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Cardiac Surgery for Arrhythmias. Cardiac arrhythmia surgery was initiated in 1968 with the first
successful division of an accessory AV connection for the Wolff-Parkinson-White syndrome. Subsequent
surgical procedures included the left atrial isolation procedure and right atrial isolation procedure for
automatic atrial tachycardias, discrete cryosurgery of the AV node for AV nodal reentrant tachycardia,
the atrial transection procedure, the corridor procedure, and the maze procedure for atrial fibrillation,
the right ventricular disconnection procedure for arrhythmogenic right ventricular tachycardia, and the
encircling endocardial ventriculotomy, subendocardial resection procedure, endocardial cryoablation, the
Jatene procedure, and the Dor procedure for ischemic ventricular tachycardia. Because of monumental
strides in the treatment of most refractory arrhythmias by endocardial catheter techniques during the past
decade, the only remaining viable surgical procedures for cardiac arrhythmias are the maze procedure for
atrial fibrillation and the Dor procedure for ischemic ventricular tachycardia. Nevertheless, the 25 to 30
years of intense activity in the field of cardiac arrhythmia surgery provided the essential foundation for
the development of these catheter techniques and represent one of the most exciting and productive eras
in the history of medicine. In one short professional career, we have witnessed the birth of arrhythmia
surgery, its adolescence as an “esoteric” specialty, its prime as an enlightening yet exhausting period, and
finally its waning years as a source of knowledge and wisdom upon which better methods of treatment have
been founded. One could hardly ask for a more rewarding experience. (J Cardiovasc Electrophysiol, Vol. 15,
pp. 250-262, February 2004)
intraoperative mapping, Wolff-Parkinson-White syndrome, left atrial isolation, cryosurgery, atrial fibrillation,
maze procedure, right ventricular disconnection, Dor procedure, mini-maze procedure
Perspective tic modality for the treatment of cardiac arrhythmias into the
21st century. The first extensive electrophysiologic mapping
During the past quarter century, surgery has played a of human atrial fibrillation4 and the subsequent development
pivotal role in the elucidation of the anatomic and elec- of a surgical technique for the treatment of atrial fibrilla-
trophysiologic abnormalities responsible for supraventric- tion in the late 1980s5-8 spawned a variety of new interven-
ular and ventricular tachyarrhythmias. The development tional and surgical approaches to the treatment of this most
of sophisticated electrophysiologic systems for intraopera- common of all cardiac arrhythmias.9-14 Critical to current
tive mapping, and the anatomically precise surgical tech- interventional techniques was the documentation that most
niques that were designed, resulted in the ability to cure paroxysmal (i.e., intermittent) atrial fibrillation is induced
the majority of medically refractory cardiac arrhythmias. by spontaneous ectopic beats originating in or near one or
Knowledge gained from the electrophysiologically guided more of the pulmonary vein orifices in the left atrium.15 The
surgical approaches to the Wolff-Parkinson-White (WPW) interventional treatment of continuous atrial fibrillation re-
syndrome and AV nodal reentrant tachycardia contributed mains problematic, but with a better understanding of the
in large part to the subsequent development of endocar- differences between intermittent and continuous atrial fib-
dial catheter techniques capable of curing those specific rillation and modifications of the original curative surgical
arrhythmias without the need for surgical intervention.1,2 techniques, the ability to cure both forms of atrial fibrillation
These intellectual and technologic advances, along with the seems imminent. Nevertheless, any discussion of the devel-
increasing sophistication and availability of antitachycar- opment and subsequent history of surgery for cardiac arrhyth-
dia pacemakers and implantable cardioverter defibrillators3 mias must begin with God’s Gift to Electrophysiology . . . the
for refractory ventricular tachyarrhythmias, eventually nar- Wolff-Parkinson-White syndrome.
rowed the indications for surgical intervention for cardiac
arrhythmias.
Despite this dramatic progress in nonsurgical interven- WPW Syndrome
tional therapy, surgery has remained an important therapeu- 16
Gaskell was the first to demonstrate that electrical activ-
ity propagated from the atrium to the ventricle via myocardial
tissue rather than nerves; his studies on the turtle heart were
Address for correspondence: James L. Cox, M.D., 13523 Rosewood Lane,
Naples, FL 34119. Fax: 239-598-4090; E-mail: jamescoxmd@aol.com
reported in 1883. Stanley Kent17 identified muscular connec-
tions between the atria and ventricles of mammals in 1893,
doi: 10.1046/j.1540-8167.2004.03656.x but he erroneously concluded that these connections were
Cox Cardiac Surgery for Arrhythmias 251
fibrillation in the operating room without understanding the that were identified, elucidated, and solved in the early days
lessons of those initial efforts. Most of the failures of con- of development of these surgical procedures.
temporary catheter and surgical techniques in ablating atrial In 1980, we described the left atrial isolation procedure
fibrillation result from a lack of knowledge of the problems (Fig. 3),33 which was capable of confining atrial fibrillation
to the left atrium while leaving the remainder of the heart in
normal sinus rhythm (Fig. 11). This procedure was success-
ful in restoring a regular ventricular rhythm without the need
for a permanent pacemaker. Unexpectedly, it also restored
normal cardiac hemodynamics as described earlier. Thus,
Figure 10. The Dor procedure. After opening the anteroseptal left ventric-
ular aneurysm, the endocardial scar over the distal ventricular septum is
undermined and resected. If either spontaneous or inducible ventricular
tachycardia was present preoperatively, the junction of the scar and normal
myocardium laterally is cryoablated. A circumferential pursestring suture
is placed around the entire base of the aneurysm at the junction of endocar- Figure 11. Following the left atrial isolation Procedure (see Fig. 3), atrial
dial scar and normal myocardium. When tied down at the proper tension, fibrillation is confined to the left atrium while the rest of the heart remains
this suture restores the normal orientation of the uninvolved muscle fibers in normal sinus rhythm. Note that the right atrium and right ventricle are
of the ventricle exclusive of the aneurysm. An endocardial patch then is an- beating in synchrony. The p waves are inconspicuous on the lead II ECG be-
chored at the level of the circumferential pursestring suture to complete the cause of loss of synchronous contraction of the left atrial mass. (Reproduced
closure of the ventricle. (Reproduced with permission from Dor V, Saab M, with permission from Williams JM, Ungerleider RM, Lofland GK, Cox JL:
Coste P, et al: Left ventricular aneurysm. A new surgical approach. Thorac Left atrial isolation: New technique for the treatment of supraventricular
Cardiovasc Surg 1989;37:11-19.) arrhythmias. J Thorac Cardiovasc Surg 19809;80:373.)
Cox Cardiac Surgery for Arrhythmias 257
the left atrial isolation procedure alleviated two of the three cisions were completed, the animals immediately converted
detrimental sequelae of atrial fibrillation, namely, the irregu- from atrial fibrillation to stable atrial flutter. Because we sus-
lar heartbeat and the compromised hemodynamics. Unfortu- pected that the atrial “flutter wave” was occurring in the right
nately, because the left atrium may continue to fibrillate, the atrium, we simply extended the medial left atriotomy across
vulnerability to systemic thromboembolism was unchanged the body of the left atrium between the superior vena cava
following this procedure. and inferior vena cava posteriorly and down to the level of the
In 1982, Scheinman introduced catheter fulguration of right free-wall tricuspid valve annulus. In the animal model
the His bundle as a means of controlling the irregular cardiac we were using, this so-called atrial transsection procedure
rhythm associated with atrial fibrillation and other refractory invariably prevented the induction and maintenance of atrial
supraventricular arrhythmias.28 This procedure was also a fibrillation or atrial flutter in every animal.7 Unfortunately,
type of isolation procedure in that it isolated the supraven- the procedure was effective but not curative in its clinical ap-
tricular arrhythmia to the atria and away from the ventricles. plication. It was apparent by this time that the surgical cure of
Catheter fulguration eventually was abandoned in favor of atrial fibrillation would require a more complete understand-
the less traumatic RF ablative techniques that still are in use ing of the underlying electrophysiology of atrial fibrillation.
today. Elective His-bundle ablation necessitates the implan-
tation of a permanent ventricular pacemaker, which restores Anatomic-Electrophysiologic Basis of Atrial Fibrillation
a normal ventricular rhythm. However, the atria continue to
Our experimental and clinical studies during the mid-
fibrillate following His-bundle ablation; therefore, this tech-
1980s documented that there are three interacting compo-
nique alleviates only one of the detrimental sequelae of atrial
nents in atrial flutter and atrial fibrillation that determine
fibrillation, the irregular heartbeat. The hemodynamic com-
the findings on the peripheral ECG and, therefore, dictate
promise due to loss of AV synchrony and the vulnerability to
the clinical diagnosis. These three components are (1) a
thromboembolism are unaffected by His-bundle ablation.
macroreentrant circuit(s), (2) passive atrial conduction in that
In 1985, Guiraudon described the corridor procedure for
portion of the atrium not involved in the macroreentrant cir-
treatment of atrial fibrillation,58 an open heart technique that
cuit, and (3) AV conduction. The electrophysiologic charac-
isolated a strip of atrial septum (the “corridor”) harboring
teristics of these three components define a spectrum of atrial
both the SA node and the AV node, thereby allowing the SA
arrhythmias, extending from simple atrial flutter, through
node to drive the ventricles. This procedure corrected the ir-
several types of transitional arrhythmias, to complex atrial
regular heartbeat associated with atrial fibrillation, but both
fibrillation.6
atria either continued to fibrillate postoperatively or devel-
In addition to elucidating the mechanism of atrial flutter
oped their own asynchronous intrinsic rhythm because they
and atrial fibrillation, these experimental and clinical elec-
both were totally isolated from the septal “corridor.” In addi-
trophysiologic studies also documented that our initial hopes
tion, both atria were isolated from their respective ventricles,
of obtaining computerized electrophysiologic maps of atrial
thereby precluding the possibility of AV synchrony on ei-
fibrillation and using them to guide the specific surgical tech-
ther side of the heart. Therefore, neither the hemodynamic
nique, as we had done in other arrhythmias, was not feasi-
compromise nor the vulnerability to thromboembolism asso-
ble. Because the macroreentrant circuits responsible for atrial
ciated with atrial fibrillation were alleviated by the corridor
flutter and atrial fibrillation are so fleeting in nature, it was
procedure, and it was soon abandoned.
recognized that it would be impossible to use activation maps
All three of the surgical and/or catheter techniques devel-
to guide surgery even with online maps. As a result, we sought
oped up to that time had attempted to isolate and confine atrial
to develop a surgical technique that would be capable of in-
fibrillation to a certain region of the atria so that its effects on
terrupting any and all macroreentrant circuits that potentially
the ventricles could be minimized. It was obvious that a much
might develop in the atria, thereby precluding the ability of
better approach would be to try to ablate the atrial fibrillation
the atrium to flutter or fibrillate. In addition, it was recognized
itself and thus restore the heart’s normal sinus rhythm. Using
that the surgical incisions would have to be placed so that the
our best canine model for atrial fibrillation,59 the first ablative
SA node could resume activity postoperatively and “direct”
surgical procedure tried was a simple incision encompassing
the propagation of the sinus impulse throughout both atria.
all of the orifices of the pulmonary veins to totally isolate
This would allow all of the atrial myocardium to be activated
them from the remainder of the heart.7 Unfortunately, this
postoperatively, resulting in preservation of atrial transport
incision had no effect whatsoever on the ability of the atria to
function, which is a prerequisite for the restoration of normal
fibrillate in any of the animals. This is particularly interesting
cardiac hemodynamics and the prevention of stasis of blood
in view of the subsequent demonstration of the importance of
flow in the left atrium with the resultant potential for throm-
the pulmonary vein orifices in serving as the “initiating site”
boembolism. The surgical procedure that was conceived to
for intermittent atrial fibrillation.
accomplish these goals is based on the concept of a maze7
The second series of experiments incorporated pulmonary
and, as a result, was named the “maze procedure.”8
vein isolation plus a lateral incision to the level of the mitral
valve annulus and a medial incision to the interatrial septum. Maze Procedure
This surgical technique also had no effect on the ability of the
atria to fibrillate. The third approach incorporated pulmonary The original surgical technique, the maze-I procedure,8
vein isolation with the left lateral incision, a medial incision was modified to become the maze-II procedure because of
from the pulmonary veins to the interatrial septum posterior late chronotropic problems with the SA node and intra-atrial
to the superior vena cava orifice and across the anterior limbus conduction delays that resulted in decreased left atrial con-
of the fossa ovalis down to the level of the tendon of Todaro. traction. However, the maze-II procedure proved to be ex-
These incisions prevented the ability of the atria to fibrillate ceedingly difficult to perform technically. As a result, it was
in every animal. However, once these left atrial and septal in- modified again to become the maze-III procedure (Fig. 12),
258 Journal of Cardiovascular Electrophysiology Vol. 15, No. 2, February 2004
which soon became the surgical technique of choice for the The most surprising of all results is the extremely low in-
treatment of medically refractory atrial flutter and atrial fib- cidence (0.7%) of perioperative neurologic events that occur
rillation.60,61 Most of the incisions originally performed as in association with the maze procedure.63 Preoperatively, all
part of the maze-III procedure eventually were replaced by of these patients of course had atrial fibrillation, and nearly
cryolesions so that the procedure could be performed by min- 20% of them had suffered some type of significant throm-
imally invasive techniques.62 boembolic event due to the atrial fibrillation. Because these
Between September 25, 1987, and April 16, 1992, 32 pa- patients have early postoperative atrial fibrillation as often as
tients underwent the maze-I procedure and 15 patients the other cardiac surgery patients, we believe that careful clo-
maze-II procedure. For the reasons mentioned earlier, the sure of the left atrial appendage during surgery most likely
maze-III procedure became the standard thereafter, and by explains this apparent paradox.
July 1, 2000, 308 patients had undergone the maze-III pro- In our series, 98% of patients were cured of atrial fibril-
cedure for treatment of atrial flutter and/or atrial fibrillation. lation by the maze procedure alone and half of the other 2%
The operative mortality rate was 2.9%, with the indepen- of patients were cured with a combination of surgery and
dent determinants of operative death being (1) preoperative postoperative drug therapy, for an overall initial cure rate of
congestive heart failure, (2) preoperative hypertension, and 99%.63 A recent study of the long-term follow-up of these pa-
(3) performance of the maze procedure concomitantly with a tients revealed a 15-year cure rate of >95%.64 Interestingly,
double-valve replacement. The most common perioperative the cure rate at 15 years was the same for patients with and
complication following the maze procedure was postoper- those without concomitant cardiac disease, putting to rest ear-
ative arrhythmias, usually atrial flutter or atrial fibrillation, lier suggestions that the maze procedure was less effective in
which occurred in 37% of patients. As described earlier, the patients with mitral valve disease than in those with no appar-
maze procedure was designed to interrupt the macroreentrant ent structural heart disease.9 Other groups that have adhered
circuits that must be able to form for the atria to fibrillate. The to the concept of the maze procedure have attained similar re-
actual physical size of these circuits is determined by the du- sults.65-67 Groups that have chosen to modify the procedure
ration of the refractory period at any given site in the atria. by violating the basic concept of the maze procedure have
Normally, atrial refractory periods are relatively long; as a re- suffered poorer results.10,68
sult, the macroreentrant circuits are relatively large, i.e., over One of the major benefits of the maze procedure is that
6 to 7 cm in diameter. During the immediate postoperative it essentially abolishes the threat of stroke associated with
period and until the atrial heal from surgery, local refractory atrial fibrillation.69,70 The long-term stroke rate following the
periods may be much shorter and, therefore, the macroreen- maze procedure is 0.1% per year (Fig. 13). Overall, 15% of
trant circuits can be much smaller. As a result, it is possible to our patients who have pacemakers required pacemakers post-
form macroreentrant circuits between the suture lines of the operatively, but virtually all of them already had pacemak-
maze procedure and, therefore, to have postoperative atrial ers implanted before surgery, were known to have sick sinus
fibrillation even after a technically perfect operation. syndrome preoperatively, or had abnormal SA nodes “un-
Because there is a critical relationship between the size of masked” by abolishing the patient’s atrial fibrillation. Never-
the macroreentrant circuits, the distance between the maze theless, the need for postoperative pacemakers is higher in our
suture lines, and the effectiveness of the procedure in curing own series than in most other series,65-67 probably because
atrial fibrillation, this same concept explains why the maze of the more extensive extracardiac dissection that we per-
procedure may fail when performed in extremely large atria. form routinely in “preparing” the field for performance of the
Because the pattern of incisions is always the same, even in maze procedure itself. This suspicion seems to be confirmed
the presence of normal long atrial refractory periods (and, by the fact that only 6% of our patients required pacemakers
therefore, of large macroreentrant circuits), the distance be- after undergoing the maze procedure using minimally inva-
tween the incisions may be so great in large atria that reentrant sive techniques in which we perform very little extracardiac
circuits still can form between them following the surgery. dissection.
This is why the “cut-and-sew” technique is recommended for In our series, all patients were documented to have both
extremely large atria so that before the incisions are closed, right atrial and left atrial transport function in the immedi-
atrial muscle can be resected to decrease the distance between ate postoperative period that contributed to forward cardiac
the maze suture lines. output. On late follow-up evaluation, 98% of patients have
Cox Cardiac Surgery for Arrhythmias 259
the pulmonary veins. This article is at once one of the most Techniques for Ablating Atrial Fibrillation
important and one of the most poorly understood articles During Mitral Valve Surgery
ever published in the electrophysiology literature. The find-
ings were completely compatible with our earlier findings; in Following the phenomenal success of RF catheter abla-
fact, they completed the picture of the electrophysiology of tion for WPW syndrome, AV nodal reentrant tachycardia,
atrial fibrillation. The article by Haissaguerre et al. showed and other supraventricular arrhythmias in the 1990s, clini-
how atrial fibrillation is induced, and our earlier work showed cal electrophysiologists began to apply it for the attempted
how atrial fibrillation is maintained. Unfortunately, the article treatment of atrial fibrillation.11,12,73 Largely because of
was taken by many to mean that all that was needed to cure the availability of RF catheters and the initial reports of
atrial fibrillation was to isolate the orifices of the pulmonary Haissaguerre’s studies, surgeons began to use these RF
veins, a misconception that led to the development of our catheters intraoperatively in an effort to ablate atrial fibril-
own first surgical procedure to ablate atrial fibrillation (see lation in patients who already were undergoing surgery for
earlier). What resulted from the misinterpretation of this ar- mitral valve disease.9,10,13,14 The major objective in some of
ticle was the development of a variety of surgical devices these approaches10,13 was no longer to create a maze pro-
and procedures designed to encircle the pulmonary veins cedure but rather to encircle the pulmonary veins. Unfortu-
as the sole treatment for atrial fibrillation, an approach that nately, the RF lesions were frequently not transmural, thereby
has resulted in a predictable and unacceptable 30% failure offering at best only a temporary barrier to electrical conduc-
rate. tion. In addition, pulmonary vein isolation was used in many
This unfortunate misinterpretation of the article by patients with continuous atrial fibrillation; thus, the 30% fail-
Haissaguerre et al. ignores the fact that chronic (continuous) ure rate was predictable. Unfortunately, without properly un-
atrial fibrillation, of say 10 years’ duration, does not require derstanding the underlying electrophysiology of atrial fibril-
any type of induction stimulus because the atria are always in lation, several medical device companies developed products
atrial fibrillation. It also ignores the seminal work of Wijffels designed to do nothing more than encircle the pulmonary
and Allessie showing that once the atria begin to fibrillate, veins even though pulmonary vein encirclement, even when
they undergo a process of electrical “remodeling” in which accomplished by completely transmural lesions, is effective
the more they fibrillate, the more they will fibrillate in the fu- in only 90% of 50% of the patients with atrial fibrillation.
ture, or, as those authors state, “Atrial fibrillation begets atrial In actuality, pulmonary vein encirclement alone occasionally
fibrillation.”71,72 These established facts negate any impor- can ablate continuous atrial fibrillation if the isolated “cuff”
tance of the pulmonary veins in continuous atrial fibrillation, of left atrium surrounding the pulmonary veins is so large that
which represents approximately one half of the patients who it inadvertently ablates all of the surrounding macroreentrant
suffer from atrial fibrillation. Therefore, simple encirclement circuits in the left atrium. The problem with this scenario is
of the pulmonary veins is not a scientifically sound surgi- that so much of the left atrium is excluded by the isolated cuff
cal approach to the treatment of chronic atrial fibrillation. that there may be not effective left atrial contraction postop-
On the other hand, pulmonary vein isolation for the treat- eratively. Nevertheless, the overall atrial fibrillation ablation
ment of paroxysmal atrial fibrillation is firmly grounded in rate of 70% to 80% that now is being accomplished in some
science because of Haissaguerre’s observations and can be centers certainly is an improvement over simply ignoring
expected to cure upward of 90% of patients with that spe- atrial fibrillation in patients undergoing mitral valve surgery,
cific type of atrial fibrillation if Haissaguerre’s observations as has been the practice in the past.
in his highly selected group of patients can be applied to We continue to apply the complete maze procedure in pa-
the general population. Thus, if surgeons are to treat atrial tients with atrial fibrillation who require mitral valve surgery,
fibrillation effectively, it is extremely important that they un- using the cryosurgical technique rather than the old “cut-
derstand the difference between induction of atrial fibrilla- and-sew” technique to avoid leaving suture lines in the pos-
tion and maintenance of atrial fibrillation and the difference terior left atrium. The technique presently advocated adds
between paroxysmal (intermittent) and chronic (continuous) only 20 minutes to the overall procedure and is, as it has
atrial fibrillation. always been, just as effective in patients with mitral valve
Cox Cardiac Surgery for Arrhythmias 261
disease as it is in patients without mitral valve or other cardiac Early success rate and atrial function recovery. (Abstract) Circulation
disease.74 1999;100:I-854.
15. Haissaguerre M, Jais P, Shah DC, Takahashi A, Hocini M, Quiniou
G, Garrigue S, Le Mouroux A, Le Metayer P, Clementy J: Sponta-
Summary neous initiation of atrial fibrillation by ectopic beats originating in the
pulmonary veins. N Engl J Med 1998;3:339:659-666.
The anatomy and physiology learned from the decades 16. Gaskell WH: On the innervation of the heart, with especial reference
of surgical treatment of WPW syndrome, AV nodal reentrant to the heart of the tortoise. J Physiol 1883;4:43.
tachycardia, automatic atrial tachycardias, ischemic and non- 17. Kent AFS: Researches on structure and function of mammalian heart.
ischemic ventricular tachyarrhythmias, and atrial fibrillation J Physiol 1893;14:233.
18. Aschoff KAL: A discussion on some aspects of heart-block. Br Med J
provided a rich basis of knowledge and experience for the 1906;2:1103.
eventual success of catheter ablative techniques for cardiac 19. Wolff L, Parkinson J, White PD: Bundle branch block with short PR
arrhythmias. A question still remains regarding the ultimate interval in healthy young people prone to paroxysmal tachycardia. Am
nonpharmacologic manner in which atrial fibrillation will be Heart J 1930;5:685.
treated. The extremely minimally invasive surgical proce- 20. Wolferth CC, Wood FC: The mechanism of production of short P-R
intervals and prolonged QRS complexes in patients with presumably
dures, including endoscopic and robotic techniques, being undamaged hearts: Hypothesis of an accessory pathway of auriculo-
developed are challenging the catheter techniques in their ventricular conduction (bundle of Kent). Am Heart J 1933;8:297.
level of noninvasiveness while proving to be much quicker 21. Durrer D, Roos JP: Epicardial excitation of the ventricles in a patient
and more effective than the catheter approaches. History has with Wolff-Parkinson-White syndrome (type B): Temporary ablation
at surgery. Circulation 1967;35:15.
shown, however, that both cardiologists and cardiac surgeons 22. Burchell HB, Frye RL, Anderson MW, et al: Atrial-ventricular and
invariably respond to a challenge by developing ingenious in- ventricular-atrial excitation in Wolff-Parkinson-White syndrome (type
terventional schemes; therefore, the next quarter century of B): Temporary ablation at surgery. Circulation 1967;36:663.
NASPE should be just as exciting as the first one has been. 23. Cobb FR, Blumenschein SD, Sealy WC, Boineau JP, Wagner GS,
Wallace AG: Successful surgical interruption of the bundle of
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