Early and Late Postoperative Tachyarrhythmias in Children and Young Adults Undergoing Congenital Heart Disease Surgery.

The population of patients with congenital heart disease is constantly growing with an increasing number of individuals reaching adulthood. A significant proportion of these children and young adults will suffer from tachyarrhythmias due to the abnormal anatomy, the hemodynamic burden, or as a sequela of surgical treatment. Depending on the underlying mechanism, arrhythmias may arise in the early postoperative period (hours to days after surgery) or in the late postoperative period (usually years after surgery). A good understanding of the electrophysiological characteristics and pathophysiological mechanisms is therefore crucial to guide the therapeutic approach. Here, we synthesize the current state of knowledge on epidemiological features, risk factors, pathophysiological insights, electrophysiological features, and therapy regarding tachyarrhythmias in children and young adults undergoing reparative surgery for congenital heart disease. The evolution and latest data on treatment options, including pharmacological therapy, ablation procedures, device therapy decision, and thromboprophylaxis, are summarized. Finally, throughout this comprehensive review, knowledge gaps and areas for future research are also identified.

Significance of post-pacing intervals shorter than tachycardia cycle length for successful catheter ablation of atypical flutter.

AIMS: During entrainment mapping of macro-reentrant tachycardias, the time difference (dPPI) between post-pacing interval (PPI) and tachycardia cycle length (TCL) is thought to be a function of the distance of the pacing site to the re-entry circuit and dPPI < 30 ms is considered within the re-entry circuit. This study assessed the importance of PPI < TCL as a successful target for atypical flutter ablation. METHODS AND RESULTS: A total of 177 ablation procedures were investigated. Surface electrocardiograms (ECGs) were evaluated and combined activation and entrainment mapping were performed to choose ablation sites. Each entrainment sequence immediately preceding static radiofrequency delivery at the same site was analysed. A total of 545 entrainment sequences were analysed. dPPI < 0 ms was observed in 45.3% (247/545) sequences. Ablation resulted in tachycardia termination more often at sites with dPPI < 0 (27.8% vs. 14.5%, P < 0.001) and with a progressively increasingly inverse correlation between dPPI duration and ablation success [odds ratio (OR): 0.974; 95% confidence interval (CI) 0.960-0.988; P < 0.001]. Tachycardia termination or cycle length prolongation also occurred more often at sites with dPPI < 0 (50.6% vs. 33.2%, P < 0.001) and with a similar inverse correlation with dPPI duration (OR: 0.972; 95% CI 0.960-0.984; P < 0.001). Twelve-lead synchronous isoelectric intervals were observed in 64.4% (163/253) flutter ECGs and were associated with a dPPI < 0 (75.3% vs. 55.8%, P < 0.001). CONCLUSION: When combined with activation mapping, a negative dPPI is a more effective parameter for identifying a target for successful ablation compared to a dPPI = 0-30 ms. Its occurrence is associated with a critical small narrow slow-conducting isthmus at the target site.

Sustained and self-terminating atrial fibrillation induced immediately after pulmonary vein isolation exhibit differences in coronary sinus electrical activity from onset.

INTRODUCTION: Little data exists on the electrophysiological differences between sustained atrial fibrillation (sAF; >5 minutes) vs self-terminating nonsustained AF (nsAF; <5 minutes). We sought to investigate the electrophysiological characteristics of coronary sinus (CS) activity during postpulmonary vein isolation (PVI) sAF vs nsAF. METHODS AND RESULTS: We studied 142 patients post-PVI for paroxysmal AF (PAF). In a 50-patient subset, CS electrograms in the first 30 seconds of induced AF were analyzed manually. A custom-made algorithm for automated electrogram annotation was derived for validation on the whole patient set. In patients with sAF post-PVI, CS fractionated potentials were ablated. Manual analysis showed that patients with sAF exhibited higher activation pattern variability (2.1 vs 0.5 changes/sec; P < .001); fewer proximal-to-distal wavefronts (25 vs 61%; P < .001); fewer unidirectional wavefronts (60 vs 86%; P < .001); more pivot locations (4.3 vs 2.1; P < .001); shorter cycle lengths (190 vs 220 ms; P < .001); and shorter cumulative isoelectric segments (35 vs 44%; P = .045) compared to nsAF. These observations were confirmed on the whole study population by automated electrogram annotation and sample entropy computation (SampEn: 0.29 ± 0.15 in sAF vs 0.15 ± 0.05 in nsAF; P < .0001). The derived model predicted sAF with 78% sensitivity, 88% specificity; agreement with manual model: 88% (Cohen's kappa= 0.76). CS defragmentation resulted in AF termination or noninducibility in 49% of sAF. CONCLUSION: In PAF patients post-PVI, induced sAF shows greater activation sequence variability, shorter cycle length, and higher SampEn in the CS compared to nsAF. Automated electrogram annotation confirmed these results and accurately distinguished self-terminating nsAF episodes from sAF based on 30-second recordings at AF onset.

Anatomical and Electrophysiological Characteristics of Dual-Loop Re-Entry in Atypical Atrial Flutter: Implications for Mapping and Catheter Ablation.

Background: Atypical atrial flutter (AFL) can be challenging to ablate, especially when involving dual-loop re-entry. We sought to assess the electroanatomical characteristics of single- and dual-loop AFLs in patients undergoing catheter ablation. Methods: We analyzed 25 non-cavotricuspid isthmus-dependent macro-re-entrant AFL in 19 consecutive patients. Three-dimensional high-density activation mapping was performed, and active re-entry loops were confirmed by entrainment mapping. Results: Of 25 AFLs (24 left, 1 right atrial), 13 (52%) exhibited dual-loop re-entry. The most common circuits included, in 6/13 (46% of dual loops), a perimitral re-entry with a second loop around the right/left pulmonary veins (PV) and, in 6/13 (46%), involved a right PV ostium with a second loop around either a functional conduction block or another PV. Ablation at the common isthmus of dual-loop AFLs and at the critical isthmus of single-loop AFLs terminated the arrhythmia more frequently than ablation at a secondary isthmus of dual-loop AFLs (5/6 (83%) and 8/11 (73%) versus 1/8 (13%), respectively, p = 0.013). Conclusions: More than half of AFLs exhibited a dual-loop re-entrant mechanism. Most critical isthmuses were found at the mitral isthmus, the left atrial roof or right PV ostia. Ablation targeting the common isthmus resulted in a higher termination rate.

Safety, Efficacy and Prognostic Benefit of Atrial Fibrillation Ablation in Heart Failure with Preserved Ejection Fraction.

Up to 65% of patients with heart failure with preserved ejection fraction (HFpEF) develop AF during the course of the disease. This occurrence is associated with adverse outcomes, including pump failure death. Because AF and HFpEF are mutually reinforcing risk factors, sinus rhythm restoration may represent a disease-modifying intervention. While catheter ablation exhibits acceptable safety and efficacy profiles, no randomised trials have compared AF ablation with medical management in HFpEF. However, catheter ablation has been reported to result in lower natriuretic peptides, lower filling pressures, greater peak cardiac output and improved functional capacity in HFpEF. There is growing evidence that catheter ablation may reduce HFpEF severity, hospitalisation and mortality compared to medical management. Based on indirect evidence, early catheter ablation and minimally extensive atrial injury should be favoured. Hence, individualised ablation strategies stratified by stepwise substrate inducibility provide a logical basis for catheter-based rhythm control in this heterogenous population. Randomised trials are needed for definitive evidence-based guidelines.

Area under the real-time contact force curve (force-time integral) predicts radiofrequency lesion size in an in vitro contractile model.

INTRODUCTION: Electrode tissue contact, radiofrequency (RF) power and duration are major determinants of RF lesion size. Since contact forces (CF) vary in the beating heart, we evaluated contact force-time integral (FTI) as a predictor of lesion size at constant RF power in a contractile bench model simulating the beating heart. METHODS AND RESULTS: An open-tip irrigated catheter was attached to a movable mount incorporating a dynamic force sensor allowing closed loop control to achieve desired force variations between the catheter tip and bovine skeletal muscle placed on a ground plate. RF energy (20 and 40 W for 60 seconds, 17 cc/min irrigation) was delivered during (1) constant contact (C) at 20 g, (2) variable contact (V) with a 20 g peak and 10 g nadir, and (3) intermittent contact (I) with a 20 g peak and 0 g nadir with loss of contact. V and I protocols were performed at 50 and 100 catheter movements/min and 2 systole:diastole time ratios (50:50 and 30:70). The area under the CF curve was calculated as the FTI. Measured FTI was highest in C, intermediate during V and lowest during I and correlated linearly with lesion volume (P < 0.0001 for 20 and 40 W). Lesion volume was highest in group C, intermediate in V and lowest in group I (P < 0.05 for C vs V, V vs I, and C vs I). CONCLUSIONS: Lesion size correlates linearly with measured contact FTI. Constant contact produces the largest and intermittent contact the smallest lesions despite constant RF power and identical peak contact forces.

Right Atrial Complexity Evolves With Stepwise Left-Sided Persistent Atrial Fibrillation Substrate Ablation and Predicts Outcomes.

OBJECTIVES: This study sought to study the relation between outcomes of modified stepwise atrial fibrillation (AF) substrate ablation and dynamic electrogram characteristics in the coronary sinus (CS) and right atrium (RA). BACKGROUND: Identifying patients with persistent AF who will benefit from limited lesion sets versus those requiring extensive substrate modification is challenging. METHODS: We studied 70 patients undergoing persistent AF ablation, 43 with acute success (successful ablation [sABL], AF termination, or noninducibility) and 27 with failure (failed ablation [fABL], no termination, or induced AF of >5 minutes). Dominant frequency (DF) and sample entropy (SampEn, increasing with signal complexity) were measured on 30-second recordings of wide-coverage simultaneous RA and CS electrograms during baseline AF and induced AF post-pulmonary vein isolation and after left-sided electrogram-guided ablation steps (on the CS with or without the left atrium [LA]). RESULTS: At baseline AF, patients with sABL exhibited lower RA SampEn (p = 0.023) and lower CS DF (p = 0.030) compared to fABL. A positive RA-to-CS SampEn gradient predicted ablation failure (48% vs. 19% for patients in fABL vs. sABL; p = 0.015). A positive RA-to-CS DF gradient developed in patients with fABL after extra-pulmonary vein substrate modification, unlike patients with sABL (p = 0.0008). At 24 months, 76% of patients were AF free, and 68% were arrhythmia free. sABL was associated with fewer AF recurrences (hazard ratio: 0.31; 95% confidence interval: 0.12-0.84; p = 0.021). A negative RA-to-CS SampEn gradient at baseline was associated with freedom from AF (-0.14 ± 0.19 vs. 0.04 ± 0.18; p = 0.002). CONCLUSIONS: RA greater than CS electrogram complexity gradients at baseline or developing during ablation are associated with unfavorable acute and long-term outcomes of persistent AF ablation. These parameters allow monitoring of the effects of left-sided substrate ablation and, therefore, a rational choice of additional RA substrate modification.

Individualised Approaches for Catheter Ablation of AF: Patient Selection and Procedural Endpoints.

Pulmonary vein isolation (PVI) is the cornerstone of AF ablation, but studies have reported improved efficacy with high rates of repeat procedures. Because of the large interindividual variability in the underlying electrical and anatomical substrate, achieving optimal outcomes requires an individualised approach. This includes optimal candidate selection as well as defined ablation strategies with objective procedure endpoints beyond PVI. Candidate selection is traditionally based on coarse and sometimes arbitrary clinical stratification such as AF type, but finer predictors of treatment efficacy including biomarkers, advanced imaging and electrocardiographic parameters have shown promise. Numerous ancillary ablation strategies beyond PVI have been investigated, but the absence of a clear mechanistic and evidence-based endpoint, unlike in other arrhythmias, has remained a universal limitation. Potential endpoints include functional ones such as AF termination or non-inducibility and substrate-based endpoints such as isolation of low-voltage areas. This review summarises the relevant literature and proposes guidance for clinical practice and future research.

Electrophysiological and Anatomical Correlates of Sites With Postpacing Intervals Shorter Than Tachycardia Cycle Length in Atypical Atrial Flutter.

Background Although entrainment mapping is an established approach to atypical atrial flutter ablation, postpacing intervals shorter than tachycardia cycle length (difference between postpacing interval and tachycardia cycle length [dPPI] <0 ms) remain of unknown significance. We sought to compare anatomic and electrophysiological properties of sites with dPPI <0, dPPI=0-30, and dPPI >30 ms. Methods We studied 24 noncavotricuspid isthmus-dependent macroreentrant atypical atrial flutter in 19 consecutive patients. Ultra high-density electroanatomic activation maps were acquired with a 64-electrode basket catheter. Entrainment mapping was performed at multiple candidate sites. Ablation was performed at the narrowest accessible slow-conducting critical isthmuses. Results Of 102 entrainment mapping sites, dPPI <30 was observed at 72 sites on complete maps of 24 atypical atrial flutter. Compared with dPPI=0-30 sites (N=45), dPPI<0 sites (N=27) were more commonly located within isthmuses <15 mm wide (67% versus 6.7%, P<0.00001; odds ratio, 28.0; 95% CI, 6.8-115.7), more frequently located within 5 mm of the leading wavefront (93% versus 64%, P=0.008), exhibited slower local conduction velocity (0.49±0.43 versus 0.93±0.57 m/s, P=0.0005), lower voltages (0.48±0.79 versus 0.92±0.97 mV, P=0.04), and more frequently fractionated electrograms (67% versus 24%, P=0.0004). High rates of arrhythmia termination or cycle length increase >15 ms by ablation were observed in both dPPI groups (94% versus 86%, P=0.53). Compared with all dPPI <30, dPPI >30 sites (N=30) were less commonly observed within isthmuses (3.3%, P<0.001) or within 5 mm of the leading wavefront (30%, P<0.0001); conduction velocity (1.0±0.7 m/s, P=0.002) and voltage (1.1±1.4 mV, P=0.049) were higher compared with dPPI<0 but similar to dPPI=0-30 sites. Conclusions In atypical atrial flutter, sites with dPPI <0 are markers of limited width critical isthmuses with slower conduction velocity, whereas sites with dPPI=0-30 ms are often not in close proximity to the reentry circuit. Virtual electrode simultaneous down and upstream (antidromic) capture of a confined isthmus of slow conduction can explain a dPPI <0. Identifying these sites may improve selective and efficient ablation strategies compared with the standard 30-ms threshold.

Evolution of post-pulmonary vein isolation atrial fibrillation inducibility at redo ablation: Electrophysiological evidence of extra-pulmonary vein substrate progression.

BACKGROUND: The electrophysiological substrate underlying atrial fibrillation (AF) progression remains difficult to identify. OBJECTIVE: The goals of this study were to study the evolution of post-pulmonary vein isolation (PVI) AF inducibility (AFI) after AF ablation and to compare patients with organized atrial tachycardia recurrence (OATr) versus those with paroxysmal or persistent AF recurrence. METHODS: We studied 99 patients who underwent de novo AF ablation (p1) and redo ablation (p2) for AF recurrence (AFr) or OATr. Stepwise AF ablation was performed at p1 and p2: (1) PVI, (2) coronary sinus defragmentation, and (3) left atrial (LA) defragmentation. Burst pacing followed each step, with AFI defined as sustained AF >5 minutes, triggering the next step. Patients with OATr underwent OAT ablation and inducibility testing post-redo PVI. Inducibility progression (IP) was defined as AFI at further steps of p2 compared to p1. RESULTS: Among patients with AFr, 34 of 72 patients (47%) exhibited post-PVI IP vs 2 of 27 (7.4%) patients with OATr (P = .0002). Stratification for persistent AF/paroxysmal AF/OATr showed a consistent association between recurrence phenotype and IP. Pulmonary vein (PV) reconnection incidence was 90%, without association with recurrence type or IP. LA volume was larger in patients with IP than in those without IP (86.7 ± 25.3 mL vs 72.0 ± 28.9 mL; P = .001). Right atrial dimensions increased between p1 and p2 in patients with IP vs no IP and in patients with AFr vs OATr. CONCLUSION: Patients with AFr after first ablation exhibit IP more frequently at redo ablation than do patients with OATr. IP correlates with more advanced AFr type, larger LA volumes, and progressive right atrial enlargement. PV reconnection is not associated with AFr. Changes in post-PVI AFI may accurately indicate progression of extra-PV AF-maintaining substrate.

Intra- and interatrial conduction abnormalities: hemodynamic and arrhythmic significance.

Alterations of normal intra- and interatrial conduction are a common outcome of multiple cardiovascular conditions. They arise most commonly in the context of advanced age, cardiovascular risk factors, organic heart disease, atrial fibrosis, and left atrial enlargement. Interatrial block (IAB), the most frequent and extensively studied atrial conduction disorder, affects up to 20% of the general primary care population. IAB can be partial (P wave duration ≥ 120 ms on any of the 12 ECG leads) or advanced (P wave ≥ 120 ms and biphasic morphology (positive-negative) in inferior leads). Advanced IAB is an independent risk factor for supraventricular tachyarrhythmias and embolic stroke in a variety of clinical settings. Advanced IAB is a cause of left atrial electromechanical dysfunction and left atrioventricular dyssynchrony and has been associated with left ventricular diastolic dysfunction. P wave duration is associated with cardiovascular and all-cause mortality in the general population. Atrial conduction abnormalities should be identified as markers of atrial remodeling, prognostic indicators, and, in the case of advanced IAB, a true arrhythmologic syndrome. IAB and other P wave abnormalities should prompt the search for associated conditions, the treatment of which may partially reverse atrial remodeling or prevent it if administered upstream. Future studies will help define the role of preventive therapeutic interventions in high-risk patients, including antiarrhythmic drug therapy and oral anticoagulation. Implications for the treatment of heart failure and for pacing should also be further investigated.

Benefit of atrial pacing in sleep apnea syndrome.

BACKGROUND: Many patients with sleep apnea syndrome have nocturnal bradycardia, paroxysmal tachyarrhythmias, or both, which can be prevented by permanent atrial pacing. We evaluated the effect of using cardiac pacing to increase the heart rate during sleep in patients with sleep apnea syndrome. METHODS: We studied 15 patients (11 men and 4 women; mean [+/-SD] age, 69+/-9 years) with central or obstructive sleep apnea who had received permanent atrial-synchronous ventricular pacemakers for symptomatic sinus bradycardia. All patients underwent three polysomnographic evaluations on consecutive nights, the first night for base-line evaluation and then, in random order, one night in spontaneous rhythm and one in dual-chamber pacing mode with atrial overdrive (basic rate, 15 beats per minute faster than the mean nocturnal sinus rate). The total duration and number of episodes of central or obstructive sleep apnea or hypopnea were analyzed and compared. RESULTS: The mean 24-hour sinus rate during spontaneous rhythm was 57 +/- 5 beats per minute at base line, as compared with 72 +/- 3 beats per minute with atrial overdrive pacing (P<0.001). The total duration of sleep was 321 +/- 49 minutes in spontaneous rhythm, as compared with 331 +/- 46 minutes with atrial overdrive pacing (P=0.48). The hypopnea index (the total number of episodes of hypopnea divided by the number of hours of sleep) was reduced from 9 +/- 4 in spontaneous rhythm to 3 +/-3 with atrial overdrive pacing (P<0.001). For both apnea and hypopnea, the value for the index was 28 +/- 22 in spontaneous rhythm, as compared with 11 +/- 14 with atrial overdrive pacing (P<0.001). CONCLUSIONS: In patients with sleep apnea syndrome, atrial overdrive pacing significantly reduces the number of episodes of central or obstructive sleep apnea without reducing the total sleep time.

Evaluation of an individualized strategy of cavotricuspid isthmus ablation as an adjunct to atrial fibrillation ablation.

OBJECTIVES: To evaluate supplementary cavotricuspid isthmus (CTI) ablation as an adjunct to atrial fibrillation (AF) ablation in selected patients. BACKGROUND: It is unclear whether routine CTI ablation is beneficial in all patients undergoing AF ablation. METHODS AND RESULTS: In patients undergoing AF ablation, additional CTI block was created only for those with typical atrial flutter (Afl) before or during the ablation. Out of 188 consecutive patients (108 male, 56 +/- 9 years), 75 underwent CTI ablation (Group CTI+) and left atrial (LA) ablation (circular mapping-guided extensive pulmonary vein isolation in all and linear LA ablation when required), while 113 underwent LA ablation alone (Group CTI-). Group CTI+ patients had smaller LA and less frequently persistent/permanent AF and linear LA ablation. Over a follow-up of 30 +/- 10 months, complications (4% vs 5%, P = NS), typical Afl occurrence (1.3% and 2.6%, P = NS) and AF recurrence (25% and 28%, P = NS) were similar. Atypical Afl was more common in Group CTI- (4 vs 14%, P = 0.026). Eighty-two percent and 79% of patients in Groups CTI+ and CTI-, respectively, remained arrhythmia free in stable sinus rhythm without antiarrhythmic drug treatment (P = NS). CONCLUSIONS: Avoiding supplementary CTI ablation in AF ablation patients without evidence of typical flutter does not result in a higher incidence of typical Afl. Despite more persistent/permanent AF and larger LA in patients without evidence of typical flutter, a strategy of selective supplementary ablation resulted in similar and low AF recurrence rates in the group without CTI ablation compared with the group with CTI ablation.

Recipient Atrial Fibrillation Manifesting as an Irregular Monomorphic Atrial Tachycardia in the Donor Heart.

A 63-year-old man presented with a 1-year history of atrial tachycardia (AT) 6 years after orthotopic heart transplantation with bicaval anastomosis. Twelve-lead electrocardiogram showed monomorphic AT with isoelectric intervals across all leads and strikingly irregular PP intervals. At electrophysiology study, the native left atrium's rhythm was atrial fibrillation (AF) while the donor atria exhibited centrifugal activation of irregular cycle length originating from a site along the left atrial anastomosis. Ablation at that site terminated AT to sinus rhythm. Recipient AF with recipient-to-donor conduction should be suspected in the presence of irregular monomorphic AT in orthotopic heart transplantation recipients. Catheter ablation of atrio-atrial conduction is safe and effective.

Atrial fibrillation originating from persistent left superior vena cava.

BACKGROUND: The left superior vena cava (LSVC) is the embryological precursor of the ligament of Marshall, which has been implicated in the initiation and maintenance of atrial fibrillation (AF). Rarely, the LSVC may persist and has been associated with some organized arrhythmias, though not with AF. We report 5 patients in whom the LSVC was a source of ectopy, initiating AF. METHODS AND RESULTS: In 5 patients (4 men; age, 46+/-11 years) with symptomatic drug-refractory AF, ectopy from the LSVC resulting in AF was observed after pulmonary vein isolation. The ectopics were spontaneous in 2 and induced by isoproterenol in the others and preceded P-wave onset by 67+/-13 ms. During multielectrode or electroanatomic mapping, venous potentials were recorded circumferentially at the proximal LSVC near its junction with the coronary sinus (CS), but at the mid-LSVC level, they were recorded only on part of the circumference. The LSVC was electrically connected to the lateral left atrium (LA) and through the CS to the right atrium, with 4.1+/-2.3 CS-LSVC and 1.6+/-0.5 LA-LSVC connections per patient. Catheter ablation in the LSVC targeting these connections resulted in electrical isolation in 4 of the 5 patients without complications. After 15+/-10 months, the 4 patients with successful isolation, including 1 who had successful reablation for LA flutter, remained in sinus rhythm without drugs. CONCLUSIONS: The LSVC can be the arrhythmogenic source of AF with connections to the CS and LA. Ablation of these connections resulted in electrical isolation.