Catheter ablation for atrial arrhythmias in adults with congenital heart disease: recurrence rates and predictors of acute procedural success

Abstract Funding Acknowledgements Type of funding sources: None. Background Heart rhythm disorders are an important cause of morbidity and emergency hospitalisation in patients with adult congenital heart disease (ACHD), and this is due to a combination of surgical scar, residual haemodynamic lesions, and cardiac chamber dilatation. The most effective available treatment is catheter ablation, although this can be extremely challenging owing to abnormal anatomy and problems accessing intra cardiac sites critical to the arrhythmia mechanism. However, outcomes of catheter ablation and analysis of factors which may predict recurrence of arrhythmia remain poorly defined. Purpose To define the cohort of ACHD patients undergoing catheter ablation for atrial arrhythmia in a large tertiary centre, characterise outcomes, and determine factors associated with arrhythmia recurrence. Methods Retrospective study of all catheter ablations for atrial arrhythmias in ACHD patients between April 13, 2016 and December 16, 2019 at our institution. Patients were identified using a field search through a centralised database; and pre-specified clinical and procedural data of interest, and time from ablation to recurrence were determined from the computerised electronic record. Binary logistical regression and cox regression analysis were used to determine potential predictors of acute procedural success and arrhythmia recurrence respectively. Results Among 90 patients (mean age 43 ± 15 years) who underwent catheter ablation for atrial arrhythmia, 39 (43%) were treated for macro-reentrant atrial tachycardia, 19 (21%) for focal atrial tachycardia, 9 (10%) for multifocal atrial tachycardia, 10 (10%) for atrial fibrillation, 7 (8%) for atrioventricular nodal reentrant tachycardia, and 6 (7%) for atrioventricular reentrant tachycardia. 35 (39%) of patients had "severe" complexity ACHD as per the Bethesda classification. 35 (39%) experienced recurrent arrhythmia with a median time to recurrence of 120 days. Age, gender, body mass index, complexity of congenital heart disease, and previous surgical repair were not identified as being significantly associated with recurrence, however univariate cox regression analysis showed a significantly longer time to recurrence in cases utilising electroanatomical mapping and demonstrating non-inducibility of arrhythmia in the lab post ablation (p < 0.001). There was 1 case of post-ablation bradycardia requiring pacemaker implantation, but no other complications. Conclusion Catheter ablation for atrial arrhythmia in ACHD patients is safe and effective, with a majority of patients achieving multiple arrhythmia-free months. Non-inducibility of arrhythmia post procedure and use of electroanatomical mapping are predictors of freedom from recurrence of atrial arrhythmia, suggesting effective characterisation and ablation of the arrhythmia mechanism is more important than the underlying substrate. These findings may aid management decisions for recurrent arrhythmia in ACHD patients.

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Catheter ablation in adults with congenital heart disease: a 15-year perspective from a tertiary center

European Heart Journal ◽

10.1093/ehjci/ehaa946.0437 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

V Waldmann ◽

D Amet ◽

A Zhao ◽

M Ladouceur ◽

C Karsenty ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Catheter Ablation ◽

Success Rate ◽

Contact Force ◽

Congenital Heart ◽

3D Mapping ◽

Density Mapping ◽

Tertiary Center ◽

Procedural Success

Abstract Background With the growing population of adults with congenital heart disease (ACHD), the number of catheter ablation procedures is expected to increase over time. Purpose We aimed to describe temporal trends in volume and outcomes of catheter ablation procedures in ACHD patients in a large tertiary center. Methods Retrospective observational study including all consecutive ACHD patients undergoing catheter ablation in a tertiary reference center over a 15-year period. Acute procedural success rate (including complete success in case of non-inducibility of any arrhythmia at the end of the procedure) as well as freedom from recurrence at 12 months were analyzed. Results From November 2004 to November 2019, 302 catheter ablations in 221 ACHD patients (43.6±15.0 years, 58.9% males) were performed. The annual number of catheter ablation increased progressively from 4 to 60 by year (p<0.001). Intra-atrial reentrant tachycardia/focal atrial tachycardia was the most common targeted arrhythmia (n=217, 71.9%). Over the study period, acute procedural success rate increased from 45.0% to 93.3% (p<0.001), including complete acute procedural success from 45.0% to 88.1% (p<0.001) (Figure 1). The use of irrigated catheters (30.0% to 94.8%, p<0.001), 3D-mapping systems (60.0% to 96.3%, p<0.001), contact force catheters (0.0% to 91.9%, <0.001), and high-density mapping (0.0% to 71.9%, p<0.01) increased significantly. Use of irrigated catheters (OR=3.96, 95% CI: 1.79–8.55), 3D-mapping system (OR=3.55, 95% CI: 1.62–7.55), contact force catheters (OR=3.46, 95% CI: 1.71–7.25), and high-density mapping (OR=3.85, 95% CI: 1.60–7.26) were associated with acute procedural success. The rate of freedom from any recurrence at 12 months increased from 29.4% to 66.2% (p=0.001). Seven (2.3%) non-fatal complications occurred. Conclusions The number of catheter ablation procedures in ACHD patients has considerably increased over the last 15 years. Advances in ablative technologies appear to be associated with a low rate of complications and a significant improvement in acute and midterm outcomes. Evolution of acute procedural success Funding Acknowledgement Type of funding source: None

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Contemporary Patients With Congenital Heart Disease: Uniform Atrial Tachycardia Substrates Allow for Clear Ablation End Points With Improved Long-Term Outcome

Circulation Arrhythmia and Electrophysiology ◽

10.1161/circep.120.009695 ◽

2021 ◽

Author(s):

Charlotte Brouwer ◽

Joachim Hebe ◽

Peter Lukac ◽

Jan-Hendrik Nürnberg ◽

Jens Cosedis Nielsen ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Atrial Tachycardia ◽

Congenital Heart ◽

Reentrant Tachycardia ◽

Term Outcome ◽

End Points ◽

Long Term Outcome ◽

Procedural Success

Background: Poor outcome after atrial tachycardia (AT) radiofrequency catheter ablation (RFCA) in repaired congenital heart disease (CHD) has been attributed to CHD complexity. This may not apply to contemporary patients. The objective of our study was to assess outcome after RFCA for AT in contemporary patients with CHD according to prior atrial surgery and predefined procedural end points. Methods: Patients with CHD referred for AT RFCA to 3 European centers were classified as no atrial surgery/cannulation only, limited or extensive prior atrial surgery. Procedural success was predefined as termination and nonreinducibility for focal AT and bidirectional block across ablation lines for intra-atrial reentrant tachycardia and after empirical substrate ablation for noninducible patients. Patients were followed for AT recurrence and mortality. Results: Ablation was performed in 290 patients (41±17 years, 59% male; 3-dimensional mapping 89%, irrigated tip catheters 90%, transbaffle access 15%). In 197, 233 AT were targeted (196 intra-atrial reentrant tachycardia [64% cavotricuspid (mitral) isthmus–dependent, 33% systemic-venous incision–dependent] and 37 focal AT). In 93 noninducible patients, empirical substrate ablation was performed. Procedural success was achieved in 209 (84%) patients. AT recurred in 148 (54%) 10 (interquartile range, 0–25) months after RFCA. AT-free survival was significantly better in patients with no atrial repair/cannulation only and in patients with complete procedural success independently of CHD complexity. From 94 patients undergoing reablation, the initially targeted substrate had recovered in 64%. Conclusions: In contemporary patients with CHD, outcome after AT ablation is associated with presence of prior atrial surgery and achievement of predefined procedural end points rather than CHD complexity. Techniques to improve lesion durability are likely to further improve long-term outcome.

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Catheter Ablation for Atrial Tachycardia in Adults With Congenital Heart Disease

JACC: Clinical Electrophysiology ◽

10.1016/j.jacep.2018.10.011 ◽

2019 ◽

Vol 5 (4) ◽

pp. 438-447 ◽

Cited By ~ 8

Author(s):

Christopher S. Grubb ◽

Matthew Lewis ◽

William Whang ◽

Angelo Biviano ◽

Kathleen Hickey ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Catheter Ablation ◽

Atrial Tachycardia ◽

Congenital Heart

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Depression Associated with Reduced Heart Rate Variability Predicts Outcome in Adult Congenital Heart Disease

Journal of Clinical Medicine ◽

10.3390/jcm10081554 ◽

2021 ◽

Vol 10 (8) ◽

pp. 1554

Author(s):

Mechthild Westhoff-Bleck ◽

Lars H. Lemke ◽

Jan-Marc S. Bleck ◽

Anja C. Bleck ◽

Johann Bauersachs ◽

...

Keyword(s):

Heart Rate ◽

Congenital Heart Disease ◽

Heart Rate Variability ◽

Regression Analysis ◽

Heart Disease ◽

Congenital Heart ◽

Cox Regression ◽

Adult Congenital Heart Disease ◽

Cox Regression Analysis ◽

Adult Congenital

In adult congenital heart disease (ACHD), major depressive disorder (MDD) represents a frequent comorbidity. In non-CHD, adverse outcome is predicted by MDD and heart rate variability (HRV), whereas in ACHD their prognostic relevance is unknown. We prospectively evaluated 171 patients (age 35.6 ± 11.4 years; male 42.7%, mean observation time 54.7 ± 14.9 months). Binary regression analysis calculated the association between MDD and HRV. Cox proportional survival analysis estimated their impact on decompensated heart failure and all-cause mortality (HF/death), supraventricular and ventricular tachycardia (SVT/VT), and hospitalization due to unexpected cardiac causes. Exclusively MDD with moderate/severe symptoms showed significantly lower HRV as derived from frequency-domain analysis (Symindex) (p = 0.013). In multivariate Cox regression analysis, patients stratified according to the lower quartile of the Symindex comorbid with MDD (n = 16) exhibited poorer prognosis regarding HF/death (Hazard Ratio (HR): 7.04 (95%CI:(1.87–26.5)), SVT/VT (HR: 4.90 (95%CI:1.74–9.25)) and hospitalization (HR: 3.80 (95%CI:1.36–10.6)). An additional independent predictor was N-terminal pro-B-type natriuretic peptide elevation (p < 0.001), indicating advanced HF and heart disease complexity (p < 0.001). Autonomic nervous system dysfunction measured by altered HRV is considered to be one of the pathways linking MDD and adverse outcomes in cardiac diseases. Our results exceed the existing literature by demonstrating that MDD with decreased HRV is associated with poorer prognosis in ACHD.

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Hospitalisations for heart failure predict mortality in pulmonary hypertension related to congenital heart disease

Heart ◽

10.1136/heartjnl-2018-313613 ◽

2018 ◽

Vol 105 (6) ◽

pp. 465-469 ◽

Cited By ~ 5

Author(s):

Despoina Ntiloudi ◽

Sotiria Apostolopoulou ◽

Konstantinos Vasiliadis ◽

Alexandra Frogoudaki ◽

Aphrodite Tzifa ◽

...

Keyword(s):

Heart Failure ◽

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Congenital Heart ◽

Cox Regression ◽

Strong Predictor ◽

Adult Congenital Heart Disease ◽

Significant Proportion ◽

Cox Regression Analysis

ObjectiveDespite the progress in the management of patients with adult congenital heart disease (ACHD), a significant proportion of patients still develop pulmonary hypertension (PH). We aimed to highlight the rate of the complications in PH-ACHD and the predicting factors of cumulative mortality risk in this population.MethodsData were obtained from the cohort of the national registry of ACHD in Greece from February 2012 until January 2018.ResultsOverall, 65 patients receiving PH-specific therapy were included (mean age 46.1±14.4 years, 64.6% females). Heavily symptomatic (New York Heart Association (NYHA) class III/IV) were 53.8% of patients. The majority received monotherapy, while combination therapy was administered in 41.5% of patients. Cardiac arrhythmia was reported in 30.8%, endocarditis in 1.5%, stroke in 4.6%, pulmonary arterial thrombosis in 6.2%, haemoptysis in 3.1% and hospitalisation due to heart failure (HF) in 23.1%. Over a median follow-up of 3 years (range 1–6), 12 (18.5%) patients died. On univariate Cox regression analysis history of HF hospitalisation emerged as a strong predictor of mortality (HR 8.91, 95% CI 2.64 to 30.02, p<0.001), which remained significant after adjustment for age and for NYHA functional class.ConclusionsLong-term complications are common among patients with PH-ACHD. Hospitalisations for HF predict mortality and should be considered in the risk stratification of this population.

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Outcomes of Atrial Arrhythmia Surgery in Patients With Congenital Heart Disease: A Systematic Review

Journal of the American Heart Association ◽

10.1161/jaha.120.016921 ◽

2020 ◽

Vol 9 (19) ◽

Author(s):

Charlotte A. Houck ◽

Natasja M. S. de Groot ◽

Isabella Kardys ◽

Christa D. Niehot ◽

Ad J. J. C. Bogers ◽

...

Keyword(s):

Atrial Fibrillation ◽

Systematic Review ◽

Congenital Heart Disease ◽

Heart Disease ◽

Atrial Tachycardia ◽

Congenital Heart ◽

Surgical Techniques ◽

Atrial Arrhythmia ◽

Number Of Patients ◽

Arrhythmia Surgery

Background The improved life expectancy of patients with congenital heart disease is often accompanied by the development of atrial tachyarrhythmias. Similarly, the number of patients requiring redo operations is expected to continue to rise as these patients are aging. Consequently, the role of arrhythmia surgery in the treatment of atrial arrhythmias is likely to become more important in this population. Although atrial arrhythmia surgery is a well‐established part of Fontan conversion procedures, evidence‐based recommendations for arrhythmia surgery for macroreentrant atrial tachycardia and atrial fibrillation in other patients with congenital heart disease are still lacking. Methods and Results Twenty‐eight studies were included in this systematic review. The median reported arrhythmia recurrence was 13% (interquartile range, 4%–26%) during follow‐up ranging from 3 months to 15.2 years. A large variation in surgical techniques was observed. Based on the acquired data, biatrial lesions are more effective in the treatment of atrial fibrillation than exclusive right‐sided lesions. Right‐sided lesions may be more appropriate in the treatment of macroreentrant atrial tachycardia; evidence for the superiority of additional left‐sided lesions is lacking. There are not enough data to support the use of exclusive left‐sided lesions. Theoretically, prophylactic atrial arrhythmia surgery may be beneficial in this population, but evidence is currently limited. Conclusions To be able to provide recommendations for arrhythmia surgery in patients with congenital heart disease, future studies should report outcomes according to the type of preoperative arrhythmia, underlying congenital heart disease, lesion set, and energy source. This is essential for determining which surgical techniques should ideally be applied under which circumstances.

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Outcomes of adults with congenital heart disease and pulmonary hypertension in the advanced pulmonary vasodilator era. A regional multicenter registry (RACCA)

European Heart Journal ◽

10.1093/ehjci/ehaa946.2213 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

A Wals Rodriguez ◽

C Federero Fernandez ◽

M.J Rodriguez Puras ◽

R Garcia Orta ◽

E Moreno Escobar ◽

...

Keyword(s):

Heart Failure ◽

Congenital Heart Disease ◽

Heart Disease ◽

Cause Of Death ◽

Congenital Heart ◽

Cox Regression ◽

Dual Therapy ◽

Cox Regression Analysis ◽

Pulmonary Vasodilator ◽

Mortality Causes

Abstract The objective of this study is to evaluate mortality, causes and risk factors of death in adults with congenital heart disease (ACHD) and PHT on Pulmonary vasodilator therapy (PVT). Methods 170 cases with PHT were identified in RACCA (Left heart disease were excluded (n=20)). We examined mortality, causes of death and complications during a mean follow-up (FU) of 7.9+4.6 years. For each patient demographic data, shunt location, clinical PHT group and functional class (FC) were collected. In an unselected sample of 103 patients, O2Sat, NT-proBNP, right ventricle function (RV) and 6-minute-test (6min) were retrospectively reviewed at 2 data collection time points: evaluation at baseline and the most recent data preceding death or at last clinical visit. Survival was assessed with Kaplan-Meier curves and differences between groups using the log-rank test. To look for predictors of death, Cox regression analysis was performed. Results Patients with PHT were predominantly women (61%), aged 45 years (IQR 33–58.5). The distribution of PHT group by underlying defects were 58 Eisenmenger (ES) (Complex 50%) and 45 non-ES (pretricuspid 46.7% (p=0,0002). PHT-pretricuspid defects occurred more in women with unrepaired simple defects and at older ages. In the sample, 85% were commenced on therapy with PVT (5% on initial dual therapy). The treated patients presented worse functional situation, more desaturation of O2 and worse TAPSE. In the group of treatment, 53% required treatment escalation during the FU. Initially, 82% were in sinus rhythm, in FC I-II 53% and in FC III-IV 39%. 10% had moderate-severe RV dysfunction. At the last visit, 38% were on monotherapy, 40% on dual therapy and 22% on triple therapy. FC improved from III-IV to I-II in 35% and 79% remained in FC-I-II without clinical deterioration. The Δ6min was +53±72 mts in those patients alive at the end of FU, and −126±26 mts in exitus cases (p=0,003), but O2 saturation did not change significantly. Over the FU, 29 patients died. The main cause of death was heart failure, followed by sudden death. The event-free survival was 84% at 5 years. Survival was significantly worst for patients presenting with arrhythmias and more advanced FC at initiation of PVT and for those who developed RV dysfunction over FU. At multivariate analysis, syndromic forms, pretricuspid shunt, non-ES physiology, PVT and monotherapy at baseline, FC III-IV and desaturation were independent predictors for death. Conclusions Although a positive clinical response was observed, mortality of PHT in CHD under advanced PVT remains high at mid-term FU. Heart failure is the leading cause of death. In terms of mortality, non ES patients and pretricuspid shunts were less responsive to treatment than patients with ES. Our results may suggest that patients with more advanced disease at the initiation of therapies do not respond properly and support the need for early treatment and initial dual therapy. Funding Acknowledgement Type of funding source: None

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Electrophysiologic Characteristics of Atrial Tachycardia Recurrence: Relevance to Catheter Ablation Strategies in Adults with Congenital Heart Disease

Heart Rhythm ◽

10.1016/j.hrthm.2021.10.003 ◽

2021 ◽

Author(s):

Jeremy P. Moore ◽

Austin Burrows ◽

Roberto G. Gallotti ◽

Kevin M. Shannon

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Catheter Ablation ◽

Atrial Tachycardia ◽

Congenital Heart

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Abstract 12140: Prognostic Value of Multiple Biomarkers on Mortality in Adults Congenital Heart Disease: Comparison of Single- and Two-Ventricle Physiology

Circulation ◽

10.1161/circ.130.suppl_2.12140 ◽

2014 ◽

Vol 130 (suppl_2) ◽

Author(s):

Kenji Miyamoto ◽

Daiji Takeuchi ◽

Kei Inai ◽

Tokuko Shinohara ◽

Toshio Nakanishi

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Cox Regression ◽

High Sensitivity ◽

Cardiovascular Death ◽

Blood Levels ◽

Type I ◽

Cox Regression Analysis ◽

Hs Crp

Introduction: Although many biomarkers are associated with heart failure, limited data is available on their prognostic predictive value in adults with congenital heart disease (ACHD). We investigate the potential of various biomarkers to predict ACHD mortality. Methods and Results: This is a single-center, retrospective cohort study. Blood levels of neurohormones (endothelin-1 [ET1], norepinephrine [NE], aldosterone, and plasma renin activity [PRA]); inflammatory biomarkers (high sensitivity C-reactive protein [hs-CRP], high sensitivity tumor necrosis factor [hs-TNF]-α, soluble TNF receptor type I and II [sTNF-RI and sTNF-RII], and interleukin-6 [IL-6]); and brain natriuretic peptide (BNP) were measured in 115 ACHD (mean age, 30 ± 10 years). NYHA class was I/ II in 86% and III/IV in 14%. The subjects were divided into two groups: patients with single-ventricle (SV group, n = 65) and with two-ventricle physiology (TV group, n = 50). We retrospectively analyzed the relationship between levels of biomarkers and cardiovascular death. During a mean follow-up period of 4.6 years, 17 (14%) cardiovascular deaths occurred, including 7 in the SV group. Univariate cox regression analysis in all subjects showed strong association between elevated levels of ET1, NE, RPA, hs-CRP, sTNF-RI and II, IL-6, and BNP and cardiovascular death (p< 0.05). In the SV group, using multivariate cox regression model, BNP and sTNF-RI were the most powerful predictors in these biomarkers (adjusted hazard ratio [aHR] of BNP: 14.84; 95%CI: 2.21-99.36 per 1 SD increase, p = 0.005) (aHR of sTNF-RI: 2.30; 95%CI: 1.91-4.55 per 1 SD increase, p = 0.017). The optimal cut-off values of BNP and sTNF-RI for mortality were 196 pg/mL and 1.26 ng/mL, respectively. Conversely, in the TV group, only IL-6 was an independent predictor of mortality (aHR: 3.24; 95%CI: 1.57-6.68 per 1 SD increase, p = 0.001), while BNP was not strongly associated with outcomes. The optimal cut-off value of IL-6 for mortality was 2.3 pg/mL. Conclusion: Various biomarkers, including sTNF-R, BNP, and IL-6, are associated with prognosis in overall ACHD. The most prominent mortality predictors might differ, due to differences in SV or TV physiology.

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