Guillaume Baudry1,2, Nicolas Mansencal3,4, Amelie Reynaud5, Pascale Richard6, Olivier Dubourg3,4, Michel Komajda1,7, Richard Isnard1,8, Patricia Réant5, Philippe Charron1,8. 1. APHP, Centre de référence pour les maladies cardiaques héréditaires, Hôpital Pitié-Salpêtrière, 47 bvd de l'hôpital, 75013 Paris, France. 2. HCL, Service Insuffisance cardiaque, Hôpital Louis Pradel, 59 Boulevard Pinel, 69500 Bron, France. 3. APHP, Service de Cardiologie, CHU Ambroise Paré, 9 av Charles de Gaulle, 92100 Boulogne Billancourt, France. 4. INSERM U-1018, CESP, Team 5 (EpReC, Renal and Cardiovascular Epidemiology), UVSQ, 94800 Villejuif, France. 5. Université de Bordeaux, CHU de Bordeaux, Service de cardiologie, Bordeaux, 33600 Pessac, France. 6. APHP, UF Cardiogénétique et Myogénétique, Service de Biochimie Métabolique, Hôpitaux Universitaires de la Pitié-Salpêtrière-Charles Foix, 47 Bvd de l'Hôpital, 75013 Paris, France. 7. Service de Cardiologie, Hôpital Saint Joseph, 75014 Paris, France. 8. Sorbonne Université, INSERM, UMR_S 1166 and ICAN Institute for Cardiometabolism and Nutrition, 91 bvd de l'hôpital, 75013 Paris, France.
Abstract
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. Published on behalf of the European Society of Cardiology. All rights reserved.
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. Published on behalf of the European Society of Cardiology. All rights reserved.
Authors: Sophie Urtado; Hélène Hergault; Stephen Binsse; Vincent Aidan; Mounir Ouadahi; Catherine Szymanski; Sophie Mallet; Marie Hauguel-Moreau; Robert Yves Carlier; Olivier Dubourg; Nicolas Mansencal Journal: J Clin Med Date: 2022-04-08 Impact factor: 4.964
Authors: Shasha Yu; Kai Jiang; Xiang Y Zhu; Christopher M Ferguson; James D Krier; Amir Lerman; Lilach O Lerman Journal: J Hypertens Date: 2021-03-01 Impact factor: 4.844