Left Ventricular Strain Is Abnormal in Preclinical and Overt Hypertrophic Cardiomyopathy: Cardiac MR Feature Tracking

Davis M Vigneault1, Eunice Yang1, Patrick J Jensen1

  • 1From the Department of Radiology and Imaging Sciences, Clinical Center, National Institutes of Health, Bethesda, Md (D.M.V., M.W.T.); Institute of Biomedical Engineering, Department of Engineering Science, University of Oxford, Oxford, England (D.M.V., M.W.T., J.A.N.); Sackler School of Graduate Biomedical Sciences, Tufts University School of Medicine, Boston, Mass (D.M.V.); Division of Cardiology (E.Y.) and Russell H. Morgan Department of Radiology and Radiological Science (L.C.), Johns Hopkins Hospital, Baltimore, Md; University of Chicago, The College, Chicago, Ill (P.J.J.); Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, Ohio (M.W.T.); Cardiovascular Division, Brigham and Women's Hospital, Boston, Mass (H.F., C.Y.H.); Departments of Internal Medicine and Pediatrics, University of Michigan, Ann Arbor, Mich (S.M.D., M.W.R.); Department of Cardiology, Boston Children's Hospital, Boston, Mass (S.D.C.); The Heart Institute and Pediatric Cardiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio (J.T.);New York University Langone Medical Center, New York, NY (M.V.S.); Department of Pediatrics, Washington University School of Medicine, St Louis, Mo (C.E.C.); Department of Nursing, College of Nursing and Health Sciences, University of Massachusetts Boston, Boston, Mass (L.S.); and School of Medicine and Public Health, University of Wisconsin-Madison, 600 Highland Ave, Madison, WI 53792 (D.A.B.).

Radiology
|December 19, 2018
PubMed

Insights

Cardiac MRI feature tracking reveals myocardial dysfunction in hypertrophic cardiomyopathy (HCM) patients, even before left ventricular hypertrophy develops. This finding highlights early contractile abnormalities in sarcomere mutation carriers.

Area of Science:

  • Cardiology
  • Medical Imaging
  • Genetics

Background:

  • Left ventricular (LV) dysfunction can signal early disease in preclinical hypertrophic cardiomyopathy (HCM), particularly in sarcomere mutation carriers without LV hypertrophy.
  • Cardiac Magnetic Resonance Imaging (CMR) feature tracking offers a method to assess myocardial strain in individuals with HCM sarcomere mutations.

Purpose of the Study:

  • To evaluate myocardial strain and circumferential transmural strain difference (cTSD) in a genotyped cohort with HCM.
  • To explore correlations between cTSD and other markers of disease severity, including anatomical and functional parameters.

Main Methods:

  • A prospective, multicenter, observational study (HCMNet) enrolled participants with HCM and their family members, all undergoing genetic testing.
  • Cardiac MRI with high temporal resolution (≤40 msec) was performed, and LV myocardial strain was analyzed using feature-tracking software.
  • Circumferential strain was measured at epicardial and endocardial surfaces to calculate cTSD. Multivariable logistic regression was used to predict HCM status.

Main Results:

  • Ninety-nine participants were analyzed: 23 controls, 34 with preclinical HCM, and 42 with overt HCM.
  • cTSD was significantly higher in both preclinical (17% ± 4) and overt HCM (22% ± 7) groups compared to controls (14% ± 4) (P < .01 for all).
  • In multivariable models, cTSD independently predicted preclinical and overt HCM status (P < .01), even after adjusting for septal thickness and N-terminal brain-type natriuretic peptide levels.

Conclusions:

  • Cardiac MRI feature tracking effectively identifies myocardial dysfunction in overt HCM.
  • This technique also detects dysfunction in sarcomere mutation carriers without LV hypertrophy, indicating early contractile abnormalities.
  • Myocardial dysfunction is present in HCM carriers even with normal LV wall thickness.

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