- Email: [email protected]
Prevalence of Atrial Fibrillation and Permanent Atrial Arrhythmias in Congenital Heart Disease
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
VOL. 70, NO. 7, 2017
ª 2017 BY THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION PUBLISHED BY ELSEVIER
ISSN 0735-1097/$36.00 http://dx.doi.org/10.1016/j.jacc.2017.06.052
EDITORIAL COMMENT
Prevalence of Atrial Fibrillation and Permanent Atrial Arrhythmias in Congenital Heart Disease Sequel or Series?* Michael J. Silka, MD, Yaniv Bar-Cohen, MD
T
he Cambridge University Press dictionary
either a new-onset sustained atrial tachycardia or AF.
defines a sequel as a book, film, or play that
The data were collected from 12 centers with a mean
continues the story of a previous work (1).
population age of 32 18 years at the time of the first
The common approach is for the events of the second
documented atrial arrhythmia. This study offers an
version to contain elements of the original work, with
epidemiological perspective on the overall types and
the sequel often based on lingering questions or un-
frequency of atrial arrhythmias in a “young” adult
certainties. At times, a sequel may lead to a series,
population with CHD and subsequent surgical in-
in which key elements appear repeatedly.
terventions. Furthermore, it provides longitudinal
By analogy, medical diagnoses and conditions may
follow-up of these patients and provides insight into
evolve over time, with new or secondary diagnoses
the propensity for these patients to develop AF or
resulting because of a primary problem that was
other persistent forms of atrial tachycardia.
ostensibly “cured,” but perhaps with lingering ques-
As the stated focus of the study was the arrhythmic
tions and uncertainties. This analogy may be partic-
consequences of structural CHD and surgical in-
ularly applicable to patients with congenital heart
terventions, the index arrhythmias were restricted to
disease (CHD), where late cardiovascular problems
3 types: 1) intra-atrial re-entrant tachycardia (IART);
may develop decades after surgery.
2) focal atrial tachycardia (FAT); and 3) AF. CHD was classified as simple, moderate, or complex based on
SEE PAGE 857
previous guidelines (3).
The study by Labombarda et al. (2) in this issue of
In the analysis of the cross-sectional data, 482 pa-
the Journal, discusses the increasing prevalence of
tients qualified for study inclusion; IART was the
atrial fibrillation (AF) and permanent atrial arrhyth-
most common initial atrial arrhythmia in 297 patients
mias in CHD. The study provides some useful insights
(61.6%), followed by AF in 139 patients (28.8%) and
and, perhaps more importantly, raises further ques-
FAT in 46 patients (9.5%). Patients presenting with
tions about the next sequel or possibly series of events
IART had a mean age of 28.8 16 years at initial
as these patients survive into and beyond middle age.
arrhythmia documentation. IART was associated with
This
multicenter,
retrospective
cohort
study
moderate or complex forms of CHD, with simple
describes patients with CHD that presented with
forms of CHD in only 42 patients with IART (14%). Patients with FAT as the index arrhythmia had the youngest mean age of onset (23 18 years) and the highest prevalence of the complex CHD.
*Editorials published in the Journal of the American College of Cardiology
Conversely, the mean age of the patients with AF
reflect the views of the authors and do not necessarily represent the
as the index arrhythmia was 41 17.2 years, with a
views of JACC or the American College of Cardiology. From the Division of Cardiology, Children’s Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, Cal-
greater percentage of simple forms of CHD. Of the total of 138 patients with AF as the index arrhythmia,
ifornia. Both authors have reported that they have no relationships
73 (53%) were >50 years of age at time of arrhythmia
relevant to the contents of this paper to disclose.
onset. This is perhaps one of the key points of this
Silka and Bar-Cohen
JACC VOL. 70, NO. 7, 2017 AUGUST 15, 2017:866–8
AF and Permanent Atrial Arrhythmias in CHD
study—although structural CHD and subsequent hy-
CHD, with onset at age 36 15 years for complex
pertrophy, dilation, and fibrosis are undoubtedly
forms, compared with 47 14 years with moderate
significant, traditional factors such as increasing age
and 59 15 years for simple forms of CHD (4).
and hypertension may equally influence the development of AF in CHD patients.
Regardless, there is the potential for AF to develop in any form of CHD, simple to complex, with age of the
In addition to the cross-sectional analysis of
patient as an important factor along with the anatomic/
the types of atrial arrhythmias identified at initial
hemodynamic substrate. Thus, as the population in-
presentation, the authors also describe the clinical
creases and age of these patients advances, it appears
course using the categorization of paroxysmal,
that perhaps the next sequel is slowly being written:
persistent, and permanent atrial arrhythmias. The
a potential AF epidemic in older patients with CHD.
overall trend reported was that IART and FAT prog-
Unfortunately, we are in the earliest stages of un-
ress from paroxysmal to persistent forms and that
derstanding the basic interplay among structural
AF becomes increasingly permanent as the patients
CHD, the coexistence of other atrial arrhythmias, and
were followed for a mean of 11.3 years post-
the traditional factors associated with the develop-
arrhythmia onset.
ment of AF. For example, it has been reported that
The second aspect of this study was the develop-
chronic IART results in shortening of the atrial
ment of new (different) atrial arrhythmias than
effective refractory period and electrical remodeling,
reported as the index arrhythmia at study entry. Of
which may facilitate the development of sustained
the 185 patients with either FAT or AF at study entry,
AF (5). Another variation in the development of AF in
only 2 subsequently developed IART during the
CHD patients may be the role of nonpulmonary vein
follow-up; likewise, only 5 of 436 patients developed
foci of continuous electrical activity, related to prior
new-onset FAT during follow-up. This suggests that
surgical procedures (6).
IART or FAT develop as primary atrial arrhythmias
A natural follow-up to defining patient groups that
related to surgical incisions, fibrosis, and hypertro-
are at risk for a disease is determining which potential
phy, and are less likely to develop as a sequel to other
therapies could be preventative. Does aggressive or
atrial arrhythmias.
earlier intervention reduce the proclivity for CHD
Conversely, new-onset AF was reported in 42 of
patients to develop AF? Does a prophylactic “Maze”
344 patients previously diagnosed with IART or FAT
or “atrial corridor” procedure at the time of CHD
(12.2%), with a total of 181 patients (37.3%) with AF at
surgery offer any benefit—or does extensive atrial
the end of the study interval. Unfortunately, factors
dissection
associated with the late or secondary development of
dispersion and an increased risk of AF? Do antiar-
AF are not defined in this study. However, a conclu-
rhythmic medications have a role in halting or
sion that persists is that AF develops in all forms of
delaying disease progression?
CHD, irrespective of complexity.
result
in
greater
electrophysiological
Although this study answers several questions, it
The development of AF and attendant comorbid-
raises an even larger number of issues and provides
ities have evolved as major clinical concerns as pa-
the rationale for future investigations into the true
tients with CHD survive into middle and advanced
risks of the late development of AF in these patients.
ages. This study adds to that possibility by demon-
As these patients are younger, with more robust AV
strating the high percentage of atrial fibrillation in
nodal conduction in the setting of impaired ventric-
those with atrial arrhythmias at age >50 years.
ular function or palliated circulatory physiology,
Although the study does not provide a denominator
the consequences of AF may be catastrophic. Thus,
with which to determine the incidence of AF in the
it becomes critical to define the relative risks for the
older CHD population, these findings suggest that AF
development of AF, to determine which specific
may eventually surpass IART as the most common
forms of CHD are associated with the development of
atrial arrhythmia in this further aging population.
AF, and finally, to establish whether there are thera-
An equally concerning observation is the gradual
peutic measures of demonstrable benefit or preven-
evolution of the arrhythmias from paroxysmal to
tion. AF may be a sequel, but advances in care will
persistent or permanent forms, which increases the
likely require a series of advances.
risks of heart failure and embolic events, and influences decisions regarding rhythm or rate control.
ADDRESS FOR CORRESPONDENCE: Dr. Michael J.
As the authors acknowledge, patients with the
Silka, Division of Cardiology, Children’s Hospital Los
more complex forms of CHD are just now beginning to
Angeles, Keck School of Medicine, University of Southern
survive to advanced age. A recent report suggests that
California, 4650 Sunset Boulevard, MS #34, Los Angeles,
AF develops earlier in patients with more complex
California 90027. E-mail: [email protected].
867
868
Silka and Bar-Cohen
JACC VOL. 70, NO. 7, 2017 AUGUST 15, 2017:866–8
AF and Permanent Atrial Arrhythmias in CHD
REFERENCES 1. Cambridge Dictionary. Sequel. Available at: http:// dictionary.cambridge.org/us/dictionary/english/ sequel. Accessed July 6, 2017. 2. Labombarda F, Hamilton R, Shohoudi A, et al., on behalf of the AARCC. Increasing prevalence of atrial fibrillation and permanent atrial arrhythmias in congenital heart disease. J Am Coll Cardiol 2017;70:857–65. 3. Warnes CA, Williams RG, Bashore TM, et al. ACA/AHA 2008 guidelines for the management of
adults with congenital heart disease. J Am Coll Cardiol 2008;52:3143–263. 4. Teuwen CP, Ramdjan TTK, de Groot NMS. Management of atrial fibrillation in adults with congenital heart defects. Expert Rev Cardiovasc Ther 2015;13:57–66. 5. Sparks PB, Jayaprakash S, Vohra JK, Kalman JM. Electrical remodeling of the atria associated with paroxysmal and chronic atrial flutter. Circulation 2000;102:1807–13.
6. Takahashi K, Shoda M, Manaka T, Nakanishi T. Successful radiofrequency catheter ablation of atrial fibrillation late after the modified Fontan operation. Europace 2008;10: 1012–4.
KEY WORDS cohort studies, congenital heart defects, electrocardiography, intra-atrial re-entrant tachycardia, tachycardia
VOL. 70, NO. 7, 2017
ª 2017 BY THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION PUBLISHED BY ELSEVIER
ISSN 0735-1097/$36.00 http://dx.doi.org/10.1016/j.jacc.2017.06.052
EDITORIAL COMMENT
Prevalence of Atrial Fibrillation and Permanent Atrial Arrhythmias in Congenital Heart Disease Sequel or Series?* Michael J. Silka, MD, Yaniv Bar-Cohen, MD
T
he Cambridge University Press dictionary
either a new-onset sustained atrial tachycardia or AF.
defines a sequel as a book, film, or play that
The data were collected from 12 centers with a mean
continues the story of a previous work (1).
population age of 32 18 years at the time of the first
The common approach is for the events of the second
documented atrial arrhythmia. This study offers an
version to contain elements of the original work, with
epidemiological perspective on the overall types and
the sequel often based on lingering questions or un-
frequency of atrial arrhythmias in a “young” adult
certainties. At times, a sequel may lead to a series,
population with CHD and subsequent surgical in-
in which key elements appear repeatedly.
terventions. Furthermore, it provides longitudinal
By analogy, medical diagnoses and conditions may
follow-up of these patients and provides insight into
evolve over time, with new or secondary diagnoses
the propensity for these patients to develop AF or
resulting because of a primary problem that was
other persistent forms of atrial tachycardia.
ostensibly “cured,” but perhaps with lingering ques-
As the stated focus of the study was the arrhythmic
tions and uncertainties. This analogy may be partic-
consequences of structural CHD and surgical in-
ularly applicable to patients with congenital heart
terventions, the index arrhythmias were restricted to
disease (CHD), where late cardiovascular problems
3 types: 1) intra-atrial re-entrant tachycardia (IART);
may develop decades after surgery.
2) focal atrial tachycardia (FAT); and 3) AF. CHD was classified as simple, moderate, or complex based on
SEE PAGE 857
previous guidelines (3).
The study by Labombarda et al. (2) in this issue of
In the analysis of the cross-sectional data, 482 pa-
the Journal, discusses the increasing prevalence of
tients qualified for study inclusion; IART was the
atrial fibrillation (AF) and permanent atrial arrhyth-
most common initial atrial arrhythmia in 297 patients
mias in CHD. The study provides some useful insights
(61.6%), followed by AF in 139 patients (28.8%) and
and, perhaps more importantly, raises further ques-
FAT in 46 patients (9.5%). Patients presenting with
tions about the next sequel or possibly series of events
IART had a mean age of 28.8 16 years at initial
as these patients survive into and beyond middle age.
arrhythmia documentation. IART was associated with
This
multicenter,
retrospective
cohort
study
moderate or complex forms of CHD, with simple
describes patients with CHD that presented with
forms of CHD in only 42 patients with IART (14%). Patients with FAT as the index arrhythmia had the youngest mean age of onset (23 18 years) and the highest prevalence of the complex CHD.
*Editorials published in the Journal of the American College of Cardiology
Conversely, the mean age of the patients with AF
reflect the views of the authors and do not necessarily represent the
as the index arrhythmia was 41 17.2 years, with a
views of JACC or the American College of Cardiology. From the Division of Cardiology, Children’s Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, Cal-
greater percentage of simple forms of CHD. Of the total of 138 patients with AF as the index arrhythmia,
ifornia. Both authors have reported that they have no relationships
73 (53%) were >50 years of age at time of arrhythmia
relevant to the contents of this paper to disclose.
onset. This is perhaps one of the key points of this
Silka and Bar-Cohen
JACC VOL. 70, NO. 7, 2017 AUGUST 15, 2017:866–8
AF and Permanent Atrial Arrhythmias in CHD
study—although structural CHD and subsequent hy-
CHD, with onset at age 36 15 years for complex
pertrophy, dilation, and fibrosis are undoubtedly
forms, compared with 47 14 years with moderate
significant, traditional factors such as increasing age
and 59 15 years for simple forms of CHD (4).
and hypertension may equally influence the development of AF in CHD patients.
Regardless, there is the potential for AF to develop in any form of CHD, simple to complex, with age of the
In addition to the cross-sectional analysis of
patient as an important factor along with the anatomic/
the types of atrial arrhythmias identified at initial
hemodynamic substrate. Thus, as the population in-
presentation, the authors also describe the clinical
creases and age of these patients advances, it appears
course using the categorization of paroxysmal,
that perhaps the next sequel is slowly being written:
persistent, and permanent atrial arrhythmias. The
a potential AF epidemic in older patients with CHD.
overall trend reported was that IART and FAT prog-
Unfortunately, we are in the earliest stages of un-
ress from paroxysmal to persistent forms and that
derstanding the basic interplay among structural
AF becomes increasingly permanent as the patients
CHD, the coexistence of other atrial arrhythmias, and
were followed for a mean of 11.3 years post-
the traditional factors associated with the develop-
arrhythmia onset.
ment of AF. For example, it has been reported that
The second aspect of this study was the develop-
chronic IART results in shortening of the atrial
ment of new (different) atrial arrhythmias than
effective refractory period and electrical remodeling,
reported as the index arrhythmia at study entry. Of
which may facilitate the development of sustained
the 185 patients with either FAT or AF at study entry,
AF (5). Another variation in the development of AF in
only 2 subsequently developed IART during the
CHD patients may be the role of nonpulmonary vein
follow-up; likewise, only 5 of 436 patients developed
foci of continuous electrical activity, related to prior
new-onset FAT during follow-up. This suggests that
surgical procedures (6).
IART or FAT develop as primary atrial arrhythmias
A natural follow-up to defining patient groups that
related to surgical incisions, fibrosis, and hypertro-
are at risk for a disease is determining which potential
phy, and are less likely to develop as a sequel to other
therapies could be preventative. Does aggressive or
atrial arrhythmias.
earlier intervention reduce the proclivity for CHD
Conversely, new-onset AF was reported in 42 of
patients to develop AF? Does a prophylactic “Maze”
344 patients previously diagnosed with IART or FAT
or “atrial corridor” procedure at the time of CHD
(12.2%), with a total of 181 patients (37.3%) with AF at
surgery offer any benefit—or does extensive atrial
the end of the study interval. Unfortunately, factors
dissection
associated with the late or secondary development of
dispersion and an increased risk of AF? Do antiar-
AF are not defined in this study. However, a conclu-
rhythmic medications have a role in halting or
sion that persists is that AF develops in all forms of
delaying disease progression?
CHD, irrespective of complexity.
result
in
greater
electrophysiological
Although this study answers several questions, it
The development of AF and attendant comorbid-
raises an even larger number of issues and provides
ities have evolved as major clinical concerns as pa-
the rationale for future investigations into the true
tients with CHD survive into middle and advanced
risks of the late development of AF in these patients.
ages. This study adds to that possibility by demon-
As these patients are younger, with more robust AV
strating the high percentage of atrial fibrillation in
nodal conduction in the setting of impaired ventric-
those with atrial arrhythmias at age >50 years.
ular function or palliated circulatory physiology,
Although the study does not provide a denominator
the consequences of AF may be catastrophic. Thus,
with which to determine the incidence of AF in the
it becomes critical to define the relative risks for the
older CHD population, these findings suggest that AF
development of AF, to determine which specific
may eventually surpass IART as the most common
forms of CHD are associated with the development of
atrial arrhythmia in this further aging population.
AF, and finally, to establish whether there are thera-
An equally concerning observation is the gradual
peutic measures of demonstrable benefit or preven-
evolution of the arrhythmias from paroxysmal to
tion. AF may be a sequel, but advances in care will
persistent or permanent forms, which increases the
likely require a series of advances.
risks of heart failure and embolic events, and influences decisions regarding rhythm or rate control.
ADDRESS FOR CORRESPONDENCE: Dr. Michael J.
As the authors acknowledge, patients with the
Silka, Division of Cardiology, Children’s Hospital Los
more complex forms of CHD are just now beginning to
Angeles, Keck School of Medicine, University of Southern
survive to advanced age. A recent report suggests that
California, 4650 Sunset Boulevard, MS #34, Los Angeles,
AF develops earlier in patients with more complex
California 90027. E-mail: [email protected].
867
868
Silka and Bar-Cohen
JACC VOL. 70, NO. 7, 2017 AUGUST 15, 2017:866–8
AF and Permanent Atrial Arrhythmias in CHD
REFERENCES 1. Cambridge Dictionary. Sequel. Available at: http:// dictionary.cambridge.org/us/dictionary/english/ sequel. Accessed July 6, 2017. 2. Labombarda F, Hamilton R, Shohoudi A, et al., on behalf of the AARCC. Increasing prevalence of atrial fibrillation and permanent atrial arrhythmias in congenital heart disease. J Am Coll Cardiol 2017;70:857–65. 3. Warnes CA, Williams RG, Bashore TM, et al. ACA/AHA 2008 guidelines for the management of
adults with congenital heart disease. J Am Coll Cardiol 2008;52:3143–263. 4. Teuwen CP, Ramdjan TTK, de Groot NMS. Management of atrial fibrillation in adults with congenital heart defects. Expert Rev Cardiovasc Ther 2015;13:57–66. 5. Sparks PB, Jayaprakash S, Vohra JK, Kalman JM. Electrical remodeling of the atria associated with paroxysmal and chronic atrial flutter. Circulation 2000;102:1807–13.
6. Takahashi K, Shoda M, Manaka T, Nakanishi T. Successful radiofrequency catheter ablation of atrial fibrillation late after the modified Fontan operation. Europace 2008;10: 1012–4.
KEY WORDS cohort studies, congenital heart defects, electrocardiography, intra-atrial re-entrant tachycardia, tachycardia