- Email: [email protected]
Insights on Atrial Fibrillation in Congenital Heart Disease
Accepted Manuscript Insights on Atrial Fibrillation in Congenital Heart Disease Mohammed A. Ebrahim, MD, Carolina A. Escudero, MD, MSc, FRCPC, Michal J. Kantoch, MD, FRCPC, Isabelle F. Vondermuhll, MD, FRCPC, Joseph Atallah, MD CM, SM, FRCPC PII:
S0828-282X(18)31009-2
DOI:
10.1016/j.cjca.2018.08.010
Reference:
CJCA 3008
To appear in:
Canadian Journal of Cardiology
Received Date: 3 July 2018 Revised Date:
1 August 2018
Accepted Date: 1 August 2018
Please cite this article as: Ebrahim MA, Escudero CA, Kantoch MJ, Vondermuhll IF, Atallah J, Insights on Atrial Fibrillation in Congenital Heart Disease, Canadian Journal of Cardiology (2018), doi: 10.1016/ j.cjca.2018.08.010. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Title: Insights on Atrial Fibrillation in Congenital Heart Disease
Authors:
RI PT
Mohammed A Ebrahim MD1 ; Carolina A Escudero MD, MSc, FRCPC 1 ; Michal J
Kantoch MD, FRCPC1 ; Isabelle F Vondermuhll MD, FRCPC2 ; Joseph Atallah MD CM, SM, FRCPC1
SC
Affiliation:
Division of Cardiology, Department of Pediatrics, University of Alberta
2
Division of Cardiology, Department of Medicine, University of Alberta
Address: 8440 112 St NW, Office 4C1.19 Edmonton, AB T6G 2B7
Mohammad Ebrahim
TE D
Corresponding Author:
Email: [email protected] P 780-407-3963
EP
F 780-407-3954
M AN U
1
AC C
Word Count: 1437
Declaration of interest: none
ACCEPTED MANUSCRIPT
Abstract
Patients with congenital heart disease (CHD) have been surviving late into adulthood, with atrial arrhythmias (AA) being the most common long-term complication. In recent
RI PT
reports, AF tended to be the most common form of AA among groups of adult CHD
(ACHD) patients older than 50 years of age. When compared to their adult counterparts without CHD, AF in ACHD patients have been characterized by a higher incidence and prevalence, younger age of onset and greater risk of progression to persistent AF. Risk
SC
factors for the development of AF are not well known but include older age, left atrial dilation, systemic hypertension and multiple cardiac surgeries. Data on management
M AN U
options such as optimal anti-arrhythmic drug therapy, indications for anticoagulation, and efficacy and safety of catheter ablation is limited. There is a crucial need for further research exploring management, prevention, and monitoring strategies for the growing ACHD patient population with AF. This report will provide a contemporary review of the epidemiology, pathophysiology and management options for AF in this complex patient
AC C
EP
TE D
population.
ACCEPTED MANUSCRIPT
Summary
Patients with congenital heart disease have increased risks for atrial fibrillation with reported higher incidence, prevalence, and progression to permanent forms when
RI PT
compared to control groups. Furthermore, there is limited data as to how to best manage this complex group of patients. This report presents comprehensive review of atrial
AC C
EP
TE D
M AN U
SC
fibrillation in congenital heart disease patients.
ACCEPTED MANUSCRIPT
Introduction
It is estimated that there are over one million adult patients living with congenital heart
RI PT
disease (CHD) in Canada and the United States, and a significant proportion has
moderate or severe/complex type of CHD [1]. With ongoing improved long-term
survival, the population of adults with CHD is expected to continue to grow significantly,
SC
with atrial arrhythmias (AA) being the most common long-term complication [1,2].
Reentrant and focal atrial tachycardias have been the main focus of medical and surgical
M AN U
interventions. However, as adult CHD (ACHD) patients age, the incidence of atrial fibrillation (AF) is expected to increase, presenting a significant clinical burden and therapeutic challenge. The latter relates to the complex underlying anatomy and physiology, presence of atypical right atrial fibrillation, and the young age of onset,
TE D
further compounded by limited evidence on risk stratification and therapy.
In this article, we provide a contemporary review of the epidemiology, pathophysiology
AC C
Epidemiology
EP
and management options for AF in ACHD patients.
Initial reports documented an increased risk for AF among adults with specific CHD such as atrial septal defect, Tetralogy of Fallot (TOF), Ebstein’s anomaly and Fontan physiology, with a prevalence ranging up to 30%, depending on age and anatomy [1,2]. A recent study by Avila et al (2017) reported a 4.8% incidence of AF among 3311 ACHD
ACCEPTED MANUSCRIPT
patients during a median follow-up of 11 years [2]. Teuwen et al (2015) reported on 199 ACHD with variable CHD complexity and AF. Regular AA (macro-reentrant or unifocal)
Atrial Fibrillation Age of Onset & Progression
RI PT
co-existed with AF in 33% and 65% initially presented with AA [3].
SC
In the same study by Teuwen et al studied (2015), the mean age of onset for AF was
49±17 years [3]. They identified that more complex CHD was associated with earlier
M AN U
onset of AF. Progression to persistent AF was observed in 26% after a median (IQR) follow-up of only 3 (1-7) years, with rapid progression despite attempted rhythm control therapy. Labombarda et al (2017) reported on 482 ACHD patients, mean age of 38±18 years, with any form of sustained atrial arrhythmia [4]. AF accounted for 29% of
TE D
arrhythmias in this cohort, and was more likely to be persistent compared to other atrial arrhythmias. Moreover in the subset of individuals over 50 years of age, AF became the predominant arrhythmia. In adult patients with TOF, a sharp increase in the prevalence of
EP
AF after age 45 years has been documented [1].
AC C
Pathophysiology
There are well-recognized risk factors for the development of AF among patients without CHD, including age, systemic hypertension, diabetes, obesity, obstructive sleep apnea, hyperthyroidism, left atrial (LA) dilation, left ventricular (LV) dysfunction, and cardiac surgery. In ACHD patients, risk factors for the development of AF include age, LA
ACCEPTED MANUSCRIPT
dilation, hypertension and multiple cardiac surgeries [1,3]. A distinguishing feature of AF in ACHD patients is the suspected presence of AF that is of right atrial (RA) origin, in contrast to typical adult-onset AF that is of left atrial origin (Figure 1). As well, AF in
RI PT
ACHD patients can be primary or secondary. Secondary AF is characterized as an
electrophysiological deterioration of re-entrant atrial tachycardia, which co-exists with
AF in one third of cases and is often associated with the presence of atrial scar [3]. For
SC
primary AF that is of LA origin, suspected CHD specific risk factors include left-sided lesions resulting in LA volume or pressure overload [3], pulmonary venous anomalies
M AN U
and persistent left superior vena cava [5]. For primary AF that is of RA origin, risk factors include pulmonary venous anomalies and lesions that cause right sided volume or pressure overload as well as Fontan physiology [2,3].
TE D
Management
Acute termination of AF can be achieved with synchronized direct-current shocks. This
EP
method can be employed urgently in the presence of hemodynamic instability, or electively in properly selected patients, assessed for specific risks such as
AC C
thromboembolic events, brady-arrhythmia, AF recurrence, etc. There is no data on pharmacological conversion of AF in ACHD.
Antiarrhythmic Drugs:
ACCEPTED MANUSCRIPT
To date there is little evidence in favor of rhythm versus rate control for AF in ACHD patients. Guidelines make a class IIa recommendation for an initial strategy rhythm control in patients with moderate or complex forms of ACHD (1). Aggressive rhythm
RI PT
control in younger individuals with more complex heart disease is reasonable as such
patients are prone to rapid ventricular response and hemodynamic compromise due to
diastolic filling impairment in the presence of stiff atrial baffles, ventricular dysfunction
SC
and/or concomitant valvular dysfunction. Unfortunately rhythm control may be
challenging in ACHD patients (3,4). On the other hand, rate control may be an adequate
M AN U
strategy for simple forms of ACHD such as atrial septal defects (1); however the target heart rate may need to be individualized based on the type of CHD (e.g. single ventricle vs systemic right ventricle).
TE D
There are no reports evaluating the efficacy and safety of antiarrhythmic medications specifically for AF in ACHD patients. Most anti-arrhythmics are associated with proarrhythmia and even increased mortality in CHD [1]. Of the two drugs of choice, Sotalol
EP
should be reserved for ACHD patients with simple structural lesions, and is considered class IIb for moderate and complex lesions, whereas amiodarone may be considered as
AC C
first line therapy for complex ACHD (class IIa recommendation) [1]. However due to the side-effect profile of antiarrhythmic drugs, ablation of AF may be considered especially in symptomatic drug refractory AF (class IIa recommendation) [1].
ACCEPTED MANUSCRIPT
Catheter & Surgical Interventions: ACHD patients in whom AF is secondary to atrial tachycardia, may benefit from catheter ablation to address the AT substrate [3]. In those with primary AF, pulmonary vein
RI PT
isolation (PVI) may have a role, particularly in older patients with simple lesions such as ASD. PVI has been shown to have a success rate of 42% at 300 days in a cohort of
mainly septal defect patients [1], which is lower than expected in non-CHD cohorts. In
SC
complex ACHD with AF, an operative bi-atrial Maze procedure is often undertaken at the time of cardiac surgery [1]. If PVI is undertaken in complex ACHD, it is important to
M AN U
understand the variations in systemic and pulmonary venous anatomy, as changes to the procedure, such as isolating the left superior vena cava, a potential trigger for AF, may be required [5]. Further study on the outcomes of PVI in complex CHD would be valuable in order to understand the utility of this therapy in these patients and to determine which
TE D
patients would benefit the most from this procedure.
Systematic evaluation of the safety and efficacy of AV node ablation as a treatment
EP
strategy for AF in patients with CHD has not been performed [1]. In ACHD patients, AV nodal ablation and the resultant need for permanent ventricular pacing poses particular
AC C
concern given the potentially fragile baseline hemodynamics and the increased risk of device complications in young CHD patients. In all patients, it is important to consider a concomitant assessment and management of underlying cardiac hemodynamic abnormalities that can be contributing to the development of AF such valvar regurgitation or stenosis, residual shunts, ventricular dysfunction, etc.
ACCEPTED MANUSCRIPT
Thrombo-prophylaxis: Patients with CHD and AF are at times assumed to be at low risk of stroke due to their younger age. However, ischemic stroke in young patients with CHD occurs at a higher
RI PT
rate than in the general population [1], therefore, all ACHD patients with AF should be
considered for some form of thrombo-prophylaxis. Certain CHD lesions are at increased risk for thromboembolic events due to factors including cyanosis, increased blood
SC
viscosity, presence of blind intra-cardiac chambers, and low flow states, thus decision
making needs to be individualized. In general, the CHADS-VASC [1] or CHADS65 risk
M AN U
scores are used in determining the intensity of thrombo-prophylaxis, albeit with limited data and level of evidence [1]. Current guidelines consider vitamin K antagonists to be the anticoagulant of choice [1]; however, data are expected to be emerging soon about the
Conclusion
TE D
safety of NOACs in ACHD.
EP
Contemporary data suggests an expected increased incidence of AF among CHD patients. Adult patients with CHD have a younger age of onset and greater risk of progression to
AC C
persistent AF than their adult counterparts without CHD. AF has become the most common arrhythmia among “older” ACHD patients and has been surpassing the prevalence of intra-atrial reentry tachycardia. This expanding clinical challenge is further compounded by the lack of data on optimal anti-arrhythmic drug therapy, indications for anticoagulation, and efficacy and safety of catheter ablation. There is a crucial need for further research exploring management, prevention, and monitoring strategies for the
ACCEPTED MANUSCRIPT
growing ACHD patient population with AF in order to optimize the outcomes for this unique and complex cohort.
RI PT
Legend
Figure 1: Pathophysiology of right and left sided atrial fibrillation in ACHD, and
associated contributing factors. AF: atrial fibrillation, RA: right atrium, LA: left atrium,
M AN U
ventricular valve, OSA: obstructive sleep apnea.
SC
APVR: anomalous pulmonary venous return, LSVC: left superior vena cava, AVV: atrio-
References:
1- Khairy P, Van Hare G, Balaji S, et al. Society Guidelines: PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in
TE D
Adults Congenital Heart Disease. Developed in Partnership Between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the Governing Bodies of PACES, HRS, the
EP
American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm
AC C
Society (CHRS), and the International Society for Adults Congenital Heart
Disease (ISACHD). Canadian Journal of Cardiology 2014;30:e1-e63.
2- Avila P, Oliver J, Gallego P, et al. Natural history and clinical predictors of atrial tachycardia in adults with congenital heart disease. Circ Arrhythm Electrophysiol 2017;10:e005396.
ACCEPTED MANUSCRIPT
3- Teuwen C, Ramdjan T, Gotte M, et al. Time course of atrial fibrillation in patients with congenital heart defects. Circ Arrhtyhm Electrophysiol 2015;8:1065-1072. 4- Labombarda F, Hamilton R, Shohoudi A, et al. Increasing prevalence of atrial
RI PT
fibrillation and permanent atrial arrhythmia in congenital heart disease. J Am Coll Cardiol 2017;70(7):857-65.
5- Refaat M, Ballout J, Mansour M. Ablation of atrial fibrillation in patients with
SC
congenital heart disease. Arrhythmia & Electrophysiology Review 2017;6(4):191-
AC C
EP
TE D
M AN U
94.
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT
S0828-282X(18)31009-2
DOI:
10.1016/j.cjca.2018.08.010
Reference:
CJCA 3008
To appear in:
Canadian Journal of Cardiology
Received Date: 3 July 2018 Revised Date:
1 August 2018
Accepted Date: 1 August 2018
Please cite this article as: Ebrahim MA, Escudero CA, Kantoch MJ, Vondermuhll IF, Atallah J, Insights on Atrial Fibrillation in Congenital Heart Disease, Canadian Journal of Cardiology (2018), doi: 10.1016/ j.cjca.2018.08.010. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT
Title: Insights on Atrial Fibrillation in Congenital Heart Disease
Authors:
RI PT
Mohammed A Ebrahim MD1 ; Carolina A Escudero MD, MSc, FRCPC 1 ; Michal J
Kantoch MD, FRCPC1 ; Isabelle F Vondermuhll MD, FRCPC2 ; Joseph Atallah MD CM, SM, FRCPC1
SC
Affiliation:
Division of Cardiology, Department of Pediatrics, University of Alberta
2
Division of Cardiology, Department of Medicine, University of Alberta
Address: 8440 112 St NW, Office 4C1.19 Edmonton, AB T6G 2B7
Mohammad Ebrahim
TE D
Corresponding Author:
Email: [email protected] P 780-407-3963
EP
F 780-407-3954
M AN U
1
AC C
Word Count: 1437
Declaration of interest: none
ACCEPTED MANUSCRIPT
Abstract
Patients with congenital heart disease (CHD) have been surviving late into adulthood, with atrial arrhythmias (AA) being the most common long-term complication. In recent
RI PT
reports, AF tended to be the most common form of AA among groups of adult CHD
(ACHD) patients older than 50 years of age. When compared to their adult counterparts without CHD, AF in ACHD patients have been characterized by a higher incidence and prevalence, younger age of onset and greater risk of progression to persistent AF. Risk
SC
factors for the development of AF are not well known but include older age, left atrial dilation, systemic hypertension and multiple cardiac surgeries. Data on management
M AN U
options such as optimal anti-arrhythmic drug therapy, indications for anticoagulation, and efficacy and safety of catheter ablation is limited. There is a crucial need for further research exploring management, prevention, and monitoring strategies for the growing ACHD patient population with AF. This report will provide a contemporary review of the epidemiology, pathophysiology and management options for AF in this complex patient
AC C
EP
TE D
population.
ACCEPTED MANUSCRIPT
Summary
Patients with congenital heart disease have increased risks for atrial fibrillation with reported higher incidence, prevalence, and progression to permanent forms when
RI PT
compared to control groups. Furthermore, there is limited data as to how to best manage this complex group of patients. This report presents comprehensive review of atrial
AC C
EP
TE D
M AN U
SC
fibrillation in congenital heart disease patients.
ACCEPTED MANUSCRIPT
Introduction
It is estimated that there are over one million adult patients living with congenital heart
RI PT
disease (CHD) in Canada and the United States, and a significant proportion has
moderate or severe/complex type of CHD [1]. With ongoing improved long-term
survival, the population of adults with CHD is expected to continue to grow significantly,
SC
with atrial arrhythmias (AA) being the most common long-term complication [1,2].
Reentrant and focal atrial tachycardias have been the main focus of medical and surgical
M AN U
interventions. However, as adult CHD (ACHD) patients age, the incidence of atrial fibrillation (AF) is expected to increase, presenting a significant clinical burden and therapeutic challenge. The latter relates to the complex underlying anatomy and physiology, presence of atypical right atrial fibrillation, and the young age of onset,
TE D
further compounded by limited evidence on risk stratification and therapy.
In this article, we provide a contemporary review of the epidemiology, pathophysiology
AC C
Epidemiology
EP
and management options for AF in ACHD patients.
Initial reports documented an increased risk for AF among adults with specific CHD such as atrial septal defect, Tetralogy of Fallot (TOF), Ebstein’s anomaly and Fontan physiology, with a prevalence ranging up to 30%, depending on age and anatomy [1,2]. A recent study by Avila et al (2017) reported a 4.8% incidence of AF among 3311 ACHD
ACCEPTED MANUSCRIPT
patients during a median follow-up of 11 years [2]. Teuwen et al (2015) reported on 199 ACHD with variable CHD complexity and AF. Regular AA (macro-reentrant or unifocal)
Atrial Fibrillation Age of Onset & Progression
RI PT
co-existed with AF in 33% and 65% initially presented with AA [3].
SC
In the same study by Teuwen et al studied (2015), the mean age of onset for AF was
49±17 years [3]. They identified that more complex CHD was associated with earlier
M AN U
onset of AF. Progression to persistent AF was observed in 26% after a median (IQR) follow-up of only 3 (1-7) years, with rapid progression despite attempted rhythm control therapy. Labombarda et al (2017) reported on 482 ACHD patients, mean age of 38±18 years, with any form of sustained atrial arrhythmia [4]. AF accounted for 29% of
TE D
arrhythmias in this cohort, and was more likely to be persistent compared to other atrial arrhythmias. Moreover in the subset of individuals over 50 years of age, AF became the predominant arrhythmia. In adult patients with TOF, a sharp increase in the prevalence of
EP
AF after age 45 years has been documented [1].
AC C
Pathophysiology
There are well-recognized risk factors for the development of AF among patients without CHD, including age, systemic hypertension, diabetes, obesity, obstructive sleep apnea, hyperthyroidism, left atrial (LA) dilation, left ventricular (LV) dysfunction, and cardiac surgery. In ACHD patients, risk factors for the development of AF include age, LA
ACCEPTED MANUSCRIPT
dilation, hypertension and multiple cardiac surgeries [1,3]. A distinguishing feature of AF in ACHD patients is the suspected presence of AF that is of right atrial (RA) origin, in contrast to typical adult-onset AF that is of left atrial origin (Figure 1). As well, AF in
RI PT
ACHD patients can be primary or secondary. Secondary AF is characterized as an
electrophysiological deterioration of re-entrant atrial tachycardia, which co-exists with
AF in one third of cases and is often associated with the presence of atrial scar [3]. For
SC
primary AF that is of LA origin, suspected CHD specific risk factors include left-sided lesions resulting in LA volume or pressure overload [3], pulmonary venous anomalies
M AN U
and persistent left superior vena cava [5]. For primary AF that is of RA origin, risk factors include pulmonary venous anomalies and lesions that cause right sided volume or pressure overload as well as Fontan physiology [2,3].
TE D
Management
Acute termination of AF can be achieved with synchronized direct-current shocks. This
EP
method can be employed urgently in the presence of hemodynamic instability, or electively in properly selected patients, assessed for specific risks such as
AC C
thromboembolic events, brady-arrhythmia, AF recurrence, etc. There is no data on pharmacological conversion of AF in ACHD.
Antiarrhythmic Drugs:
ACCEPTED MANUSCRIPT
To date there is little evidence in favor of rhythm versus rate control for AF in ACHD patients. Guidelines make a class IIa recommendation for an initial strategy rhythm control in patients with moderate or complex forms of ACHD (1). Aggressive rhythm
RI PT
control in younger individuals with more complex heart disease is reasonable as such
patients are prone to rapid ventricular response and hemodynamic compromise due to
diastolic filling impairment in the presence of stiff atrial baffles, ventricular dysfunction
SC
and/or concomitant valvular dysfunction. Unfortunately rhythm control may be
challenging in ACHD patients (3,4). On the other hand, rate control may be an adequate
M AN U
strategy for simple forms of ACHD such as atrial septal defects (1); however the target heart rate may need to be individualized based on the type of CHD (e.g. single ventricle vs systemic right ventricle).
TE D
There are no reports evaluating the efficacy and safety of antiarrhythmic medications specifically for AF in ACHD patients. Most anti-arrhythmics are associated with proarrhythmia and even increased mortality in CHD [1]. Of the two drugs of choice, Sotalol
EP
should be reserved for ACHD patients with simple structural lesions, and is considered class IIb for moderate and complex lesions, whereas amiodarone may be considered as
AC C
first line therapy for complex ACHD (class IIa recommendation) [1]. However due to the side-effect profile of antiarrhythmic drugs, ablation of AF may be considered especially in symptomatic drug refractory AF (class IIa recommendation) [1].
ACCEPTED MANUSCRIPT
Catheter & Surgical Interventions: ACHD patients in whom AF is secondary to atrial tachycardia, may benefit from catheter ablation to address the AT substrate [3]. In those with primary AF, pulmonary vein
RI PT
isolation (PVI) may have a role, particularly in older patients with simple lesions such as ASD. PVI has been shown to have a success rate of 42% at 300 days in a cohort of
mainly septal defect patients [1], which is lower than expected in non-CHD cohorts. In
SC
complex ACHD with AF, an operative bi-atrial Maze procedure is often undertaken at the time of cardiac surgery [1]. If PVI is undertaken in complex ACHD, it is important to
M AN U
understand the variations in systemic and pulmonary venous anatomy, as changes to the procedure, such as isolating the left superior vena cava, a potential trigger for AF, may be required [5]. Further study on the outcomes of PVI in complex CHD would be valuable in order to understand the utility of this therapy in these patients and to determine which
TE D
patients would benefit the most from this procedure.
Systematic evaluation of the safety and efficacy of AV node ablation as a treatment
EP
strategy for AF in patients with CHD has not been performed [1]. In ACHD patients, AV nodal ablation and the resultant need for permanent ventricular pacing poses particular
AC C
concern given the potentially fragile baseline hemodynamics and the increased risk of device complications in young CHD patients. In all patients, it is important to consider a concomitant assessment and management of underlying cardiac hemodynamic abnormalities that can be contributing to the development of AF such valvar regurgitation or stenosis, residual shunts, ventricular dysfunction, etc.
ACCEPTED MANUSCRIPT
Thrombo-prophylaxis: Patients with CHD and AF are at times assumed to be at low risk of stroke due to their younger age. However, ischemic stroke in young patients with CHD occurs at a higher
RI PT
rate than in the general population [1], therefore, all ACHD patients with AF should be
considered for some form of thrombo-prophylaxis. Certain CHD lesions are at increased risk for thromboembolic events due to factors including cyanosis, increased blood
SC
viscosity, presence of blind intra-cardiac chambers, and low flow states, thus decision
making needs to be individualized. In general, the CHADS-VASC [1] or CHADS65 risk
M AN U
scores are used in determining the intensity of thrombo-prophylaxis, albeit with limited data and level of evidence [1]. Current guidelines consider vitamin K antagonists to be the anticoagulant of choice [1]; however, data are expected to be emerging soon about the
Conclusion
TE D
safety of NOACs in ACHD.
EP
Contemporary data suggests an expected increased incidence of AF among CHD patients. Adult patients with CHD have a younger age of onset and greater risk of progression to
AC C
persistent AF than their adult counterparts without CHD. AF has become the most common arrhythmia among “older” ACHD patients and has been surpassing the prevalence of intra-atrial reentry tachycardia. This expanding clinical challenge is further compounded by the lack of data on optimal anti-arrhythmic drug therapy, indications for anticoagulation, and efficacy and safety of catheter ablation. There is a crucial need for further research exploring management, prevention, and monitoring strategies for the
ACCEPTED MANUSCRIPT
growing ACHD patient population with AF in order to optimize the outcomes for this unique and complex cohort.
RI PT
Legend
Figure 1: Pathophysiology of right and left sided atrial fibrillation in ACHD, and
associated contributing factors. AF: atrial fibrillation, RA: right atrium, LA: left atrium,
M AN U
ventricular valve, OSA: obstructive sleep apnea.
SC
APVR: anomalous pulmonary venous return, LSVC: left superior vena cava, AVV: atrio-
References:
1- Khairy P, Van Hare G, Balaji S, et al. Society Guidelines: PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in
TE D
Adults Congenital Heart Disease. Developed in Partnership Between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the Governing Bodies of PACES, HRS, the
EP
American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm
AC C
Society (CHRS), and the International Society for Adults Congenital Heart
Disease (ISACHD). Canadian Journal of Cardiology 2014;30:e1-e63.
2- Avila P, Oliver J, Gallego P, et al. Natural history and clinical predictors of atrial tachycardia in adults with congenital heart disease. Circ Arrhythm Electrophysiol 2017;10:e005396.
ACCEPTED MANUSCRIPT
3- Teuwen C, Ramdjan T, Gotte M, et al. Time course of atrial fibrillation in patients with congenital heart defects. Circ Arrhtyhm Electrophysiol 2015;8:1065-1072. 4- Labombarda F, Hamilton R, Shohoudi A, et al. Increasing prevalence of atrial
RI PT
fibrillation and permanent atrial arrhythmia in congenital heart disease. J Am Coll Cardiol 2017;70(7):857-65.
5- Refaat M, Ballout J, Mansour M. Ablation of atrial fibrillation in patients with
SC
congenital heart disease. Arrhythmia & Electrophysiology Review 2017;6(4):191-
AC C
EP
TE D
M AN U
94.
AC C
EP
TE D
M AN U
SC
RI PT
ACCEPTED MANUSCRIPT