Arrhythmogenic Right Ventricular Cardiomyopathy and Differential Diagnosis with Diseases Mimicking Its Phenotypes

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease, which is characterized by fibro-fatty replacement of predominantly the right ventricle (RV). The disease can result in ventricular tachyarrhythmias and sudden cardiac death. Our understanding of the pathophysiology and clinical expressivity of ARVC has been continuously evolving. The diagnosis can be challenging due to its variable expressivity, incomplete penetrance and the lack of specific diagnostic criteria. Idiopathic RV outflow tract tachycardia, Brugada Syndrome, athlete's heart, dilated cardiomyopathy, myocarditis, cardiac sarcoidosis, congenital aneurysms and diverticula may mimic clinical phenotypes of ARVC. This review aims to provide an update on the differential diagnosis of ARVC

Management of Patients with Atrial Fibrillation: Specific Considerations for the Old Age

Atrial fibrillation (AF) is the commonest of all sustained arrhythmias, and most of the patients seeking medical therapy are in the elderly age group. The management of these patients is particularly difficult due to associated comorbidities. Hypertension, congestive heart failure, left ventricular hypertrophy, and coronary artery disease are often present in the elderly patient population, and therefore, antiarrhythmic drugs often fail due to side effects, proarrhythmia, or poor rhythm control. Recently, radiofrequency catheter ablation has been widely performed as an efficient therapy for recurrent, drug-refractory AF. Nevertheless, patients at old age were underrepresented in prior AF ablation trials, and the current guidelines for catheter ablation of AF recommend a noninvasive approach in the elderly patient group due to the lack of clinical data supporting ablation therapy. However, study results of our group and others are suggesting that catheter ablation is a safe and effective treatment for patients over the age of 65 years with symptomatic, drug-refractory AF, and therefore, patients should not be precluded from catheter ablation only on the basis of age. This paper discusses the pharmacological (rhythm control, rate control, and anticoagulation) and catheter management of AF in the elderly population

Focal atrial tachycardia originating from mirror position of fossa ovalis: A case report

In patients undergoing ablation of focal atrial tachycardia (FAT) arising from the interatrial septum (IAS), mapping of both atria is required. Electroanatomical mapping of the IAS and identification of earliest activation are necessary for assessing arrhythmia origin, leading to an effective radiofrequency ablation. The fossa ovalis (FOv) is an anatomical structure of the right atrium (RA). However, FAT may also arise from the corresponding left IAS. The present case is a FAT originating from the left IAS mirror position of the FOv, corresponding to the earliest activation of the RA. The earliest RA electrogram with double potentials identified in the area of the FOv predicts the mirror position within the left atrium

Heart Failure in Patients with Arrhythmogenic Cardiomyopathy

Arrhythmogenic cardiomyopathy (ACM) is a rare inherited cardiomyopathy characterized as fibro-fatty replacement, and a common cause for sudden cardiac death in young athletes. Development of heart failure (HF) has been an under-recognized complication of ACM for a long time. The current clinical management guidelines for HF in ACM progression have nowadays been updated. Thus, a comprehensive review for this great achievement in our understanding of HF in ACM is necessary. In this review, we aim to describe the research progress on epidemiology, clinical characteristics, risk stratification and therapeutics of HF in ACM

Medical therapy with flecainide and propafenone in atrial fibrillation: Long-term clinical experience in the tertiary care setting

BACKGROUND: Flecainide and propafenone are Class Ic antiarrhythmic drugs that block the cardiac fast inwards Na+ current and are used for rhythm control in patients with atrial fibrillation (AF). However, data on long-term clinical efficacy and safety of these drugs in a real-world setting are scarce. METHODS: Patients with AF who received chronic flecainide or propafenone therapy were retrospectively studied from the database of a tertiary care center. The primary outcome of the study was clinical efficacy of Class Ic antiarrhythmics, which was assessed based on the improvement of arrhythmia-related symptoms at the time of last follow-up. RESULTS: Among the 361 patients (261 males, 72.3%) with a mean age of 56 ± 12 years, 287 (79.5%) were using long-term flecainide, and 74 (20.5%) patients propafenone. The majority of the patients had paroxysmal AF (n = 331, 91.7%) and had an atrioventricular-nodal blocking co-medication (n = 287, 79.5%). A total of 117 (32%) patients discontinued therapy after a median of 210 days (interquartile range 62-855 days). Clinical efficacy was observed in 188 patients (52%). The most common reason for therapy discontinuation was adverse drug effects, particularly proarrhythmic effects (48% for flecainide and 33% for propafenone). Patients who did not clinically benefit from Class Ic antiarrhythmics more often underwent pulmonary vein isolation (p = 0.02). CONCLUSIONS: Long-term therapy with Class Ic antiarrhythmics showed clinical efficacy in approximately half of the patients with paroxysmal or persistent AF. However, these drugs were also associated with a relatively high rate of adverse events, and in particular proarrhythmic effects, which often resulted in therapy discontinuation rendering appropriate patient selection and therapy surveillance essential

Ablation of atrial fibrillation after the retirement age: considerations on safety and outcome

Background: Although the incidence of atrial fibrillation (AF) progressively increases with age, the vast majority of AF ablation is done in middle-aged patients. We evaluated the feasibility and safety of catheter ablation in patients older than 65years of age with paroxysmal and persistent AF. Methods: Out of a total of 230 consecutive AF ablation procedures, 45 patients were older than 65years of age and underwent 53 procedures. The ablation strategy consisted of wide-area circumferential lines around both ipsilateral pulmonary veins using a three-dimensional mapping system. Results: The mean age was 69 ± 3.5years (35 males). The mean duration for AF was 8.7 ± 6.5years. Thirty-nine had paroxysmal and six persistent AF despite use of 1.38 ± 0.77 antiarrhythmic drugs. All patients had a structurally normal heart. Eleven had systemic hypertension. Mean procedure time was 187 ± 33min. Acute procedural success rate with abolition of all pulmonary vein potentials was achieved in all patients. Pericardial tamponade requiring percutaneous drainage occurred in one (1.9%) patient. There were no cardioembolic events. Among the 43 patients whose clinical outcome was assessed at 6months, 34 (79%) had a significant reduction (>90%) of the total symptomatic AF burden, compared to pre-ablation, with a complete lack of symptomatic AF in 32 (74%) patients. The success rate was higher for patients with paroxysmal versus persistent AF (81 vs. 67%). Six patients (11%) underwent repeat procedures. Conclusions: Catheter ablation is a safe and effective treatment for patients over the age of 65years with symptomatic, drug-refractory AF. Therefore, patients should not be excluded from undergoing AF catheter ablation on the basis of age alon

Transesophageal Echocardiography-Guided Transseptal Left Atrial Access to Improve Safety in Patients Undergoing Pulmonary Vein Isolation

BACKGROUND: Endovascular pulmonary vein isolation (PVI) has become an important strategy for rhythm control in patients with symptomatic atrial fibrillation (AF). Transseptal access is a critical step of this procedure and can result in potentially life-threatening complications. This retrospective study evaluates the safety of standardized, transesophageal echocardiography (TEE)-guided transseptal access to the left atrium in consecutive patients who underwent PVI. METHODS: After the implementation of a standardized, TEE-guided procedure for transseptal access, the data of 404 consecutive PVI procedures using radiofrequency ablation and 3D-mapping were prospectively collected over 5 years. TEE-guided transseptal punctures were performed on 375 patients undergoing one to three PVIs. The patient cohort was retrospectively analyzed for major and minor complications, fluoroscopy time, fluoroscopy dose and ablation outcomes. RESULTS: No single complication related to transseptal access occurred, affirming the safety of the TEE-guided approach. Fluoroscopy time and fluoroscopy dose decreased significantly after 152 procedures. PVI-related minor complications occurred in 11 procedures (2.6%) and included 10 vascular-access-related complications (2.4%) and 1 TEE-related esophageal hematoma (0.2%), which healed spontaneously. CONCLUSION: Our single-center study shows that TEE guidance may allow safe transseptal access to the left atrium in patients undergoing PVI