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• W4281809487 abstract "Hypertrophic cardiomyopathy (HCM) is defined by the presence of left ventricular hypertrophy in the absence of other potentially causative cardiac, systemic, syndromic, or metabolic diseases. Symptoms can be related to a range of pathophysiologic mechanisms including left ventricular outflow tract obstruction with or without significant mitral regurgitation, diastolic dysfunction with heart failure with preserved and heart failure with reduced ejection fraction, autonomic dysfunction, ischemia, and arrhythmias. Appropriate understanding and utilization of multimodality imaging is fundamental to accurate diagnosis as well as longitudinal care of patients with HCM. Resting and stress imaging provide comprehensive and complementary information to help clarify mechanism(s) responsible for symptoms such that appropriate and timely treatment strategies may be implemented. Advanced imaging is relied upon to guide certain treatment options including septal reduction therapy and mitral valve repair. Using both clinical and imaging parameters, enhanced algorithms for sudden cardiac death risk stratification facilitate selection of HCM patients most likely to benefit from implantable cardioverter-defibrillators. Hypertrophic cardiomyopathy (HCM) is defined by the presence of left ventricular hypertrophy in the absence of other potentially causative cardiac, systemic, syndromic, or metabolic diseases. Symptoms can be related to a range of pathophysiologic mechanisms including left ventricular outflow tract obstruction with or without significant mitral regurgitation, diastolic dysfunction with heart failure with preserved and heart failure with reduced ejection fraction, autonomic dysfunction, ischemia, and arrhythmias. Appropriate understanding and utilization of multimodality imaging is fundamental to accurate diagnosis as well as longitudinal care of patients with HCM. Resting and stress imaging provide comprehensive and complementary information to help clarify mechanism(s) responsible for symptoms such that appropriate and timely treatment strategies may be implemented. Advanced imaging is relied upon to guide certain treatment options including septal reduction therapy and mitral valve repair. Using both clinical and imaging parameters, enhanced algorithms for sudden cardiac death risk stratification facilitate selection of HCM patients most likely to benefit from implantable cardioverter-defibrillators. Attention ASE Members:Login at www.ASELearningHub.org to earn continuing medical education credit through an online activity related to this article. Certificates are available for immediate access upon successful completion of the activity and postwork. This activity is free for ASE Members, and $25 for nonmembers.This document is endorsed by the following ASE International Alliance Partners: Argentine Federation of Cardiology; Argentine Society of Cardiology; ASEAN Society of Echocardiography; Australasian Society for Ultrasound in Medicine; Canadian Society of Echocardiography; Cardiovascular Imaging Department of the Brazilian Society of Cardiology; Chinese Society of Echocardiography; Indian Academy of Echocardiography; Indonesian Society of Echocardiography; Iranian Society of Echocardiography; Israel Working Group on Echocardiography; Italian Association of Cardiothoracic Anaesthesiologists; Japanese Society of Echocardiography; Korean Society of Echocardiography; Mexican Society of Echocardiography and Cardiovascular Imaging; Society of Cardiovascular Images of the Inter-American Society of Cardiology; Thai Society of Echocardiography; The Pan-African Society of Cardiology; and Vietnam Society of Echocardiography. Attention ASE Members: Login at www.ASELearningHub.org to earn continuing medical education credit through an online activity related to this article. Certificates are available for immediate access upon successful completion of the activity and postwork. This activity is free for ASE Members, and $25 for nonmembers. This document is endorsed by the following ASE International Alliance Partners: Argentine Federation of Cardiology; Argentine Society of Cardiology; ASEAN Society of Echocardiography; Australasian Society for Ultrasound in Medicine; Canadian Society of Echocardiography; Cardiovascular Imaging Department of the Brazilian Society of Cardiology; Chinese Society of Echocardiography; Indian Academy of Echocardiography; Indonesian Society of Echocardiography; Iranian Society of Echocardiography; Israel Working Group on Echocardiography; Italian Association of Cardiothoracic Anaesthesiologists; Japanese Society of Echocardiography; Korean Society of Echocardiography; Mexican Society of Echocardiography and Cardiovascular Imaging; Society of Cardiovascular Images of the Inter-American Society of Cardiology; Thai Society of Echocardiography; The Pan-African Society of Cardiology; and Vietnam Society of Echocardiography. Since the publication of the recommendations for multimodality cardiovascular imaging of patients with hypertrophic cardiomyopathy (HCM) in 2011, an impressive growth and evolution of imaging techniques has occurred, enhancing both the recognition and management of the disease.1Nagueh S.F. Bierig S.M. Budoff M.J. Desai M. Dilsizian V. Eidem B. et al.American Society of Echocardiography clinical recommendations for multimodality cardiovascular imaging of patients with hypertrophic cardiomyopathy: Endorsed by the American Society of Nuclear Cardiology, Society for Cardiovascular Magnetic Resonance, and Society of Cardiovascular Computed Tomography.J Am Soc Echocardiogr. 2011; 24: 473-498Abstract Full Text Full Text PDF PubMed Scopus (225) Google Scholar This update from the American Society of Echocardiography (ASE), American Society of Nuclear Cardiology (ASNC), Society of Cardiovascular Magnetic Resonance (SCMR), and Society of Cardiovascular Computed Tomography (SCCT) provides a contemporary practical framework for the utilization of multimodality imaging in the care of HCM patients. Metabolic disorders that mimic HCM are only included in the differential diagnosis, where appropriate, and are not the focus of this document. HCM is defined by the presence of left ventricular hypertrophy (LVH) in the absence of other potentially causative cardiac, systemic, syndromic, or metabolic diseases.2Ommen S.R. Mital S. Burke M.A. Day S.M. Deswal A. Elliott P. et al.2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.J Am Coll Cardiol. 2020; 162: e23-e106Google Scholar It is the most common genetic abnormality of the myocardium, with an estimated prevalence ranging from 1:500 to as high as 1:200.3Maron B.J. Gardin J.M. Flack J.M. Gidding S.S. Kurosaki T.T. Bild D.E. Prevalence of hypertrophic cardiomyopathy in a general population of young adults. Echocardiographic analysis of 4111 subjects in the CARDIA Study. Coronary Artery Risk Development in (Young) Adults.Circulation. 1995; 92: 785-789Crossref PubMed Google Scholar, 4Maron B.J. Mathenge R. Casey S.A. Poliac L.C. Longe T.F. Clinical profile of hypertrophic cardiomyopathy identified de novo in rural communities.J Am Coll Cardiol. 1999; 33: 1590-1595Crossref PubMed Scopus (86) Google Scholar, 5Semsarian C. Ingles J. Maron M.S. Maron B.J. New perspectives on the prevalence of hypertrophic cardiomyopathy.J Am Coll Cardiol. 2015; 65: 1249-1254Crossref PubMed Scopus (559) Google Scholar Predominantly recognized as a disease caused by mutations in genes encoding sarcomeric proteins, HCM has a wide range of clinical expression and disease penetrance. Many individuals with HCM have a normal life expectancy and are relatively free of symptoms, while an important minority suffers debilitating symptoms and/or premature mortality.2Ommen S.R. Mital S. Burke M.A. Day S.M. Deswal A. Elliott P. et al.2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.J Am Coll Cardiol. 2020; 162: e23-e106Google Scholar,6Maron B.J. Rowin E.J. Casey S.A. Maron M.S. How hypertrophic cardiomyopathy became a contemporary treatable genetic disease with low mortality: shaped by 50 years of clinical research and practice.JAMA Cardiol. 2016; 1: 98-105Crossref PubMed Scopus (127) Google Scholar Symptoms can be related to a range of pathophysiologic mechanisms including diastolic dysfunction,7Villemain O. Correia M. Mousseaux E. Baranger J. Zarka S. Podetti I. et al.Myocardial stiffness evaluation using noninvasive shear wave imaging in healthy and hypertrophic cardiomyopathic adults.JACC Cardiovasc Imaging. 2019; 12: 1135-1145Crossref PubMed Scopus (57) Google Scholar,8Soullier C. Obert P. Doucende G. Nottin S. Cade S. Perez-Martin A. et al.Exercise response in hypertrophic cardiomyopathy: blunted left ventricular deformational and twisting reserve with altered systolic-diastolic coupling.Circ Cardiovasc Imaging. 2012; 5: 324-332Crossref PubMed Scopus (45) Google Scholar heart failure with preserved or reduced ejection fraction (EF),9Pasqualucci D. Fornaro A. Castelli G. Rossi A. Arretini A. Chiriatti C. et al.Clinical spectrum, therapeutic options, and outcome of advanced heart failure in hypertrophic cardiomyopathy.Circ Heart Fail. 2015; 8: 1014-1021Crossref PubMed Scopus (45) Google Scholar,10Rowin E.J. Maron B.J. Carrick R.T. Patel P.P. Koethe B. Wells S. et al.Outcomes in patients with hypertrophic cardiomyopathy and left ventricular systolic dysfunction.J Am Coll Cardiol. 2020; 75: 3033-3043Crossref PubMed Scopus (38) Google Scholar left ventricular outflow tract (LVOT) obstruction11Sorajja P. Nishimura R.A. Gersh B.J. Dearani J.A. Hodge D.O. Wiste H.J. et al.Outcome of mildly symptomatic or asymptomatic obstructive hypertrophic cardiomyopathy: a long-term follow-up study.J Am Coll Cardiol. 2009; 54: 234-241Crossref PubMed Scopus (70) Google Scholar,12Maron M.S. Olivotto I. Zenovich A.G. Link M.S. Pandian N.G. Kuvin J.T. et al.Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction.Circulation. 2006; 114: 2232-2239Crossref PubMed Scopus (656) Google Scholar with or without significant mitral regurgitation (MR),13Sherrid M.V. Balaram S. Kim B. Axel L. Swistel D.G. The mitral valve in obstructive hypertrophic cardiomyopathy: a test in context.J Am Coll Cardiol. 2016; 67: 1846-1858Crossref PubMed Scopus (128) Google Scholar autonomic dysfunction,14Patel V. Critoph C.H. Finlay M.C. Mist B. Lambiase P.D. Elliott P.M. Heart rate recovery in patients with hypertrophic cardiomyopathy.Am J Cardiol. 2014; 113: 1011-1017Abstract Full Text Full Text PDF PubMed Google Scholar ischemia,15Raphael C.E. Cooper R. Parker K.H. Collinson J. Vassiliou V. Pennell D.J. et al.Mechanisms of myocardial ischemia in hypertrophic cardiomyopathy: insights from wave intensity analysis and magnetic resonance.J Am Coll Cardiol. 2016; 68: 1651-1660Crossref PubMed Scopus (55) Google Scholar,16Sharzehee M. Chang Y. Song J.P. Han H.C. Hemodynamic effects of myocardial bridging in patients with hypertrophic cardiomyopathy.Am J Physiol Heart Circ Physiol. 2019; 317: H1282-H1291Crossref PubMed Scopus (10) Google Scholar and arrhythmias.17van Velzen H.G. Theuns D.A. Yap S.C. Michels M. Schinkel A.F. Incidence of device-detected atrial fibrillation and long-term outcomes in patients with hypertrophic cardiomyopathy.Am J Cardiol. 2017; 119: 100-105Abstract Full Text Full Text PDF PubMed Google Scholar,18Wilke I. Witzel K. Munch J. Pecha S. Blankenberg S. Reichenspurner H. et al.High incidence of de novo and subclinical atrial fibrillation in patients with hypertrophic cardiomyopathy and cardiac rhythm management device.J Cardiovasc Electrophysiol. 2016; 27: 779-784Crossref PubMed Google Scholar Appropriate understanding and utilization of multimodality imaging is fundamental to accurate diagnosis as well as longitudinal care of patients with HCM. Novel echocardiographic and cardiac magnetic resonance (CMR) imaging techniques have improved differentiation of HCM from other causes of LVH.19Hinojar R. Varma N. Child N. Goodman B. Jabbour A. Yu C.Y. et al.T1 mapping in discrimination of hypertrophic phenotypes: hypertensive heart disease and hypertrophic cardiomyopathy: findings from the international T1 multicenter cardiovascular magnetic resonance study.Circ Cardiovasc Imaging. 2015; 8: e003285Crossref PubMed Scopus (0) Google Scholar, 20Schnell F. Matelot D. Daudin M. Kervio G. Mabo P. Carre F. et al.Mechanical dispersion by strain echocardiography: a novel tool to diagnose hypertrophic cardiomyopathy in athletes.J Am Soc Echocardiogr. 2017; 30: 251-261Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar, 21Williams L.K. Forero J.F. Popovic Z.B. Phelan D. Delgado D. Rakowski H. et al.Patterns of CMR measured longitudinal strain and its association with late gadolinium enhancement in patients with cardiac amyloidosis and its mimics.J Cardiovasc Magn Reson. 2017; 19: 61Crossref PubMed Scopus (38) Google Scholar, 22Phelan D. Thavendiranathan P. Popovic Z. Collier P. Griffin B. Thomas J.D. et al.Application of a parametric display of two-dimensional speckle-tracking longitudinal strain to improve the etiologic diagnosis of mild to moderate left ventricular hypertrophy.J Am Soc Echocardiogr. 2014; 27: 888-895Abstract Full Text Full Text PDF PubMed Google Scholar Such refinements in conjunction with serial imaging have broadened our perspective of the penetrance of disease expression in HCM mutation carriers.23Lorenzini M. Norrish G. Field E. Ochoa J.P. Cicerchia M. Akhtar M.M. et al.Penetrance of hypertrophic cardiomyopathy in sarcomere protein mutation carriers.J Am Coll Cardiol. 2020; 76: 550-559Crossref PubMed Scopus (31) Google Scholar Resting and stress imaging provides both comprehensive and complementary information to help clarify mechanism(s) responsible for nonspecific symptoms such that appropriate and timely treatment strategies may be implemented. Advanced imaging is relied upon to guide certain treatment options including septal reduction therapy (SRT) and mitral valve repair. Using both clinical and imaging parameters, enhanced algorithms for sudden cardiac death (SCD) risk stratification facilitate selection of HCM patients most likely to benefit from implantable cardioverter-defibrillators (ICD), resulting in dramatically improved prognosis.6Maron B.J. Rowin E.J. Casey S.A. Maron M.S. How hypertrophic cardiomyopathy became a contemporary treatable genetic disease with low mortality: shaped by 50 years of clinical research and practice.JAMA Cardiol. 2016; 1: 98-105Crossref PubMed Scopus (127) Google Scholar,24Maron B.J. Rowin E.J. Maron M.S. Evolution of Risk Stratification and Sudden Death Prevention in Hypertrophic Cardiomyopathy: 20 Years with the Implantable Cardioverter-Defibrillator.Heart Rhythm. 2021; 18: 1012-1023Abstract Full Text Full Text PDF PubMed Google Scholar Accurate quantification of the magnitude, location, and pattern of LVH is essential for the diagnosis and management of patients. Wall thickness ≥15 mm in the absence of other causes of hypertrophy in a non-dilated left ventricle (LV) defines HCM.2Ommen S.R. Mital S. Burke M.A. Day S.M. Deswal A. Elliott P. et al.2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines.J Am Coll Cardiol. 2020; 162: e23-e106Google Scholar End diastolic wall thickness ≥13 mm can be diagnostic if there is a family history of HCM or a known disease-causing genetic mutation. Risk of SCD correlates with the magnitude of hypertrophy. Massive LVH (≥30 mm, which occurs in about 10% of individuals with HCM) has been used as a threshold for ICD implantation, although the relationship between the degree of hypertrophy and the risk of SCD is a continuous one. In addition, the location and pattern of increased wall thickness can predict the probability of a positive genetic test.25Bos J.M. Towbin J.A. Ackerman M.J. Diagnostic, prognostic, and therapeutic implications of genetic testing for hypertrophic cardiomyopathy.J Am Coll Cardiol. 2009; 54: 201-211Crossref PubMed Scopus (264) Google Scholar In the pediatric population, it is essential to consider that because of somatic growth, a single cut-off value cannot be applied across patients of different ages and sizes. Cardiac measurements are expressed as z-scores, representing standard deviations from a patient size-specific mean value, with hypertrophy diagnosed at z score >2.26Colan S.D. Normal echocardiographic values for cardiovascular structures. Echocardiography in Pediatric and Congenital Heart Disease: From Fetus to Adult. John Wiley & Sons Ltd, 2016: 883-901Google Scholar,27Lopez L. Colan S. Stylianou M. Granger S. Trachtenberg F. Frommelt P. et al.Relationship of Echocardiographic Z Scores Adjusted for Body Surface Area to Age, Sex, Race, and Ethnicity: The Pediatric Heart Network Normal Echocardiogram Database.Circ Cardiovasc Imaging. 2017; 10: e006979Crossref PubMed Scopus (108) Google Scholar The development of hypertrophy is a dynamic process. An abnormal increase in LV wall thickness in childhood is uncommon and should prompt consideration of phenocopies (Table 1) or double gene mutations. Hypertrophy usually accelerates in adolescence. While there are many different phenotypic expressions of HCM, hypertrophy is usually asymmetric, affecting non-contiguous LV segments, and occasionally the right ventricle (RV).28Klues H.G. Schiffers A. Maron B.J. Phenotypic spectrum and patterns of left ventricular hypertrophy in hypertrophic cardiomyopathy: morphologic observations and significance as assessed by two-dimensional echocardiography in 600 patients.J Am Coll Cardiol. 1995; 26: 1699-1708Crossref PubMed Scopus (516) Google Scholar Focal asymmetric hypertrophy of the basal anterior septum, defined as septal/posterior wall thickness >1.3 in a normotensive patient, is the most common pattern of hypertrophy. Variants include a sigmoid septum, reversed septal curvature, concentric, mid-wall, and apical hypertrophy (Figure 1 and Video 1). The positivity rate of genetic testing differs with each pattern of increased wall thickness, with the lowest yield occurring in patients with sigmoid septum and highest in patients with a reversed septal curvature pattern.25Bos J.M. Towbin J.A. Ackerman M.J. Diagnostic, prognostic, and therapeutic implications of genetic testing for hypertrophic cardiomyopathy.J Am Coll Cardiol. 2009; 54: 201-211Crossref PubMed Scopus (264) Google Scholar Furthermore, patients with a pathogenic mutation have greater wall thickness compared with mutation-negative individuals, which may partially account for the higher rate of adverse events in this cohort.29Ho C.Y. Day S.M. Ashley E.A. Michels M. Pereira A.C. Jacoby D. et al.Genotype and lifetime burden of disease in hypertrophic cardiomyopathy: insights from the sarcomeric human cardiomyopathy registry (SHaRe).Circulation. 2018; 138: 1387-1398Crossref PubMed Scopus (219) Google Scholar All imaging modalities should report the pattern and distribution of hypertrophy along with the location and magnitude of maximal wall thickness at end-diastole.Table 1Typical Features and Findings in Phenocopies of HCMMost Common PhenocopiesClinical FeaturesTypical ECG FindingsTypical Echo FindingsTypical CMR FindingsGenetics and Additional FeaturesChildren/Adolescents Danon DiseaseMild skeletal myopathy, ophthalmic abnormalities, intellectual disability.May reveal pre-excitation syndrome.Massive concentric LVH, occasionally dilated cardiomyopathy.LVH is often severe. LGE can be extensive, but often conspicuously sparing of the mid septumX-linked dominant disorder, although isolated cardiac form can present in older females.Diagnosis based on elevated CK, muscle biopsy, genetic testing (LAMP2 gene mutation)Adults <40 years PRKAG2Proximal myopathy, myalgia, epilepsy, early-onset hypertensionPre-excitation syndrome, bundle branch block, high voltages. Atrial fibrillation, atrial flutter. Advanced atrioventricular blocks, marked sinus bradycardia, or sinus blockVariable degree of increased LV wall thickness.Diastolic and systolic dysfunctionHighly variable findings from minimal asymmetric hypertrophy without LGE in early stages to severe hypertrophy with extensive LGE in advanced stagesAutosomal dominant, PRKAG2 gene mutation Friedrichs AtaxiaProgressive ataxia, loss of deep tendon reflexes, motor weakness, cerebral dysarthria, diabetes mellitusLateral T wave flattening or inversion.Supraventricular and ventricular arrhythmiaMild concentric remodeling, followed by hypertrophy, less often eccentric, hypertrophy. Impaired relaxation. Ultimately dilatation with systolic dysfunction. Sparkling texture.In early and intermediate disease: concentric remodeling or hypertrophy. In late disease: replacement fibrosis.Autosomal recessive, serum alpha-tocopherol level, brain MRI Anderson- Fabry DiseaseMulti-system disease: peripheral neuropathy, cutaneous lesions, progressive renal insufficiency with proteinuria, coronary small vessel disease.LVH with repolarization abnormalities, conduction abnormalities, preexcitation, atrial and ventricular arrhythmia.Concentric, asymmetric, and eccentric hypertrophy. Impaired relaxation. Normal ejection fraction. Thinned basal inferolateral LV wall in advanced disease.RV hypertrophy. Prominent papillary muscle. Aortic dilatation.LGE typically involves the mid segments of the lateral wall with subendocardial sparing. Involvement of the basal third of other LV walls in severe cases. Short T1 relaxation time may be present in the septum.X-linked recessive, deficiency of alpha-galactosidase A activity. Males present at a younger age. Athlete’s HeartAsymptomaticSinus bradycardia, LVH, early repolarization, first degree heart bock, Wenckebach, ectopic atrial or junctional rhythm.End-diastolic wall thickness typically below 15 mm. Balanced four chambers dilation. Normal/low-normal biventricular ejection fraction with normal/supranormal diastolic function.LGE absent except occasionally at the RV insertion points. Normal ECV.Supranormal functional capacity.Adults > 40 Hypertensive Heart DiseaseMay be asymptomatic or develop heart failure symptoms related to diastolic dysfunctionLVH (low sensitivity for detecting anatomic LVH), repolarization abnormalities, prolonged QTc and QRS durationMost commonly concentric hypertrophy or remodeling with varying degrees of diastolic dysfunction depending on the severity and duration of hypertensionPatchy LGE can be seen.Increased extracellular volume fraction in some patients Cardiac AmyloidosisClinical features include heart failure, peripheral neuropathy, atrial arrhythmias, and carpal tunnel syndromeLow QRS voltages relative to LV wall thickness.Conduction abnormalities, supraventricular arrhythmias.Concentric increase in LV wall thickness, sometimes with septal predominance. Restrictive LV filling pattern with more advanced disease. Normal to progressively reduced systolic function. Reduced global longitudinal strain with apical sparing. Bi-atrial dilatation. Thickening of valve leaflets. Small pericardial effusion.Subendocardial and transmural late gadolinium enhancement with relative apical sparing. Characteristic simultaneous myocardial and blood nulling or suboptimal myocardial nulling.Prolonged native myocardial T1 relaxation time. Increased extra-cellular volume fraction.Technetium-based bone scintigraphy for ATTR amyloidosis. Monoclonal proteins assay in blood and urine in patients with AL amyloidosis. Tissue biopsy may be needed.Alpha Gal-A, alpha galactosidase A; CK, creatine kinase; LAMP2, lysosome-associated membrane protein 2; LGE, Late Gadolinium Enhancement; LVH, Left Ventricular Hypertrophy; PRKAG2, gamma 2 regulatory subunit of AMP-activated protein kinase. Open table in a new tab Alpha Gal-A, alpha galactosidase A; CK, creatine kinase; LAMP2, lysosome-associated membrane protein 2; LGE, Late Gadolinium Enhancement; LVH, Left Ventricular Hypertrophy; PRKAG2, gamma 2 regulatory subunit of AMP-activated protein kinase. Transthoracic echocardiography (TTE) is the initial imaging modality of choice in HCM. Care should be taken to avoid LV foreshortening in the apical views, especially in cases with apical hypertrophy. The apical, anterior, and anterolateral walls can be challenging to visualize and accurately measure their thickness. Thus, there should be a low threshold to use ultrasound enhancing agents (UEA) if visualization is not optimal. This is particularly important if the pre-test probability is high, such as when screening family members of gene-positive individuals or in the presence of concerning electrocardiographic (ECG) patterns such as deep lateral T-wave inversion. Wall measurements obtained with UEA may be more reproducible than those obtained without and are more closely aligned to measurements obtained by CMR.30Urbano-Moral J.A. Gonzalez-Gonzalez A.M. Maldonado G. Gutierrez-Garcia-Moreno L. Vivancos-Delgado R. De Mora-Martin M. et al.Contrast-enhanced echocardiographic measurement of left ventricular wall thickness in hypertrophic cardiomyopathy: comparison with standard echocardiography and cardiac magnetic resonance.J Am Soc Echocardiogr. 2020; 33: 1106-1115Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar Additionally, UEA may facilitate identification of myocardial crypts, apical aneurysms, and ventricular thrombi, and can facilitate myocardial target localization for alcohol septal ablation. Systematic identification and exclusion of RV structures (including trabeculation, moderator band, and crista supraventricularis) is advised when measuring the interventricular septum (IVS). Long-axis views can overestimate wall thickness due to tangential cuts through the wall and potential inclusion of other structures such as trabeculations or papillary muscles in the measurements (Figure 2). Therefore, it is important to integrate and cross-reference short- and long-axis views to optimally align measurements.31Phelan D. Sperry B.W. Thavendiranathan P. Collier P. Popovic Z.B. Lever H.M. et al.Comparison of ventricular septal measurements in hypertrophic cardiomyopathy patients who underwent surgical myectomy using multimodality imaging and implications for diagnosis and management.Am J Cardiol. 2017; 119: 1656-1662Abstract Full Text Full Text PDF PubMed Scopus (7) Google Scholar,32Lang R.M. Badano L.P. Mor-Avi V. Afilalo J. Armstrong A. Ernande L. et al.Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging.Eur Heart J Cardiovasc Imaging. 2015; 16: 233-270Crossref PubMed Scopus (3652) Google Scholar High-quality three-dimensional (3D) echocardiography is superior to 2D in the evaluation of LV mass and more closely correlated with CMR assessment.33Chang S.A. Kim H.K. Lee S.C. Kim E.Y. Hahm S.H. Kwon O.M. et al.Assessment of left ventricular mass in hypertrophic cardiomyopathy by real-time three-dimensional echocardiography using single-beat capture image.J Am Soc Echocardiogr. 2013; 26: 436-442Abstract Full Text Full Text PDF PubMed Scopus (0) Google Scholar,34Avegliano G.P. Costabel J.P. Asch F.M. Sciancalepore A. Kuschnir P. Huguet M. et al.Utility of real time 3D echocardiography for the assessment of left ventricular mass in patients with hypertrophic cardiomyopathy: comparison with cardiac magnetic resonance.Echocardiography. 2016; 33: 431-436Crossref PubMed Scopus (9) Google Scholar The RV free wall should be measured in subcostal views at end-diastole with care to avoid inclusion of epicardial fat.32Lang R.M. Badano L.P. Mor-Avi V. Afilalo J. Armstrong A. Ernande L. et al.Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging.Eur Heart J Cardiovasc Imaging. 2015; 16: 233-270Crossref PubMed Scopus (3652) Google Scholar CMR’s contribution is of particular importance because of high spatial resolution and tissue characterization.35Hindieh W. Chan R. Rakowski H. Complementary role of echocardiography and cardiac magnetic resonance in hypertrophic cardiomyopathy.Curr Cardiol Rep. 2017; 19: 81Crossref PubMed Scopus (8) Google Scholar Still, care should be taken to avoid erroneous measurements from long-axis views (Figure 3). These capabilities confer diagnostic sensitivity when LVH is near diagnostic or therapeutic thresholds, atypical or complex in distribution, and in patients with technically challenging echocardiographic studies. As a component of the wider clinical and imaging assessment, tissue characterization (late gadolinium enhancement [LGE], T1 and T2 mapping) can help differentiate between increased wall thickness due to HCM and other conditions such as athletic remodeling, hypertension, inflammation, and infiltration (Table 1).36Haaf P. Garg P. Messroghli D.R. Broadbent D.A. Greenwood J.P. Plein S. Cardiac T1 Mapping and Extracellular Volume (ECV) in clinical practice: a comprehensive review.J Cardiovasc Magn Reson. 2016; 18: 89Crossref PubMed Scopus (301) Google Scholar,37Captur G. Manisty C. Moon J.C. Cardiac MRI evaluation of myocardial disease.Heart. 2016; 102: 1429-1435Crossref PubMed Scopus (42) Google ScholarFigure 4Integration of the 12-lead ECG in individuals with increased LV wall thickness to aid in the differential diagnosis. Image courtesy of Dr John Sy" @default.
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• W4281809487 title "Recommendations for Multimodality Cardiovascular Imaging of Patients with Hypertrophic Cardiomyopathy: An Update from the American Society of Echocardiography, in Collaboration with the American Society of Nuclear Cardiology, the Society for Cardiovascular Magnetic Resonance, and the Society of Cardiovascular Computed Tomography" @default.
• W4281809487 cites W109657961 @default.
• W4281809487 cites W118603018 @default.
• W4281809487 cites W134901354 @default.
• W4281809487 cites W1432820760 @default.
• W4281809487 cites W1543797453 @default.
• W4281809487 cites W1554269902 @default.
• W4281809487 cites W1561938212 @default.
• W4281809487 cites W1873150723 @default.
• W4281809487 cites W1915630878 @default.
• W4281809487 cites W1963947481 @default.
• W4281809487 cites W1966190213 @default.
• W4281809487 cites W1968427188 @default.
• W4281809487 cites W1973108468 @default.
• W4281809487 cites W1973685427 @default.
• W4281809487 cites W1978023625 @default.
• W4281809487 cites W1978480291 @default.
• W4281809487 cites W1985818915 @default.
• W4281809487 cites W1987736934 @default.
• W4281809487 cites W1990737568 @default.
• W4281809487 cites W1992088338 @default.
• W4281809487 cites W1993710397 @default.
• W4281809487 cites W1994771690 @default.
• W4281809487 cites W1996038459 @default.
• W4281809487 cites W1996065283 @default.
• W4281809487 cites W1996636073 @default.
• W4281809487 cites W1998117193 @default.
• W4281809487 cites W1998983714 @default.
• W4281809487 cites W2003964271 @default.
• W4281809487 cites W2004316705 @default.
• W4281809487 cites W2004337623 @default.
• W4281809487 cites W2004957860 @default.
• W4281809487 cites W2005202532 @default.
• W4281809487 cites W2005229135 @default.
• W4281809487 cites W2008480712 @default.
• W4281809487 cites W2010477218 @default.
• W4281809487 cites W2012637960 @default.
• W4281809487 cites W2017034231 @default.
• W4281809487 cites W2019584999 @default.
• W4281809487 cites W2022588850 @default.
• W4281809487 cites W2025818746 @default.
• W4281809487 cites W2028440344 @default.
• W4281809487 cites W2029162346 @default.
• W4281809487 cites W2031737275 @default.
• W4281809487 cites W2034409872 @default.
• W4281809487 cites W2036940365 @default.
• W4281809487 cites W2037098361 @default.
• W4281809487 cites W2037971966 @default.
• W4281809487 cites W2039627471 @default.
• W4281809487 cites W2041432334 @default.
• W4281809487 cites W2042811509 @default.
• W4281809487 cites W2045630430 @default.
• W4281809487 cites W2046122390 @default.
• W4281809487 cites W2047273318 @default.
• W4281809487 cites W2049057276 @default.
• W4281809487 cites W2050620846 @default.
• W4281809487 cites W2051066326 @default.
• W4281809487 cites W2051787463 @default.
• W4281809487 cites W2051967821 @default.
• W4281809487 cites W2052308215 @default.
• W4281809487 cites W2052617955 @default.
• W4281809487 cites W2054209395 @default.
• W4281809487 cites W2058027699 @default.
• W4281809487 cites W2060718803 @default.
• W4281809487 cites W2063893017 @default.
• W4281809487 cites W2064475524 @default.
• W4281809487 cites W2064759633 @default.
• W4281809487 cites W2068044592 @default.
• W4281809487 cites W2076034570 @default.
• W4281809487 cites W2082292539 @default.
• W4281809487 cites W2083189718 @default.
• W4281809487 cites W2087447794 @default.
• W4281809487 cites W2087542228 @default.
• W4281809487 cites W2087844906 @default.
• W4281809487 cites W2090876786 @default.
• W4281809487 cites W2091306472 @default.
• W4281809487 cites W2091383199 @default.
• W4281809487 cites W2094023228 @default.
• W4281809487 cites W2094109107 @default.
• W4281809487 cites W2095094855 @default.
• W4281809487 cites W2096579994 @default.

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