Multichamber Strain Imaging and Biomarker Profiling for 1-Year Risk Stratification in Pediatric Dilated Cardiomyopathy

TL;DR

The study finds that multichamber strain imaging and biomarkers like NT-proBNP can predict short-term risks in children with dilated cardiomyopathy.

Contribution

The study introduces a novel approach combining multichamber strain imaging and age-adjusted biomarkers for risk stratification in pediatric DCM.

Findings

Children with DCM events had significantly impaired LVGLS, LASr, and RVFWSL compared to controls.

Zlog NT-proBNP was markedly higher in children who experienced events.

LVGLS, Zlog NT-proBNP, and LASr showed excellent discrimination for 1-year events with specific cut-offs.

Abstract

Dilated cardiomyopathy (DCM) in children is rare, but carries a high risk of progression to advanced heart failure (HF) and heart transplant (HTx). Improved short-term risk stratification is essential; however, robust pediatric prognostic tools remain limited. We aimed to evaluate the 1-year prognostic value of multichamber speckle-tracking echocardiography (STE) and biomarkers, including age-adjusted N-terminal pro-B-type natriuretic peptide (NT-proBNP) and vitamin D, in children with DCM. In this single-centre prospective cohort study, 29 children with idiopathic DCM and 27 age- and sex-matched healthy controls underwent standardised clinical, laboratory, and echocardiographic assessment. The primary endpoint was a 12-month composite of implantation of an implantable cardioverter-defibrillator (ICD), left-ventricular assist device (LVAD), HTx, or all-cause mortality. During a 1-year follow-up, 9/29 (31%) DCM patients experienced major events. Compared with event-free patients and controls, children with events had more impaired LVGLS (−5.99 ± 2.45% vs. −13.44 ± 6.88% and −19.98 ± 3.25%), lower LASr (10.97 ± 7.67% vs. 25.36 ± 10.28% and 44.0 ± 11.43%), and reduced RVFWSL (−15.32 ± 5.24% vs. −23.13 ± 8.55% and −24.78 ± 4.45%; all p < 0.01). Zlog NT-proBNP was markedly higher in the event group (5.37 [5.00–6.08] vs. 2.28 [0.71–3.68] and 0.14 [−0.02–0.88]). LVGLS, Zlog NT-proBNP, and LASr showed excellent discrimination for 1-year events (AUC 0.91, 0.91, and 0.87, respectively), with clinically applicable cut-offs (LVGLS ≥ −8%, Zlog NT-proBNP ≥ 4.6, LASr ≤ 21%). In conclusion, multichamber strain imaging combined with age-adjusted NT-proBNP provides clinically relevant, exploratory markers for short-term risk stratification in pediatric DCM, supporting earlier intensification of follow-up and timely referral for advanced heart failure therapies. These findings warrant validation in larger multicenter cohorts.

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