Utility of Cardiac Magnetic Resonance Imaging in the Diagnosis of Hypertrophic Cardiomyopathy

Background—Two-dimensional echocardiography is currently the standard test for the clinical diagnosis of hypertrophic cardiomyopathy (HCM). The present study was undertaken to determine whether cardiac MRI (CMR) affords greater accuracy than echocardiography in establishing the diagnosis and assessing the magnitude of left ventricular (LV) hypertrophy in HCM. Methods and Results—Forty-eight patients (age 34±16 years) suspected of having HCM (or with a confirmed diagnosis) were imaged by both echocardiography and CMR to assess LV wall thickness in 8 anatomic segments (total n=384 segments) and compared in a blinded fashion. Maximum LV thickness was similar by echocardiography (21.7±9.1 mm) and CMR (22.5±9.6 mm; P=0.21). However, in 3 (6%) of the 48 patients, echocardiography did not demonstrate LV hypertrophy, and CMR identified otherwise undetected areas of wall thickening in the anterolateral LV free wall (17 to 20 mm), which resulted in a new diagnosis of HCM. In the overall study group, compared with CMR, echocardiography also underestimated the magnitude of hypertrophy in the basal anterolateral free wall (by 20±6%; P=0.001), as well as the presence of extreme LV wall thickness (≥30 mm) in 10% of patients (P<0.05). Conclusions—CMR is capable of identifying regions of LV hypertrophy not readily recognized by echocardiography and was solely responsible for diagnosis of the HCM phenotype in an important minority of patients. CMR enhances the assessment of LV hypertrophy, particularly in the anterolateral LV free wall, and represents a powerful supplemental imaging test with distinct diagnostic advantages for selected HCM patients.

[1]  J. Seidman,et al.  Preclinical diagnosis of familial hypertrophic cardiomyopathy by genetic analysis of blood lymphocytes. , 1991, The New England journal of medicine.

[2]  Dudley J Pennell,et al.  Comparison of interstudy reproducibility of cardiovascular magnetic resonance with two-dimensional echocardiography in normal subjects and in patients with heart failure or left ventricular hypertrophy. , 2002, The American journal of cardiology.

[3]  B. Maron Hypertrophic cardiomyopathy: a systematic review. , 2002, JAMA.

[4]  A. Weyman Principles and Practice of Echocardiography , 1994 .

[5]  Barry J Maron,et al.  American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice G , 2003, Journal of the American College of Cardiology.

[6]  D. Pennell,et al.  Toward clinical risk assessment in hypertrophic cardiomyopathy with gadolinium cardiovascular magnetic resonance. , 2003, Journal of the American College of Cardiology.

[7]  B. Maron,et al.  Athlete's heart in women. Echocardiographic characterization of highly trained elite female athletes. , 1996, JAMA.

[8]  F. Carreras,et al.  Comparison of morphologic assessment of hypertrophic cardiomyopathy by magnetic resonance versus echocardiographic imaging. , 1997, The American journal of cardiology.

[9]  H Niimura,et al.  Development of left ventricular hypertrophy in adults in hypertrophic cardiomyopathy caused by cardiac myosin-binding protein C gene mutations. , 2001, Journal of the American College of Cardiology.

[10]  J. Gottdiener,et al.  Patterns and significance of distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy. A wide angle, two dimensional echocardiographic study of 125 patients. , 1981, The American journal of cardiology.

[11]  J. Lima,et al.  Cardiovascular magnetic resonance imaging: current and emerging applications. , 2004, Journal of the American College of Cardiology.

[12]  D. Pennell,et al.  Breath-hold FLASH and FISP cardiovascular MR imaging: left ventricular volume differences and reproducibility. , 2002, Radiology.

[13]  B. Maron,et al.  Relation of electrocardiographic abnormalities to evolving left ventricular hypertrophy in hypertrophic cardiomyopathy during childhood. , 1989, The American journal of cardiology.

[14]  B. Maron,et al.  Development and progression of left ventricular hypertrophy in children with hypertrophic cardiomyopathy. , 1986, The New England journal of medicine.

[15]  E. E. van der Wall,et al.  Assessment of quantitative hypertrophy scores in hypertrophic cardiomyopathy: magnetic resonance imaging versus echocardiography. , 1996, American heart journal.

[16]  J. Gottdiener,et al.  Hypertrophic Cardiomyopathy with Unusual Locations of Left Ventricular Hypertrophy Undetectable, by M‐mode Echocardiography: Identification by Wide‐angle Two‐dimensional Echocardiography , 1981, Circulation.

[17]  Dudley J Pennell,et al.  The histologic basis of late gadolinium enhancement cardiovascular magnetic resonance in hypertrophic cardiomyopathy. , 2004, Journal of the American College of Cardiology.

[18]  J Paul Finn,et al.  Left ventricular mass: manual and automatic segmentation of true FISP and FLASH cine MR images in dogs and pigs. , 2004, Radiology.

[19]  H W Woo,et al.  Advantages and applications of the centerline method for characterizing regional ventricular function. , 1986, Circulation.

[20]  P. Elliott,et al.  Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. , 2000, Journal of the American College of Cardiology.

[21]  A. Devlin,et al.  A comparison of MRI and echocardiography in hypertrophic cardiomyopathy. , 1999, The British journal of radiology.

[22]  R J van der Geest,et al.  Assessment of regional left ventricular wall parameters from short axis magnetic resonance imaging using a three-dimensional extension to the improved centerline method. , 1997, Investigative radiology.

[23]  Raymond J Kim,et al.  Gadolinium-enhanced magnetic resonance imaging in hypertrophic cardiomyopathy: in vivo imaging of the pathologic substrate for premature cardiac death? , 2003, Journal of the American College of Cardiology.

[24]  D. Pennell,et al.  Comparison of interstudy reproducibility of cardiovascular magnetic resonance with two-dimensional echocardiography in normal subjects and in patients with heart failure or left ventricular hypertrophy. , 2002, The American journal of cardiology.

[25]  B. Maron,et al.  Magnitude of left ventricular hypertrophy and risk of sudden death in hypertrophic cardiomyopathy. , 2000, The New England journal of medicine.

[26]  D. Pennell,et al.  Detection of apical hypertrophic cardiomyopathy by cardiovascular magnetic resonance in patients with non-diagnostic echocardiography , 2004, Heart.

[27]  B. Maron,et al.  Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. , 2003, The New England journal of medicine.

[28]  J. Seidman,et al.  Mutations in the genes for cardiac troponin T and alpha-tropomyosin in hypertrophic cardiomyopathy. , 1995, The New England journal of medicine.

[29]  E. Fleck,et al.  Noninvasive diagnosis of ischemia-induced wall motion abnormalities with the use of high-dose dobutamine stress MRI: comparison with dobutamine stress echocardiography. , 1999, Circulation.

[30]  Heiko Mahrholdt,et al.  Myocardial scarring in asymptomatic or mildly symptomatic patients with hypertrophic cardiomyopathy. , 2002, Journal of the American College of Cardiology.

[31]  M. Link,et al.  Primary prevention of sudden death as a novel treatment strategy in hypertrophic cardiomyopathy. , 2003, Circulation.

[32]  S. Plein,et al.  Steady‐state free precession magnetic resonance imaging of the heart: Comparison with segmented k‐space gradient‐echo imaging , 2001, Journal of magnetic resonance imaging : JMRI.

[33]  Masami Shimizu,et al.  Diagnostic value of abnormal Q waves for identification of preclinical carriers of hypertrophic cardiomyopathy based on a molecular genetic diagnosis. , 2004, European heart journal.

[34]  P. Elliott,et al.  Hypertrophic Cardiomyopathy: Histopathological Features of Sudden Death in Cardiac Troponin T Disease , 2001, Circulation.

[35]  H. Watkins,et al.  Sudden death due to troponin T mutations. , 1997, Journal of the American College of Cardiology.

[36]  B. Maron,et al.  Phenotypic spectrum and patterns of left ventricular hypertrophy in hypertrophic cardiomyopathy: morphologic observations and significance as assessed by two-dimensional echocardiography in 600 patients. , 1995, Journal of the American College of Cardiology.

[37]  K. Hayashi,et al.  Chronologic electrocardiographic changes in patients with hypertrophic cardiomyopathy associated with cardiac troponin 1 mutation. , 2002, American heart journal.

[38]  M. Link,et al.  Efficacy of implantable cardioverter-defibrillators for the prevention of sudden death in patients with hypertrophic cardiomyopathy. , 2000, The New England journal of medicine.

[39]  J. Seidman,et al.  The Genetic Basis for Cardiomyopathy from Mutation Identification to Mechanistic Paradigms , 2001, Cell.

[40]  M. Jerosch-Herold,et al.  An approach to the three-dimensional display of left ventricular function and viability using MRI , 2003, The International Journal of Cardiovascular Imaging.

[41]  L. McLain,et al.  Sudden death in young athletes. , 2003, Pediatric annals.