Advanced echocardiographic phenotyping reveals distinct functional patterns in cardiomyopathies with increased LV mass

Background

Heart diseases characterized by increased left ventricular (LV) mass often progress to symptomatic heart failure. Advanced echocardiography, including myocardial work (MyW) analysis, can provide insights into the distinct patterns of LV remodeling and function, thereby advancing the pathophysiological understanding of these diseases.

Methods and results

We analyzed a cohort of 141 patients from five different cardiomyopathy subgroups, all with preserved LV ejection fraction and no pacemaker devices: AL amyloidosis (n=25), ATTR amyloidosis (n=48), hypertrophic obstructive cardiomyopathy (HOCM; n=25), hypertrophic non-obstructive cardiomyopathy (HNCM; n=23), and Fabry cardiomyopathy (n=25). For comparison, we also included data from healthy subjects (n=439). LV morphology, systolic and diastolic performance including strain and myocardial work (MyW) parameters as well as biomarkers (NT-proBNP) were assessed (table). In HOCM patients, we added the average of the mean and maximal gradient to systolic blood pressure to calculate MyW. Group differences were assessed using ANCOVA with adjustment for age and sex.

LV mass index was lowest in Fabry disease and highest in ATTR amyloidosis. All patient groups had elevated E/e´ and increased NT-proBNP levels, with NT-proBNP highest in AL amyloidosis (Table). Functional and deformation-based measures revealed distinct subgroup-specific patterns: Both AL and ATTR amyloidosis exhibited low constructive and wasted myocardial work. In contrast, HOCM showed high constructive work, whereas HNCM had high wasted work. Nevertheless, global work efficiency was reduced in all patient groups (Figure).

Conclusions

Detailed echocardiographic phenotyping using MyW analysis demonstrated distinct functional patterns across heart diseases with increased LV mass. Extremes of MyW, i.e. both increased and reduced MyW values, were associated with reduced global work efficiency and increased LV filling pressures. These MyW patterns may aid in a structured approach to phenotypic differentiation among heart diseases with increased LV mass.For image description, please refer to the figure legend and surrounding text.For image description, please refer to the figure legend and surrounding text.