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- W109657961 abstract "Attention ASE Members:The ASE has gone green! Visit www.aseuniversity.org to earn free continuing medical education credit through an online activity related to this article. Certificates are available for immediate access upon successful completion of the activity. Nonmembers will need to join the ASE to access this great member benefit!Table of ContentsAbbreviations 474Organization of the Writing Group and Evidence Review 4741.Introduction 4742.Echocardiography 474A.Cardiac Structure 474B.Assessment of LV Systolic Function 475C.Assessment of LV Diastolic Function 477D.Dynamic Obstruction and Mitral Valve Abnormalities 477E.Mitral Regurgitation in HCM 480F.Myocardial Ischemia, Fibrosis, and Metabolism 481G.Guidance of Septal Reduction Procedures 481i.Surgical Myectomy 481ii.Alcohol Septal Ablation 481iii.Permanent Pacing 483H.Screening and Preclinical Diagnosis 4833.Nuclear Imaging 484A.Cardiac Structure 484B.Radionuclide Angiography for LV Systolic Function 484C.Radionuclide Angiography for LV Diastolic Function 484D.Dynamic Obstruction and Mitral Valve Abnormalities 484E.Mitral Regurgitation in HCM 484F.Myocardial Ischemia, Fibrosis, and Metabolism 484i.SPECT 484ii.Positron Emission Tomography (PET) 485iii.Imaging Metabolism 486G.Guidance of Septal Reduction Procedures 486H.Screening and Preclinical Diagnosis 4863.Cardiovascular Magnetic Resonance 486A.Cardiac Structure 486B.Assessment of LV Systolic Function 487C.Assessment of LV Diastolic Function 487D.Dynamic Obstruction and Mitral Valve Abnormalities 487E.Mitral Regurgitation in HCM 488F.Myocardial Ischemia, Fibrosis, and Metabolism 488i.Ischemia 488ii.Fibrosis 488iii.Imaging Metabolism 489G.Guidance of Septal Reduction Procedures 489H.Screening and Preclinical Diagnosis 4894.Cardiac Computed Tomography 489A.Cardiac Structure 489B.Assessment of LV Systolic Function 490C.Assessment of LV Diastolic Function 490D.Dynamic Obstruction and Mitral Valve Abnormalities 490E.Mitral Regurgitation in HCM 490F.Myocardial Ischemia, Fibrosis, and Metabolism 490G.Guidance of Septal Reduction Procedures 490H.Screening and Preclinical Diagnosis 4915.Hypertrophic Cardiomyopathy Imaging in the Pediatric Population 4916.Role of Imaging in the Differential Diagnosis of Hypertrophic Cardiomyopathy 4917.Recommendations for Clinical Applications 492A.Cardiac Structure 492B.Assessment of LV Systolic and Diastolic Function 493C.Assessment of LVOT Obstruction 493D.Evaluation of Patients Undergoing Invasive Therapy 493E.Diagnosis of CAD in Patients With HCM 494F.Screening 494G.Role of Imaging in Identifying Patients at High Risk for Sudden Cardiac Death 494Organization of the Writing Group and Evidence ReviewThe writing group was composed of acknowledged experts in hypertrophic cardiomyopathy (HCM) and its imaging representing the ASE, the American Society of Nuclear Cardiology, the Society for Cardiovascular Magnetic Resonance, and the Society of Cardiovascular Computed Tomography. The document was reviewed by the ASE Guidelines and Standards Committee and four official reviewers nominated by the American Society of Nuclear Cardiology, Society for Cardiovascular Magnetic Resonance, Society of Cardiovascular Computed Tomography, and the American College of Cardiology Foundation.The purpose of this document is to review the strengths and applications of the current imaging modalities and provide recommendation guidelines for using these techniques to optimize the management of patients with HCM. The recommendations are based on observational studies, sometimes obtained in a small number of patients, and from the clinical experience of the writing group members, given the scarcity of multimodality imaging comparative effectiveness studies. Notwithstanding these recommendations, the writing group believes that the selection of a given imaging modality must be individualized.1. IntroductionHCM is the most common genetic cardiomyopathy. Across multiple geographies and ethnicities, the prevalence is approximately 0.2%.1Maron B.J. McKenna W.J. Danielson G.K. Kappenberger L.J. Kuhn H.J. Seidman C.E. et al.ACC/ESC clinical expert consensus panel on hypertrophic cardiomyopathy: a report of the American College of Cardiology Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines (Committee to Develop an Expert Consensus Panel on Hypertrophic Cardiomyopathy).J Am Coll Cardiol. 2003; 42: 1687-1713Abstract Full Text Full Text PDF PubMed Scopus (816) Google Scholar HCM is transmitted in an autosomal dominant inheritance pattern. The natural history is benign in the majority of patients, with a near normal life span. However, adverse outcomes, including sudden cardiac death, lifestyle-limiting symptoms secondary to dynamic left ventricular (LV) outflow tract (LVOT) obstruction and/or diastolic filling abnormalities, atrial fibrillation, and LV systolic dysfunction, occur in some patients.1Maron B.J. McKenna W.J. Danielson G.K. Kappenberger L.J. Kuhn H.J. Seidman C.E. et al.ACC/ESC clinical expert consensus panel on hypertrophic cardiomyopathy: a report of the American College of Cardiology Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines (Committee to Develop an Expert Consensus Panel on Hypertrophic Cardiomyopathy).J Am Coll Cardiol. 2003; 42: 1687-1713Abstract Full Text Full Text PDF PubMed Scopus (816) Google ScholarThe clinical diagnosis of HCM is based on the demonstration of LV hypertrophy in the absence of another disease process that can reasonably account for the magnitude of hypertrophy present.1Maron B.J. McKenna W.J. Danielson G.K. Kappenberger L.J. Kuhn H.J. Seidman C.E. et al.ACC/ESC clinical expert consensus panel on hypertrophic cardiomyopathy: a report of the American College of Cardiology Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines (Committee to Develop an Expert Consensus Panel on Hypertrophic Cardiomyopathy).J Am Coll Cardiol. 2003; 42: 1687-1713Abstract Full Text Full Text PDF PubMed Scopus (816) Google Scholar Many patients are diagnosed serendipitously when a cardiac murmur or electrocardiographic abnormality prompts echocardiographic evaluation. Others present with dyspnea, chest pain, and/or presyncope. Sudden cardiac death occurs in approximately 1% of patients with HCM each year, and detecting patients at risk for sudden cardiac death is one of the most challenging clinical dilemmas. At the current time, a set of clinical risk factors1Maron B.J. McKenna W.J. Danielson G.K. Kappenberger L.J. Kuhn H.J. Seidman C.E. et al.ACC/ESC clinical expert consensus panel on hypertrophic cardiomyopathy: a report of the American College of Cardiology Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines (Committee to Develop an Expert Consensus Panel on Hypertrophic Cardiomyopathy).J Am Coll Cardiol. 2003; 42: 1687-1713Abstract Full Text Full Text PDF PubMed Scopus (816) Google Scholar and imaging results are considered in the context of each patient’s specific circumstances to help each patient decide whether an implantable cardioverter-defibrillator (ICD) represents an appropriate choice for that patient.1Maron B.J. McKenna W.J. Danielson G.K. Kappenberger L.J. Kuhn H.J. Seidman C.E. et al.ACC/ESC clinical expert consensus panel on hypertrophic cardiomyopathy: a report of the American College of Cardiology Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines (Committee to Develop an Expert Consensus Panel on Hypertrophic Cardiomyopathy).J Am Coll Cardiol. 2003; 42: 1687-1713Abstract Full Text Full Text PDF PubMed Scopus (816) Google ScholarThe management of HCM is based on a thorough understanding of the underlying anatomy and pathophysiology. In addition, careful assessment for concomitant structural heart disease is crucial to allow appropriate patient selection for advanced therapies.Various imaging modalities can be used to assess cardiac structure and function, the presence and severity of dynamic obstruction, the presence of mitral valve abnormalities, and the severity of mitral regurgitation, as well as myocardial ischemia, fibrosis, and metabolism. In addition, imaging can be used to guide treatment, screening and preclinical diagnosis and to detect phenocopies.2. EchocardiographyA Cardiac StructureLV volumes and the pattern of hypertrophy can be well defined by echocardiography (Figure 1, Video 1 [ view video clip online], Table 1). Ventricular volumes in HCM are usually normal or slightly reduced. Traditionally, the biplane Simpson’s method has been applied to the measurement of LV volumes and ejection fraction (EF).2Lang R.M. Bierig M. Devereux R.B. Flachskampf F.A. Foster E. Pellikka P.A. et al.Recommendations for chamber quantification: a report from the American Society of Echocardiography’s Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology.J Am Soc Echocardiogr. 2005; 18: 1440-1463Abstract Full Text Full Text PDF PubMed Scopus (3836) Google Scholar Recently, real-time three-dimensional (3D) echocardiography has been shown to provide more accurate means of quantification,3Caselli S. Pelliccia A. Maron M. Santini D. Puccio D. Marcantonio A. et al.Differentiation of hypertrophic cardiomyopathy from other forms of left ventricular hypertrophy by means of three-dimensional echocardiography.Am J Cardiol. 2008; 102: 616-620Abstract Full Text Full Text PDF PubMed Scopus (17) Google Scholar though there is a paucity of data on its accuracy in HCM. All imaging windows should be used to accurately define the areas of increased wall thickness. Hypertrophied segments often have slightly increased brightness in comparison with segments having normal end-diastolic wall thickness.Table 1Echocardiographic evaluation of patients with HCM1. Presence of hypertrophy and its distribution; report should include measurements of LV dimensions and wall thickness (septal, posterior, and maximum)2. LV EF3. RV hypertrophy and whether RV dynamic obstruction is present4. LA volume indexed to body surface area5. LV diastolic function (comments on LV relaxation and filling pressures)6. Pulmonary artery systolic pressure7. Dynamic obstruction at rest and with Valsalva maneuver; report should identify the site of obstruction and the gradient8. Mitral valve and papillary muscle evaluation, including the direction, mechanism, and severity of mitral regurgitation; if needed, TEE should be performed to satisfactorily answer these questions9. TEE is recommended to guide surgical myectomy, and TTE or TEE for alcohol septal ablation10. Screening Open table in a new tab LV hypertrophy, although usually asymmetric, can also be concentric. The distribution of hypertrophy can be in any pattern and at any location, including the right ventricle. Although septal predominance is more common, hypertrophy can be isolated to the LV free wall or apex (Figure 1). The presence of hypertrophy localized to the anterolateral wall can be missed, and careful imaging and extra care during interpretation are needed. When the extent of hypertrophy is difficult to visualize, having a high index of suspicion and meticulous imaging of the LV apex and/or the use of LV cavity opacification by intravenous contrast aids in the accurate diagnosis4Olszewski R. Timperley J. Szmigielski C. Monaghan M. Nihoyannopoulos P. Senior R. et al.The clinical applications of contrast echocardiography.Eur J Echocardiogr. 2007; 8: S13-S23Crossref PubMed Scopus (40) Google Scholar (Videos 2 and 3 [ view video clips online]). In particular, apical HCM and apical aneurysms can be missed without contrast. Transthoracic echocardiography (TTE) combined with the intravenous injection of an echocardiographic contrast agent should be performed in patients with HCM with suspected apical hypertrophy, to define the extent of hypertrophy and to diagnose apical aneurysms and clots.4Olszewski R. Timperley J. Szmigielski C. Monaghan M. Nihoyannopoulos P. Senior R. et al.The clinical applications of contrast echocardiography.Eur J Echocardiogr. 2007; 8: S13-S23Crossref PubMed Scopus (40) Google Scholar, 5Wigle E.D. Sasson Z. Henderson M.A. Ruddy T.D. Fulop J. Rakowski H. et al.Hypertrophic cardiomyopathy: the importance of the site and the extent of hypertrophy. A review.Prog Cardiovasc Dis. 1985; 28: 1-83Abstract Full Text PDF PubMed Google Scholar, 6Spirito P. Maron B.J. Relation between extent of left ventricular hypertrophy and occurrence of sudden cardiac death in hypertrophic cardiomyopathy.J Am Coll Cardiol. 1990; 15: 1521-1526Abstract Full Text PDF PubMed Google Scholar, 7Thaman R. Gimeno J.R. Murphy R.T. Kubo T. Sachdev B. Mogensen J. et al.Prevalence and clinical significance of systolic impairment in hypertrophic cardiomyopathy.Heart. 2005; 91: 920-925Crossref PubMed Scopus (54) Google Scholar, 8Mulvagh S.L. Rakowski H. Vannan M.A. Abdelmoneim S.S. Becher H. Bierig S.M. et al.American Society of Echocardiography consensus statement on the clinical applications of ultrasonic contrast agents in echocardiography.J Am Soc Echocardiogr. 2008; 21: 1179-1201Abstract Full Text Full Text PDF PubMed Scopus (141) Google Scholar It is possible to express the severity of hypertrophy using semiquantitative scores,5Wigle E.D. Sasson Z. Henderson M.A. Ruddy T.D. Fulop J. Rakowski H. et al.Hypertrophic cardiomyopathy: the importance of the site and the extent of hypertrophy. A review.Prog Cardiovasc Dis. 1985; 28: 1-83Abstract Full Text PDF PubMed Google Scholar, 6Spirito P. Maron B.J. Relation between extent of left ventricular hypertrophy and occurrence of sudden cardiac death in hypertrophic cardiomyopathy.J Am Coll Cardiol. 1990; 15: 1521-1526Abstract Full Text PDF PubMed Google Scholar which are based on wall thickness measurements by two-dimensional (2D) imaging in parasternal short-axis views at end-diastole. In the presence of adequate-quality images and expertise, 3D echocardiography provides the most accurate echocardiographic approach for quantifying LV mass.B Assessment of LV Systolic FunctionLV EF is usually normal or increased in patients with HCM and should be assessed in all imaging studies. Of note, patients with HCM with significant hypertrophy can have small LV end-diastolic volumes and therefore reduced stroke volumes despite having normal EFs. Overt LV systolic dysfunction, termed the “dilated or progressive phase of HCM,” “end-stage HCM,” or “burnt-out HCM,” is usually defined as an LV EF < 50% and occurs in a minority (2%–5%) of patients. Prognosis is markedly worse in the presence of LV systolic dysfunction.7Thaman R. Gimeno J.R. Murphy R.T. Kubo T. Sachdev B. Mogensen J. et al.Prevalence and clinical significance of systolic impairment in hypertrophic cardiomyopathy.Heart. 2005; 91: 920-925Crossref PubMed Scopus (54) Google Scholar Likewise, the development of an apical aneurysm is an uncommon but important complication that can be readily recognized with contrast echocardiography.8Mulvagh S.L. Rakowski H. Vannan M.A. Abdelmoneim S.S. Becher H. Bierig S.M. et al.American Society of Echocardiography consensus statement on the clinical applications of ultrasonic contrast agents in echocardiography.J Am Soc Echocardiogr. 2008; 21: 1179-1201Abstract Full Text Full Text PDF PubMed Scopus (141) Google ScholarIn addition to 2D and 3D imaging, Doppler methods have been used to assess for the presence of subclinical LV systolic dysfunction. Doppler tissue imaging measures the velocity of myocardial motion in systole and in diastole. Reduced systolic (Sa) and reduced early diastolic (Ea or e′) velocities can occur before the onset of overt hypertrophy.9Nagueh S.F. Bachinski L.L. Meyer D. Hill R. Zoghbi W.A. Tam J.W. et al.Tissue Doppler imaging consistently detects myocardial abnormalities in patients with hypertrophic cardiomyopathy and provides a novel means for an early diagnosis before and independently of hypertrophy.Cirulation. 2001; 104: 128-130Crossref PubMed Google Scholar, 10Cardim N. Perrot A. Ferreirat T. Pereira A. Osterziel K.J. Reis R.P. et al.Usefulness of Doppler myocardial imaging for identification of mutation carriers of familial hypertrophic cardiomyopathy.Am J Cardiol. 2002; 90: 128-132Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar Doppler tissue imaging can also be used to measure myocardial strain and strain rate, which unlike tissue Doppler velocities are not affected by translation and tethering. Strain rate imaging has been shown to be useful in differentiating nonobstructive HCM from hypertensive LV hypertrophy.11Kato T.S. Noda A. Izawa H. Yamada A. Obata K. Nagata K. et al.Discrimination of nonobstructive hypertrophic cardiomyopathy from hypertensive left ventricular hypertrophy on the basis of strain rate imaging by tissue Doppler ultrasonography.Circulation. 2004; 110: 3808-3814Crossref PubMed Scopus (98) Google Scholar However, tissue Doppler–derived strain imaging has technical limitations due to its angle dependence. Speckle-tracking echocardiography (STE) directly assesses myocardial motion from B-mode (2D) images and is independent of angulation between the ultrasound beam and the plane of motion. Several studies have shown reductions in strain (Figure 2, Figure 3) in patients with HCM compared with controls.12Serri K. Reant P. Lafitte M. Berhouet M. Le Bouffos V. Roudaut R. et al.Global and regional myocardial function quantification by two-dimensional strain: application in hypertrophic cardiomyopathy.J Am Coll Cardiol. 2006; 47: 1175-1181Abstract Full Text Full Text PDF PubMed Scopus (212) Google Scholar, 13Carasso S. Yang H. Woo A. Vannan M.A. Jamorski M. Wigle E.D. et al.Systolic myocardial mechanics in hypertrophic cardiomyopathy: novel concepts and implications for clinical status.J Am Soc Echocardiogr. 2008; 21: 675-683Abstract Full Text Full Text PDF PubMed Scopus (72) Google Scholar In terms of rotational motion, STE allows for quantification of the twisting (or wringing) motion of the heart. Observing LV torsion in normal subjects from an apical perspective, the base rotates clockwise while the apex rotates counterclockwise, creating a coordinated “wringing” motion of the left ventricle. Rotation velocities of twisting and untwisting are usually similar in patients with HCM as a group and in control subjects (Figure 4), although individual variations exist. Although the extent of rotation is usually normal, there can be differences in the direction of rotation. For example, mid-LV rotation in patients with HCM occurs in a clockwise direction, opposite to the direction seen in normal subjects.13Carasso S. Yang H. Woo A. Vannan M.A. Jamorski M. Wigle E.D. et al.Systolic myocardial mechanics in hypertrophic cardiomyopathy: novel concepts and implications for clinical status.J Am Soc Echocardiogr. 2008; 21: 675-683Abstract Full Text Full Text PDF PubMed Scopus (72) Google ScholarFigure 2LV global longitudinal strain by STE in a control subject (left) and a patient with HCM and hyperdynamic left ventricle (right). LV global strain is markedly reduced at 7% in the patient with HCM. AVC, Aortic valve closure.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 3(Left) Radial strain in the LV short-axis view from six myocardial segments by STE in a control subject. (Right) Strain from a patient with HCM and hyperdynamic left ventricle. Radial strain is markedly reduced in all six segments in the patient with HCM. AVC, Aortic valve closure.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 4Twist by STE in a control subject (left) and a patient with HCM (right). Both exhibit an initial clockwise rotation followed by a counterclockwise rotation of 17°.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Although STE is a promising method to evaluate myocardial function, there are significant differences between strain values across the 17 LV segments in normal individuals. Therefore, the variation of regional strain across the left ventricle necessitates the use of site-specific normal ranges, and the routine use of STE is not recommended at the present time.C Assessment of LV Diastolic FunctionLV and left atrial (LA) filling abnormalities have been reported in patients with HCM irrespective of the presence and extent of LV hypertrophy. The assessment of LV diastolic function in HCM can be limited by the relatively weak correlations between the mitral inflow and pulmonary venous flow velocities and invasive parameters of LV diastolic function.14Nishimura R.A. Appleton C.P. Redfield M.M. Ilstrup D.M. Holmes Jr., D.R. Tajik A.J. Noninvasive Doppler echocardiographic evaluation of left ventricular filling pressures in patients with cardiomyopathies: a simultaneous Doppler echocardiographic and cardiac catheterization study.J Am Coll Cardiol. 1996; 28: 1226-1233Abstract Full Text PDF PubMed Scopus (220) Google Scholar, 15Nagueh S.F. Lakkis N.M. Middleton K.J. Spencer III, W.H. Zoghbi W.A. Quinones M.A. Doppler estimation of left ventricular filling pressures in patients with hypertrophic cardiomyopathy.Circulation. 1999; 99: 254-261Crossref PubMed Google Scholar However, the atrial reversal velocity and its duration (Figure 5) recorded from the pulmonary veins have a significant correlation with LV end-diastolic pressure.15Nagueh S.F. Lakkis N.M. Middleton K.J. Spencer III, W.H. Zoghbi W.A. Quinones M.A. Doppler estimation of left ventricular filling pressures in patients with hypertrophic cardiomyopathy.Circulation. 1999; 99: 254-261Crossref PubMed Google ScholarFigure 5Assessment of LV diastolic function in a patient with HCM with elevated LV end-diastolic pressure but normal LA pressure. Mitral inflow shows a short mitral A duration at the level of the mitral annulus, whereas the Ar velocity in pulmonary venous flow is increased in amplitude and duration. Lateral annular e′ velocity is normal, and the ratio of peak E velocity (at the level of mitral tips) to e′ velocity is <8, consistent with normal LA pressure. (Right) Tissue Doppler (TD) velocities. A, Peak mitral late diastolic velocity; a′, late diastolic TD velocity; Ar, atrial reversal signal in pulmonary veins; E, peak mitral early diastolic velocity; e′, early diastolic TD velocity; D, diastolic velocity in pulmonary veins; S, systolic velocity in pulmonary veins.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Previous studies have noted reasonable correlations between E/e′ ratio and LV filling pressures.15Nagueh S.F. Lakkis N.M. Middleton K.J. Spencer III, W.H. Zoghbi W.A. Quinones M.A. Doppler estimation of left ventricular filling pressures in patients with hypertrophic cardiomyopathy.Circulation. 1999; 99: 254-261Crossref PubMed Google Scholar This was found across a wide range of annular velocities, including in patients in whom lateral annular e′ velocity was >8 cm/sec (Figure 5, Figure 6). A recent study noted modest correlations in patients with HCM with severely impaired LV relaxation and markedly reduced annular velocities.16Geske J.B. Sorajja P. Nishimura R.A. Ommen S.R. Evaluation of left ventricular filling pressures by Doppler echocardiography in patients with hypertrophic cardiomyopathy: correlation with direct left atrial pressure measurement at cardiac catheterization.Circulation. 2007; 116: 2702-2708Crossref PubMed Scopus (92) Google Scholar The E/e′ ratio has also been correlated with exercise tolerance in adults17Matsumura Y. Elliott P.M. Virdee M.S. Sorajja P. Doi Y. McKenna W.J. Left ventricular diastolic function assessed using Doppler tissue imaging in patients with hypertrophic cardiomyopathy: relation to symptoms and exercise capacity.Heart. 2002; 87: 247-251Crossref PubMed Google Scholar and children18McMahon C.J. Nagueh S.F. Pignatelli R.H. Denfield S.W. Dreyer W.J. Price J.F. et al.Characterization of left ventricular diastolic function by tissue Doppler imaging and clinical status in children with hypertrophic cardiomyopathy.Circulation. 2004; 109: 1756-1762Crossref PubMed Scopus (87) Google Scholar with HCM. In addition, septal e′ velocity appears to be an independent predictor of death and ventricular dysrhythmia in children with HCM.18McMahon C.J. Nagueh S.F. Pignatelli R.H. Denfield S.W. Dreyer W.J. Price J.F. et al.Characterization of left ventricular diastolic function by tissue Doppler imaging and clinical status in children with hypertrophic cardiomyopathy.Circulation. 2004; 109: 1756-1762Crossref PubMed Scopus (87) Google ScholarFigure 6Assessment of LV diastolic function in a patient with HCM with elevated LA pressure. Mitral inflow shows a restrictive inflow pattern (E velocity, 140 cm/sec). The arrow points to an L velocity in middiastole, which is observed in the presence of impaired relaxation and increased filling pressures. Lateral annular and septal annular tissue Doppler (TD) velocities (both e′ and a′) are markedly reduced consistent with severely impaired LV relaxation. The markedly increased E/e′ ratio is consistent with increased LA pressure > 20 mm Hg. The reduced mitral A velocity with its short deceleration time and the severely reduced a′ velocity are consistent with increased LV end-diastolic pressure. A, Peak mitral late diastolic velocity; a′, late diastolic TD velocity; E, peak mitral early diastolic velocity; e′, early diastolic TD velocity.View Large Image Figure ViewerDownload Hi-res image Download (PPT)A comprehensive approach is recommended when predicting LV filling pressures in patients with HCM,19Nagueh S.F. Appleton C.P. Gillebert T.C. Marino P.N. Oh J.K. Smiseth O.A. et al.Recommendations for the evaluation of left ventricular diastolic function by echocardiography.J Am Soc Echocardiogr. 2009; 22: 107-133Abstract Full Text Full Text PDF PubMed Scopus (756) Google Scholar taking into consideration the above velocities and ratios, as well as pulmonary artery pressures and LA volume, particularly in the absence of significant mitral regurgitation and atrial fibrillation, as the latter two conditions lead to LA enlargement in the presence of a normal LA pressure.LA size provides important prognostic information in HCM.20Nistri S. Olivotto I. Betocchi S. Losi M.A. Valsecchi G. Pinamonti B. et al.Participating CentersPrognostic significance of left atrial size in patients with hypertrophic cardiomyopathy (from the Italian Registry for Hypertrophic Cardiomyopathy).Am J Cardiol. 2006; 98: 960-965Abstract Full Text Full Text PDF PubMed Scopus (56) Google Scholar, 21Woo A. Williams W.G. Choi R. Wigle E.D. Rozenblyum E. Fedwick K. et al.Clinical and echocardiographic determinants of long-term survival after surgical myectomy in obstructive hypertrophic cardiomyopathy.Circulation. 2005; 111: 2033-2041Crossref PubMed Scopus (118) Google Scholar, 22Losi M.A. Betocchi S. Barbati G. Parisi V. Tocchetti C.G. Pastore F. et al.Prognostic significance of left atrial volume dilatation in patients with hypertrophic cardiomyopathy.J Am Soc Echocardiogr. 2009; 22: 76-81Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar LA enlargement in HCM is multifactorial in origin, with important contributions from the severity of mitral regurgitation, the presence of diastolic dysfunction, and possibly atrial myopathy.1Maron B.J. McKenna W.J. Danielson G.K. Kappenberger L.J. Kuhn H.J. Seidman C.E. et al.ACC/ESC clinical expert consensus panel on hypertrophic cardiomyopathy: a report of the American College of Cardiology Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines (Committee to Develop an Expert Consensus Panel on Hypertrophic Cardiomyopathy).J Am Coll Cardiol. 2003; 42: 1687-1713Abstract Full Text Full Text PDF PubMed Scopus (816) Google Scholar because LA volume has been shown to be the more accurate index of LA size, LA volume indexed to body surface area should be assessed in accordance with ASE guidelines.2Lang R.M. Bierig M. Devereux R.B. Flachskampf F.A. Foster E. Pellikka P.A. et al.Recommendations for chamber quantification: a report from the American Society of Echocardiography’s Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology.J Am Soc Echocardiogr. 2005; 18: 1440-1463Abstract Full Text Full Text PDF PubMed Scopus (3836) Google ScholarThere are three main mechanical functions of the left atrium: (1) reservoir function (during ventricular systole and isovolumic relaxation), (2) conduit function (during early diastole), and (3) contractile (booster pump) function (during atrial systole). The assessment of" @default.
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