In hypertrophic cardiomyopathy, myocardial replacement fibrosis was weakly correlated with left ventricular hypertrophy at cardiac MRI, was inhomogeneous and asymmetric, and was of prognostic signi... Background Myocardial replacement fibrosis is one of the major histologic features of hypertrophic cardiomyopathy (HCM), but its characteristics have not been well delineated. Purpose To clarify th...

Childhood Takayasu’s arteritis (c-TA) is scarcely reported but is characterized by devastating morbidity and mortality. This study aims to investigate the clinical course of c-TA and prognostic factors associated with rehospitalization and events including vascular complications, flares, and death. An ambispective study of 101 c-TA patients satisfying the American College of Rheumatology (ACR) criteria and/or the European League against Rheumatism (EULAR)/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS) criteria was conducted from January 2002 to December 2017. Data on demographic, clinical, laboratory, imaging, and therapeutic features were collected. Event-free survival, complication-free survival, flare-free survival, rehospitalization-free survival, and associated prognostic factors were assessed by Kaplan-Meier survival curve and propensity score analysis. The median age at c-TA onset was 14 (interquartile range (IQR) 12–16) years and 76.2% were female. Hypertension (70.3%), blood pressure discrepancy (55.4%), bruits (51.5%), and pulse deficits (37.6%) were core presentations. Major vascular involvement included the renal artery (62.4%), abdominal aorta (42.6%), subclavian artery (43.6%), and carotid artery (42.6%). Glucocorticoids (78.2%), antihypertensive drugs (72.3%), antiplatelet agents (72.3%), and revascularization (57.4%) were made up the majority administered. At a median 2.4 (IQR 0.7–6.1) years of follow-up, events, rehospitalization, vascular complications, flares and death were observed in 44.6%, 37.6%, 44.6%, 26.7%, and 3%, respectively. The 5-year event-free survival, rehospitalization-free survival, vascular complication-free survival, and flare-free survival were 42.8%, 55.8%, 45.9%, and 62.3%, respectively. Body mass index (BMI) (hazard ratio (HR) = 0.49, 95% confidence interval (CI) 0.30–0.81, p = 0.005), stroke (HR = 7.37, 95% CI 2.35–23.1, p = 0.001), and revascularization (HR = 0.51, 95% CI 0.27–0.94, p = 0.032) were independent prognostic predictors of events. Predictors for rehospitalization include age at admission (HR = 0.81, 95% CI 0.69–0.94, p = 0.006), renal artery involvement (HR = 0.49, 95% CI 0.25–0.96, p = 0.037), and elevated C-reactive protein (CRP; HR = 2.50, 95% CI 1.24–5.00, p = 0.01). BMI level (p = 0.024) and renal artery involvement (p = 0.015) were also associated with vascular complications, while revascularization (p = 0.002) independently correlated with re-flares. This large ambispective study of c-TA revealed an early 3% mortality at the first year and around 50% morbidity within 5 years after diagnosis. Hypertension, renal artery involvement, and revascularization based on anti-inflammation, antihypertension, and antiplatelet medications dominated c-TA with indications for optimistic prognosis. Patients with initial lower BMI level, a younger age at admission, stroke, and elevated CRP have a high risk of poor outcomes, requiring close c-TA monitoring and more aggressive management. NCT03199183 , unique protocol ID: 2016-ZX43. June 26, 2017

Aims: The predictive value of white blood cells in triple-vessel coronary artery disease (TVD) remains unclear. This study aimed to examine the relationship between WBC counts and long-term prognosis of TVD. Methods: A total of 8943 consecutive patients with triple-vessel coronary artery disease were enrolled from April 2004 to February 2011. The primary endpoint was all-cause death and the secondary endpoints were major adverse cardiovascular and cerebrovascular events (MACCEs; a composite of all-cause death, myocardial infarction or stroke). Results: After a median of 7.5 years of follow-up, 7678 patients were included in the final analysis. Multivariable analysis showed that the white blood cell count was an independent predictor of death (hazard ratio: 1.04, p < 0.01) and MACCE (hazard ratio: 1.03, p = 0.02). In white blood cell differential analysis, increased monocytes (hazard ratio: 1.93, p = 0.001) and eosinophils (hazard ratio: 1.82, p < 0.01), and decreased lymphocytes (hazard ratio: 0.89, p = 0.02) were independent predictors of death. Increased monocytes (hazard ratio: 1.62, P = 0.002) and eosinophils (hazard ratio: 1.55, p < 0.01) were independent predictors of MACCE. A combination of monocyte, lymphocyte and eosinophil counts with the Synergy between Percutaneous Coronary Intervention with Taxus and Cardiac Surgery (SYNTAX) score improved the predictive value for mortality (area under the curve from 0.569 to 0.611; integrated discrimination improvement = 0.012; net reclassification improvement = 0.299) and improved slightly with SYNTAX score II (all p < 0.05). Conclusion: Total and differential white blood cell counts are independent prognostic factors of long-term mortality and MACCE in triple-vessel coronary artery disease. A combination of monocyte, lymphocyte and eosinophil counts improved the predictive value for mortality with the SYNTAX score, and improved it slightly with SYNTAX score II.

Aims: The present study sought to determine the rate and prognostic implications of post-procedural physiologically significant residual ischemia according to Murray law-based quantitative flow ratio (μQFR) after left main (LM) bifurcation percutaneous coronary intervention (PCI). Methods and results: Consecutive patients undergoing LM bifurcation stenting at a large tertiary care center between January 2014 and December 2016 with available post-PCI μQFR were included. Physiologically significant residual ischemia was defined by post-PCI μQFR values ≤0.80 in the left anterior descending (LAD) or left circumflex artery (LCX). The primary outcome was 3-year cardiovascular death. The major secondary outcome was 3-year bifurcation-oriented composite endpoint (BOCE). Among 1170 included patients with analyzable post-PCI μQFR, 155 (13.2%) had residual ischemia in either LAD or LCX. Patients with vs. those without residual ischemia had a higher risk of 3-year cardiovascular mortality [5.4% vs. 1.3%; adjusted hazard ratio (HR) 3.20, 95% confidence interval (CI): 1.16–8.80]. The 3-year risk of BOCE was significantly higher in the residual ischemia group (17.8% vs. 5.8%; adjusted HR 2.79, 95% CI: 1.68–4.64), driven by higher incidence of the composite of cardiovascular death and target bifurcation-related myocardial infarction (14.0% vs. 3.3%; adjusted HR 4.06, 95% CI: 2.22–7.42). A significant, inverse association was observed between continuous post-PCI μQFR and the risk of clinical outcomes (per 0.1 μQFR decrease, HR of cardiovascular death 1.27, 95% CI: 1.00–1.62; HR of BOCE 1.29, 95% CI: 1.14–1.47). Conclusion: After angiographically successful LM bifurcation PCI, residual ischemia assessed by μQFR was identified in 13.2% of patients and was associated with higher risk of 3-year cardiovascular death, indicating the superior prognostic value of post-PCI physiological assessment.