Download Right Ventricular Dysfunction and Pulmonary Valve Replacement

Right Ventricular Dysfunction and Pulmonary
Valve Replacement After Correction of Tetralogy
of Fallot
Frank T. H. de Ruijter, MD, Ineke Weenink, MD, Francois J. Hitchcock, MD,
Erik J. Meijboom, MD, PhD, and Ger B. W. E. Bennink, MD, PhD
Children’s Heart Center, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, The Netherlands
Background. Correction of tetralogy of Fallot often
leads to pulmonary regurgitation, sometimes warranting
pulmonary valve replacement, for which indications and
timing to achieve optimal results are not yet clear. This
retrospective study describes follow-up and reinterventions in our tetralogy of Fallot population.
Methods. Review of all consecutive patients operated
on for tetralogy of Fallot between 1977 and 2000 was
conducted. Included are date and type of repair, Doppler
echocardiography (two-dimensional echocardiography),
electrocardiographs, reoperations, and physical
condition.
Results. Total repair was performed in 171 patients at a
mean age 1.9 ⴞ 2.5 years, follow-up time counted 9.6 ⴞ
7.0 years. Right ventriculotomy was used in 92%, and
transatrial ventricular septal defect closure was used in
8%; 74% received a transannular outflow patch. Twentyyear survival was 91%. Last follow-up electrocardiographs showed right bundle branch block in 67% and
serious arrhythmias in 11%. Two-dimensional echocardiography demonstrated severe pulmonary insufficiency
and dilated right ventricle in 31% and 38%, respectively,
increasing with postrepair age (p < 0.001). Poor clinical
condition (New York Heart Association class IIⴙ) and
echocardiographic proof of right atrial dilatation (p ⴝ
0.012) and arrhythmias (p ⴝ 0.03) were significantly
associated. Furthermore, the influence of residual hemodynamic lesions, such as a remaining ventricular septal
defect or pulmonary stenosis, or right ventricular dilatation was important (p ⴝ 0.04). Reintervention was necessary in 32 patients (19%; 10-year freedom, 83%), including
angioplasty for residual stenosis and pulmonary valve
replacement. At a mean age of 9.2 years after correction,
14 patients received a homograft, and 2 patients received
a heterograft. In 7 patients the right ventricle returned to
normal dimensions and symptoms disappeared. The incidence of right ventricular dilatation was considerably
higher (p ⴝ 0.020) in patients with a transannular patch;
the transatrial approach showed the opposite (p ⴝ 0.03),
and patients presented with lower QRS duration (p ⴝ
0.007), although no difference could be found between
survival after both surgical techniques. Effects of early
timing (correction < 6 months) on right ventricular
dysfunction could not be established.
Conclusions. Severe right ventricular dilatation and
pulmonary regurgitation secondary to outflow tract repair in tetralogy of Fallot are frequently occurring sequelae developing slowly over time. Indications for
pulmonary valve replacement remain controversial because echocardiographic findings or arrhythmias are not
always accompanied by deterioration of clinical condition. However, right atrial dilatation and additional hemodynamic lesions demand increased vigilance. Transatrial repair is associated with a favorable outcome.
(Ann Thorac Surg 2002;73:1794 – 800)
© 2002 by The Society of Thoracic Surgeons
N
up, pulmonary valve insufficiency is frequently encountered even in asymptomatic patients [5–7]. Severe longstanding pulmonary regurgitation (PR) can lead to
deleterious complications [1, 2, 8 –15]. In a small number
of patients with both deterioration of symptomatic status
and objective evidence of progressive right heart failure,
pulmonary valve replacement (PVR) is considered to
relieve symptoms [1, 4, 16 –20]; this number is expected to
grow [16, 21], especially those in whom a transannular
patch was used [22]. But specific indications and timing
of PVR remain unclear. This study is an inventory of our
tetralogy of Fallot population from initial repair to
present status, focusing on the development of RV indicators and occurrence of reinterventions in follow-up
with special attention to PVR.
owadays, clinical management of tetralogy of Fallot
(obstruction of right ventricular [RV] outflow, malalignment ventricular septum defect, and RV hypertrophy) involves a total surgical correction in early childhood. This decreases the necessity of prior palliative
shunt procedures and has a low operative mortality and
excellent long-term results [1–5]. However, in the follow-
Presented at the Forty-eighth Annual Meeting of the Southern Thoracic
Surgical Association, San Antonio, TX, Nov 8 –10, 2001.
Address reprint requests to Dr Bennink, Department of Cardiothoracic
Surgery, Pediatric Heart Center, Lundlaan 6, KG 01.319.0 3584 EA Utrecht,
The Netherlands; e-mail: [email protected].
© 2002 by The Society of Thoracic Surgeons
Published by Elsevier Science Inc
0003-4975/02/$22.00
PII S0003-4975(02)03586-5
Ann Thorac Surg
2002;73:1794 – 800
DE RUIJTER ET AL
RIGHT VENTRICULAR DYSFUNCTION AND PULMONARY VALVE REPLACEMENT
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Material and Methods
Population
The reports of all consecutive surviving patients, operated on in the Children’s Heart Center Utrecht between
1977 and 2000 and residing in the Netherlands, were
examined retrospectively. The population (n ⫽ 171) consisted of 106 (62%) male and 65 female patients with a
mean follow-up time of 9.6 ⫾ 7.0 years (range, 0.2 to 32.6
years; median, 8.0) and mean age of 13.8 ⫾ 8.0 years
(range, 0.2 to 37.8 years). Excluded from this survey were
99 patients with follow-up abroad directly after operation
(82), pulmonary atresia (13), complete atrioventricular
septal defect, subaortic conus, or death before complete
correction (2).
Uncomplicated tetralogy of Fallot was encountered in
150 patients, 12% (n ⫽ 21) had additional cardiac complications (abnormal coronary artery, systemic to pulmonary collaterals, pulmonary artery hypoplasia, branch
stenosis). Pentalogy (with atrial septal defect) was reported in 23 (13%), 12 had a persistent ductus arteriosus,
and 21 a bicuspid pulmonary valve. Comorbidity included Down syndrome, velocardiofacial syndrome, and
VATER association (vertebral defects, imperforate anus,
tracheoesophageal fistula, and radial and renal dysplasia) in 47 patients (27%). In 33 patients (19%) a palliative
shunt was initially required to overcome symptoms (age,
0.9 ⫾ 1.4 years; Blalock-Taussig, 8; central aortopulmonary, 25).
Total correction was performed at a mean age of 1.9 ⫾
2.5 years (range, 0.2 to 21.7 years). Most patients were
corrected using a transannular patch (n ⫽ 126, 74%) in
the RV outflow tract. In the remaining 26% infundibulectomy or pulmonary valve commissurotomy was sufficient
to overcome the obstruction. In the majority the ventricular septal defect (VSD) was repaired through right
ventriculotomy (157 patients, 92%); only 14 patients underwent transatrial repair. Mean follow-up time postoperatively was 8.0 ⫾ 6.5 years (range, 0 to 27.9 years;
median, 6.3 years).
Doppler echocardiographic data (especially RV dimensions, pulmonary stenosis and regurgitation, right atrial
dilatation, and tricuspid insufficiency), cardiac catheterizations, electrocardiographs, and 24-hour Holter recordings (QRS complex duration, dysrhythmias) were reviewed. Furthermore, physical condition (New York
Heart Association functional class I to IV), cause and time
of death, age, indications, and type of reintervention
were recorded.
Right Ventricular Dilatation and Pulmonary
Regurgitation
Echocardiographic data (two-dimensional views) describing the RV were categorized as follows: a normal RV,
ie, left ventricle to RV ratio of 3:1, was attributed the
number 0 or none, a severely dilated RV (RV size equal or
even exceeding left ventricular dimensions) was assigned
the value 2, and anything in between was classified as a
(mildly) dilated RV, associated with the number 1. Pulmonary valve incompetence was classified by the cardi-
Fig 1. Actuarial survival as a function of age.
ologist on a scale from 1 to 4, 1 meaning trivial, 2 mild, 3
and 4 are in this study regarded as severe.
Statistical Analysis
All data are presented as the mean value ⫾ one standard
deviation (range). Probability of survival, freedom of
reintervention, and PVR after repair were calculated by
Kaplan-Meier analysis with the use of the SPSS program
(SPSS Inc, OH). Differences among groups were calculated with Fisher’s exact test, log rank test, and Student’s
t test. For the time analysis of RV dilatation and PR, data
were clustered and adjusted for dependence of the outcome within a patient. The Mixor program (version 2)
[23] then provided a graph from the mean probability in
every category and year after corrective operation.
Results
Follow-Up
A total of 16 patients died during follow-up after complete repair. However, of the 11 deaths almost immediately after the operation, 2 patients had tracheobronchomalacia, 1 suffered from cystic fibrosis combined with
septic shock, and 2 from trisomy 13 and VATER association. Late deaths included 3 unrelated deaths (eg, car
accident) and 2 patients with sudden cardiac death.
Actuarial survival was 91% at both 10 and 20 years (Fig 1).
Total mortality attributed only to the heart disease itself
counted 4.7%.
At latest follow-up date most patients are in New York
Heart Association class I, yet a considerable number of
them presented with some insufficiency of the pulmonary valve, RV dilatation, tricuspid valve regurgitation,
and right atrial dilatation (Table 1).
A significant association between poor clinical condition (New York Heart Association functional class II⫹)
and objective echocardiographic measurements was only
found in the demonstration of right atrial dilatation (p ⫽
0.012); tricuspid insufficiency came close (p ⫽ 0.08). The
presence of arrhythmias was also related to poor condition (p ⫽ 0.03).
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Ann Thorac Surg
2002;73:1794 – 800
Table 1. Patient Data at Last Follow-Up
PVR
Characteristic
n
Deaths (early ⫹ lost to FU)
Follow-up age postoperatively PVR
Reinterventions except PVR
n
Significant arrhythmias
Severe PR
Severe RV dilatation
Tricuspid insufficiency
RA dilatation
Pulmonary stenosis ⬎30 mm Hg
All Patients
Without PVR
171
16 (15)
8.0 ⫾ 6.5
22
NYHA I
NYHA II⫹
155
14
8.1 ⫾ 9.3
16
9.2 ⫾ 6.7
5
4.4 ⫾ 6.6
1
133
13
10
48
56
63
19
20
0
15
6
5
13
12
5
6
12
3
7
1
4
5
3
6
140
16
11 (8%)
44 (31%)
53 (38%)
56 (40%)
16 (11%)
20 (14%)
6 (38%)
5 (31%)
7 (43%)
12 (75%)
6 (38%)
6 (38%)
FU ⫽ follow-up;
NYHA ⫽ New York Heart Association;
atrial;
RV ⫽ right ventricular.
NYHA I
NYHA II⫹
PR ⫽ pulmonary regurgitation;
In 15% (26 patients), RV outflow tract aneurysms were
detected (ranging from trivial to mild, only one requiring
reoperation). In 26% (44 patients) echocardiography
showed a residual VSD; 9 were closed and 35 were very
small or closed spontaneously. Postrepair cardiac catheterization was performed in 38 patients, mostly associated with PVR or to quantify pulmonary stenosis before
angioplasty.
Arrhythmias
Cardiac arrhythmias on routine electrocardiographic examinations were prevalent, especially complete right
bundle branch block (67%). First-degree atrioventricular
block was found in 16 patients; in 6 patients this preceded
the onset of more complex rhythm disturbances. Furthermore, 1 patient with Wolf-Parkinson-White syndrome
and 1 with sick sinus syndrome were encountered. Holter
monitoring was deemed necessary in 17 patients with
severe arrhythmias, such as (supra)ventricular tachycardia runs or multiple and multiform VES. Three patients
received medication and 1, a pacemaker implantation. Of
these patients 6 had PVR.
ⱍ
Before
After
16
2 (1)
PVR ⫽ pulmonary valve replacement;
RA ⫽ right
and fatigue; indications for and outcome of repeated
operative solution are depicted in Table 2. Most valves
used were homografts, although two were Hancock heterografts. Good results were reported in 7 patients: an RV
that returned to normal dimensions and disappearance
of symptoms. A QRS complex exceeding 180 ms predicted serious problems in at least 2 of 3 patients. All
important arrhythmias present before reoperation persisted after the procedure.
Four patients had a reintervention for residual pulmonary stenosis first. Tricuspid insufficiency as indication
for PVR was documented in patients with both good and
less favorable outcome.
Time and Type of Repair and Outcome
Figure 3 demonstrates that RV dimensions in patients
without or before PVR slowly increase over time. A
Reinterventions
A total of 32 patients (19%) underwent reoperation at a
mean age of 8.7 ⫾ 5.6 years (range, 1.3 to 22.3 years; at
6.3 ⫾ 5.8 years after correction), of which 8 did it more
than once (Fig 2). Follow-up time after first reintervention
was 6.0 ⫾ 5.8 years. The majority of reinterventions, PVR
excluded (10-year freedom, 83%), consisted of angioplasty for residual pulmonary stenosis (n ⫽ 12) and
residual VSD closure (n ⫽ 6).
Pulmonary Valve Replacement
In 16 patients (9%) both physical and heart condition
deteriorated to such an extent that PVR was considered
(mean age after repair, 9.2 ⫾ 6.7 years). Predominant
clinical symptoms include diminished exercise tolerance
Fig 2. Freedom of reintervention or pulmonary valve replacement
(PVR) as a function of years after operation.
Ann Thorac Surg
2002;73:1794 – 800
Table 2. Details of Pulmonary Valve Replacements
Indications for PVR
Postrepair Clinical
RV
Tricuspid
Pulmonary
QRS ⬎
Patient
Age (y)
Condition PR Dilatation Insufficiency Stenosis Arrhythmias 180 ms Homograft
1
0.1
2
x
x
x
x
x
x
x
1.0
1.2
x
x
x
4
5
6
7
2.7
4.5
5.0
6.0
x
x
x
x
x
x
x
x
x
x
x
x
x
x
8
6.5
x
x
x
x
x
9
10
11
9.0
11.9
14.0
x
x
x
x
x
x
x
x
x
x
x
x
12
15.2
x
x
x
x
13
14
15
16
15.8
16.6
17.5
21.0
x
x
x
x
94%
x
x
x
x
75%
x
x
x
x
x
x
x
x
100% 100%
DCRV ⫽ double chamber right ventricle;
patch;
VSD ⫽ ventricular septal defect.
x
x
Dilatation pulmonary
artery
Abnormal lung vein,
extra TAP,
pacemaker
x
x
x
x
x
x
x
x
x
x
x
x
x
x
x
x
x
x
PR ⫽ pulmonary regurgitation;
x
38%
19%
PS ⫽ pulmonary stenosis;
DCRV
Residual VSD
Angioplasty
pulmonary artery
x
38%
Angioplasty
pulmonary artery
Mitral valve
correction
Angioplasty
pulmonary artery
x
x
x
x
88%
PVR ⫽pulmonary valve replacement;
Outcome
0.1
Death shortly postoperatively
2nd PVR 7 yr later, sudden
cardiac death 12 y later
19.0
0.0
3.6
1.9
0.1
21.9
Residual RV dilatation
1.8
Residual PS
Good
Good
Heart transplant 19 y
postoperatively
Good
9.8
2.8
2.8
Residual RV dilatation
Residual PS
Good
0.1
Good
3.6
3.0
3.0
0.6
Good
Residual RV dilation
Residual PS
Good
RV ⫽ right ventricular;
TAP ⫽ transannular
DE RUIJTER ET AL
RIGHT VENTRICULAR DYSFUNCTION AND PULMONARY VALVE REPLACEMENT
3
Other Problems
Follow-up
After PVR
(y)
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DE RUIJTER ET AL
RIGHT VENTRICULAR DYSFUNCTION AND PULMONARY VALVE REPLACEMENT
Fig 3. Right ventricular (RV) dilatation index as a function of years
after operation.
similar plot could be drawn for pulmonary valve insufficiency. Statistical analysis showed that the increase was
significant in both cases (p ⬍ 0.001).
In 126 patients corrected with a transannular patch, 50
(39%) had developed severe RV dilatation at last followup; if the surgeon did not use this patch, this number
decreased to 26% (n ⫽ 12). Statistically, the incidence of
RV dilatation was thus considerably higher in patients
who underwent correction with the use of a transannular
outflow patch (p ⫽ 0.020).
The group in which the VSD was closed by transatrial
approach (n ⫽ 14) showed the opposite (p ⫽ 0.03): no
patients with severe RV dilatation. Only 1 patient underwent reoperation for residual VSD, and 1 patient had
PVR; moreover, this group presented with clearly lower
QRS duration (p ⫽ 0.007; mean, 108.0 ms compared with
131.6 ms in right ventriculotomy group).
Despite these effects, no difference could be found
between survival of both transannular patching and
transatrial VSD repair and their surgical opposites.
Results of patients with early correction (ie, before 6
months of age) demonstrated two reoperations, of which
one was PVR. Effects of early timing on RV dysfunction
could not be established. Finally, the influence of residual
hemodynamic lesions such as a remaining VSD, small
RV outflow tract aneurysm, or pulmonary stenosis more
than 30 mm Hg on RV dilatation could not be neglected
(p ⫽ 0.04).
Comment
In conjunction with recent large studies, the prognosis of
a newborn with tetralogy of Fallot is excellent [1– 4, 21].
Most patients were in New York Heart Association functional class I, and we found a 20-year actuarial survival of
91%. This is lower than the general population, as
pointed out by Murphy and colleagues [1], but it is still a
very reasonable outcome compared with other serious
congenital heart defects. The group of patients with
pulmonary atresia needs to be distinguished, as they
Ann Thorac Surg
2002;73:1794 – 800
present with more problems in follow-up, confirming the
results of Knott-Craig and associates [3].
We doubt whether changes toward earlier timing of
the initial repair greatly affect the late outcome. Our data
show that patients operated on after the age of 6 months
have a comparable probability of needing reintervention
or PVR, and there is no difference in RV dimensions. The
fact that their follow-up time was significantly longer and
most operations were performed in the early days of our
center certainly militate for a time-dependent or surgically dependent effect. Evaluating transannular patching,
this time factor was ruled out, and our results met those
of previous observations [22], indicating that both severe
PR and RV dilatation were more prevalent in those
patients who received this outflow patch. The positive
impact of the transatrial approach was confirmed, although this technique was not used extensively in our
population; we have more recently changed to a more
frequent implementation of this technique.
The shorter duration of the QRS complex, did not
result in fewer dysrhythmias in follow-up, in accordance
with Oechslin and coworkers [19], pointing out that the
site of ventricular arrhythmia is predominantly around
the margins of the VSD patch and not the ventriculotomy
per se, therefore putting every patient at equal risk. In
regard to the severity of late RV outflow tract complications, our results did confirm those of Dietl and colleagues [24], who are in favor of the right atrial technique.
Recommending preservation of the pulmonary valve or,
if a transannular patch is necessary, restricting its width
(minimizing regurgitant flow) and having a preference
for the transatrial approach seems logical.
Right ventricular dysfunction and pulmonary valve insufficiency slowly develop after repair of tetralogy of Fallot
and become present in almost all patients to some degree.
Similar to Meijboom and coworkers [21], Zakha and associates [9], Jonsson and colleagues [7], and Horneffer and
associates [2], who also used echocardiography in followup, we found evidence of more than trivial PR in a considerable number of patients. Meijboom and colleagues [21],
however, did not confirm the increase we found with time
of both PR and RV dilatation. Exercise capacity is found to
be decreased in patients with dilated RV [13, 21], but most
patients remain asymptomatic. Although some argue that
long-standing PR has no adverse effect on survival [1, 2, 21,
22, 25, 26], PVR will become a more frequent consideration
as the population ages. Shimazaki and coworkers [27]
reported that in patients without operation and with anatomically normal hearts, except for a congenitally incompetent pulmonary valve, symptoms of right heart failure will
develop 30 to 40 years later in life.
A considerable variation exists among patients in ability to tolerate PR and, as a result, development of RV
dilatation. Especially additional lesions make the detrimental effects more difficult to endure: 5 of our patients
already had one reoperation before undergoing PVR [8,
13, 22, 26]. Furthermore, Norgard and colleagues [28] and
Gatzoulis and associates [29] demonstrated the protective effect (limited PR, less ventricular dilatation, and
superior exercise performance) of a poorly compliant RV.
Ann Thorac Surg
2002;73:1794 – 800
DE RUIJTER ET AL
RIGHT VENTRICULAR DYSFUNCTION AND PULMONARY VALVE REPLACEMENT
A high percentage of our population (9%) underwent
PVR. In a few patients symptoms clearly diminished and
the severity of regurgitation and dilatation improved, but
unlike other reports [16, 18 –20], objective outcome was
not as good as expected. Some can be explained by
normal deterioration of the implanted valve (in this case,
mostly homografts), as also seen in groups with pulmonary atresia. Discovering a high-risk individual in an
earlier stage, and offering PVR before the onset of myocardial degeneration, is now becoming the ultimate
quest. Answers to this search are to be found in more
specific indications for the replacement.
Some authors have emphasized the importance of
coexisting tricuspid valve insufficiency [17, 26], resulting
from progressive RV dilatation, and advocate earlier
replacement because the onset of tricuspid regurgitation
may represent a very late and possibly irreversible stage
of RV failure [20].
Scar tissue is an important factor in the development of
malignant ventricular arrhythmias. When this is combined with poor hemodynamics, a high-risk situation can
evolve [30]. In contrast to Niezen and associates [11],
Meijboom and colleagues [21] could not show a correlation between RV dilatation and arrhythmias (and sudden
death). Neither could be confirmed in our study, but we
agree with the findings of Gatzoulis and coworkers [31],
that QRS prolongation (⬎ 180 ms) is their most sensitive
predictor. The presence of significant arrhythmias by
itself alone cannot be an indication for PVR.
Perhaps a great factor in this problem is not the
indication itself, but the evidence of its presence. Different patients have different ways of responding to hemodynamics after complete repair, and this should be accounted for in diagnostic methods. Doubts about a
deteriorated RV function by echocardiographic data
should best be confirmed by magnetic resonance imaging mapping studies to allow optimal timing in the
decision-making process tailored to the individual patient [11, 32].
Limitations
In the 23 years of cardiac surgery for tetralogy of Fallot in
our center, techniques and timing of the operation have
changed and the experience of the surgeons has increased, suggesting a more favorable outcome in the
youngest patients. During follow-up, electrocardiograph
and echocardiography devices were modernized, presenting clearer pictures and allowing detection of problems earlier. Moreover, observations were performed by
different cardiologists. Comparison of results may also be
hazardous, because no standardized method is used in
literature to quantify either PR or RV dilatation.
Conclusions
Severe RV dilatation and PR secondary to outflow tract
repair in tetralogy of Fallot are frequently occurring
sequelae in respectively 38% and 32% of our population,
developing slowly with time. Indications for PVR remain
controversial because warning echocardiographic findings or arrhythmias are not always accompanied by
1799
deterioration of clinical condition. However, right atrial
dilatation and additional hemodynamic lesions demand
increased vigilance. Transatrial repair is associated with a
favorable outcome.
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DISCUSSION
DR DARRYL S. WEIMAN (Memphis, TN): Doctor Kanter seems
to think that the porcine valve replacement is superior to the
homograft. Did you have a chance to look at your data in that
regard?
DR BENNINK: It is very interesting. In Holland, research was
done by the Leiden group in which they put in the growing pig
a biologic valve, mainly the Freestyle Medtronic valve, and
because of the disastrous results, we did not dare to use that
valve in humans. Now that I have seen this result of Dr Kanter,
I can see that the pig is a little bit different from the human
being. I knew that before, but it is not always wise to extrapolate
all the results you get from animal experiments. But mainly it is
homograft insertion that we do, and the Groningen group in
Holland even implants mechanical valves in the pulmonary
position.
DR CONSTANTINE MAVROUDIS (Chicago, IL): Some of the
very important work on postoperative arrhythmias in these
patients has been done by Michael Gatzoulis in Great Britain.
He showed that atrial reentry tachycardia is more common than
ventricular tachycardia in these patients. You did note in one of
your slides that you used arrhythmias as part of your indications
for operation. But did you do anything about these arrhythmias
at the time of operation? In other words, did you perform a maze
procedure in any of these patients? It would be interesting to
hear what your thoughts are on this subject.
DR BENNINK: Thank you for your comments, Dr Mavroudis.
We have not done anything with it yet, but, as you probably
know, one of your former fellows is now working in our institute
and we are planning to come and visit you in Chicago in January
to see exactly how to do those procedures, so we are really
considering it. One of our cardiologists is interested in electrophysiology. I think it is a wise thing to do, especially if the
procedure is not too much prolonged. Then I think it will
contribute to the outcome of the patients. Up until now we did
not perform any arrhythmia surgical procedures.