Multilayer Myocardial Mechanics in Genotype-Positive Left Ventricular Hypertrophy-Negative Patients With Hypertrophic Cardiomyopathy

• Published in: The American journal of cardiology 2018-11, Vol.122 (10), p.1754-1760
• Main Authors: Williams, Lynne K., Misurka, James, Ho, Carolyn Y., Chan, Wan-Xian, Agmon, Yoram, Seidman, Christine, Rakowski, Harry, Carasso, Shemy
• Format: Article
• Language: English
• Subjects: Abnormalities; Adult; Cardiomyopathy; Cardiomyopathy, Hypertrophic - diagnosis; Cardiomyopathy, Hypertrophic - genetics; Cardiomyopathy, Hypertrophic - physiopathology; Cardiovascular disease; Diastole; Echocardiography; Endocardium - diagnostic imaging; Female; Fibrosis; Genotype; Genotypes; Heart; Heart Ventricles - diagnostic imaging; Heart Ventricles - physiopathology; Humans; Hypertrophy; Hypertrophy, Left Ventricular - diagnosis; Hypertrophy, Left Ventricular - genetics; Hypertrophy, Left Ventricular - physiopathology; Magnetic Resonance Imaging, Cine; Male; Mechanics; Mechanics (physics); Multilayers; Mutation; Myocardial Contraction - physiology; Myocardium; Myocardium - pathology; Patients; Pericardium - diagnostic imaging; Reproducibility of Results; Rotation; Sarcomeres - genetics; Strain analysis; Strain rate; Stroke Volume - physiology; Systole; Variance analysis; Velocity; Ventricle
• ISSN: 0002-9149; 1879-1913
• DOI: 10.1016/j.amjcard.2018.08.008

It is unknown whether the presence of a sarcomeric mutation alone is sufficient to result in abnormal myocardial force generation, or whether additional changes in myocardial architecture (hypertrophy, disarray, and fibrosis) are required to impair systolic function. Speckle tracking echocardiography allows quantification of global strain/strain rates, twist, and dyssynchrony. In the present study we sought to further elucidate early abnormalities of myocardial mechanics in sarcomeric mutation carriers without evidence of clinical disease. Sixty genotype-positive left ventricular hypertrophy-negative (G+left ventricular hypertrophy [LVH]−) patients and 60 normal controls were studied. Velocity vector imaging was applied retrospectively to echocardiographic images to quantify global longitudinal and circumferential strain/strain rate, and rotation parameters. The G+LVH− group demonstrated both smaller left ventricular diastolic cavity dimensions (4.5 ± 0.6 cm vs 4.8 ± 0.4 cm) and a higher LVEF (66 ± 6% vs 60 ± 5%) compared with controls. An increase in circumferential subendocardial systolic strain (−30 ± 5 vs −27 ± 3%) and both systolic and diastolic subendocardial strain rate was seen in the G+LVH− group. Peak rotation angles were higher at the base and apex, with an increase in total twist (9.0 ± 3.8 vs 6.9 ± 2.9). In the control group, global and average segmental strain were similar, suggesting no/minimal dyssynchrony (global mechanical synchrony index [GMSi] 0.97–0.98). In the G+LVH− group GMSi was significantly lower (subendocardial GMSi 0.95; subepicardial GMSi 0.60), suggesting increasing subendocardial to subepicardial dyssynchrony. In conclusion, utilizing multilayer strain analysis, we demonstrate that G+LVH− subjects have enhanced subendocardial systolic strain rate and twist, as well as mechanical dyssynchrony within the left ventricular myocardium. These results demonstrate that abnormalities in myocardial mechanics precede the development of clinical hypertrophy.