Two-dimensional echocardiography showed regional longitudinal strain was reduced in mutation carriers before hypertrophy, with basal anteroseptal strain <16.5% providing 90% specificity.
Observational (n=140)
Does two-dimensional echocardiographic strain assessment identify early left ventricular abnormalities in sarcomeric mutation carriers before the hypertrophic stage compared to healthy controls?
Regional, but not global, longitudinal strain is significantly reduced in the early stage of hypertrophic cardiomyopathy before left ventricular hypertrophy, aiding in the identification of mutation carriers.
Absolute Event Rate: 20.6% vs 22.9%
AIMS: Hypertrophic cardiomyopathy (HCM) is a genetic disease with delayed cardiac expression. Our objective was to characterize left ventricular (LV) myocardial strain by two-dimensional echocardiography in sarcomeric mutation carriers before the hypertrophic stage. METHODS AND RESULTS: We studied 140 adults derivation cohort (n = 79), validation cohort (n = 61). The derivation cohort comprised 38 confirmed HCM patients with hypertrophy (LVH+/Gen+), 20 mutation carriers without LV hypertrophy (LVH-/Gen+), and 21 healthy controls. LV global longitudinal strain was not different in LVH-/Gen+ compared with controls 20.6%, interquartile (IQ): 18.3/24.2 vs. 22.9%, IQ: 20.9/26.8 but was reduced in LVH+/Gen+ patients (14.1%, IQ: 11.8/18.5, P < 0.001). Regional peak longitudinal strain was significantly decreased in LVH-/Gen+ when compared with controls in four segments: basal anteroseptal (BAS) wall (P = 0.018), basal inferoseptal wall (P = 0.047), basal inferior wall (P = 0.006), and mid anteroseptal wall (P = 0.022). Receiver operating characteristic analysis identified that BAS strain <16.5% had a sensitivity (Se), specificity (Sp), positive and negative predictive values (PPV, NPV) of 57%, 90%, 82%, and 67%, respectively, to differentiate LVH-/G+ patients from controls. Similarly, the accuracy of a ratio between basal inferoseptal/basal anterolateral (BIS/BAL) strain <0.76 was 73%, 92%, 82%, and 64%, respectively (Se/Sp/PPV/NPV). In the validation cohort, the accuracy of BAS and BIS/BAL was 39%/93%/87%/57% and 55%/96%/95%/64% (Se/Sp/PPV/NPV), respectively, to differentiate the LVH-/Gen+ group from controls. CONCLUSION: Regional longitudinal strain, but not global strain, was significantly reduced at the early stage of HCM before LV hypertrophy. This suggests that the inclusion of strain (BAS < 16.5%; BIS/BAL < 0.76) in the evaluation of HCM relatives would help identify mutation carriers and early LV abnormalities.
Baudry et al. (Wed,) conducted a observational in Hypertrophic cardiomyopathy (HCM) due to sarcomeric mutations (n=140). Two-dimensional echocardiographic strain imaging vs. Healthy controls was evaluated on Left ventricular global longitudinal strain in mutation carriers without hypertrophy versus controls. Two-dimensional echocardiography showed regional longitudinal strain was reduced in mutation carriers before hypertrophy, with basal anteroseptal strain <16.5% providing 90% specificity.
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