Fragmented endocardial signals and early afterdepolarizations during torsades de pointes tachycardia.

BACKGROUND: Bradycardia-induced torsade de pointes (TdP) tachycardia in patients with spontaneous high-degree atrioventricular block (AVB) is common. The aim of this study was to analyze endocardial recordings during TdP in spontaneous high-degree AVB in humans to better understand the electrophysiological mechanisms underlying this phenomenon. METHODS: The study group consisted of 5 patients with typical episodes of TdP during spontaneous high-degree AVB. A standard (USCI) temporary bipolar endocardial catheter positioned at the apex of the right ventricle (RV) and bipolar chest leads from two precordial leads V1 and V4 were used to record the tracings during TdP. RESULTS: The presence of a wide spectrum of fragmentations was noted on endocardial electrograms (EGMs), which were invisible on the surface electrocardiogram (ECG) tracing. Endocardial signals indicated that TdP started in the proximity of the RV apex, since the local EGM began prior to the QRS complex on the surface ECG. Early afterdepolarizations (EADs) were observed in 2 out of 5 cases confirming a common opinion about the mechanism of TdP. However, this phenomenon was not observed in 3 other patients suggesting that the arrhythmia was the result of a different mechanism originating in proximity to the RV apex. CONCLUSIONS: This work demonstrated early endocardial signals in the RV apex during TdP associated with high-degree AVB in humans, and exhibits a spectrum of fragmented signals in this area occurring on a single or multiple beats. These fragmentations indicate areas of poor conduction and various degrees of intramyocardial block, and therefore a new mechanism of TdP tachycardia in some patients with spontaneous high-degree AVB.

Mechanisms of torsade de pointes tachycardia in patients with spontaneous high-degree atrioventricular block: A modern look at old data.

INTRODUCTION: The mechanisms of torsade de pointes tachycardia (TdP) are incompletely understood. We aimed to investigate the mechanisms underlying TdP tachycardia in patients with spontaneous high-degree atrioventricular block (AVB). METHODS AND RESULTS: This retrospective study reviewed old TdP recordings after ventricular temporary pacing interruption in 16 patients with spontaneous high-degree AVB. Five of them had also bipolar endocardial right ventricular (RV) apex recordings. The QT interval during AVB at a mean heart rate of 38.9 ±â€¯7.5 bpm was 653.0 ±â€¯67.2 ms. The critical coupling interval (CCI) between the last escape QRS during AVB and the first premature ventricular complex (PVC) was significantly shorter before the onset of TdP than before single PVCs and couplets. A morphologic crescendo of the escape T wave was observed before the onset of TdP, followed by a rhythmic and morphologic crescendo of PVCs. The escape RV apex electrograms (EGMs) showed the constant pattern of a rapid deflection similar to a Purkinje potential 40 to 80 ms after the onset of the QRS, superimposed on a smooth low amplitude signal in 4 out of 5 patients. CONCLUSIONS: The major endocardial T wave prolongation and augmentation (morphologic crescendo) of the escape beat prior to the first PVC suggests a phase 2 reentry mechanism due to early afterdepolarization. The induced TdP can be due to the changing outputs from one or two simultaneous RV moving reentry circuits between depressed fibers and fast conducting ones, possibly located in the thin crista supraventricularis structure which has several connections with the septum and the RV free wall.

Right atrial pathology in arrhythmogenic right ventricular dysplasia.

BACKGROUND: Atrial fibrillation (AF) is the most common atrial arrhythmia in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVD). Considering the histologic changes known in the right ventricular (RV) in ARVD, the aim of the present study was to examine right atrial (RA) pathology in patients with ARVD. METHODS: Histology of RA and RV was assessed from autopsy material in 3 patients with ARVD without persistent atrial arrhythmia. RA histology in 3 patients with permanent AF without ARVD and 5 patients without cardiovascular disease was also studied. Staining with hematoxylin phloxine saffron was performed for the ARVD patients to identify fibrosis, and hematoxylin-eosin for identification of lymphocytes. Masson's trichrome staining was performed for control groups taken from a collection of standard glass slides. RESULTS: In all 3 ARVD cases, RA anomalies were observed that revealed a reduction of cardiomyocytes, the presence of adipocytes, some of them inside the mediomural atrial layer and interstitial fibrosis. In 2 ARVD cases, interstitial fibrosis was also associated with a focus of replacement fibrosis, which was also observed in patients with permanent AF without ARVD. The histologic specimen of the RA and RV from the control group without cardiovascular disease did not display any evidence of fat or fibrosis with a preserved cardiomyocyte architecture. CONCLUSIONS: A similar histopathological substrate, as can be observed in the RV of patients with ARVD can also be seen in the RA of these patients. This may explain the high prevalence of atrial arrhythmias, particularly AF, in patients with ARVD.

Naxos disease: from the origin to today.

Naxos disease, first described by Dr. Nikos Protonotarios and colleagues on the island of Naxos, Greece, is a special form of arrhythmogenic right ventricular dysplasia (ARVD). It is an inherited condition with a recessive form of transmission and a familial penetrance of 90%. It is associated with thickening of the skin of the hands and sole, and a propensity to woolly hair. The cardiac anomalies characterized by ventricular arrhythmias with ventricular extrasystoles and tachycardia and histologic features of the myocardium are consistent with ARVD, but in a more severe form of dysplasia with major dilatation of the right ventricle. The identification of the responsible first gene on chromosome 17, and its product plakoglobin as the responsible protein for Naxos disease proved to be a milestone in the study of ARVD, which opened a new field of research. Thanks to those with the determination to discover Naxos disease, there is and will be more clarity in understanding the mechanisms of juvenile sudden death in the young who have an apparently otherwise normal heart.

Intramyocardial block in patients with atrioventricular block.

Atrioventricular (AV) block has been extensively studied. However, conduction inside the myocardium in patients with AV block has not been reported. In this study, we aimed to demonstrate the presence of intramyocardial block in patients with AV block. Five consecutive patients with spontaneous high-grade AV block and Torsades de pointes (TdP) were prospectively studied with standard United States Catheter Instruments (USCI) endocardial temporary catheter located at the right ventricle (RV) apex. The morphology of endocardial potentials observed in the basic QRS complexes as well as during episodes of TdP was studied. The electrogram (EGM) of the basic rhythm showed a sharp deflection of high amplitude preceded and/or followed by a smooth potential of low amplitude interpreted as far-field potentials in all patients. The sharp potential can be observed at the beginning, in the middle or at the end of the smooth potential. All these potentials were reproduced from beat to beat and were falling inside the QRS complex of the surface ECG. Therefore, these aspects are zones of electrically depressed or silent myocardium larger than the interelectrode distance of 12 mm. This situation is in agreement with recent genetic factors. In this study, we demonstrated for the first time that patients with spontaneous AV block also have trouble in ventricular activation located on the AV conduction system and inside the myocardium. It is then possible to speculate that the presence of diffuse non-conducting myocardium explains why most TdPs do not degenerate into ventricular fibrillation (VF) and generally stop spontaneously.

Endomyocardial biopsy in patients with acute myocarditis, idiopathic dilated cardiomyopathy, and arrhythmogenic right ventricular dysplasia.

Endomyocardial biopsy (EMB) is useful for the diagnosis of myocarditis, cardiac sarcoidosis, and non-ischemic cardiomyopathy. In this mini-review, we discuss the diagnostic potential of EMB in cases of acute/chronic-active myocarditis, sarcoidosis, idiopathic dilated cardiomyopathy and arrhythmogenic right ventricular dysplasia. We also summarize the complications caused by endomyocardial biopsy procedures. Importantly, we finally review the emerging molecular biology technologies as well as biological engineering techniques that can help improve the diagnostic accuracy of EMB to diagnose myocarditis and cardiomyopathies, promoting the management of these diseases.

The electrocardiographic manifestations of arrhythmogenic right ventricular dysplasia.

The ECG is abnormal in most patients with arrhythmogenic right ventricular dysplasia (ARVD). Right ventricular parietal block, reduced QRS amplitude, epsilon wave, T wave inversion in V1-3 and ventricular tachycardia in the morphology of left bundle branch block are the characteristic changes that reflect the underlying genetic predetermined pathology and pathoelectrophysiology. Recognizing the characteristic ECG changes in ARVD will be of help in making a correct diagnosis of this rare disease.