Cardiac MRI-Derived Right Ventricular Strain for Risk Stratification in Pediatric and Adolescent Arrhythmogenic Cardiomyopathy: A Multicenter Cohort Study.
Pediatric and adolescent ACM manifests a malignant phenotype with high burden of heart failure and arrhythmia, and RVGLS provides independent and incremental prognostic value beyond conventional CMR markers and could improve risk stratification in this high-risk population.
Abstract
Background
Pediatric and adolescent arrhythmogenic cardiomyopathy (ACM) is a clinically severe phenotype that remains underrepresented in cardiac magnetic resonance (CMR) research. The prognostic value of CMR-derived right ventricular deformation analysis in this population is unclear.
Objectives
This study sought to describe the burden of severe cardiac outcomes in pediatric and adolescent ACM, and to assess the prognostic and incremental value of CMR-derived right ventricular global longitudinal strain (RVGLS) for risk stratification when incorporated into conventional clinical and CMR markers.
Methods
This multicenter study included consecutive pediatric and adolescent patients (aged ≤21 years) with ACM who underwent baseline CMR evaluation. We assessed clinical, electrocardiographic, and CMR parameters according to the 2010 Task Force Criteria, along with biventricular strain parameters. Severe cardiac outcomes were defined as a composite of malignant ventricular arrhythmias (MVAs), heart transplantation, and heart failure-related death.
Results
The cohort included 102 patients (16.3 ± 3.4 years; 72% male) and 95 controls (mean age: 15.6 ± 3.3 years; 70% male). During a median of 58-month follow-up, 51 (50%) patients experienced severe cardiac outcomes and 28 (27.5%) MVA outcomes. Reduced RVGLS was independently associated with severe cardiac outcomes (HR: 1.14; P = 0.004) and MVA outcomes (subdistribution HR: 1.15; P < 0.001) after adjustment for clinical and conventional CMR parameters. Incorporation of RVGLS significantly improved prediction of severe cardiac outcomes beyond both right ventricular-based and left ventricular-based multivariable models, with significant improvement in discrimination and reclassification. Kaplan-Meier analysis further showed that RVGLS ≥-12.2% identified patients at higher risk of severe cardiac outcomes and MVA outcomes (both log-rank P < 0.001).
Conclusions
Pediatric and adolescent ACM manifests a malignant phenotype with high burden of heart failure and arrhythmia. RVGLS provides independent and incremental prognostic value beyond conventional CMR markers and could improve risk stratification in this high-risk population.
BACKGROUND
The left atrium is an early sensor of left ventricular (LV) dysfunction. The prognostic value of left atrial (LA) strain derived from CMR Feature-Tracking has been demonstrated in adult patients with hypertrophic cardiomyopathy (HCM); however, its significance in pediatric patients with HCM remains unknown. Our study aims to assess LA myocardial function using multi-parametric CMR and to explore the relationship between LA strains with adverse outcomes in pediatric patients with HCM.
METHODS
This retrospective study included pediatric patients with HCM who underwent CMR from January 2011 to December 2020. Strain parameters were assessed using CMR -FT analysis. Cox regression analysis was performed to assess the associations between variables and clinical outcomes which included sudden cardiac death (SCD) or equivalent events and heart failure-related events.
RESULTS
The cohort comprised 284 patients (median age, 15.0 years [interquartile range (IQR) 13.0-18.0]; 184 men) and 42 age- and sex-matched normal controls. Compare with normal controls, pediatric patients with HCM had impaired LA reservoir and conduit strains (all P<0.001), with no evidence of a difference in LA booster strain. After a median follow-up period of 62.8 months, a total of 41 (14.4%) patients reached composite outcomes. After adjusting clinical and imaging parameters in multivariable cox analysis, LA reservoir strain (HR: 0.95, 95% CI: 0.91-0.98, P = 0.007), and conduit strain (HR: 0.92, 95% CI: 0.87-0.97, P = 0.002) remained significantly independent predictors of composite outcomes, but not showed in booster strain. Adding LA reservoir strain or conduit strain in turn to the clinical and imaging model resulted in significant improvements in model performance for both composite outcomes and SCD events, respectively (all p < 0.001).
CONCLUSION
LA reservoir and conduit strains derived from CMR -FT were impaired in pediatric patients with HCM and showed important prognostic value for adverse clinical outcomes, providing incremental prognostic value when combined in a model with clinical and conventional risk factors.
Xing-Rui Chen, Yun Tang, Xuan Ma et al.· Journal of Cardiovascular Ma...· 0 citations
Strain parameters, particularly LVGLS, remained independently associated with outcomes in 34 of 36 studies after multivariable adjustment, often providing incremental value beyond LVEF and late gadolinium enhancement, and support strain imaging, especially LVGLS, as an adjunct to conventional risk stratification in diverse NICM populations.
Dina Mohammed, Humza Saeed, Abdallah Rayyan et al.· Cardiology in Review· 0 citations
Background: Risk stratification for ventricular arrhythmias in hypertrophic cardiomyopathy (HCM) remains challenging, particularly in patients who do not fulfill conventional high-risk criteria. This study aimed to investigate the association between cardiovascular magnetic resonance (CMR)-derived phenotypic characteristics and non-sustained ventricular tachycardia (NSVT) in patients with HCM. Methods: A total of 69 consecutive patients with HCM who underwent comprehensive CMR imaging and 24 h Holter monitoring were retrospectively analyzed. CMR assessment included phenotypic characteristics of the left ventricle, mitral valve apparatus, and papillary muscles, as well as late gadolinium enhancement (LGE). Ventricular arrhythmic events were defined as the presence of a premature ventricular complex burden >10%, NSVT, and/or sustained ventricular tachycardia. Univariable logistic regression and multivariable Firth penalized logistic regression analyses were performed to identify variables associated with NSVT. Results: The study population had a mean age of 57.5 years, and 23 patients (33.3%) were female. Ventricular arrhythmic events were detected in 16 patients (23.1%), all of whom had NSVT. Median LGE burden was 4%. LGE was present in 43 patients (62.3%), including papillary muscle (PM)-LGE in 13 patients (19.0%). Anterolateral PM–septum systolic contact was observed in 7 patients (10.0%). In univariable analysis, maximal left ventricular wall thickness (OR 1.29, 95% CI 1.09–1.53; p = 0.004), abnormal chordal attachment (OR 5.56, 95% CI 1.09–28.19; p = 0.039), anterolateral PM mobility (OR 1.45 per 0.1-unit increase, 95% CI 1.10–1.91; p = 0.009), anterolateral PM–septum systolic contact (OR 31.20, 95% CI 3.38–288.0; p = 0.002), and PM-LGE (OR 9.60, 95% CI 2.50–36.84; p < 0.001) were associated with NSVT. In the four-variable Firth penalized logistic regression model, abnormal chordal attachment (OR 9.09, 95% CI 1.18–67.42; p = 0.036), anterolateral PM–septum systolic contact (OR 23.08, 95% CI 2.18–385.17; p = 0.008), and PM-LGE (OR 17.26, 95% CI 2.37–176.64; p = 0.004) remained associated with NSVT. Given the limited number of events, these multivariable findings should be considered exploratory. Conclusions: CMR-derived PM abnormalities were associated with NSVT in patients with HCM. In particular, PM-LGE, abnormal chordal attachment, and anterolateral PM–septum systolic contact emerged as exploratory CMR characteristics that warrant further evaluation in larger prospective studies.
Barış Güven, Furkan M. Deniz, F. Özkan et al.· Diagnostics· 0 citations
Summary Background The risk stratification of sudden cardiac death (SCD) in patients with heart failure (HF) with mildly reduced ejection fraction (HFmrEF) remains suboptimal. This study aims to evaluate the role of late gadolinium enhancement (LGE) and T1 mapping in predicting SCD in patients with HFmrEF and to provide an improved clinical risk stratification algorithm. Methods A total of 1855 patients with HFmrEF from five tertiary medical centers were enrolled in this study, all of whom underwent echocardiography and cardiovascular magnetic resonance (CMR). Data analysis utilized a multicenter development cohort (n = 1417) and a multicenter external validation cohort (n = 438). The primary endpoints were sudden cardiac death, appropriate ICD shocks, and resuscitated cardiac arrest. Secondary endpoints were a composite of HF death, unplanned HF hospitalization, or cardiac transplantation. Based on validated MRI predictors, a risk algorithm and corresponding clinical workflow were developed for SCD risk assessment. Findings During a median follow-up of 42 months in the development cohort, 142 and 140 patients experienced primary and secondary endpoints, respectively. Adjusted analyses identified the following as significant predictors of SCD-related events: LGE ≥6.3% (HR 5.33, 95% CI: 3.74–7.60; P < 0.001), ECV ≥28.4% (HR 3.44, 95% CI: 2.32–5.11; P < 0.001), and a native T1 z-score ≥2.7 (HR 2.40, 95% CI 1.67–3.48; P < 0.001). Patients with ECV >28.4% and no LGE showed higher SCD risk compared to those with ECV <28.4% and LGE <6.3% or mid-wall LGE. Conversely, patients with ln (NT-proBNP) <6.2, LGE <6.3%, and ECV <28.4% had a lower SCD risk, with an annual event rate of 0.4%. Notably, LGE ≥6.3% was associated with a high annual SCD event rate of 7.9%, irrespective of ECV, NT-proBNP levels, and LGE distribution. Interpretation In HFmrEF, at least 6.3% of LGE serves as a strong predictor of SCD risk, irrespective of its distribution. ECV significantly enhances risk stratification, particularly in patients with negative or mid-wall LGE. Funding This study was supported by the National Natural Science Foundation of China (Grant Nos. 81871354, 81571672) and Key R&D Program of Shandong Province, China (2025CXPT106, 2025CXGC020305).
Wen-Xian Wang, Runze Zhu, Yan Gao et al.· The Lancet Regional Health -...· 0 citations
Background Differentiating cardiac amyloidosis (CA) from hypertrophic cardiomyopathy (HCM) is clinically challenging due to overlapping phenotypes of left ventricular hypertrophy. This study aimed to evaluate the diagnostic value of cardiac magnetic resonance (CMR)-derived left atrial strain parameters in differentiating CA from HCM. Methods A retrospective analysis was performed on CMR data obtained from 37 patients with HCM, 41 patients with CA, and 29 individuals in the normal control group. Left ventricular and left atrial functions were quantified using CVI42 software (version 5.14.2). Left atrial strain parameters were assessed using the tissue tracking module, including left atrial reservoir strain (εs) and strain rate (SRs), conduit strain (εe) and strain rate (SRe), and booster strain (εa) and strain rate (SRa). Differences in left ventricular and left atrial functional parameters, as well as left atrial strain parameters among the three groups, were analyzed using the Kruskal-Wallis test. Pairwise comparisons were conducted using the Mann-Whitney U test. Receiver operating characteristic (ROC) curve analysis was performed using MedCalc (version 15.2.2) to determine the diagnostic efficacy of left atrial strain parameters in differentiating CA from HCM. Results The absolute values of εs, εe, εa, SRs, SRe, and SRa were significantly lower in both the HCM and CA groups compared with the normal control group (P<0.05). Furthermore, the absolute values of these parameters were markedly lower in CA compared with HCM (P<0.05). Among these parameters, εs demonstrated the highest diagnostic performance for distinguishing CA from HCM, with an area under the ROC curve of 0.921. Conclusions CMR-derived left atrial strain parameters, particularly εs, might serve as a reliable means for differentiating between HCM and CA, which need further prospective study including patients with all HCM types and conducting internal and external validation to confirm credibility and expand generalizability.
Ya-Nan Jin, Xiao-Ning Shao, Yong Zhang et al.· Quantitative Imaging in Medi...· 0 citations
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