell line and, possibly, stem cell transplantation. Hereditary ATTR is a disease of the liver, with production of mutant TTR protein that congeals into amyloid fibrils. The treatment for hereditary ATTR may include liver transplantation. Wild-type ATTR lacks the genetic mutation seen in the hereditary form. Nonetheless, there is misfolding of the TTR proteins for unknown reason and deposition in the tissues as amyloid fibrils. Medical therapy for ATTR focuses on three mechanisms. TTR silencers (inotersen and patisiran) are designed to decrease the amount of TTR protein produced to 10%-to-15% of the normally produced amount. Strategies used in both hereditary and wild-type ATTR include TTR stabilizer and fibril disrupters. The only approved TTR stabilizer for patients with CA is tafamidis. Diflunisal is a TTR inhibitor that is approved in patients with amyloid neuropathy, but many patients cannot tolerate treatment if they have renal dysfunction, fluid overload, or bleeding tendency. Because these drugs are in early clinical use, knowledge of immediate and long-term side effects and specific anesthetic considerations are evolving. With expanding diagnostic techniques and treatment options, the number of patients diagnosed with CA likely will increase. ATTR CA has been demonstrated in 13.3% of older patients hospitalized with HFpEF, and it is likely that anesthesiologists already are providing care to these patients in the perioperative environment more than expected.34Gonzalez-Lopez E Gallego-Delgado M Guzzo-Merello G et al.Wild-type transthyretin amyloidosis as a cause of heart failure with preserved ejection fraction.Eur Heart J. 2015; 36: 2585-2594Crossref PubMed Scopus (414) Google Scholar In addition, coexisting CA in the presence of aortic stenosis increasingly has been recognized in the elderly.35Ternacle J Krapf L Mohty D et al.Aortic stenosis and cardiac amyloidosis: JACC review topic of the week.J Am Coll Cardiol. 2019; 74: 2638-2651Crossref PubMed Scopus (43) Google Scholar A systemic disease with amyloid infiltration into multiple tissues, including the heart, kidneys, and nervous system, involves direct implications on the conduct of an anesthetic in these patients. Table 3 summarizes important clinical features and conditions associated with CA. Autonomic and sensorineural neuropathy are common, leading to orthostatic hypotension and placing these patients at risk for hemodynamic instability. Infiltration of the heart leads to ventricular stiffness and high sensitivity to volume and heart-rate changes. Atrial fibrillation is common in CA patients, and irregular heart rhythms are poorly tolerated. Atrial thrombosis risk is elevated, and patients may develop thrombosis and stroke even in the presence of sinus rhythm.36El-Am EA Dispenzieri A Melduni RM et al.Direct current cardioversion of atrial arrhythmias in adults with cardiac amyloidosis.J Am Coll Cardiol. 2019; 73: 589-597Crossref PubMed Scopus (50) Google Scholar,37Dubrey S Pollak A Skinner M et al.Atrial thrombi occurring during sinus rhythm in cardiac amyloidosis: Evidence for atrial electromechanical dissociation.Br Heart J. 1995; 74: 541-544Crossref PubMed Scopus (96) Google Scholar Coronary microvascular dysfunction present in AL and ATTR CA may manifest as angina in the absence of obstructive coronary artery disease. Perivascular amyloid deposits may account for microvascular dysfunction.38Dorbala S Vangala D Bruyere Jr, J et al.Coronary microvascular dysfunction is related to abnormalities in myocardial structure and function in cardiac amyloidosis.JACC Heart Fail. 2014; 2: 358-367Crossref PubMed Scopus (83) Google Scholar,39Migrino RQ Truran S Gutterman DD et al.Human microvascular dysfunction and apoptotic injury induced by AL amyloidosis light chain proteins.Am J Physiol Heart Circ Physiol. 2011; 301: H2305-H2312Crossref PubMed Scopus (54) Google Scholar With the neurocardiac alterations, patients with CA are highly sensitive to hemodynamic changes and loading conditions such that negative inotropes and vasodilators may predispose these patients to hypotension and/or cardiovascular collapse. The heterogeneity of clinical response for these patients continues into the perioperative environment because of variable organ system dysfunction and disease.Table 3Clinical Features Associated with AmyloidosisHeart failure with preserved ejection fractionPeripheral and autonomic neuropathyNephrotic syndromePeriorbital purpuraMacroglossiaHistory of bilateral carpal tunnel syndromeHistory of biceps tendon ruptureHistory of spinal stenosis Open table in a new tab Because amyloidosis is a systemic disease involving amyloid fibril deposition into multiple organ systems, it is important for the cardiothoracic anesthesiologist to understand this disease process and its associated end-organ effects. A once rapidly fatal condition, CA is being diagnosed with increased frequency and has become better understood. Clinical and imaging assessment is necessary for diagnosis and important in monitoring response to newly available treatment. As diagnostic and treatment options expand, it remains important for the anesthesiologist to be aware of the diagnosis and treatment of this multisystem disease." @default.
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• W3132299792 title "Imaging Cardiac Amyloidosis: An Update for the Cardiothoracic Anesthesiologist" @default.
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