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

Einfluss der Nierenfunktion auf Mortalität und Prognose bei Leberzirrhose

Die Leberzirrhose ist durch eine steigende Inzidenz sowie hohe Morbidität und Mortalität gekennzeichnet. Eine renale Dysfunktion stellt bei Patienten mit fortgeschrittener Lebererkrankung eine schwerwiegende Komplikation dar, daher sollte in der vorliegenden Untersuchung die Bedeutung einer renalen Funktionsstörung für das Outcome von Patienten mit manifester Leberzirrhose untersucht werden. In einer retrospektiven Analyse wurden 245 Patienten erfasst, die im Zeitraum von 2004-2011 eine orthotope Lebertransplantation (OLTx) erhielten, oder mit selbiger Diagnose einer Dialyse zugeführt werden mussten. Die Patienten wurden hinsichtlich prädiktiver Parameter auf das 1-Jahres-Outcome untersucht, wobei die Nierenfunktion mittels MDRD4 und CKD-EPI-Formel abgeschätzt wurde und Kaplan-Meier sowie multivariate Cox-Hazard-Analysen erstellt wurden. Die 1-Jahres-Mortalität in der Gruppe der transplantierten Patienten betrug mehr als 22 %; bei Dialysepflichtigkeit stieg sie je nach Subgruppe auf 54-64 % an. Als führende Todesursache war in allen Gruppen eine Sepsis mit konsekutivem Multiorganversagen auszumachen. Auffällig war dabei, dass über 90 % der verstorbenen Patienten bei stationärer Aufnahme eine eine geschätzte GFR <30ml/min/1,73m2 aufwiesen und im Verlauf eine entzündliche Komponente in Form eines SIRS oder einer Sepsis entwickelten. Die Analysen zeigten, dass mit abnehmender Nierenfunktion die Mortalität signifikant zunimmt. Dies gilt sowohl für das Überleben nach Transplantation, als auch für das Überleben ohne OLTx. Die höchste Mortalität fand sich bei dialysepflichtigen Patienten; bei diesen Patienten konnte keine signifikante Verbesserung des Überlebens durch eine OLTx nachgewiesen werden. Niereninsuffizienz und Dialysepflicht konnten als stärkste prädiktive und unabhängige Faktoren für das Überleben bei Patienten mit Leberzirrhose ermittelt werden

The effect of first step right atrial mapping (FRAM) on ablation duration and fluoroscopy exposure during cavotricuspid isthmus ablation of atrial flutter

AIM To investigate the clinical significance of right atrial mapping prior to cavotricuspid isthmus (CTI) ablation in patients with typical atrial flutter (AFL). METHODS Clinical and ablation parameters were retrospectively assessed and compared in patients undergoing CTI ablation with or without a first-step right atrial mapping (FRAM) by using the CARTO 3D mapping system. RESULTS CTI block by radiofrequency ablation (RFA) was achieved in all 143 patients. In the FRAM group there was a shorter ablation duration and fluoroscopy exposure compared with the non-FRAM group. CHA$_{2}$DS$_{2}$-VASc score was associated with higher ablation durations, more ablation applications and increased fluoroscopy exposure. Body mass index (BMI) was associated with longer ablation duration and more ablation applications. Furthermore, patients with reduced left ventricular ejection fraction (LVEF) had longer ablation durations and more fluoroscopy exposure. One patient in the non-FRAM group developed cardiac effusion after ablation. None of the patients had recurrence after 6 months of follow-up. CONCLUSIONS Patients with high BMI, high CHA$_{2}$DS$_{2}$-VASc score and reduced LVEF may benefit from the FRAM approach by reducing ablation duration, number of ablation applications and fluoroscopy exposure

A desmoplakin variant associated with isolated arrhythmogenic left ventricular cardiomyopathy with rapid monomorphic ventricular tachycardia at first presentation

Arrhythmogenic cardiomyopathy (ACM) encompasses heart muscle diseases associated with potentially life-threatening ventricular tachyarrhythmias occurring out of proportion to the degree of underlying disease. The most classical disease is arrhythmogenic right ventricular cardiomyopathy (ARVC), whereas biventricular ACM as well as left-dominant forms (arrhythmogenic left ventricular cardiomyopathy, ALVC) have been increasingly recognized. Genetic variants in the desmoplakin (DSP) gene, coding for an integral part of the desmosome and the resultant disruption of intermediate filament binding, were shown to be associated with ACM, including ALVC.1 In this paper, we report a DSP variant associated with rapid sustained monomorphic ventricular tachycardia as first manifestation in a young female patient with isolated ALVC without right ventricle (RV) involvement

Swiss National Registry on Catheter Ablation Procedures: Changing Trends over the Last 20 Years

The Swiss Ablation Registry provides a national database for electrophysiologic studies and catheter ablations. We analyzed the database to provide an in-depth look at changing trends over the last 20 years. During the study period a total of 78622 catheter ablations (age 61.0 ± 1.2 years; 63.7% male) were performed in 29 centers. The number of ablations increased by approximately ten-fold in 20 years. Ablation for atrial fibrillation (AF) was the main driver behind this increase, with more than hundred-fold (39.7% of all ablations in 2019). Atrioventricular-nodal-reentrant-tachycardia (AVNRT) and accessory pathways, being the main indications for ablation in 2000 (44.1%/25.1%, respectively), made up of only a small proportion (15.2%/3.5%,) respectively in 2019. Fluoroscopy, ablation, and procedure durations were reduced for all ablations over time. The highest repeat ablations were performed for ventricular tachycardia and AF (24.4%/24.3%). The majority of ablations (63.0%) are currently performed in private hospitals and non-university public hospitals whereas university hospitals had dominated (82.4%) at the turn of the century. A pronounced increase in the number of catheter ablations in Switzerland was accompanied by a marked decrease in fluoroscopy, ablation, and procedure durations. We observed a shift toward more complex procedures in older patients with comorbidities

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&rsquo;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

CME-EKG 67/Antworten: Arrhythmie bei Belastung

CME ECG 67/Answers: Arrhythmia on Exertion Abstract. Ventricular tachycardias are potentially life-threatening cardiac arrhythmias with a heart rate >100 beats/min, originating from the specific conduction system below the His or the ventricular myocardium. The morphology of the surface ECG can provide valid information about the underlying mechanism and the associated cardiac disorder. The according pathomechanism is of paramount importance for further management. This article is intended to provide an insight into the various causes and treatment options as well as the differential diagnosis of ventricular tachycardias

CME-EKG 67: Arrhythmie bei Belastung

Ventrikuläre Tachykardien sind potenziell lebensbedrohliche Herzrhythmusstörungen mit einer Herzfrequenz >100 Schläge/min und einem Ursprungsort der Arrhythmie unterhalb des Hisbündels im spezifischen Reizleitungssystem oder im ventrikulären Myokard. Die Morphologie im Oberflächen-EKG kann bereits Hinweise auf den zugrundeliegenden Mechanismus und die damit assoziierte Grunderkrankung liefern. Der jeweilige Pathomechanismus ist entscheidend für die weiterführende Therapie. Dieser Artikel soll einen Einblick in die verschiedenen Ursachen und Therapieoptionen sowie die Differenzialdiagnose der ventrikulären Tachykardien geben. = CME ECC 67: Arrhythmia on Exertion Abstract. Ventricular tachycardias are potentially life-threatening cardiac arrhythmias with a heart rate >100 beats/min, originating from the specific conduction system below the His or the ventricular myocardium. The morphology of the surface ECG can provide valid information about the underlying mechanism and the associated cardiac disorder. The according pathomechanism is of paramount importance for further management. This article is intended to provide an insight into the various causes and treatment options as well as the differential diagnosis of ventricular tachycardias