Background and Objectives: Inherited arrhythmia (IA) is a more common cause of sudden cardiac death in Asian population, but little is known about the genetic background of Asian IA probands. We aimed to investigate the clinical characteristics and analyze the genetic underpinnings of IA in a Korean cohort. Methods: This study was conducted in a multicenter cohort of the Korean IA Registry from 2014 to 2017. Genetic testing was performed using a next-generation sequencing panel including 174 causative genes of cardiovascular disease. Results: Among the 265 IA probands, idiopathic ventricular fibrillation (IVF) and Brugada Syndrome (BrS) was the most prevalent diseases (96 and 95 cases respectively), followed by long QT syndrome (LQTS, n=54). Two-hundred-sixteen probands underwent genetic testing, and 69 probands (31.9%) were detected with genetic variant, with yield of pathogenic or likely pathogenic variant as 6.4%. Left ventricular ejection fraction was significantly lower in genotype positive probands (54.7±11.3 vs. 59.3±9.2%, p=0.005). IVF probands showed highest yield of positive genotype (54.0%), followed by LQTS (23.8%), and BrS (19.5%). Conclusions There were significant differences in clinical characteristics and genetic yields among BrS, LQTS, and IVF. Genetic testing did not provide better yield for BrS and LQTS. On the other hand, in IVF, genetic testing using multiple gene panel might enable the molecular diagnosis of concealed genotype, which may alter future clinical diagnosis and management strategies.

BACKGROUND: Vitamin K antagonist (VKA) to prevent thromboembolism in non-valvular atrial fibrillation (NVAF) patients has limitations such as drug interaction. This study investigated the clinical characteristics of Korean patients treated with VKA for stroke prevention and assessed quality of VKA therapy and treatment satisfaction. METHODS: We conducted a multicenter, prospective, non-interventional study. Patients with CHADS2 ≥ 1 and treated with VKA (started within the last 3 months) were enrolled from April 2013 to March 2014. Demographic and clinical features including risk factors of stroke and VKA treatment information was collected at baseline. Treatment patterns and international normalized ratio (INR) level were evaluated during follow-up. Time in therapeutic range (TTR) > 60% indicated well-controlled INR. Treatment satisfaction on the VKA use was measured by Treatment Satisfaction Questionnaire for Medication (TSQM) after 3 months of follow-up. RESULTS: A total of 877 patients (age, 67; male, 60%) were enrolled and followed up for one year. More than half of patients (56%) had CHADS2 ≥ 2 and 83.6% had CHA2DS2-VASc ≥ 2. A total of 852 patients had one or more INR measurement during their follow-up period. Among those patients, 25.5% discontinued VKA treatment during follow-up. Of all patients, 626 patients (73%) had poor-controlled INR (TTR < 60%) measure. Patients' treatment satisfaction measured with TSQM was 55.6 in global satisfaction domain. CONCLUSION: INR was poorly controlled in Korean NVAF patients treated with VKA. VKA users also showed low treatment satisfaction.
Atrial Fibrillation* ; Drug Interactions ; Follow-Up Studies ; Humans ; International Normalized Ratio ; Male ; Prospective Studies ; Risk Factors ; Stroke ; Thromboembolism ; Vitamin K* ; Vitamins*

PURPOSE: The aim of this study was to analyze anatomical popliteal artery entrapment syndrome (PAES) and to individualize the treatment of this condition according to the anatomical status of the artery and the adjacent structure. METHODS: A total of 35 anatomical PAES legs in 23 consecutive patients treated within the Asan Medical Center, Seoul, Korea between 1995 and 2011 were analyzed retrospectively. Anatomical PAES was diagnosed by MRI and/or CT scans of the knee joint, and CT or conventional transfemoral arteriography of the lower extremities. RESULTS: We noted a type II gastrocnemius medial head (GNM) anomaly, a type III GNM anomaly, or an aberrant plantaris muscle in 51.4%, 20%, and 28.6% of PAES legs, respectively. In assessments of the arterial lesions, popliteal or tibial artery occlusion was noted in 19 of 26 symptomatic PAES legs. For cases without popliteal artery lesions, myotomy of the anatomically deranged muscle was performed in 5 of 7 symptomatic and 4 of 9 asymptomatic PAES legs. For occluded popliteal arteries, we performed ten direct repairs of the pathological popliteal artery and 4 femoro-below the knee popliteal bypass surgeries. As a result of the arterial Surgery, 9 direct procedures with myotomy yielded a patent artery, while 3 graft failures were noted in the bypass group. The median follow-up period was 84 months (range, 12–206 months). CONCLUSION: We recommend that treatment of PAES should be individualized based on pathology, symptoms, and various imaging studies.
Angiography ; Arteries ; Chungcheongnam-do ; Follow-Up Studies ; Head ; Humans ; Knee ; Knee Joint ; Korea ; Leg ; Lower Extremity ; Magnetic Resonance Imaging ; Muscle, Skeletal ; Pathology ; Popliteal Artery* ; Retrospective Studies ; Seoul ; Tibial Arteries ; Tomography, X-Ray Computed ; Transplants

BACKGROUND AND OBJECTIVES: Despite significant advances in the treatment of acute myocardial infarction (MI), the prevention of sudden cardiac death (SCD), the most common mode of death in patients with MI, remains challenging. Furthermore, previous Korean MI registries did not address the issue of post-MI SCD. Additional risk stratifiers of post-MI SCD are still required to compensate for the limitation of using left ventricular ejection fraction to predict lethal arrhythmic events. SUBJECTS AND METHODS: We designed the first Korean prospective nationwide multicenter registry primarily focused on SCD; the Korean noninvasive Risk Evaluation study for sudden cardiac DEath From INfarction or heart failurE (K-REDEFINE). The registry consists of 2 groups of patients presenting with (1) acute MI or (2) acute heart failure (HF) at 25 tertiary referral cardiovascular centers. The primary endpoint of the MI group study of K-REDEFINE registry is the incidence and risk factors of post-MI SCD. In particular, the association between the risk of SCD and non-invasive Holter-based electrocardiogram (ECG) variables will be evaluated, such as T-wave alternans (marker of repolarization heterogeneity) and heart rate turbulence/variability (a marker of autonomic function). Other secondary study outcomes include atrioventricular arrhythmias, HF-related admission, repeated myocardial ischemic events, stroke, and overall deaths. CONCLUSION AND PERSPECTIVE: The K-REDEFINE registry will provide new prospects for the better management of MI patients with high risk of SCD by clarifying the burden and predictors of SCD and the clinical utility of various non-invasive ambulatory ECG-based variables in risk stratification for SCD in this patient population.
Arrhythmias, Cardiac ; Death, Sudden, Cardiac* ; Electrocardiography ; Heart Failure* ; Heart Rate ; Heart* ; Humans ; Incidence ; Infarction* ; Myocardial Infarction* ; Prospective Studies ; Referral and Consultation ; Registries ; Risk Factors ; Stroke ; Stroke Volume

BACKGROUND AND OBJECTIVES: Sudden cardiac death (SCD) is one of the most common causes of death in patients with heart failure (HF). However, there are no available data on SCD in previous Korean HF registries. Additionally, although widely used, the utility of left ventricular (LV) ejection fraction (EF) in risk stratification for SCD is limited. SUBJECTS AND METHODS: The Korean non-invasive Risk Evaluation study for sudden cardiac DEath From INfarction or heart failurE (KREDEFINE) is the first Korean prospective, nationwide multicenter registry, primarily focused on SCD. The registry consists of 2 groups of patients presenting with (1) acute HF or (2) acute myocardial infarction (MI) at 25 tertiary referral cardiovascular centers. Using the HF-group data of the K-REDEFINE registry, the incidence and risk factors of SCD in patients with HF will be assessed. In particular, the efficacy of Holter-based ECG variables, such as T-wave alternans (marker of repolarization heterogeneity) and heart rate turbulence/ variability (maker of autonomic function), in risk stratification for SCD will be evaluated. Other cardiovascular outcomes will also be analyzed, including atrioventricular arrhythmias, HF-related admission, stroke, and overall deaths. CONCLUSION AND PERSPECTIVE: The K-REDEFINE registry will pave the way for better management of patients with HF at high risk of SCD by elucidating the burden and risk factors of SCD and the clinical utility of various non-invasive ambulatory ECG-based parameters in risk stratification for SCD in this patient population.
Arrhythmias, Cardiac ; Cause of Death ; Death, Sudden, Cardiac* ; Electrocardiography ; Heart Failure* ; Heart Rate ; Heart* ; Humans ; Incidence ; Infarction* ; Myocardial Infarction ; Prospective Studies ; Referral and Consultation ; Registries ; Risk Factors ; Stroke

Neurogenic pulmonary edema (NPE) leading to cardiopulmonary dysfunction is a potentially life-threatening complication in patients with central nervous system lesions. This case report describes a 28-yr woman with life-threatening fulminant NPE, which was refractory to conventional respiratory treatment, following the rupture of an aneurysm. She was treated successfully with extracorporeal membrane oxygenation (ECMO), although ECMO therapy is generally contraindicated in neurological injuries such as brain trauma and diseases that are likely to require surgical intervention. The success of this treatment suggests that ECMO therapy should not be withheld from patients with life-threatening fulminant NPE after subarachnoid hemorrhage.
Adult ; Brain/radiography ; Decompressive Craniectomy ; Extracorporeal Membrane Oxygenation ; Female ; Humans ; Intracranial Aneurysm/complications/*diagnosis ; Pulmonary Edema/*diagnosis/etiology/therapy ; Subarachnoid Hemorrhage/etiology ; Tomography, X-Ray Computed

The purpose of this study was to investigate the anti-hyperlipidemic effect of soy bean extract solution fermented by Bacillus subtilis MORI (BTD-1E) in obese db/db mice. Eight-week-old male db/db mice were administered 33.3 mg/kg BTD-1E solution orally once a day for four weeks. The BTD-1E group showed significantly lower body weight compared with the db control group (P<0.05). The BTD-1E group showed significantly lower serum total cholesterol and LDL cholesterol levels compared with the db control group, respectively (P<0.05, P<0.01). The BTD-1E group showed significantly decreased liver weight relative to final body weight compared with the db control group (P<0.01). After four weeks of BTD-1E administration, lipid droplets in the liver were apparently decreased in the BTD-1E group compared to the db control group. In summary, our results suggest that BTD-1E has an anti-hyperlipidemic effect in the obese mouse model.
1-Deoxynojirimycin ; Animals ; Bacillus ; Bacillus subtilis ; Body Weight ; Cholesterol ; Cholesterol, LDL ; Humans ; Liver ; Male ; Mice ; Mice, Obese ; Soybeans

OBJECTIVE: To optimize the recanalization of acute cerebral stroke that were not effectively resolved by conventional intraarterial thrombolysis (IAT), we designed a double device technique to allow for rapid and effective reopening. In this article, we describe the feasibility and efficacy of this technique. METHODS: From January 2008 to September 2009, twenty patients with acute cerebral arterial occlusion (middle cerebral artery : n=12; internal carotid artery terminus : n=5; basilar artery : n=3) were treated by the double device technique. This technique was applied when conventional thrombolytic methods using drug, microwires, microcatheters and balloons did not result in recanalization. In the double device technique, two devices are simultaneously placed at the lesion (for example, one microcatheter and one balloon or two microcatheters). Chemicomechanical or mechanicomechanical thrombolysis was performed simultaneously using various combinations of two devices. Recanalization rates, procedural time, complications, and clinical outcomes were analyzed. RESULTS: The initial median National Institute of Health Stroke Scale (NIHSS) was 16 (range 5-26). The double device technique was applied after conventional IAT methods failed. Recanalization was achieved in 18 patients (90%). Among them, 55% (11 cases) were complete (thrombolysis in cerebral infarction 2B, 3). The median thrombolytic procedural time including the conventional technique was 135+/-83.7 minutes (range 75-427). Major symptomatic hemorrhages (neurological deterioration > or =4 points in NIHSS) developed in two patients (10%). Good long term outcomes (modified Rankin Scale < or =2 at 90 days) occurred in 25% (n=5) of the cases. Mortality within 90 days developed in two cases (10%). CONCLUSION: The double device technique is a feasible and effective technical option for large vessel occlusion refractory to conventional thrombolysis.
Basilar Artery ; Carotid Artery, Internal ; Cerebral Arteries ; Cerebral Infarction ; Glycosaminoglycans ; Hemorrhage ; Humans ; Stroke

Two male patients who presented with altered mental states and hemiparesis were treated by retrieval thrombectomy. The occlusion sites were M1 in both patients. During each thrombectomy, a self-expanding and fully retrievable Solitaire stent was partially deployed to cover the whole intra-arterial clot and then was retrieved slowly while occluding the internal cerebral artery with a balloon-guiding catheter. Complete recanalization (defined as thrombolysis of cerebral infarction grade 2b or 3) was achieved in both patients. The procedural time from groin puncture to recanalization was 17 min and 30 min, respectively. Immediate post-operative National Institutes of Health Stroke scores improved to 17 from 22 in one patient and to 19 from 24 in the other patient. There were no procedure-related complications including distal embolisms or post-operative intracranial hemorrhages. We suggest that this technique may be a simple, rapid, and safe thrombolytic method for acute ischemic stroke patients with large artery occlusions.
Arteries ; Catheters ; Cerebral Arteries ; Cerebral Infarction ; Embolism ; Groin ; Humans ; Intracranial Hemorrhages ; Male ; National Institutes of Health (U.S.) ; Paresis ; Punctures ; Stents ; Stroke ; Thrombectomy

A young woman who had a delivery history 3 months previously presented with dyspnea and orthopnea. Initial findings of physical examination, chest radiography, and echocardiogram showed typical congestive heart failure with severe left ventricular (LV) dysfunction. At first, we considered peripartum cardiomyopathy because she had given birth to a baby 3 months previously. However, even though we massively tried conventional drug therapy for 10 days, the patient still remained with refractory heart failure. We performed additional laboratory studies such as complement level and autoantibodies, of which the results supported systemic lupus erythematosus. We could make the diagnosis of acute lupus myocarditis and treated her with corticosteroid. The symptoms were dramatically disappeared and LV function also improved.
Acute Disease ; Administration, Oral ; Adult ; Echocardiography ; Female ; Glucocorticoids/administration & dosage ; Heart Failure/*diagnosis ; Humans ; Lupus Erythematosus, Systemic/complications/*diagnosis/radiography ; Methylprednisolone/administration & dosage ; Myocarditis/*diagnosis/etiology/radiography ; Prednisolone/administration & dosage