Korean Circulation Journal最新文献

Background and objectives: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a life-threatening inherited arrhythmogenic disorder. Recently, RYR2, the major CPVT-causative gene, was associated with neuropsychiatric manifestations. We aimed to analyze the clinical presentations, neuropsychiatric manifestations, and treatment outcomes of children with CPVT.

Methods: We retrospectively reviewed 23 patients diagnosed with CPVT before 19 years of age. Genetic analysis, history of neuropsychiatric manifestations, changes in ventricular arrhythmia burden before and after treatment, occurrence of cardiac events, and overall survival (OS) were investigated.

Results: RYR2 variants were identified in 17 patients, and 14 were classified as pathogenic or likely pathogenic. Neuropsychiatric manifestations, including intellectual disability and attention deficit hyperactivity disorder, were identified in 10 patients (43.5%). The 5-year cardiac event-free survival rate was 31.2%, and the 10-year OS rate was 73.1%. Patients diagnosed since 2009 had a higher cardiac event-free survival rate than those diagnosed before 2009 (p=0.0028). Combined beta-blocker and flecainide therapy demonstrated a lower risk of cardiac events than beta-blocker monotherapy (hazard ratio [HR], 0.08; 95% confidence interval [CI], 0.02-0.38; p=0.002). Left cardiac sympathetic denervation (LCSD) reduced the ventricular arrhythmia burden in Holter monitoring. Occurrence of near-fatal cardiac events after diagnosis was an independent predictor of death (HR, 33.40; 95% CI, 6.23-179.95; p<0.001).

Conclusions: Neuropsychiatric manifestations are common in children with CPVT. Flecainide and/or LCSD, when added to beta-blocker therapy, reduce the ventricular arrhythmia burden and cardiac events, thereby improving treatment outcomes in recent years.

Background and objectives: A comprehensive survey of congenital heart disease (CHD) prevalence has not yet been conducted in South Korea. This study aimed to investigate the prevalence of CHDs in Korean children and lay the foundation for national CHD epidemiology.

Methods: Target patients were infantile crucial CHDs, which include critical CHDs (requiring urgent procedures after birth with common hypoxemic defects) and diverse categorical defects excluding simple shunt defects. Data were obtained from the National Health Insurance Service over a 5-year period (2014-2018). Birth prevalence (new cases per 1,000 live births) of CHDs in Korea was analyzed and compared with that of other countries.

Results: The birth prevalences of right heart obstructive defects (pulmonary valve stenosis and pulmonary atresia), conus anomalies (tetralogy of Fallot and double outlet right ventricle), and total anomalous pulmonary venous return showed significant increases in the East Asian group (P<0.001), whereas those of left heart obstructive defects (coarctation of aorta, aortic stenosis, and hypoplastic left heart syndrome), truncus anomalies (D-transposition of great artery and persistent truncus arteriosus), atrioventricular septal defect, and hypoplastic right heart syndrome were significantly decreased in the East Asian group (P<0.001).

Conclusions: The overall birth prevalence of crucial CHDs in Korea was similar to that of critical CHDs in previous studies from other countries. Some subtypes of right heart obstructive defects, left heart obstructive defects, and conotruncal anomalies showed significant differences between East Asian and Western populations. This study contributes to a foundation for national CHD epidemiology in Korean children.

Heart failure (HF) in children is a complex syndrome with multiple diverse etiologies and both acute and chronic presentations. Chronic presentations can persist throughout childhood and adolescence, and require diligent management with ongoing reassessment to maximize survival and quality of life. Stages of HF are key to recognize as they guide both management and inform prognosis. In more severe cases, children can present with signs of low cardiac output and circulatory collapse with potential to transition either to a chronic HF stage or progress to a need for advanced HF therapies. Morbidity and mortality are high. Managing HF requires a multi-disciplinary approach that can adapt to the needs of the different phases of childhood and adolescence. Treatment can include medications, nutritional support, activity modifications, and potentially surgical intervention, pacemaker, respiratory or mechanical support, or even heart transplantation. Limited evidence exists for almost all medical therapies used in the management of HF in children and approaches are predominantly extrapolated from extensive adult experience. There are multiple maladaptive pathways in the failing heart; medications that modify these maladaptive pathways promote "reverse remodelling" of the myocardium and are key to the management, forming the basis for "guideline directed medical therapy". The purpose of this review is to summarize the current state of the art management of systolic HF in children.

Many countries have published clinical practice guidelines for appropriate clinical decisions, optimal treatment, and improved clinical outcomes in patients with acute coronary syndrome. Developing guidelines that are specifically tailored to the Korean environment is crucial, considering the treatment system, available medications and medical devices, racial differences, and level of language communication. In 2017, the Korean Society of Myocardial Infarction established a guideline development committee. However, at that time, it was not feasible to develop guidelines, owing to the lack of knowledge and experience in guideline development and the absence of methodology experts. In 2022, the National Evidence-Based Healthcare Collaborating Agency collaborated with a relevant academic association to develop internationally reliable guidelines, with strict adherence to the methodology for evidence-based guideline development. The first Korean acute coronary syndrome guideline starts from the 9 key questions for pharmacotherapy.

Background and objectives: We investigated the clinical benefit of anticoagulation with non-vitamin K antagonist oral anticoagulant (NOAC) in very elderly atrial fibrillation (AF) patients through national healthcare insurance registry.

Methods: Clinical data was acquired from the National Health Insurance Service of south Korea. Medical records of 862,935 patients who were diagnosed with AF from 2015 to 2020 were collected for analysis. Patients under the age of 85, prior history of intracranial hemorrhage, gastrointestinal bleeding and prior prescription days of aspirin, warfarin or NOAC exceeding 90 along with follow up period less than 90 days were excluded.

Results: A total of 10,625 patients were eligible for analysis. Patients with oral anticoagulant (hazard ratio [HR], 0.60, 95% confidence interval [CI], 0.53-0.69, p<0.001) showed higher efficacy regarding cerebrovascular accident (CVA) compared to aspirin (HR, 0.84, 95% CI, 0.74-0.95, p=0.008) and no treatment group. Individual comparison of NOAC and aspirin via propensity score matching showed that patients with NOAC (HR, 0.71, 95% CI, 0.61-0.85, p<0.001) showed higher event free survival regarding CVA compared to aspirin. Bleeding risk was also higher for NOAC (HR, 1.28, 95% CI, 1.07-1.56, p=0.006) group but did not result in commensurate increase in mortality (HR, 0.60, 95% CI, 0.45-0.81, p<0.001).

Conclusions: Anticoagulation with NOAC in very elderly patient showed higher event free survival regarding CVA. Despite having higher event rate of bleeding, eventual death was lower for NOAC.

Background and objectives: Guidelines recommend target levels of low-density lipoprotein cholesterol (LDL-C) and intensive lipid-lowering therapy (LLT) in high-risk patients. However, the value of escalating LLT when the LDL-C targets are achieved with moderate-intensity statins is unknown. We aimed to evaluate the benefits of LLT escalation in this population.

Methods: In this retrospective propensity score-matched study, we screened data from two university hospitals between 2006 and 2021. Of the 54,069 patients with atherosclerotic cardiovascular disease (ASCVD), 3,205 who achieved LDL-C levels <70 mg/dL with moderate-intensity statins were included. After 1:3 matching, 1,315 patients (339 with LLT escalation and 976 without) were ultimately examined. The primary outcomes were major adverse cardiovascular and cerebrovascular events (MACCE)1 (cardiovascular death, nonfatal myocardial infarction, and nonfatal ischemic stroke) and all-cause death.

Results: During a median follow-up of 5.7 years, the MACCE1 rate was not significantly lower in the escalation group than in the non-escalation group (9.8 and 14.3/1,000 person-years, respectively; hazard ratio [HR], 0.68; 95% confidence interval [CI], 0.43-1.09; p=0.11). Kaplan-Meier curves showed similar results (log-rank p=0.11). The risk of all-cause death did not differ between the groups. MACCE2 rate, which additionally includes coronary/peripheral revascularization, was lower in the escalation group (24.5 and 35.4/1,000 person-years, respectively; HR, 0.70; 95% CI, 0.52-0.94; p=0.017).

Conclusions: LLT escalation did not significantly lower hard cardiovascular outcomes and all-cause death in patients with ASCVD achieving LDL-C levels <70 mg/dL with moderate-intensity statins. However, it had benefit in reducing revascularization rates in this population.

Background and objectives: There is no dedicated occlusive device for closing coronary artery fistulas (CAFs), and specific efficacy and safety data of various off-label occlusive devices for CAFs closure are scarce.

Methods: Patients undergoing transcatheter closure of CAFs from January 2011 to December 2022 were included in the single-center retrospective study. The study population was divided into 2 groups: coils group (n=35) and patent ductus arteriosus (PDA) occluders group (n=66).

Results: No significant intergroup differences were observed in demographic characteristics except age. The presence of multiple CAF origins (54.3% vs. 4.5%, p<0.001) and multiple draining sites (51.4% vs. 3.0%, p<0.001) were more common in the coils group. In contrast, the presence of aneurysm (72.7% vs. 14.3%, p<0.001), and large fistula (75.8% vs. 37.1%, p<0.001) were more prevalent in the PDA occluders group. The acute procedural success rate of the PDA occluders group was higher compared to that of the coils group (87.9% vs. 62.9%, adjusted odds ratio [OR], 7.20; 95% confidence interval, 1.59-32.64; p=0.01). In addition, no significant intergroup differences were noted in both the recanalization rate (7.8% vs. 20%, p=0.107) and the reintervention rate (3.1% vs. 8.6%, p=0.342).

Conclusions: Transcatheter closure of CAFs using PDA occluders was associated with significantly higher acute procedural success rates compared to coil embolization with comparable late outcomes.

Background and objectives: This study aimed to investigate the roles of lncRNA uc003pxg.1 and miR-339-5p in regulating the occurrence and development of coronary heart disease.

Methods: First, the expression levels of uc003pxg.1 and miR-339-5p were verified in peripheral blood mononuclear cells of clinical samples. Then, the target gene was identified using high-throughput sequencing combined with bioinformatics. Human umbilical vein endothelial cells (HUVECs) were transfected with si-uc003pxg.1, miR-339-5p mimic and miR-339-5p inhibitor, and the expression of related genes was detected by reverse transcription-quantitative polymerase chain reaction and western blotting. EdU, CCK-8, Cell scratch and Transwell assays were used to analyze the effects of uc003pxg.1 and miR-339-5p on cell proliferation and migration.

Results: The expression of uc003pxg.1 and miR-339-5p was negatively correlated in clinical samples and HUVECs. The si-uc003pxg.1 and miR-339-5p mimic decreased the proliferation and migration of HUVECs and decreased the expression of transforming growth factor (TGF)-β1 and α-smooth muscle actin (SMA). The protein expression levels of TGF-β1, α-SMA, CD31, collagen I, collagen III and endoglin were decreased, and angiogenesis was weakened. The miR-339-5p inhibitor had the opposite effect.

Conclusions: Our study revealed that upregulation of uc003pxg.1 and downregulation of miR-339-5p in vitro promote cell proliferation, cell migration and angiogenesis and upregulate the expression of TGF-β1, α-SMA, CD31, collagen I, collagen III and endoglin, which may lead to the development of vascular atherosclerosis.