Pediatr Res. 2026 Jun 22. doi: 10.1038/s41390-026-05198-8. Online ahead of print.
ABSTRACT
BACKGROUND: Pediatric left ventricular non-compaction (LVNC) has a heterogeneous prognosis, with cardiovascular death as a major threat. This study explored predictive markers and aimed to construct a simplified risk stratification model.
METHODS: In this multicenter longitudinal cohort study, 157 LVNC patients aged < 18 years were enrolled. Baseline data were collected. The primary endpoint was cardiovascular death. Independent predictors were identified by Cox regression, with significant indicators adjusted for body surface area (BSA) and stratified into tertiles. Restricted cubic splines, Kaplan-Meier survival curves, subgroup analyses, and ROC curves were employed to assess their predictive performance and robustness.
RESULTS: The mean follow-up duration was 3.34 ± 2.86 years. Cardiovascular death occurred in 26 patients (16.6%). Left atrial diameter (LAD) (HR = 1.052, P = 0.046) and NT-proBNP (HR = 1.159, P = 0.039) were independent predictors. BSA-adjusted LAD/BSA showed improved prediction (AUC 0.702-0.768). Patients in the highest tertiles for LAD/BSA ( > 29.55 mm/m²; HR = 4.242) and NT-proBNP ( > 1362.40 pg/mL; HR = 1.720) had significantly increased risk, with a continuous positive dose-response relationship. Survival curves and subgroup analyses confirmed the robustness of these associations.
CONCLUSION: LAD and NT-proBNP independently predict cardiovascular death in pediatric LVNC. A model using BSA-adjusted LAD/BSA and NT-proBNP tertiles can identify high-risk children, aiding early clinical risk stratification.
IMPACT: Identifies left atrial diameter (LAD) and N-terminal pro B-type natriuretic peptide (NT‑proBNP) as independent predictors of cardiovascular death in pediatric left ventricular non‑compaction (LVNC). Demonstrates that body‑surface‑area‑adjusted LAD (LAD/BSA) significantly improves predictive performance over unadjusted LAD. Proposes a simplified, clinically accessible risk-stratification model using tertiles of LAD/BSA and NT-proBNP to identify high-risk children and guide individualized management. Reveals a continuous, positive dose-response relationship for both markers, with the rate of risk increase tending to flatten at higher levels. Shows consistent predictive value across key clinical subgroups, supporting the robustness of the findings.
PMID:42324339 | DOI:10.1038/s41390-026-05198-8