Jitendra K. Vohra

Electrical Remodeling of the Atria in Congestive Heart Failure Electrophysiological and Electroanatomic Mapping in Humans

Background—Atrial fibrillation (AF) frequently complicates congestive heart failure (CHF). However, the electrophysiological substrate for AF in humans with CHF remains unknown. We evaluated the electrophysiological and electroanatomic characteristics of the atria in patients with CHF. Methods and Results—Twenty-one patients (aged 53.7±13.6 years) with symptomatic CHF (left ventricular ejection fraction 25.5±6.0%) and 21 age-matched controls were studied. The following were evaluated: effective refractory periods (ERPs) from the high and low lateral right atrium (LRA), high septal right atrium, and distal coronary sinus (CS); conduction time along the CS and LRA; corrected sinus node recovery times; P-wave duration; and conduction at the crista terminalis. In a subset, electroanatomic mapping was performed to determine atrial activation, regional conduction velocity, double potentials, fractionated electrograms, regional voltage, and areas of electrical silence. Patients with CHF demonstrated an increase in atrial ERP with no change in the heterogeneity of refractoriness, an increase of atrial conduction time along the LRA and the CS, prolongation of the P-wave duration and corrected sinus node recovery times, and greater number and duration of double potentials along the crista terminalis. Electroanatomic mapping demonstrated regional conduction slowing with a greater number of electrograms with fractionation or double potentials, associated with areas of low voltage and electrical silence (scar). Patients with CHF demonstrated an increased propensity for AF with single extrastimuli, and induced AF was more often sustained. Conclusions—Atrial remodeling due to CHF is characterized by structural changes, abnormalities of conduction, sinus node dysfunction, and increased refractoriness. These abnormalities may be responsible in part for the increased propensity for AF in CHF.

Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes

This international consensus statement is the collaborative effort of three medical societies representing electrophysiology in North America, Europe, and Asian-Pacific area: the Heart Rhythm Society (HRS), the European Heart Rhythm Association (EHRA), and the Asia Pacific Heart Rhythm Society. The objective of the consensus document is to provide clinical guidance for diagnosis, risk stratification, and management of patients affected by inherited primary arrhythmia syndromes. It summarizes the opinion of the international writing group members based on their own experience and on a general review of the literature with respect to the clinical data on patients affected by channelopathies. This document does not address the indications of genetic testing in patients affected by inherited arrhythmias and their family members. Diagnostic, prognostic, and therapeutic implications of the results of genetic testing are also not included in this document because this topic has been covered by a recent publication1 coauthored by some of the contributors of this consensus document, and it remains the reference text on this topic. Guidance for the evaluation of patients with idiopathic ventricular fibrillation, sudden arrhythmic death …

Electrophysiological and Electroanatomic Characterization of the Atria in Sinus Node Disease Evidence of Diffuse Atrial Remodeling

Background—The normal sinus pacemaker complex is an extensive structure within the right atrium. We hypothesized that patients with sinus node disease (SND) would have evidence of diffuse atrial abnormalities. Methods and Results—Sixteen patients with symptomatic SND and 16 age-matched controls were studied. The following were evaluated: effective refractory periods (ERPs) from the high and low lateral right atrium (RA), high septal RA, and distal coronary sinus (CS); conduction time along the CS and lateral RA; P-wave duration; and conduction at the crista terminalis. Electroanatomic mapping was performed to define the sinus node complex and determine regional conduction velocity, double potentials, fractionated electrograms, regional voltage, and areas of electrical silence. Patients with SND demonstrated significant increase in atrial ERP at all right atrial sites, increased atrial conduction time along the lateral RA and CS, prolongation of the P-wave duration, and greater number and duration of double potentials along the crista terminalis. Electroanatomic mapping demonstrated the sinus node complex in SND to be more often unicentric, localized to the low crista terminalis at the site of the largest residual voltage amplitude. There was significant regional conduction slowing with double potentials and fractionation associated with areas of low voltage and electrical silence (or scar). Conclusions—SND is associated with diffuse atrial remodeling characterized by structural change, conduction abnormalities, and increased right atrial refractoriness. There was a change in the nature of sinus pacemaker activity with loss of the normal multicentric pattern of activation, caudal shift of the pacemaker complex, and abnormal and circuitous conduction around lines of conduction block.

Electrophysiological and electrocardiographic characteristics of focal atrial tachycardia originating from the pulmonary veins: acute and long-term outcomes of radiofrequency ablation.

Background—The objective of this study was to describe the electrophysiological characteristics, anatomic distribution, and long-term outcome after focal ablation (RFA) of pulmonary vein (PV) atrial tachycardia (AT). Both atrial fibrillation (AF) and AT may be due to a rapidly firing focus in the PVs. Whether these represent two aspects of the same process is unknown. Methods and Results—Twenty-seven patients with 28 PV(16%) ATs of a consecutive series of 172 undergoing RFA for focal AT are reported. The mean age was 39±16 years, with symptoms for 9±14 years resistant to 1.7±0.8 medications. AT occurred spontaneously or with isoproterenol in all patients and was not inducible with PES in any. The distribution of PV ATs was right superior PV, 11; left superior PV, 11; left inferior PV, 5; and right inferior PV, 1; 26of 28 foci (93%) were ostial. RFA was successful in 28 of 28 PV ATs acutely. RFA was focal in 25 of 28, with PV isolation of a single target vein in 3. There were 4 recurrences at a mean of 3.3 months. Repeat RFA was performed in all 4 and successful in 3 of 4. All but one recurrence occurred from the same site. Long-term success was achieved in 26 of 27 (96%) patients at mean follow-up of 25±22 months. No patients have had subsequent development of AF or AT from a different site. Conclusions—PV AT has a distribution similar to PV AF, with a propensity to upper veins. However, the majority of foci are ostial, and only a small percentage occur from deep in the PV. Focal RFA is associated with high long-term success, with freedom from both AT from other sites and from AF. PV AT is a localized process and therefore may be different from PV AF.

A Prospective Study of Sudden Cardiac Death among Children and Young Adults

BACKGROUND Sudden cardiac death among children and young adults is a devastating event. We performed a prospective, population-based, clinical and genetic study of sudden cardiac death among children and young adults. METHODS We prospectively collected clinical, demographic, and autopsy information on all cases of sudden cardiac death among children and young adults 1 to 35 years of age in Australia and New Zealand from 2010 through 2012. In cases that had no cause identified after a comprehensive autopsy that included toxicologic and histologic studies (unexplained sudden cardiac death), at least 59 cardiac genes were analyzed for a clinically relevant cardiac gene mutation. RESULTS A total of 490 cases of sudden cardiac death were identified. The annual incidence was 1.3 cases per 100,000 persons 1 to 35 years of age; 72% of the cases involved boys or young men. Persons 31 to 35 years of age had the highest incidence of sudden cardiac death (3.2 cases per 100,000 persons per year), and persons 16 to 20 years of age had the highest incidence of unexplained sudden cardiac death (0.8 cases per 100,000 persons per year). The most common explained causes of sudden cardiac death were coronary artery disease (24% of cases) and inherited cardiomyopathies (16% of cases). Unexplained sudden cardiac death (40% of cases) was the predominant finding among persons in all age groups, except for those 31 to 35 years of age, for whom coronary artery disease was the most common finding. Younger age and death at night were independently associated with unexplained sudden cardiac death as compared with explained sudden cardiac death. A clinically relevant cardiac gene mutation was identified in 31 of 113 cases (27%) of unexplained sudden cardiac death in which genetic testing was performed. During follow-up, a clinical diagnosis of an inherited cardiovascular disease was identified in 13% of the families in which an unexplained sudden cardiac death occurred. CONCLUSIONS The addition of genetic testing to autopsy investigation substantially increased the identification of a possible cause of sudden cardiac death among children and young adults. (Funded by the National Health and Medical Research Council of Australia and others.).

The Response of the QT Interval to the Brief Tachycardia Provoked by Standing: A Bedside Test for Diagnosing Long QT Syndrome

OBJECTIVES This study was undertaken to determine whether the short-lived sinus tachycardia that occurs during standing will expose changes in the QT interval that are of diagnostic value. BACKGROUND The QT interval shortens during heart rate acceleration, but this response is not instantaneous. We tested whether the transient, sudden sinus tachycardia that occurs during standing would expose abnormal QT interval prolongation in patients with long QT syndrome (LQTS). METHODS Patients (68 with LQTS [LQT1 46%, LQT2 41%, LQT3 4%, not genotyped 9%] and 82 control subjects) underwent a baseline electrocardiogram (ECG) while resting in the supine position and were then asked to get up quickly and stand still during continuous ECG recording. The QT interval was studied at baseline and during maximal sinus tachycardia, maximal QT interval prolongation, and maximal QT interval stretching. RESULTS In response to brisk standing, patients and control subjects responded with similar heart rate acceleration of 28 +/- 10 beats/min (p = 0.261). However, the response of the QT interval to this tachycardia differed: on average, the QT interval of controls shortened by 21 +/- 19 ms whereas the QT interval of LQTS patients increased by 4 +/- 34 ms (p < 0.001). Since the RR interval shortened more than the QT interval, during maximal tachycardia the corrected QT interval increased by 50 +/- 30 ms in the control group and by 89 +/- 47 ms in the LQTS group (p < 0.001). Receiver-operating characteristic curves showed that the test adds diagnostic value. The response of the QT interval to brisk standing was particularly impaired in patients with LQT2. CONCLUSIONS Evaluation of the response of the QT interval to the brisk tachycardia induced by standing provides important information that aids in the diagnosis of LQTS.

Pulmonary Vein Antral Isolation for Paroxysmal Atrial Fibrillation: Results from Long-Term Follow-Up

Long‐Term Follow‐Up After Atrial Fibrillation Ablation. Introduction: Pulmonary veins play an important role in triggering atrial fibrillation (AF). Pulmonary vein isolation (PVI) is an effective treatment for patients with paroxysmal AF. However, the late AF recurrence rate in long‐term follow‐up of circumferential PV antral isolation (PVAI) is not well documented. We sought to determine the time to recurrence of arrhythmia after PVAI, and long‐term rates of sinus rhythm after circumferential PVAI.

Focal atrial tachycardia arising from the mitral annulus: electrocardiographic and electrophysiologic characterization.

OBJECTIVES The study was done to characterize the electrocardiographic and electrophysiologic features of focal atrial tachycardia originating at the mitral annulus (MA). BACKGROUND Though the majority of left atrial tachycardias originate around the ostia of the pulmonary veins, only isolated reports have described focal tachycardia originating from the MA. METHODS Seven patients of a consecutive series of 172 patients undergoing radiofrequency ablation for focal atrial tachycardia are reported. Electrophysiologic study involved catheters positioned along the coronary sinus (CS), crista terminalis (CT), His bundle, and a mapping/ablation catheter. RESULTS All seven patients had tachycardia foci originating from the superior region of the MA in close proximity to the left fibrous trigone and mitral-aortic continuity. These foci demonstrated a characteristic P-wave morphology and endocardial activation pattern. The P-wave morphology in the precordial leads typically showed a biphasic pattern, with an inverted component followed by an upright component. The P-wave was consistently of low amplitude in the limb leads. Earliest endocardial activity occurred at the His bundle region in all seven patients. In general, CS activation was proximal to distal, and mid-CT activation was earlier than high or low CT. Ablation was successful at the superior aspect of the MA in all patients. CONCLUSIONS The MA is an unusual but important site of origin for focal atrial tachycardia, with a propensity to be localized to the superior aspect. It can be suspected as a potential anatomic site of tachycardia origin from analysis of P-wave morphology and the atrial endocardial activation sequence map. Using mapping targeted to anatomic structures achieved a high success rate for ablation.

Focal Atrial Tachycardia Arising from the Tricuspid Annulus: Electrophysiologic and Electrocardiographic Characteristics

Tricuspid Annular Tachycardia. Introduction: Focal right atrial tachycardia (RAT) arising from the crista terminalis, para‐Hisian, and coronary sinus os regions are well described. Less information exists regarding RAT arising from the nonseptal region of the tricuspid annulus (TA).

Tachycardia-Mediated Cardiomyopathy Secondary to Focal Atrial Tachycardia: Long-Term Outcome After Catheter Ablation

OBJECTIVES This study aimed to characterize the incidence, clinical and electrophysiologic features, and long-term outcomes of patients with tachycardia-mediated cardiomyopathy (TCM) secondary to focal atrial tachycardia (AT). BACKGROUND TCM is known to complicate atrial tachyarrhythmias. Little is known of the patient and tachycardia characteristics associated with the development of left ventricular (LV) dysfunction and the long-term outcomes after cure of tachycardia. METHODS A total of 345 patients with focal AT underwent radiofrequency ablation between January 1997 and July 2008. A retrospective analysis was performed to identify patients with LV dysfunction, defined as an ejection fraction <50% on echocardiography. Patients with pre-existing structural heart disease (n = 14) were excluded. Patients with TCM (n = 30) and without TCM (n = 301) were compared. Recovery of LV function was also assessed. RESULTS The incidence of TCM was 10%. Incessant or very frequent paroxysmal tachycardia was strongly associated with TCM, compared to patients without TCM (100% vs. 20%, p < 0.001). Patients in the TCM group were younger (mean age 39 +/- 22 years vs. 51 +/- 17 years, p = 0.0006) and more frequently male (60% vs. 38%, p < 0.001). Patients with TCM had a longer mean tachycardia cycle length (502 +/- 131 ms vs. 402 +/- 105 ms, p < 0.0001) and slower ventricular rate (117 +/- 21 beats/min vs. 141 +/- 33 beats/min, p = 0.0007) during tachycardia compared with patients who did not have TCM. Appendage sites are associated with a high incidence of incessant tachycardia (84%) and LV dysfunction (42%). After successful ablation, LV function was restored in 97% of patients at a mean of 3 months. CONCLUSIONS Cardiomyopathy occurs in 10% of patients with focal AT. A slower incessant tachycardia is more frequently complicated by cardiomyopathy. Long-term restoration of LV function can be achieved after successful catheter ablation of the tachycardia focus.