Current perspectives of sudden cardiac death management in hypertrophic cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is an autosomal dominant disorder characterized by left ventricular hypertrophy. Sudden cardiac death (SCD) is a rare but the most catastrophic complication in patients with HCM. Implantable cardioverter-defibrillators (ICDs) are widely recognized as effective preventive measures for SCD. Individualized risk stratification and early intervention in HCM can significantly improve patient prognosis. In this study, we review the latest findings regarding pathogenesis, risk stratification, and prevention of SCD in HCM patients, highlighting the clinic practice of cardiovascular magnetic resonance imaging for SCD management.

Glycogen storage disease with massive left ventricular hypertrophy and increased native T1: a case report.

Background: Glycogen storage disease (GSD) type Ⅲa is a rare autosomal recessive disorder resulting in the accumulation of abnormally structured glycogen in the liver, skeletal muscle, and cardiac muscle. Cardiovascular magnetic resonance (CMR) tissue characteristics in GSD have rarely been reported. Case summary: We report a 24-year-old male patient suffering from recurrent palpitation and atypical chest pain for 5 years with suspected hypertrophic cardiomyopathy. Laboratory tests revealed an elevated creatine kinase, and physical exam revealed hepatosplenomegaly. Cardiovascular magnetic resonance demonstrated asymmetrical massive left ventricular hypertrophy with a maximal thickness of 34.6 mm in the septum. In the regions with focal late gadolinium enhancement (LGE) in the anterior septum, both native T1 and extracellular volume (ECV) are elevated. However, in the LGE-negative regions of the myocardium, native T1 was elevated without elevation in ECV (septum, 22.7%; free wall, 20.9%). Whole exome sequencing revealed a novel pathogenic homozygous nonsense variant of the AGL gene (c.4284 T > G, p. Tyr1428*), confirming the diagnosis of the patients as GSD type Ⅲa. Discussion: This case showed increased diffuse native T1 but not ECV on CMR in LGE-negative myocardium in GSD, which indicates that the T1 value is increased with an accumulation of glycogen in the myocardium, but the ECV space was not expanded in this process. Genetic testing should be obtained in severe LV hypertrophy when multi-organ involvement is present, and myocardial tissue characterization is discrepant between T1 elevation and normal ECV to consider glycogen storage disorder.

Validation of the Risk Stratification for Sudden Cardiac Death in Chinese Patients With Hypertrophic Cardiomyopathy.

Accurate identification of hypertrophic cardiomyopathy (HCM) patients at high risk of sudden cardiac death (SCD) event is challenging. The objective of this study was to validate the three SCD risk stratifications recommended by the 2014 European Society of Cardiology (ESC) guideline, the 2020 American Heart Association /American College of Cardiology (AHA/ACC) guideline, and the 2022 ESC guideline in Chinese patients with HCM. Our study population are made up of a cohort of 856 HCM patients without prior SCD events. The endpoint was defined as SCD or equivalent events (successful resuscitation after cardiac arrest or appropriate ICD shock for ventricular tachycardia or ventricular fibrillation). During a median follow-up of 43 months, SCD endpoints occurred in 44 (5.1%) patients. A total of 34 (77.3%) patients suffering from SCD events were classified correctly into high-risk groups by the 2020 AHA/ACC guideline, 27(61.4%) by the 2022 ESC guideline, and 13 (29.6%) by the 2014 ESC guideline. The C-statistic of the 2020 AHA/ACC guideline was 0.68 (95% CI, 0.60-0.76), which performed better than the 2022 ESC guideline (0.65: 95% CI, 0.56-0.73), and the 2014 ESC guideline (0.58: 95% CI, 0.48-0.67). The 2020 AHA/ACC guideline displayed better discrimination for SCD risk stratification in Chinese HCM patients than the other two guidelines, with a higher sensitivity but lower specificity.

Incremental significance of myocardial oedema for prognosis in hypertrophic cardiomyopathy.

AIMS: To explore the prognosis of myocardial oedema measured by T2 mapping in hypertrophic cardiomyopathy (HCM). METHODS AND RESULTS: A total of 674 patients with HCM (age: 50 ± 15 years, 60.5% males) who underwent cardiovascular magnetic resonance were prospectively enrolled from 2011 to 2020. One hundred healthy controls (age: 48 ± 19 years, 58.0% males) were included for comparison. Myocardial oedema was quantitatively measured by T2 mapping in both global and segmental myocardium. The endpoints were defined as a combination of cardiovascular death and appropriate implantable cardioverter defibrillator discharge. During a median follow-up of 36 months (interquartile range: 24-60 months), 55 patients (8.2%) had cardiovascular events. Patients with cardiovascular events had a higher T2 max, T2 min, and T2 global values (all P < 0.001) than patients who remained event free. Survival analysis demonstrated that patients with HCM with late gadolinium enhancement [LGE(+)] and T2 max ≥44.9 ms had a higher risk of developing cardiovascular events (P < 0.001). A multivariate Cox regression analysis showed that T2 max, T2 min, and T2 global provided significant prognostic value to predict cardiovascular events (all P < 0.001). According to the C-index (0.825, 0.814), net reclassification index (0.612, 0.536, both P < 0.001), and integrative discrimination index (0.029, 0.029, both P < 0.05), T2 max or T2 min significantly increased the predictive performance of established risk factors, including extensive LGE. CONCLUSION: Patients with HCM with LGE(+) and higher T2 had worse prognosis than those with LGE(+) and lower T2.

The predictive value of fast semi-automated left atrial long-axis strain analysis for atrial fibrillation in hypertrophic cardiomyopathy.

OBJECTIVES: Hypertrophic cardiomyopathy (HCM) patients are closely related to LA functional impairment. Left atrial (LA) strain provides more insight into LA function. The study aimed to investigate the left atrial dysfunction of HCM patients by rapid semi-automatic method and determine the predictive value of left atrial long-axis strain (LAS) for atrial fibrillation (AF). METHODS: We enrolled 372 HCM patients and 100 healthy participants to assess the LA functional parameters. LAS was obtained by semi-automated tracking of the distance between the mid-posterior point of LA wall which is defined as the intersection of the LA long axis and the posterior wall, and the origins of the mitral valve. The inferior and anterior mitral valve annular insertion points on the 2-chamber view and the lateral and septal insertion points on the 4-chamber view were chosen as the origins of the mitral valve. The clinical outcome was defined as detecting the onset of AF. RESULTS: The LA strain values were analyzed as 20.8 ± 7.48% for εs, 9.81 ± 5.09% for εe, and 10.91 ± 4.24% for εa in HCM patients, which decreased significantly compared with normal controls. Significant correlations were detected between LV functional parameters and LA strain. During a median follow-up of 61 months, 44 (11.8%) patients developed AF. In the Cox regression analysis, LA strain was identified as a significant predictor of the onset of AF. CONCLUSIONS: HCM patients had impairment of LA strain before LA enlargement and reduced EF. LA-LAS can be used as a predictive value for predicting the occurrence of AF in HCM patients. KEY POINTS: • Fast semi-automated long-axis strain analysis by CMR is feasible and effective for evaluating the LA longitudinal function. • Hypertrophic cardiomyopathy patients show significant impairment of left atrial strain before LA enlargement. • The left atrial long-axis strain was an independent predictor of atrial fibrillation in hypertrophic cardiomyopathy.

Prognostic value of fast semi-automated left atrial long-axis strain analysis in hypertrophic cardiomyopathy.

BACKGROUND: The prognostic value of left atrial (LA) size and function in hypertrophic cardiomyopathy (HCM) is well recognized, but LA function is difficult to routinely analyze. Fast LA long-axis strain (LA-LAS) analysis is a novel technique to assess LA function on cine cardiovascular magnetic resonance (CMR). We aimed to assess the association between fast LA-LAS and adverse clinical outcomes in patients with HCM. METHODS: 359 HCM patients and 100 healthy controls underwent routine CMR imaging. Fast LA-LAS was analyzed by automatically tracking the length between the midpoint of posterior LA wall and the left atrioventricular junction based on standard 2- and 4-chamber balanced steady-state free precession cine-CMR. Three strain parameters including reservoir strain (εs), conduit strain (εe), and active strain (εa) were assessed. The endpoint was set as composite adverse events including cardiovascular death, resuscitated cardiac arrest, sudden cardiac death aborted by appropriate implantable cardioverter-defibrillator discharge, and hospital admission related to heart failure. RESULTS: During an average follow-up of 40.9 months, 59 patients (19.7%) reached endpoints. LA strains were correlated with LA diameter, LA volume index (LAVI) and LA empty fraction (LAEF) (all p < 0.05). In the stepwise multivariate Cox regression analysis, εs and εe (hazard ratio, 0.94 and 0.89; p = 0.019 and 0.006, respectively) emerged as independent predictors of the composite adverse events. Fast LA εs and LA εe are stronger prognostic factors than LA size, LAVI and the presence of left ventricular late gadolinium enhancement. CONCLUSIONS: Fast LA reservoir and conduit strains are independently associated with adverse outcomes in HCM.

Prognostic value of myocardial extracellular volume fraction evaluation based on cardiac magnetic resonance T1 mapping with T1 long and short in hypertrophic cardiomyopathy.

OBJECTIVE: To investigate the prognostic significance of T1 mapping using T1 long and short in hypertrophic cardiomyopathy (HCM) patients. METHODS: A total of 263 consecutive patients with HCM referred for cardiovascular magnetic resonance (CMR) imaging were enrolled in this study. The imaging protocol consisted of cine, late gadolinium enhancement (LGE), and T1 mapping with T1 long and short. All patients were followed up prospectively. Outcome events were divided into the primary and secondary endpoint events. Primary endpoint events included cardiac death, heart transplant, aborted sudden death, and cardiopulmonary resuscitation after syncope. The secondary endpoint event was defined as unplanned rehospitalization for heart failure. RESULT: The average follow-up duration was 28.3 ± 12.1 (range: 1-78) months. In all, 17 patients (7.0%) experienced a primary endpoint including 13 cardiovascular deaths, three aborted sudden deaths, and one resuscitation after syncope, and 34 patients experienced a secondary endpoint. Patients with primary endpoints showed a trend towards more extensive LGE (p < 0.001), significantly higher ECV (p < 0.001), and native T1 (p = 0.028) than those without events. In multivariate Cox regression analysis, ECV was independently associated with primary and secondary endpoints (p < 0.001 and p = 0.047, respectively). For every 3% increase, ECV portended a 1.374-fold increase risk of a primary endpoint occurring (p < 0.001). In the Kaplan-Meier survival analysis, the incidence of primary and secondary endpoint events was significantly higher in HCM with increased ECV (p < 0.001 and p = 0.009, respectively). CONCLUSION: In patients with HCM, ECV is a strong imaging marker for predicting adverse outcome. KEY POINTS: • ECV is a potent imaging index which has a strong correlation with LVEF and LVEDVI and can evaluate myocardial tissue structure and function. • ECV and LGE can provide a prognostic value in patients with hypertrophic cardiomyopathy. • ECV has stronger predictive effectiveness than LGE; even in the subgroup with LGE, ECV shows independent predictive significance for adverse events.

The Prognostic Value of Inflammation Factors in Hepatocellular Carcinoma Patients with Hepatic Artery Interventional Treatments: A Retrospective Study.

BACKGROUND: Hepatic artery interventional therapy has been recognized as the first choice for advanced liver cancer. However, reliable prognostic markers are still lacking. In the present study, we aimed to evaluate the prognostic value of inflammation factors including neutrophil to lymphocyte ratio (NLR), platelet to lymphocyte ratio (PLR) and monocyte to lymphocyte ratio (MLR) in hepatocellular carcinoma (HCC) patients with hepatic artery interventional treatments. METHODS: Patients undergoing hepatic artery interventional therapy after being diagnosed with HCC between 2007 and 2014 were enrolled. Pre-treatment NLR, PLR and MLR were calculated, and all factors including gender, age, TNM stage, BCLC staging, inflammation factors, LDH, ALP, CEA, AFP, hepatitis, liver cirrhosis, portal vein involvement, surgical history and hepatic artery interventional treatment on overall survival (OS) were evaluated by the univariate and multivariate Cox proportional hazards analyses. RESULTS: Overall, 407 patients were included. The optimal cutoff values determined by receiver operating characteristic (ROC) curve analyses for NLR, PLR and MLR were 3.82, 140.00 and 0.27, respectively. High NLR was associated with worse OS (median survival time: high NLR group 9 vs low NLR group 19 months, HR 1.842, 95% CI: 1.457-2.329, P<0.001). Elevated PLR was negatively correlated with OS (8 vs 18 months, HR 1.677, 95% CI: 1.302-2.161, P<0.001). Patients in high MLR group had a worse OS (10 vs 21 months, HR 1.626, 95% CI: 1.291-2.048, P<0.001). In multivariate analysis, NLR, LDH, ALP and portal vein involvement were independent prognostic factors for OS of HCC patients after hepatic artery interventional therapy. In addition, for patients in BCLC stage A and B, higher NLR, PLR and MLR were all significantly negatively correlated to median survival time (NLR: 17 vs 26 months, HR: 1.739 (95% CI: 1.279-2.365), P<0.001; PLR: 18 vs 26 months, HR: 1.681 (95% CI: 1.245-2.271), P=0.001; MLR: 20 vs 26 months, HR: 1.589 (95% CI: 1.185-2.129), P=0.002). CONCLUSION: Elevated pre-treatment NLR, PLR and MLR were associated with worse survival time in HCC patients after hepatic artery interventional therapy. Among them, NLR was an independent prognostic factor for OS.

Prediction of Overall Survival and Progression-Free Survival by the 18F-FDG PET/CT Radiomic Features in Patients with Primary Gastric Diffuse Large B-Cell Lymphoma.

Purpose. To determine whether the radiomic features of 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) contribute to prognosis prediction in primary gastric diffuse large B-cell lymphoma (PG-DLBCL) patients. Methods. This retrospective study included 35 PG-DLBCL patients who underwent PET/CT scans at West China Hospital before curative treatment. The volume of interest (VOI) was drawn around the tumor, and radiomic analysis of the PET and CT images, within the same VOI, was conducted. The metabolic and textural features of PET and CT images were evaluated. Correlations of the extracted features with the overall survival (OS) and progression-free survival (PFS) were evaluated. Univariate and multivariate analyses were conducted to assess the prognostic value of the radiomic parameters. Results. In the univariate model, many of the textural features, including kurtosis and volume, extracted from the PET and CT datasets were significantly associated with survival (5 for OS and 7 for PFS (PET); 7 for OS and 14 for PFS (CT)). Multivariate analysis identified kurtosis (hazard ratio (HR): 28.685, 95% confidence interval (CI): 2.067-398.152, p=0.012), metabolic tumor volume (MTV) (HR: 26.152, 95% CI: 2.089-327.392, p=0.011), and gray-level nonuniformity (GLNU) (HR: 14.642, 95% CI: 2.661-80.549, p=0.002) in PET and sphericity (HR: 11.390, 95% CI: 1.360-95.371, p=0.025) and kurtosis (HR: 11.791, 95% CI: 1.583-87.808, p=0.016), gray-level nonuniformity (GLNU) (HR: 6.934, 95% CI: 1.069-44.981, p=0.042), and high gray-level zone emphasis (HGZE) (HR: 9.805, 95% CI: 1.359-70.747, p=0.024) in CT as independent prognostic factors. Conclusion. 18F-FDG PET/CT radiomic features are potentially useful for survival prediction in PG-DLBCL patients. However, studies with larger cohorts are needed to confirm the clinical prognostication of these parameters.

Pretreatment Inflammatory Indexes as Prognostic Predictors of Survival in Patients Suffering From Synovial Sarcoma.

Background: Inflammatory indexes have been considered as important prognostic factors in various types of cancers. This study aimed to evaluate prognostic values of neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), lymphocyte-to-monocyte ratio (LMR) in patients with synovial sarcoma (SS). Methods: One hundred and three patients diagnosed with SS were collected during 2006-2017 and divided into high or low NLR, PLR, and LMR groups based on receiver operating characteristic curve analysis. Data of clinical variables were collected for univariate and multivariate analyses. The Kaplan-Meier method was used to analyze OS and PFS of SS patients and significance was evaluated by the log-rank test. Results: The optimal cut-off values of NLR, PLR, and LMR were 2.70, 154.99, and 4.16, respectively. Univariate analyses identified resection surgery, distant metastasis, NLR, PLR, and LMR as the potential predictors of progression-free survival (PFS) and overall survival (OS). In the multivariate analyses, NLR was independent predictors for OS (HR 5.074, 95% CI 1.200-21.463, p = 0.027). Resection surgery, metastasis and LMR was independent predictors for PFS (HR 5.328, p = 0.017; HR 3.114, p = 0.04 and HR 0.202, p = 0.025, respectively). Conclusion: Resection surgery, distant metastasis, NLR, and LMR were independent prognostic factors of PFS and OS in patients with synovial sarcoma. Surgery as an effective treatment strategy, other than radiotherapy and chemotherapy, can significantly prolong survival of synovial patients. Clinical utility of these inflammatory biomarkers should be validated in a larger sample size study.