Key result
Apical-to-basal myocardial work ratio predicts adverse outcomes in hATTR-CM better than global work index.
Why the study?
Most prognostic echocardiographic data in transthyretin cardiac amyloidosis derive from wild-type or mixed cohorts, limiting applicability to genetically distinct hereditary populations. The prognostic value of myocardial work parameters, which integrate left ventricular deformation with afterload, needed evaluation in a genetically characterised hATTR-CM cohort.
Does echocardiographic assessment of global myocardial work index and derived parameters (such as relative apical-to-basal work ratio) predict adverse clinical outcomes in adults with hereditary transthyretin cardiac amyloidosis?
Cohort (n=42)
Does echocardiographic assessment of global myocardial work index and derived parameters (such as relative apical-to-basal work ratio) predict adverse clinical outcomes in adults with hereditary transthyretin cardiac amyloidosis?
In hereditary transthyretin cardiac amyloidosis with preserved ejection fraction, the echocardiographic relative apical-to-basal myocardial work ratio outperforms global myocardial work indices in predicting adverse clinical outcomes.
Relative apical-to-basal work ratio may aid prognostication in hATTR-CM; extends prior echocardiographic data to hereditary cohorts but remains hypothesis-generating.
Background Hereditary transthyretin cardiac amyloidosis (hATTR-CM) is a progressive infiltrative cardiomyopathy characterised by heart failure with preserved ejection fraction and marked phenotypic variability according to genotype. Most prognostic echocardiographic data derive from wild-type or myxed cohorts, limiting applicability to genetically distinct populations. Myocardial work indices integrate left ventricular deformation with afterload and may provide a more physiologically relevant assessment of myocardial performance in amyloidosis, particularly in the presence of autonomic dysfunction and altered loading conditions. Purpose To evaluate the prognostic value of global myocardial work index (GWI) and derived myocardial work parameters in a genetically characterised cohort of patients with hATTR-CM. Methods Retrospective longitudinal study including 42 adults with confirmed hATTR-CM. Comprehensive echocardiography with pressure–strain loop analysis was performed to derive GWI, global constructive work (GCW), global wasted work (GWW), global work efficiency (GWE), and the relative apical-to-basal work ratio (RAB). The primary composite endpoint included heart failure hospitalisation, ventricular arrhythmia or device implantation, and all-cause mortality. Prognostic performance was assessed using ROC curves and Kaplan–Meier survival analysis. Results Mean age was 47.5 years; 59% were female. A single predominant pathogenic variant accounted for 70% of cases (Ser70Arg), reflecting genetic homogeneity within this cohort. Despite preserved left ventricular ejection fraction (median 60%) and relatively early functional class (50% in NYHA II), global longitudinal strain was already reduced (mean 13.9%), indicating subclinical systolic impairment. During a median follow-up of 25 months (IQR 9–35), 17 patients (40%) reached the composite endpoint. Patients with events were older, had higher natriuretic peptide levels and more advanced neurological impairment. They demonstrated lower global longitudinal strain (11.9% vs 15.3%, p<0.01), higher E/e′, and significantly reduced GWI and GCW (p<0.04). GWI predicted events (AUC 0.74; cut-off 1170 mmHg%; sensitivity 82%, specificity 68%). RAB demonstrated the highest discriminatory capacity (AUC 0.77; cut-off 1.62; sensitivity 76%, specificity 84%), outperforming global indices and showing early and significant separation of Kaplan–Meier curves (log-rank p=0.001). Conclusion In this genetically characterised hATTR-CM cohort, myocardial work indices were associated with adverse outcomes despite preserved ejection fraction and early clinical stage. The relative apical-to-basal work ratio showed superior prognostic performance compared with global myocardial work, suggesting that regional distribution of myocardial work captures disease heterogeneity and may enhance risk stratification in hereditary amyloid cardiomyopathy.
No takes yet. Share an insight, caveat, or question.
Barba et al. (2026) conducted a cohort in Hereditary transthyretin cardiac amyloidosis (hATTR-CM) (n=42). Myocardial work indices (global myocardial work index [GWI] and relative apical-to-basal work ratio [RAB]) vs. Global myocardial work indices / patients without adverse events was evaluated on Composite of heart failure hospitalisation, ventricular arrhythmia or device implantation, and all-cause mortality (AUC 0.77 for RAB; AUC 0.74 for GWI, p=0.001). Relative apical-to-basal myocardial work ratio predicted adverse outcomes in hATTR-CM (AUC 0.77; log-rank p=0.001), outperforming global myocardial work index (AUC 0.74).
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: