Aims: The association between left ventricular (LV) systolic dysfunction and adverse outcomes in pediatric cardiomyopathies (CMs) has been well established, but little is known about the prognostic value of the diastolic function in this population. This study aims to evaluate the association between left atrial (LA) function, assessed through 2D speckle-tracking echocardiography (2D-STE), and adverse outcomes in pediatric CM patients. Methods: A retrospective study of 138 pediatric CM patients (dilated, hypertrophic, and restrictive CMs) and 45 controls from 3 institutions was conducted. Echocardiographic parameters, including LA reservoir strain (LAS), were measured from each patient's oldest complete echocardiogram at each institution. The primary composite end point was cardiovascular death or aborted death, the need for a ventricular assist device, or cardiac transplantation. Associations between LAS and outcomes were assessed using Cox proportional hazards models and Kaplan-Meier analysis; complementary receiver operating characteristic curves were computed after adjustment for follow-up duration. Results: During a median follow-up of 78.8 months, 25 patients experienced the composite end point. Lower LAS (P < .001), impaired global longitudinal strain (GLS; P = .003), reduced LV ejection fraction (LVEF; P = .003), and increased LA volume index (LAVi; P = .027) and E/e' (P = .024) were significantly associated with adverse outcomes. Multivariable Cox analysis demonstrated that LAS (hazard ratio, 0.90 [0.84-0.96]; P = .001) is independently associated with the combined end point in model A adjusted for E/A and E/e', in model B (hazard ratio, 0.94 [0.89-0.99]; P = .018) adjusted for LAVi and LVEF, and in model C (hazard ratio, 0.91 [0.87-0.95]; P < .001) adjusted for GLS and LVEF. Left atrial reservoir strain performed better than LAVi in determining the association with outcomes, with an area under the curve of 0.739 (P < .001) and a cutoff of 15.3% (sensitivity, 0.58; specificity, 0.86). Conclusion: Left atrial reservoir strain showed an adjusted association with adverse outcomes in pediatric patients within the specified models, providing incremental information beyond conventional echocardiographic parameters. Incorporating LAS into routine evaluations may improve risk stratification and guide early interventions in pediatric CM. (J Am Soc Echocardiogr 2026;39:398-409.)
Left Atrial Function in Pediatric Cardiomyopathies: A Multicenter Study
Sabatino J.;Avesani M.;Castaldi B.;Cattapan I.;Di Salvo G.
2026
Abstract
Aims: The association between left ventricular (LV) systolic dysfunction and adverse outcomes in pediatric cardiomyopathies (CMs) has been well established, but little is known about the prognostic value of the diastolic function in this population. This study aims to evaluate the association between left atrial (LA) function, assessed through 2D speckle-tracking echocardiography (2D-STE), and adverse outcomes in pediatric CM patients. Methods: A retrospective study of 138 pediatric CM patients (dilated, hypertrophic, and restrictive CMs) and 45 controls from 3 institutions was conducted. Echocardiographic parameters, including LA reservoir strain (LAS), were measured from each patient's oldest complete echocardiogram at each institution. The primary composite end point was cardiovascular death or aborted death, the need for a ventricular assist device, or cardiac transplantation. Associations between LAS and outcomes were assessed using Cox proportional hazards models and Kaplan-Meier analysis; complementary receiver operating characteristic curves were computed after adjustment for follow-up duration. Results: During a median follow-up of 78.8 months, 25 patients experienced the composite end point. Lower LAS (P < .001), impaired global longitudinal strain (GLS; P = .003), reduced LV ejection fraction (LVEF; P = .003), and increased LA volume index (LAVi; P = .027) and E/e' (P = .024) were significantly associated with adverse outcomes. Multivariable Cox analysis demonstrated that LAS (hazard ratio, 0.90 [0.84-0.96]; P = .001) is independently associated with the combined end point in model A adjusted for E/A and E/e', in model B (hazard ratio, 0.94 [0.89-0.99]; P = .018) adjusted for LAVi and LVEF, and in model C (hazard ratio, 0.91 [0.87-0.95]; P < .001) adjusted for GLS and LVEF. Left atrial reservoir strain performed better than LAVi in determining the association with outcomes, with an area under the curve of 0.739 (P < .001) and a cutoff of 15.3% (sensitivity, 0.58; specificity, 0.86). Conclusion: Left atrial reservoir strain showed an adjusted association with adverse outcomes in pediatric patients within the specified models, providing incremental information beyond conventional echocardiographic parameters. Incorporating LAS into routine evaluations may improve risk stratification and guide early interventions in pediatric CM. (J Am Soc Echocardiogr 2026;39:398-409.)
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