ABSTRACT
Hypertrophic cardiomyopathy (HCM) is a genetic disease associated with a risk of malignant ventricular tachyarrhythmias and sudden cardiac death (SCD). Assessment of the SCD risk is crucial for its clinical management, and there has been considerable interest in developing risk stratification strategies. An implantable cardioverter-defibrillator (ICD) is a life-saving treatment for patients with HCM who are at a high-risk of ventricular tachyarrhythmias and SCD. However, a substantial number of ICD recipients experience adverse effects arising from inappropriate device therapy and implant-related complications. This has led to numerous investigations of the risk of SCD and the indications for ICD implantation. American guidelines were recently updated to include new risk markers, including left ventricular systolic dysfunction, apical aneurysm, and extensive late gadolinium enhancement, while European guidelines recommend individualized estimated 5-year SCD risk assessment models. Studies evaluating other risk factors for SCD in patients with HCM have also been published. Drawing on recent guidelines and publications on clinical risk factors, we focus this review on updated risk assessments for SCD with ICD therapy in patients with HCM.
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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.
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Optimized management of atrial fibrillation requires patient-oriented decision making with a multidisciplinary approach. This report incorporates recent authoritative studies to provide detailed recommendations for managing atrial fibrillation in specific clinical settings. The principles of the Atrial fibrillation Better Care (ABC) pathway apply in these clinical settings. In addition, specific considerations are discussed for each of these conditions and populations.
ABSTRACT
Optimized management of atrial fibrillation requires patient-oriented decision making with a multidisciplinary approach. This report incorporates recent authoritative studies to provide detailed recommendations for managing atrial fibrillation in specific clinical settings. The principles of the Atrial fibrillation Better Care (ABC) pathway apply in these clinical settings. In addition, specific considerations are discussed for each of these conditions and populations.
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Background@#This study evaluated the status of single nucleotide polymorphisms (SNPs) in Korean patients with early-onset (< 40 years old) atrial fibrillation (AF) and their effects on the outcome after catheter ablation. @*Methods@#A total of 89 patients (35.7 ± 3.7 years, 81 males) with drug-refractory AF (paroxysmal 64.0%) who underwent catheter ablation were included in this study. Sixteen SNPs, including rs13376333, rs10465885, rs10033464, rs2200733, rs17042171, rs6843082, rs7193343, rs2106261, rs17570669, rs853445, rs11708996, rs6800541, rs251253, rs3807989, rs11047543, and rs3825214, were genotyped. Serial 48-hour Holter monitoring was conducted to detect AF recurrences during long-term follow up. @*Results@#Wild-type genotypes of rs11047543 (GG; 26/69 [37.7%] vs. GA; 13/18 [72.2%] vs.AA; 0/0 [0%], P = 0.009) and rs7193343 (CC; 0/7 [0%] vs. CT; 22/40 [55.0%] vs. TT; 18/41 [43.9%], P = 0.025) and the homozygous variant of rs3825214 (AA; 16/31 [51.6%] vs. AG; 22/43 [51.2%] vs. GG; 2/13 [15.4%], P = 0.056) were significantly associated with a lower rate of late recurrence. When the patients were assigned to four groups according to the number of risk alleles (n = 0–3), there were significant differences in recurrence rate (n = 0; 0/3 vs. n = 1; 2/13 [15.4%] vs. n = 2; 24/52 [46.2%] vs. n = 3; 13/17 [76.5%], P = 0.003). When correcting for multiple variables, rs11047543 (hazard ratio [HR], 2.723; 95% confidence interval [CI], 1.358–5.461; P = 0.005) and the number of risk alleles (HR, 2.901; 95% CI, 1.612–5.219; P < 0.001) were significantly associated with recurrence of AF after catheter ablation. @*Conclusion@#Polymorphisms on rs7193343 closest to ZFHX3 (16q22), rs3825214 near to TBX5 (12q24), and rs11047543 near to SOX5 (12p12) modulate the risk for AF recurrence after catheter ablation. The number of risk alleles of these 3 SNPs was an independent predictor of recurrence during long-term follow up in Korean patients with early-onset AF.
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Background@#There is little data regarding types of idiopathic premature ventricular complex (PVC) according to heart rate dependence. @*Methods@#One hundred and sixty-eight patients with idiopathic PVC were enrolled in this study. Evaluation of the number of PVCs and total ventricular beats, and the density of PVC was done using 24 h Holter monitoring. Patients were divided into groups as having: fast rate-dependent (Group I), slow rate-dependent (Group II), and heart rateindependent PVC (Group III) based on the relationship between the number of hourly PVC and hourly heart rate. After ß-blocker medication, 24 h Holter monitoring was repeated. @*Results@#Among the 168 subjects, 66 (39.3%) patients were in Group I, 18 (10.7%) in Group II, and 84 (50.0%) in Group III. There were no significant differences in the baseline number of PVCs and total ventricular beats, and the density of PVC among the three groups. The number of PVCs was significantly reduced in patients with Group I (14,030 ± 11,463 beats/day vs. 7401 ± 10,464 beats/day, p < 0.001), and total ventricular beat was significantly reduced in patients with Group I (109,223 ± 17,564 beats/day vs. 96,182 ± 15,594 beats/day, p < 0.001) and Group III (106,515 ± 13,468 beats/ day vs. 97,995 ± 12,960 beats/day, p < 0.001) after ß-blocker medication. The density of PVC was significantly reduced only in patients of Group I (12.9 ± 10.3% vs. 7.4 ± 10.3%, p = 0.001) after ß-blocker medication. @*Conclusions@#The type of PVC according to the heart rate dependence should be considered when treating idi‑ opathic PVC with ß-blockers.
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Background@#This study evaluated the status of single nucleotide polymorphisms (SNPs) in Korean patients with early-onset (< 40 years old) atrial fibrillation (AF) and their effects on the outcome after catheter ablation. @*Methods@#A total of 89 patients (35.7 ± 3.7 years, 81 males) with drug-refractory AF (paroxysmal 64.0%) who underwent catheter ablation were included in this study. Sixteen SNPs, including rs13376333, rs10465885, rs10033464, rs2200733, rs17042171, rs6843082, rs7193343, rs2106261, rs17570669, rs853445, rs11708996, rs6800541, rs251253, rs3807989, rs11047543, and rs3825214, were genotyped. Serial 48-hour Holter monitoring was conducted to detect AF recurrences during long-term follow up. @*Results@#Wild-type genotypes of rs11047543 (GG; 26/69 [37.7%] vs. GA; 13/18 [72.2%] vs.AA; 0/0 [0%], P = 0.009) and rs7193343 (CC; 0/7 [0%] vs. CT; 22/40 [55.0%] vs. TT; 18/41 [43.9%], P = 0.025) and the homozygous variant of rs3825214 (AA; 16/31 [51.6%] vs. AG; 22/43 [51.2%] vs. GG; 2/13 [15.4%], P = 0.056) were significantly associated with a lower rate of late recurrence. When the patients were assigned to four groups according to the number of risk alleles (n = 0–3), there were significant differences in recurrence rate (n = 0; 0/3 vs. n = 1; 2/13 [15.4%] vs. n = 2; 24/52 [46.2%] vs. n = 3; 13/17 [76.5%], P = 0.003). When correcting for multiple variables, rs11047543 (hazard ratio [HR], 2.723; 95% confidence interval [CI], 1.358–5.461; P = 0.005) and the number of risk alleles (HR, 2.901; 95% CI, 1.612–5.219; P < 0.001) were significantly associated with recurrence of AF after catheter ablation. @*Conclusion@#Polymorphisms on rs7193343 closest to ZFHX3 (16q22), rs3825214 near to TBX5 (12q24), and rs11047543 near to SOX5 (12p12) modulate the risk for AF recurrence after catheter ablation. The number of risk alleles of these 3 SNPs was an independent predictor of recurrence during long-term follow up in Korean patients with early-onset AF.
ABSTRACT
Background@#There is little data regarding types of idiopathic premature ventricular complex (PVC) according to heart rate dependence. @*Methods@#One hundred and sixty-eight patients with idiopathic PVC were enrolled in this study. Evaluation of the number of PVCs and total ventricular beats, and the density of PVC was done using 24 h Holter monitoring. Patients were divided into groups as having: fast rate-dependent (Group I), slow rate-dependent (Group II), and heart rateindependent PVC (Group III) based on the relationship between the number of hourly PVC and hourly heart rate. After ß-blocker medication, 24 h Holter monitoring was repeated. @*Results@#Among the 168 subjects, 66 (39.3%) patients were in Group I, 18 (10.7%) in Group II, and 84 (50.0%) in Group III. There were no significant differences in the baseline number of PVCs and total ventricular beats, and the density of PVC among the three groups. The number of PVCs was significantly reduced in patients with Group I (14,030 ± 11,463 beats/day vs. 7401 ± 10,464 beats/day, p < 0.001), and total ventricular beat was significantly reduced in patients with Group I (109,223 ± 17,564 beats/day vs. 96,182 ± 15,594 beats/day, p < 0.001) and Group III (106,515 ± 13,468 beats/ day vs. 97,995 ± 12,960 beats/day, p < 0.001) after ß-blocker medication. The density of PVC was significantly reduced only in patients of Group I (12.9 ± 10.3% vs. 7.4 ± 10.3%, p = 0.001) after ß-blocker medication. @*Conclusions@#The type of PVC according to the heart rate dependence should be considered when treating idi‑ opathic PVC with ß-blockers.
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Non-vitamin K antagonist oral anticoagulants (NOACs) are an alternative to vitamin K antagonists (VKAs) and have emerged as the treatment of choice in Korea. However, several questions remain regarding the optimal use of these agents in specific clinical situations. In this paper we discuss 1) patients with atrial fibrillation (AF) and coronary artery disease, 2) avoiding confusion with NOAC dosing across indications, 3) cardioversion in a patient treated with a NOAC, 4) AF patients who present with acute stroke while on NOACs, 5) NOACs in special situations, 6) anticoagulation in AF patients with a malignancy, and 7) optimizing VKA dose adjustments.
Subject(s)
Humans , Anticoagulants , Atrial Fibrillation , Coronary Artery Disease , Electric Countershock , Heart , Korea , Stroke , Vitamin KABSTRACT
Non-vitamin K antagonist oral anticoagulants (NOACs) are an alternative to vitamin K antagonists (VKAs) and have emerged as the treatment of choice in Korea. However, several questions remain regarding the optimal use of these agents in specific clinical situations. In this paper we discuss 1) patients with atrial fibrillation (AF) and coronary artery disease, 2) avoiding confusion with NOAC dosing across indications, 3) cardioversion in a patient treated with a NOAC, 4) AF patients who present with acute stroke while on NOACs, 5) NOACs in special situations, 6) anticoagulation in AF patients with a malignancy, and 7) optimizing VKA dose adjustments.
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Atrial fibrillation (AF) is the most common form of sustained arrhythmia in elderly patients. However, AF is often detected during health screening, or accidentally during testing for other diseases; some patients lack clinical symptoms. Nevertheless, AF increases the incidence of ischemic stroke and other thrombotic events, and compromises cardiovascular prognosis in terms of heart failure, dementia, and hospitalization. Therefore, initial AF management should be performed at the point of primary care, not only in specialized medical centers. We wish to propose a five-step management protocol for AF. We review the evidence supporting integrated management by primary care physicians new to AF, and by specialized physicians who often diagnose and manage AF. Further, we also outline a structured goal-based follow-up protocol; this is an important part of integrated management.
Subject(s)
Aged , Humans , Arrhythmias, Cardiac , Atrial Fibrillation , Dementia , Follow-Up Studies , Heart Failure , Heart , Hospitalization , Incidence , Integrative Medicine , Mass Screening , Physicians, Primary Care , Primary Health Care , Prognosis , StrokeABSTRACT
Oral anticoagulants (OAC) are necessary to prevent thromboembolism in patients with atrial fibrillation (AF). OACs used in Korea are composed of warfarin and non-vitamin K antagonist OAC. Risk stratification and selection of OACs in patients with AF is usually performed by international guidelines for AF management. However, these guidelines do not always reflect the unique characteristics of AF patients in Korea as they were established based on a small portion of the Asian population and, therefore, have limited application to Korean patients. In addition, under certain conditions, the choice of OACs and doses according to the international guidelines are unsuitable for Korean AF patients. Recently, robust clinical data of Korean AF patients have become available. The Korean AF Management Guideline Committee, as part of the Korean Heart Rhythm Society, analyzed all available studies regarding management of AF including those focusing on Korean patients. Expert consensus and guidelines for optimal management of AF patients in Korea were established following systematic reviews and intensive discussions. This article provides the appropriate choice of OACs and dose for management of Korean AF patients with various clinical conditions.
Subject(s)
Humans , Anticoagulants , Asian People , Atrial Fibrillation , Consensus , Heart , Korea , Stroke , Thromboembolism , WarfarinABSTRACT
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.
Subject(s)
Humans , Arrhythmias, Cardiac , Death, Sudden, Cardiac , Electrocardiography , Heart Failure , Heart Rate , Heart , Incidence , Infarction , Myocardial Infarction , Prospective Studies , Referral and Consultation , Registries , Risk Factors , Stroke , Stroke VolumeABSTRACT
We described a case of a 54-year-old male who presented with dizziness and dyspnea due to cardiac metastasis of leiomyosarcoma. Cardiac metastasis of leiomyosarcoma caused both bradyarrhythmia and tachyarrhythmia in the patient. He was treated with implantation of a permanent pacemaker for management of complete atrio-ventricular block and anti-arrhythmic drug that suppressed ventricular tachycardia successfully.
Subject(s)
Humans , Male , Middle Aged , Bradycardia , Dizziness , Dyspnea , Heart , Leiomyosarcoma , Neoplasm Metastasis , Tachycardia , Tachycardia, VentricularABSTRACT
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.
Subject(s)
Humans , Arrhythmias, Cardiac , Cause of Death , Death, Sudden, Cardiac , Electrocardiography , Heart Failure , Heart Rate , Heart , Incidence , Infarction , Myocardial Infarction , Prospective Studies , Referral and Consultation , Registries , Risk Factors , StrokeABSTRACT
In the article, the 3rd author's affiliation was misspelled.
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PURPOSE: To compare the effectiveness of device closure and medical therapy in prevention of recurrent embolic event in the Korean population with cryptogenic stroke and patent foramen ovale (PFO). MATERIALS AND METHODS: Consecutive 164 patients (men: 126 patients, mean age: 48.1 years, closure group: 72 patients, medical group: 92 patients) were enrolled. The primary end point was a composite of death, stroke, transient ischemic attack (TIA), or peripheral embolism. RESULTS: Baseline characteristics were similar in the two groups, except age, which was higher in the medical group (45.3±9.8 vs. 50.2±6.1, p<0.0001), and risk of paradoxical embolism score, which was higher in the closure group (6.2±1.6 vs. 5.7±1.3, p=0.026). On echocardiography, large right-to-left shunt (81.9% vs. 63.0%, p=0.009) and shunt at rest/septal hypermobility (61.1% vs. 23.9%, p<0.0001) were more common in the closure group. The device was successfully implanted in 71 (98.6%) patients. The primary end point occurred in 2 patients (2 TIA, 2.8%) in the closure group and in 2 (1 death, 1 stroke, 2.2%) in the medical group. Event-free survival rate did not differ between the two groups. CONCLUSION: Compared to medical therapy, device closure of PFO in patients with cryptogenic stroke did not show difference in reduction of recurrent embolic events in the real world's setting. However, considering high risk of echocardiographic findings in the closure group, further investigation of the role of PFO closure in the Asian population is needed.
Subject(s)
Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Cardiac Catheterization/adverse effects , Disease-Free Survival , Embolism/etiology , Fibrinolytic Agents/adverse effects , Foramen Ovale, Patent/complications , Ischemic Attack, Transient/drug therapy , Republic of Korea/epidemiology , Risk , Secondary Prevention/methods , Septal Occluder Device/adverse effects , Stroke/etiology , Treatment OutcomeABSTRACT
BACKGROUND AND OBJECTIVES: Empirical evidence is lacking on the cumulative disease burden of obesity and hypertension and its impact on cardiac function and exercise capacity. The purpose of this study was to determine whether the presence of obesity and hypertension together was associated with cardiac dysfunction and exercise capacity. SUBJECTS AND METHODS: Using a retrospective study design, medical records were reviewed for echocardiographic and treadmill exercise stress test data. Subjects were grouped according to four categories: normal control, obese, hypertensive, or obese and hypertensive. RESULTS: Obese, hypertensive persons showed significantly lower Ea and E/A ratio and greater E/Ea ratio, deceleration time, left ventricular (LV) mass, and LV mass index compared to their counter parts (normal control, obese and/or hypertensive) (all p<0.05), after controlling for age and sex. After controlling for age and sex, significant differences in exercise capacity indices were found, with the obese group having shorter exercise time, lower metabolic equivalents, and lower maximal oxygen uptake than the normal control, hypertensive, or both groups (all p<0.05). The hypertensive or obese and hypertensive group had greater maximal blood pressure compared with the normal control group (all p<0.001). Obese and hypertensive persons were approximately three times more likely to have diastolic dysfunction (odd ratio=2.96, p=0.001), when compared to the reference group (normotensive, non-obese, or hypertensive only persons). CONCLUSION: Diastolic dysfunction was associated with obesity and/or hypertension. The cumulative risk of obesity and hypertension and their impact on diastolic dysfunction which could be modifiable could reduce exercise capacity.
Subject(s)
Humans , Blood Pressure , Deceleration , Echocardiography , Exercise Test , Hypertension , Medical Records , Metabolic Equivalent , Obesity , Oxygen , Retrospective StudiesABSTRACT
PURPOSE: Pulmonary arterial hypertension (PAH) is an orphan disease showing poor prognosis. The purpose of study was to evaluate clinical factors influencing outcomes in PAH. MATERIALS AND METHODS: Patients who were diagnosed with PAH at a single center were reviewed retrospectively. Forty patients (34.9+/-14.5 years, 80% of female) were enrolled. RESULTS: Causes were congenital heart disease in 24 (60%), connective tissue disease in 8 (20%) and idiopathic PAH in 6 (15%). Sixteen patients (40%) were WHO functional class III or IV at the time of diagnosis. Twenty seven patients (67.5%) received molecular targeted therapy. During follow-up (53.6+/-45.5 months), 10 patients (25%) died and 1-, 2-, and 8 year survival rates were 91.3%, 78.7%, and 66.8%, respectively. As expected, median survival of patients with functional class I or II were significantly longer than patients with III or IV (p=0.041). Interestingly, patients with molecular targeted therapy showed longer survival than conventional therapy (p=0.021). CONCLUSION: WHO functional class at the time of diagnosis was the strong predictor of survival, and molecular targeted therapy could significantly improve the survival. Therefore, early screening and intensive management would be crucial to improve the prognosis in the patient with PAH.
Subject(s)
Adult , Female , Humans , Male , Middle Aged , Antihypertensive Agents/therapeutic use , Disease Management , Familial Primary Pulmonary Hypertension , Heart Defects, Congenital/complications , Hypertension/complications , Hypertension, Pulmonary/classification , Kaplan-Meier Estimate , Molecular Targeted Therapy/methods , Prognosis , Retrospective Studies , Survival RateABSTRACT
Chronic obstructive pulmonary disease (COPD) is one of the causes of cor pulmonale. Cor pulmonale patients with pulmonary hypertension have a significant lower survival rate than patients without. However, there is no conclusive treatment options in cor pulmonale and pulmonary hypertension associated with COPD until now. We report a patient with cor pulmonale and pulmonary hypertension associated with severe form of COPD and tuberculous destroyed lung who achieved marked clinical, functional and echocardiographic hemodynamic improvements with inhaled iloprost for six months.