Systematic quantification of histologic ventricular fibrosis in isolated mitral valve prolapse and sudden cardiac death.

BACKGROUND: Cardiac fibrosis in mitral valve prolapse (MVP) is implicated in the development of sudden cardiac death (SCD); however, the pattern remains poorly characterized. OBJECTIVE: The purpose of this study was to systematically quantify left and right ventricular fibrosis in individuals with isolated MVP and SCD (iMVP-SCD), whereby other potential causes of death are excluded, compared to a control cohort. METHODS: Individuals with iMVP-SCD were identified from the Victorian Institute of Forensic Medicine, Australia, and matched for age, sex, and body mass index to control cases with noncardiac death. Cardiac tissue sections were analyzed to determine collagen deposition in the left ventricular free wall (anterior, lateral, and posterior portions), interventricular septum, and right ventricle. Within the iMVP-SCD cases, the endocardial-to-epicardial distribution of fibrosis within the left ventricle was specifically characterized. RESULTS: Seventeen cases with iMVP-SCD were matched 1:1 with 17 controls, yielding 149 samples and 1788 histologic regions. The iMVP-SCD group had increased left ventricular (anterior, lateral, and posterior; all P <.001) and interventricular septum fibrosis (P <.001), but similar amounts of right ventricular fibrosis (P = .62) compared to controls. In iMVP-SCD, left ventricular fibrosis was significantly higher in the lateral and posterior walls compared to the anterior wall and interventricular septum (all P <.001). Within the lateral and posterior walls, iMVP-SCD cases had a significant endocardial-to-epicardial gradient of cardiac fibrosis (P <.01) similar to other known conditions that cause cardiac remodeling. CONCLUSION: Our study indicates that nonuniform left ventricular remodeling with both localized and generalized left ventricular fibrosis is important in the pathogenesis of SCD in individuals with MVP.

Characteristic Histopathological Findings and Cardiac Arrest Rhythm in Isolated Mitral Valve Prolapse and Sudden Cardiac Death.

Background The association between mitral valve prolapse (MVP) and sudden death remains controversial. We aimed to describe histopathological changes in individuals with autopsy-determined isolated MVP (iMVP) and sudden death and document cardiac arrest rhythm. Methods and Results The Australian National Coronial Information System database was used to identify cases of iMVP between 2000 and 2018. Histopathological changes in iMVP and sudden death were compared with 2 control cohorts matched for age, sex, height, and weight (1 group with noncardiac death and 1 group with cardiac death). Data linkage with ambulance services provided cardiac arrest rhythm for iMVP cases. From 77 221 cardiovascular deaths in the National Coronial Information System database, there were 376 cases with MVP. Individual case review yielded 71 cases of iMVP. Mean age was 49±18 years, and 51% were women. Individuals with iMVP had higher cardiac mass (447 g versus 355 g; P<0.001) compared with noncardiac death, but similar cardiac mass (447 g versus 438 g; P=0.64) compared with cardiac death. Individuals with iMVP had larger mitral valve annulus compared with noncardiac death (121 versus 108 mm; P<0.001) and cardiac death (121 versus 110 mm; P=0.002), and more left ventricular fibrosis (79% versus 38%; P<0.001) compared with noncardiac death controls. In those with iMVP and witnessed cardiac arrest, 94% had ventricular fibrillation. Conclusions Individuals with iMVP and sudden death have increased cardiac mass, mitral annulus size, and left ventricular fibrosis compared with a matched cohort, with cardiac arrest caused by ventricular fibrillation. The histopathological changes in iMVP may provide the substrate necessary for development of malignant ventricular arrhythmias.

Mitral valve prolapse and sudden cardiac death: a systematic review and meta-analysis.

OBJECTIVES: Mitral valve prolapse (MVP) is commonly observed as a benign finding. However, the literature suggests that it may be associated with sudden cardiac death (SCD). We performed a meta-analysis and systematic review to determine the: (1) prevalence of MVP in the general population; (2) prevalence of MVP in all SCD and unexplained SCD; (3) incidence of SCD in MVP and (4) risk factors for SCD. METHODS: The English medical literature was searched for: (1) MVP community prevalence; (2) MVP prevalence in SCD cohorts; (3) incidence SCD in MVP and (4) SCD risk factors in MVP. Thirty-four studies were identified for inclusion. This study was registered with PROSPERO (CRD42018089502). RESULTS: The prevalence of MVP was 1.2% (95% CI 0.5 to 2.0) in community populations. Among SCD victims, the cause of death remained undetermined in 22.1% (95% CI 13.4 to 30.7); of these, MVP was observed in 11.7% (95% CI 5.8 to 19.1). The incidence of SCD in the MVP population was 0.14% (95% CI 0.1 to 0.3) per year. Potential risk factors for SCD include bileaflet prolapse, ventricular fibrosis complex ventricular ectopy and ST-T wave abnormalities. CONCLUSION: The high prevalence of MVP in cohorts of unexplained SCD despite low population prevalence provides indirect evidence of an association of MVP with SCD. The absolute number of people exposed to the risk of SCD is significant, although the incidence of life-threatening arrhythmic events in the general MVP population remains low. High-risk features include bileaflet prolapse, ventricular fibrosis, ST-T wave abnormalities and frequent complex ventricular ectopy. TRIAL REGISTRATION: PROSPERO (CRD42018089502).

Long-Term Arrhythmic and Nonarrhythmic Outcomes of Lamin A/C Mutation Carriers.

BACKGROUND: Mutations in LMNA are variably expressed and may cause cardiomyopathy, atrioventricular block (AVB), or atrial arrhythmias (AAs) and ventricular arrhythmias (VA). Detailed natural history studies of LMNA-associated arrhythmic and nonarrhythmic outcomes are limited, and the prognostic significance of the index cardiac phenotype remains uncertain. OBJECTIVES: This study sought to describe the arrhythmic and nonarrhythmic outcomes of LMNA mutation carriers and to assess the prognostic significance of the index cardiac phenotype. METHODS: The incidence of AVB, AA, sustained VA, left ventricular systolic dysfunction (LVD) (= left ventricular ejection fraction ≤50%), and end-stage heart failure (HF) was retrospectively determined in 122 consecutive LMNA mutation carriers followed at 5 referral centers for a median of 7 years from first clinical contact. Predictors of VA and end-stage HF or death were determined. RESULTS: The prevalence of clinical manifestations increased broadly from index evaluation to median follow-up: AVB, 46% to 57%; AA, 39% to 63%; VA, 16% to 34%; and LVD, 44% to 57%. Implantable cardioverter-defibrillators were placed in 59% of patients for new LVD or AVB. End-stage HF developed in 19% of patients, and 13% died. In patients without LVD at presentation, 24% developed new LVD, and 7% developed end-stage HF. Male sex (p = 0.01), nonmissense mutations (p = 0.03), and LVD at index evaluation (p = 0.004) were associated with development of VA, whereas LVD was associated with end-stage HF or death (p < 0.001). Mode of presentation (with isolated or combination of clinical features) did not predict sustained VA or end-stage HF or death. CONCLUSIONS: LMNA-related heart disease was associated with a high incidence of phenotypic progression and adverse arrhythmic and nonarrhythmic events over long-term follow-up. The index cardiac phenotype did not predict adverse events. Genetic diagnosis and subsequent follow-up, including anticipatory planning for therapies to prevent sudden death and manage HF, is warranted.

Temporal distribution of arrhythmic events in chronic kidney disease: Highest incidence in the long interdialytic period.

BACKGROUND: Chronic kidney disease (CKD) patients undergoing hemodialysis (HD) have a high risk of sudden cardiac death (SCD). A unique risk factor may be a longer interval between HD sessions (interdialytic period). Inherent in conventional HD (thrice-weekly) are two 48-hour short breaks (SIDP) and one 72-hour long break (LIDP) between HD sessions. OBJECTIVE: We used an implantable cardiac monitor (ICM) to define the incidence and timing of significant arrhythmias in an HD population. METHODS: Fifty CKD patients undergoing HD with left ventricular ejection fraction >35% had an ICM inserted, with intensive follow-up to record SCD events and predefined bradyarrhythmias and tachyarrhythmias. RESULTS: Mean age of the patients was 67 ± 11 years; 72% were male, and the mean follow-up was 18 ± 4 months. There were 8 unexpected SCDs (16%), all during the LIDP. The terminal event was severe bradycardia with asystole in each recorded case. No episodes of polymorphic ventricular tachycardia (VT) occurred. A total of 7686 arrhythmia events were recorded in 43 patients (86%), including bradycardia in 15 patients (30%), sinus arrest in 14 (28%), second-degree atrioventricular block in 4 (8%), nonsustained VT in 10 (20%), and new-onset paroxysmal atrial fibrillation in 14 (28%). The LIDP was the highest-risk period for all arrhythmias (P < .001). The arrhythmia event rate per hour was greatest during the first pre-HD period of the week compared with any other peri-HD period (P < .001). CONCLUSION: Risk of SCD and significant arrhythmias is greatest during the LIDP. SCD was attributable to severe bradycardia and asystole. Interventions to prevent this type of SCD or shorten the LIDP deserve further evaluation. CLINICAL TRIAL REGISTRATION INFORMATION: URL: https://www.anzctr.org.au (Unique identifier: ACTRN12613001326785).

Familial cardiological and targeted genetic evaluation: low yield in sudden unexplained death and high yield in unexplained cardiac arrest syndromes.

BACKGROUND: It has been reported that cardiological screening and genetic evaluation in relatives of families with sudden unexplained death syndrome and unexplained cardiac arrest (UCA) may uncover a heritable etiology in a significant proportion of families. OBJECTIVE: To evaluate the yield of a comprehensive evaluation protocol of a large unselected cohort of consecutive families with autopsy-negative sudden unexplained death syndrome (termed sudden arrhythmic death syndrome [SADS]) and UCA. METHODS: We studied (1) 109 consecutive families (411 relatives) referred with 1 or more sudden deaths in the family and (2) 52 consecutive probands with UCA (91 relatives) referred by cardiologists between January 2007 and December 2012. A comprehensive cardiological screening was performed followed by targeted genetic evaluation if a clinical phenotype was proven or suspected. Diagnosis was made by a multidisciplinary team using published clinical criteria. RESULTS: A diagnosis was made in 19 of 109 families with SADS (yield 18%), with the majority having long QT syndrome (LQTS). Diagnosis varied according to proband age, with LQTS most common in the very young (≤20 years) and Brugada syndrome in the older age probands (≥40 years) (P = .03). In contrast, a diagnosis was made in 32 of 52 families with UCA (yield 62%), the majority of which had LQTS and Brugada syndrome. No clinical or circumstantial factors increased the likelihood of diagnosis in families with either SADS or UCA. CONCLUSIONS: In contrast to previously published series, a comprehensive strategy of cardiological evaluation and targeted genetic testing in more than 100 families with SADS was found to have a lower diagnostic yield (18%). Diagnostic yield in families with UCA was approximately 4 times higher (62%), which is consistent with the published literature.

The phenomenon of "QT stunning": the abnormal QT prolongation provoked by standing persists even as the heart rate returns to normal in patients with long QT syndrome.

BACKGROUND: Patients with long QT syndrome (LQTS) have inadequate shortening of the QT interval in response to the sudden heart rate accelerations provoked by standing-a phenomenon of diagnostic value. We now validate our original observations in a cohort twice as large. We also describe that this abnormal QT-interval response persists as the heart rate acceleration returns to baseline. OBJECTIVES: To describe a novel observation, termed "QT stunning" and to validate previous observations regarding the "QT-stretching" phenomenon in patients with LQTS by using our recently described "standing test." METHODS: The electrocardiograms of 108 patients with LQTS and 112 healthy subjects were recorded in the supine position. Subjects were then instructed to stand up quickly and remain standing for 5 minutes during continuous electrocardiographic recording. The corrected QT interval was measured at baseline (QTc(base)), when heart rate acceleration without appropriate QT-interval shortening leads to maximal QT stretching (QTc(stretch)) and upon return of heart rate to baseline (QTc(return)). RESULTS: QTc(stretch) lengthened significantly more in patients with LQTS (103 ± 80 ms vs 66 ± 40 ms in controls; P <.001) and so did QTc(return) (28 ± 48 ms for patients with LQTS vs -3 ± 32 ms for controls; P <.001). Using a sensitivity cutoff of 90%, the specificity for diagnosing LQTS was 74% for QTc(base), 84% for QTc(return), and 87% for QTc(stretch). CONCLUSIONS: The present study extends our previous findings on the abnormal response of the QT interval in response to standing in patients with LQTS. Our study also shows that this abnormal response persists even after the heart rate slows back to baseline.

Predictors of acute and long-term success of slow pathway ablation for atrioventricular nodal reentrant tachycardia: a single center series of 1,419 consecutive patients.

BACKGROUND: Atrioventricular nodal reentrant tachycardia (AVNRT) is the most common mechanism of supraventricular tachycardia. Slow pathway (SP) ablation is the first-line treatment approach with a high acute success rate and a low risk of inadvertent complete atrioventricular (AV) block. However, there is still some uncertainty as to the most appropriate procedural endpoints and the impact of these on risk of recurrence. We report the acute and long-term results of SP ablation in a large single-center consecutive series and analyze predictors of acute success and late recurrence. METHODS: The study included 1,448 consecutive procedures in 1,419 patients with AVNRT (mean age 49 ± 17 years, 66% women) who underwent SP ablation using a combined electrophysiologic and anatomic approach. Univariate and multivariate analysis was performed for potential predictors of acute success and late recurrence. RESULTS: Acute success was achieved in 98.1%. Transient (first, second, or third degree) AV block occurred during the procedure in 20 (1.41%) patients. One patient (0.07%) had persistent first-degree and transient second-degree AV block after ablation and underwent pacemaker implant at day 21. Of the 1,391 patients with successful ablation, 22 patients (1.5%) developed AVNRT recurrence during a follow-up period of 63 ± 38 months. The only independent predictor of reduced procedural success was the presence of atypical AVNRT (hazard ratio 3.1, P = 0.04). Independent predictors of AVNRT recurrence were age <20 years and female gender (hazard ratios 14.1 and 3.7, respectively). No significant difference in the incidence of late recurrence was observed in patients with or without residual slow-pathway conduction, or according to use of isoproterenol testing or general anesthesia. However, patients with a single echo with recurrence had a significantly larger echo window (median 85 ms) than those without (median 30 ms, P = 0.01). CONCLUSIONS: This study demonstrates in a large consecutive single-center series that SP ablation using radiofrequency energy is a highly effective procedure with an extremely low risk of inadvertent AV block and a low recurrence rate. We found that single-AV nodal echo beats represented a procedural endpoint that did not predict AVNRT recurrence but that a large echo window is associated with recurrence. Recurrence rates in this series were higher in young women, possibly reflecting a more conservative approach to ablation in this age group.

Dissociated pulmonary vein potentials following antral pulmonary vein isolation for atrial fibrillation: impact on long-term outcome.

BACKGROUND: Pulmonary vein (PV) reconnection is the Achilles heel of pulmonary vein isolation (PVI) for atrial fibrillation (AF). Dissociated pulmonary vein potentials (dPVP) may reflect abnormal PV automaticity, indicate more extensive PV muscular sleeve or may simply be an epiphenomenon. OBJECTIVE: This study sought to determine the incidence, characteristics and prognostic significance of dPVP following PVI for AF. METHODS: 89 consecutive patients (mean age 58.2 ± 8.4 years, 75% male, 74% paroxysmal, 26% persistent AF) underwent antral PVI using three-dimensional mapping systems with image integration with the endpoint of bidirectional PV block. Following PV electrical isolation the presence and characteristics of dPVP were recorded. Holter monitoring was performed at 3, 6 and 12 months. Acute PV reconnection was assessed over a 30-min waiting period. RESULTS: Electrical isolation was achieved in all 372 PV targeted for ablation. 69 of 372 isolated PV (19%) demonstrated dPVP after acute electrical isolation. Sites of dPVP origin were the left superior in 36%, left inferior in 20%, right superior in 31% and right inferior in 12%. All 69 dPVP demonstrated slow activity (cycle length >1500 ms) with only four persisting more than 30 min after acute isolation. There was no difference in the clinical characteristics between dPVP-positive vs dPVP-negative patients. At a mean follow-up of 21 ± 8 months the single procedure success was 25/33 (76%) in dPVP-positive versus 39/60 (64%) in dPVP-negative patients (p = -0.3). In the eight dPVP-positive patients who underwent a second procedure, 11 of the 14 (79%) veins with initial dPVP demonstrated PV-left atrial reconnection. CONCLUSION: dPVP are present in 19% of PV following acute antral electrical isolation. The presence of dPVP did not predict recurrent AF following PVI.

Long-term outcome following ablation of atrial flutter occurring late after atrial septal defect repair.

AIMS: In patients with surgical atrial septal defect (ASD) repair, late atrial flutters (AFLs), including cavotricuspid isthmus (CTI)-dependent and non-CTI-dependent scar-related flutter (AFL), are common. Radiofrequency ablation (RFA) of these arrhythmias has a high acute success rate. We aimed to characterize the long-term freedom from atrial arrhythmias in this population. METHODS: Twenty consecutive patients undergoing RFA for AFL late after ASD repair were included. Electrophysiological assessment included multipolar activation, entrainment, and electroanatomic mapping. Clinical, electrocardiograph, and Holter monitoring follow-up was conducted every 6 months. RESULTS: Mean age was 53 ± 13 years. Time from surgical repair to RFA was 29 ± 15 years. All patients had CTI-dependent AFL (20/20). There were 1.6 ± 0.7 arrhythmias per patient; other arrhythmias included non-CTI-dependent AFL (14), focal atrial tachycardia (two), and atrioventricular nodal reentry tachycardia (two) . Acute success was obtained in 100%. Five patients with recurrent AFL (three CTI dependent, two non-CTI dependent) at 13 ± 8 months had successful repeat RFA. At 3.2 ± 1.6 years follow-up since the last procedure, 90% of patients with successful RFA for AFL remained free of their clinical arrhythmia. However, 30% of the original 20 patients had documented atrial fibrillation (AF) 2.1 ± 1.6 years after the last procedure; five (25%) required AF intervention. One stroke (5%) occurred in the context of late AF. CONCLUSION: RFA of AFL occurring late after surgical ASD repair has a low long-term risk of recurrence, although 25% of patients required two procedures. However, there is a high late incidence of AF (30%), with an additional 25% of patients requiring intervention for AF.

Low risk of major complications associated with pulmonary vein antral isolation for atrial fibrillation: results of 500 consecutive ablation procedures in patients with low prevalence of structural heart disease from a single center.

OBJECTIVES: To report the major complication rate associated with pulmonary vein antral isolation (PVAI) in a consecutive series of 500 patients from a single center. BACKGROUND: Catheter ablation for atrial fibrillation (AF) is an established procedure for refractory AF. However, the risk of major complications has been reported to range from 3.9% to 4.5% and continues to represent a cause for concern. We hypothesized that these studies may have overestimated the rate of major complications associated with PVAI in patients with a low prevalence of structural heart disease (SHD). METHODS: Data were prospectively collected from 500 consecutive AF ablation procedures on 424 patients (mean age 55 ± 11 years, 79% men, paroxysmal AF-80% and persistent AF-20%, CHADS2 scores of 0, 1, 2, 3 present in 64%, 28%, 7%, 1%, respectively), performed between July 2006 and September 2009. All procedures were performed under general anesthesia with intraoperative transesophageal echo. PVAI was performed using a nonfluoroscopic mapping system with an endpoint of PV isolation. Adjunctive left atrial ablation was performed in 21% of patients only. Major complications were defined from a compilation of those reported in 5 prior studies reporting complications. RESULTS: In 500 procedures, there were no instances of death, stroke/TIA, cardiac tamponade, atrioesophageal fistula, or PV stenosis. Major complications occurred in 4 procedures (0.8%): esophageal hematoma (TEE probe)--2; pharyngeal trauma--1; and retroperitoneal hematoma-1. CONCLUSIONS: AF ablation can be performed safely in young patients without structural heart disease with a low risk (<1%) of major complications when using a strategy of PVAI.

Prevalence of fractionated electrograms in the coronary sinus: comparison between patients with persistent or paroxysmal atrial fibrillation and a control population.

BACKGROUND: Complex fractionated atrial electrograms (CFAEs) are often identified as targets for radiofrequency ablation in the coronary sinus (CS) of patients with atrial fibrillation (AF). OBJECTIVE: The purpose of this study was to determine whether similar features are present in a normal control population. METHODS: Twenty-four patients with AF (12 paroxysmal, 12 persistent) were compared with 12 controls (undergoing radiofrequency ablation for supraventricular tachycardia) in whom at least 1 minute of AF was induced by rapid atrial pacing. Electrogram comparisons during sinus rhythm and AF were made offline. A random 10-second window of AF was used for analysis of fractionation and dominant frequency. RESULTS: The three groups were age matched. CFAEs during AF were less prevalent in the control versus the AF groups (control = 30% +/- 28%, paroxysmal AF = 63% +/- 34%, persistent AF = 62% +/- 29%, P = .01). This difference was significant for the proximal to mid-CS only. Conduction velocity within the CS was slower in AF versus control patients (paroxysmal AF = 51 +/- 6 cm/s, persistent AF = 52 +/- 6 cm/s, control = 73 +/- 11 cm/s, P <.001). Minimum AF cycle length was shorter in the AF groups versus the control group (paroxysmal AF = 132 +/- 34 ms, persistent AF = 127 +/- 34 ms, control = 168 +/- 30 ms, P = .01). No differences in dominant frequency or prevalence of sinus rhythm CFAE was seen among the three groups. CONCLUSION: AF patients have a higher prevalence of CFAE and short cycle length activation within the proximal CS than control patients with nonclinical AF. CFAE are associated with slowed CS conduction in AF patients. No difference in the dominant frequency during AF was seen. CS CFAEs are common in a control population with induced AF and are unlikely to signify clinically important AF drivers in this setting.

Long-term outcome following successful catheter ablation of atrial tachycardia originating from the pulmonary veins: absence of late atrial fibrillation.

OBJECTIVES: This study aimed to characterize the long-term outcome and incidence of atrial fibrillation (AF) in patients following catheter ablation of focal atrial tachycardia (AT) from the pulmonary veins (PV). BACKGROUND: Although both AT and AF may originate from ectopic foci within PVs, it is unknown whether PV AT patients subsequently develop AF. METHODS: Twenty-eight patients with 29 PV ATs (14%) from a consecutive series of 194 patients who underwent RFA for focal AT were included. Patients with concomitant AF prior to the index procedure were excluded. RESULTS: The minimum follow-up duration was 4 years; mean age 38 +/- 18 years with symptoms for 6.5 +/- 10 years, having tried 1.5 +/- 0.9 antiarrhythmic drugs. The distribution of foci was: left superior 12 (41%), right superior 10 (34%), left inferior 5 (17%), and right inferior 2 (7%). The focus was ostial in 93% and 2-4 cm distally within the vein in 7%. Mean tachycardia cycle length was 364 +/- 90 ms. Focal ablation was performed in 25 of 28 patients. There were 6 recurrences with 5 from the original site. Twenty-six patients were available for long-term clinical follow-up. At a mean of 7.2 +/- 2.1 years, 25 of 26 (96%) were free from recurrence off antiarrhythmic drugs. No patients developed AF. CONCLUSIONS: Focal ablation for tachycardia originating from the PVs is associated with long-term freedom from both AT and AF. Therefore, although PV AT and PV AF share a common anatomic distribution, PV AT is a distinct clinical entity successfully treated with focal RFA and not associated with AF in the long term.

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.

Vagal paroxysmal atrial fibrillation: prevalence and ablation outcome in patients without structural heart disease.

INTRODUCTION: The prevalence of vagal and adrenergic atrial fibrillation (AF) and the success rate of pulmonary vein isolation (PVI) are not well defined. We investigated the prevalence of vagal and adrenergic AF and the ablation success rate of antral pulmonary vein isolation (APVI) in patients with these triggers compared with patients with random AF. METHODS AND RESULTS: Two hundred and nine consecutive patients underwent APVI due to symptomatic drug refractory paroxysmal AF. Patients were diagnosed as vagal or adrenergic AF if >90% of AF episodes were related to vagal or adrenergic triggers; otherwise, a diagnosis of random AF was made. Clinical, electrocardiogram (ECG), and Holter follow-up was every 3 months in the first year and every 6 months afterward and for symptoms. Of 209 patients, 57 (27%) had vagal AF, 14 (7%) adrenergic AF, and 138 (66%) random AF. Vagal triggers were sleep (96.4%), postprandial (96.4%), late post-exercise (51%), cold stimulus (20%), coughing (7%), and swallowing (2%). At APVI, 94.3% of patients had isolation of all veins. Twenty-five (12%) patients had a second APVI. At a follow-up of 21 +/- 15 months, the percentage of patients free of AF was 75% in the vagal group, 86% in the adrenergic group, and 82% for random AF (P = 0.51). CONCLUSION: In patients with PAF and no structural heart disease referred for APVI, vagal AF is present in approximately one quarter. APVI is equally effective in patients with vagal AF as in adrenergic and random AF.

Linear ablation of right atrial free wall flutter: demonstration of bidirectional conduction block as an endpoint associated with long-term success.

INTRODUCTION: Ablation for atypical atrial flutter (AFL) is often performed during tachycardia, with termination or noninducibility of AFL as the endpoint. Termination alone is, however, an inadequate endpoint for typical AFL ablation, where incomplete isthmus block leads to high recurrence rates. We assessed conduction block across a low lateral right atrial (RA) ablation line (LRA) from free wall scar to the inferior vena cava (IVC) or tricuspid annulus in 11 consecutive patients with atypical RA free wall flutter. METHOD AND RESULTS: LRA block was assessed following termination of AFL, by pacing from the ablation catheter in the low lateral RA posterior to the ablation line and recording the sequence and timing of activation anterior to the line with a duodecapole catheter, and vice versa for bidirectional block. LRA block resulted in a high to low activation pattern on the halo and a mean conduction time of 201 +/- 48 ms to distal halo. LRA conduction block was present in only 2 out of 6 patients after termination of AFL by ablation. Ablation was performed during sinus rhythm (SR) in 9 patients to achieve LRA conduction block. No recurrence of AFL was observed at long-term follow-up (22 +/- 12 months); 3 patients developed AF. CONCLUSION: Termination of right free wall flutter is often associated with persistent LRA conduction and additional radiofrequency ablation (RFA) in SR is usually required. Low RA pacing may be used to assess LRA conduction block and offers a robust endpoint for atypical RA free wall flutter ablation, which results in a high long-term cure rate.