European Journal of Cardio-thoracic Surgery 25 (2004) 728–734

www.elsevier.com/locate/ejcts

Early replacement of pulmonary valve after repair

Downloaded from https://academic.oup.com/ejcts/article-abstract/25/5/728/456208 by guest on 06 September 2019

of tetralogy: is it really beneficial?q
Cheong Lima, Jae Young Leeb,*, Woong-Han Kimc, Soo-Cheol Kimd, Jin-Young Songb,
Soo-Jin Kimb, Joong-Haeng Choha, Chong Whan Kime
a
Department of Thoracic and Cardiovascular Surgery, Seoul National University Bundang Hospital, Sungnam-shi, Kyungki-do, South Korea
b
Department of Pediatric Cardiology, Sejong Heart Institute, Sejong General Hospital, 91-121 Sosa Bon 2-dong,
Sosa-ku, Bucheon-shi, Kyungki-do 422-232, South Korea
c
Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul, South Korea
d
Department of Thoracic and Cardiovascular Surgery, Kyunghee University Medical Center, Seoul, South Korea
e
Department of Cardiovascular Surgery, Sejong Heart Institute, Sejong General Hospital, Bucheon-shi, Kyungki-do, South Korea
Received 21 September 2003; received in revised form 10 January 2004; accepted 23 January 2004

Abstract
Objectives: Debate on the proper timing of pulmonary valve replacement (PVR) after repair of tetralogy of Fallot (TOF) is still
continuing. Significant pulmonary regurgitation (PR) could result in right ventricular (RV) dysfunction, exercise intolerance, arrhythmia, and
sudden death. We analyzed clinical results of PVR after repair of TOF to investigate potential risk factor for late outcomes. Methods: From
January 1993 to July 2002, 58 patients (38 males and 20 females) received PVR after repair of TOF. More than moderate degree of PR was
observed in these patients by echocardiography. Mean age at PVR was 13.5 ^ 9.6 years (1.2– 44) and TOF repair was performed at
5.2 ^ 7.1 years of age (0.5– 34). Therefore, PVR was performed at 8.3 ^ 5.2 years (4 months – 28 years) after repair. Preoperative
electrocardiogram showed complete right bundle branch block in 49 patients (84.5%). Mean duration of QRS complex was 142 ^ 30 ms.
Major arrhythmia occurred in eight patients. Twenty-nine patients complained decreased physical activity and 10 patients showed clinical
signs of right heart failure. Results: Early death occurred in one patient (2.5%). Major complication occurred in three patients (complete
heart block in two, aortic rupture in one). Follow-up was performed for 2.5 ^ 2.4 years (46 days – 10.3 years). There was no late death.
Postoperative cardiothoracic ratio was significantly decreased (0.61 ^ 0.07 to 0.55 ^ 0.06, P , 0:001). Marked symptomatic improvement
was noted in all patients. Postoperative symptomatic group ðn ¼ 14Þ showed older age at repair of TOF (12.5 ^ 10.7 vs 2.6 ^ 2.3 years,
P ¼ 0:003), older age at PVR (23.2 ^ 12.8 vs 10.1 ^ 5.0 years, P ¼ 0:001), longer interval between repair of TOF and PVR (10.6 ^ 7.0 vs
7.5 ^ 4.2 years, P , 0:05), higher degree of functional class (2.4 ^ 0.5 vs 1.4 ^ 0.8, P , 0:001), and longer duration of hospitalization
(30.0 ^ 14.2 vs 18.9 ^ 11.4 days, P ¼ 0:004) than postoperative asymptomatic group ðn ¼ 43Þ: Conclusions: In patients with significant
PR after repair of TOF, PVR had clinical benefits including symptomatic improvement with low mortality and morbidity. Proper timing must
be carefully selected according to objective evaluation of RV function. In our study, earlier PVR prior to symptomatic manifestation showed
beneficial effects.
q 2004 Elsevier B.V. All rights reserved.
Keywords: Right ventricle; Pulmonary regurgitation; Congenital

1. Introduction most of the patients [1]. However, chronic significant PR

may result in progressive right ventricular (RV) dilatation
Pulmonary regurgitation (PR) after repair of tetralogy of and failure, exercise intolerance, and it may be accompanied
Fallot (TOF) is generally well tolerated for a long time in by ventricular arrhythmia and sudden death [2 – 4]. Asso-
ciated problems such as residual ventricular septal defect,
tricuspid valve regurgitation (TR), and distal pulmonary
q
Presented at the joint 17th Annual Meeting of the European Association artery stenosis can aggravate those adverse effects of PR [5].
for Cardio-thoracic Surgery and the 11th Annual Meeting of the European
Society of Thoracic Surgeons, Vienna, Austria, October 12 –15, 2003.
Therefore, the necessity of pulmonary valve replacement
* Corresponding author. Tel.: þ82-32-340-1123; fax: þ 82-32-340-1236. (PVR) in patients with significant PR is generally
E-mail address: [email protected] (J.Y. Lee). accepted [6 – 8].
1010-7940/$ - see front matter q 2004 Elsevier B.V. All rights reserved.
doi:10.1016/j.ejcts.2004.01.036
 C. Lim et al. / European Journal of Cardio-thoracic Surgery 25 (2004) 728–734 729

However, debate on the proper timing of PVR after failure, or (2) associated lesions besides PR such as TR
repair of TOF is still continuing [9 –12]. In a recent report or pulmonary artery stenosis, or residual ventricular
by Therrien et al. [9], RV functional recovery after PVR for septal defect, or (3) symptomatic arrhythmia, or (4) RV
chronic PR was compromised in the adult population, and dilatation and dysfunction assessed by qualitative 2D
the authors suggested that earlier PVR should be considered echocardiography or right ventriculography.
before RV function deteriorated. Leiden group [12 – 14] also Before PVR, 29 (50%) patients had reduced exercise
showed the rationale of earlier PVR using dobutamine stress tolerance and were in New York Heart Association
magnetic resonance imaging (MRI) and brain natriuretic (NYHA) functional class II ðn ¼ 20Þ; III ðn ¼ 7Þ; or IV
peptide level. ðn ¼ 2Þ: Ten patients showed clinical signs of right heart

Downloaded from https://academic.oup.com/ejcts/article-abstract/25/5/728/456208 by guest on 06 September 2019

The precise assessment of RV function is difficult failure such as generalized edema. Tricuspid regurgitation
because of its structural complexity and altered loading of more than moderate degree was observed in 18
conditions, especially in patients with PR and associated patients. Branch pulmonary artery stenosis or distortion
lesions. Preoperative parameter predicting postoperative was observed in 38 patients. RV outflow track obstruc-
RV performance and long-term outcome after PVR in tion with more than 40 mmHg of pressure gradient was
patients with chronic PR was not clearly defined yet. And observed in two patients. Five patients had residual
there is still a concern about the risk of later reoperation for
ventricular septal defect of more than moderate size.
prosthetic valve failure.
RV dysfunction and enlargement were subjectively
The purposes of our study were to examine the results
classified into mild, moderate, and severe grades. In these
and effectiveness of PVR after repair of TOF, and to find out
criteria, preoperative echocardiography ðn ¼ 58Þ showed
potential risk factors contributing to poor late outcomes.
moderate to severe RV dilatation in 44 patients (76%),
Our primary focus on this study was to investigate the
and moderate to severe RV dysfunction in seven patients
parameters regarding the proper surgical timing of PVR.
(12%).
Eight patients had symptomatic arrhythmias (atrial flutter
2. Materials and methods and/or fibrillation in six, and sustained ventricular tachy-
cardia in two). Six of them were more than 20 years of age.
2.1. Patients profile Preoperative electrocardiogram showed complete right
bundle branch block in 49 patients (84.5%). Mean duration
A retrospective study was done in 58 consecutive of QRS complex was 142 ^ 30 ms. Long QRS duration
patients (38 males and 20 females), who underwent PVR (more than 180 ms) was observed in eight patients. Patients’
for PR after previous valveless repair of TOF or Fallot-type demographic data are summarized in Table 1.
double outlet right ventricle from January 1993 to July 2002
at the Sejong Heart Institute, Sejong General Hospital. Full
medical records including cardiac database of our institution
were reviewed. All patients had more than moderate degree Table 1
of PR. The severity of PR was assessed by pulse-wave Demographic data
Doppler characteristics on the main pulmonary artery as Parameters Value (range or percentage)
previously described [15] and was graded as moderate or
severe if the regurgitant fraction was more than 40%. Demography
Preoperative right ventriculography was done in 55 patients Sex (male/female) 38/20
and also showed more than moderate degree of PR in all. Age (years) at TOF repair 5.2 ^ 7.1 (0.5–34)
Age (years) at PVR 13.5 ^ 9.6 (1.2–44)
The initial TOF repair was done at a mean age of
Interval, TOF repair–PVR (years) 8.3 ^ 5.2 (0.3–28)
5.2 ^ 7.1 years (range 0.5 –34 years). PVR was performed
at a mean age of 13.5 ^ 9.6 years (range 1.2 –44 years) with Preoperative status
Number, PR $ moderate 58 (100)
a mean duration from repair to PVR of 8.3 ^ 5.2 years
Number, PA stenosis/distortion 38 (66)
(range 4 months – 28 years). Fifteen patients had undergone Number, TR $ moderate 18 (31)
palliative procedures before intracardiac repair. A trans- Number, significant arrhythmia 8 (14)
annular patch had been used in 49 patients (84.5%). Sixteen QRS duration (ms) 142 ^ 30 (80–200)
patients had additional interventional or surgical procedures Number, NYHA FC $ II 29 (50)
Number, right heart failure 10 (17)
before PVR; balloon pulmonary angioplasty and/or stent
CT ratio (%) 60.8 ^ 7.3 (47.3–77.0)
implantation in 11 and surgical pulmonary angioplasty in RVEDP (mmHg) 10.0 ^ 3.2 (3–18)
three for branch pulmonary artery stenosis, and RV
outflow track reconstruction with pulmonary artery angio- TOF, tetralogy of Fallot; PVR, pulmonary valve replacement; PR,
pulmonary valve regurgitation; PA, pulmonary artery; TR, tricuspid valve
plasty in two. regurgitation; QRS, QRS complex on electrocardiogram; NYHA FC, New
PVR was generally considered in patients with (1) York Heart Association functional class; CT, cardiothoracic; RVEDP, right
exercise intolerance or signs and symptoms of right heart ventricular end-diastolic pressure.
730 C. Lim et al. / European Journal of Cardio-thoracic Surgery 25 (2004) 728–734

2.2. Preoperative and postoperative workup 2.4. Statistical analysis

The patients who underwent initial TOF repair were Statistical analysis was performed with the Statistical
followed-up by the pediatric cardiologist in every 6- or Analysis System software package (version 6.12; SAS
12-month interval. Careful review of symptoms, physical Institute, Cary, NC). Comparison between preoperative and
postoperative continuous variables such as cardiothoracic
activities and signs of right heart failure was performed in
ratio was performed by paired t-test. The significance of
every visit. Progressive increase of cardiothoracic ratio, new
differences between two groups was assessed by Student’s
onset of symptoms, and decreasing tendency of physical
t-test, x 2 -test or Fisher’s exact test as appropriate. Freedom

Downloaded from https://academic.oup.com/ejcts/article-abstract/25/5/728/456208 by guest on 06 September 2019

activity as well as changes in electrocardiogram such as
from reoperation was analyzed using Kaplan – Meier
arrhythmia and QRS prolongation were considered indi-
method. All results were expressed as mean ^ standard
cations of diagnostic workup including echocardiography,
deviation and a value of P , 0:05 was considered
cardiac catheterization, 24 h Holter monitoring, and exer- statistically significant. Multivariate analysis was not done.
cise test. Recently, we performed cardiac MRI with cine-
MRI to evaluate the RV function objectively. Postoperative
follow-up was performed in the same manner. 3. Results

Cardiopulmonary bypass time and aortic cross clamping

2.3. Surgical technique time were 164 ^ 53 (range 88 – 308 min) and 82 ^ 46 min
(range 14 – 173 min), respectively. Associated procedures
After the induction of general anesthesia and full were done in 42 patients (72%) and were summarized in
invasive monitoring, redo midline sternotomy incision was Table 2. Duration of hospital stay was 21.6 ^ 13.0 days
made using oscillating saw. Femorofemoral bypass was (range 7 – 62 days) postoperatively.
used when appropriate, i.e. bleeding during redo-sterno- Early death occurred in one patient (2.5%). He was
tomy. Careful dissection of substernal adhesion was 38-year-old male who suffered from dyspnea, orthopnea,
performed using both blunt and sharp dissections with ascites and pleural effusion due to ventricular failure, atrial
electrocautery. After institution of routine cannulation and fibrillation, and cardiac cirrhosis. He had been operated on
moderate systemic hypothermia around 30 8C, electrical at the age of 33 years for TOF repair. He received PVR and
ventricular fibrillation was induced and the RV outflow tract tricuspid valve replacement with Carpentier – Edwards
was opened longitudinally over the annulus of pulmonary pericardial bioprostheses. Concomitant aortic valvuloplasty
valve. In case of residual ventricular septal defect or other and closure of ventricular septal defect were performed.
intracardiac problem, aortic cross clamping and antegrade Immediate postoperative vital signs were stable and
intermittent cold blood cardioplegia was used. echocardiography showed fair to good RV contractility.
PVR was performed using various prosthesis including Left ventricular ejection fraction was estimated up to 44%.
At the 11th postoperative day, he died of sudden massive
bioprosthesis, mechanical prosthesis and homograft as
tracheal bleeding and multiorgan failure due to intractable
available. There was no randomization in choice of
valve, and it usually depended on individual surgeon’s
preference. There were three mechanical valves, 23 Table 2
porcine bioprostheses, 20 pericardial bioprostheses, 11 Associated procedures
stentless bioprostheses, and 1 homograft valve.
Procedures Number of cases
Valve substitute was inserted into the true pulmonary
valve annulus using 4 – 0 polyprophylene continuous PA angioplasty 38
sutures. Stentless valves and homograft were inserted RVOT aneurysmorrhaphy 21
more distally to avoid sternal compression. Aneurysmal TVP or TVR 18 (1: TVR)
dilatation of RV outflow tract was excised or plicated. In RVOTO relief 16
VSD closure 11
order to insert larger pulmonic valve, anterior aspect of RA cryoablation 4
valve substitute was covered with bovine pericardial patch. RA reduction plasty 3
Any kind of pulmonary artery stenosis was concurrently Pacemaker implantation 3
enlarged with the same patch. Patients with significant ASD closure 1
Aortic valvuloplasty 1
arrhythmia received anti-arrhythmic surgery using cryo-
LVOTO relief 1
ablation and right side maze procedure [16]. Recently, we
used synthetic patch made of enhanced-polytetrafluoro- PA, pulmonary artery; RVOT, right ventricular outflow tract; TVP,
tricuspid valvuloplasty; TVR, tricuspid valve replacement; RVOTO, right
ethylene (Gore-Texe) to cover the anterior surface of the
ventricular outflow tract obstruction; VSD, ventricular septal defect; RA,
heart in order to facilitate safer and faster re-entrance of right atrium; ASD, atrial septal defect; LVOTO, left ventricular outflow
sternum during inevitable future re-operations. tract obstruction.
 C. Lim et al. / European Journal of Cardio-thoracic Surgery 25 (2004) 728–734 731

Table 3
Postoperative complications

Complications Number

Wound problem 3
Complete atrioventricular block 2
Aortic rupture 1
Low cardiac output syndrome 1
Torsade de Pointes 1

Downloaded from https://academic.oup.com/ejcts/article-abstract/25/5/728/456208 by guest on 06 September 2019

Transient neurologic deficit 1
Prolonged chest tube drainage 1
Pneumothorax 1

coagulopathy. Postoperative complications were sum-

marized in Table 3.
Follow-up was performed for 2.5 ^ 2.4 years (46 days – Fig. 1. Actuarial freedom from redo-PVR in patients who underwent PVR
10.3 years). There was no late death. Postoperative after TOF repair. PVR, pulmonary valve replacement; TOF, tetralogy of
cardiothoracic ratio was significantly decreased at the last Fallot.
follow-up (0.61 ^ 0.07 to 0.55 ^ 0.06, P , 0:001). Post- was 5.3 ^ 2.5 years (range 1.1 –8.2 years). Two of them had
operative echocardiography was performed at a median of first PVR with mechanical valves, two with pericardial
20 months after PVR (range 1 month – 10 years). It showed valves, one with FreeStylee stentless valve, and one with
improved RV function and RV dilatation. Moderate to aortic homograft. Actuarial freedom from redo-PVR at 5
severe RV dysfunction was observed in 1.8% (vs 12% years post-PVR was 87.9 ^ 9.5% (Fig. 1).
preoperatively, P , 0:05), and moderate to severe RV Comparison of perioperative parameters was done
dilatation in 28% (vs 76% preoperatively, P , 0:001). TR between postoperatively symptomatic and asymptomatic
of more than moderate degree was observed in three patients groups (Table 4). Postoperative symptomatic group
(vs 18 patients preoperatively, P , 0:001). Symptomatic ðn ¼ 14Þ showed older age at repair of TOF, older age at
improvement was marked and the average validity class was PVR, longer interval between repair and PVR, longer
significantly decreased (mean NYHA functional class, duration of hospitalization, higher degree of preoperative
1.72 ^ 0.83 to 1.25 ^ 0.43, P , 0:001). Fourteen patients functional class, larger cardiothoracic ratio and more
(26%) were still symptomatic (NYHA class II in 13 and frequent preoperative arrhythmia than postoperative asymp-
III in 1). tomatic group ðn ¼ 43Þ: The incidence of TR and RV
Four out of six patients, who had atrial arrhythmia
preoperatively, underwent anti-arrhythmia surgery includ-
ing cryoablation and right-side maze procedure, two with Table 4
concomitant pacemaker insertion due to sinus node Comparison between groups according to validity after pulmonary valve
replacement
dysfunction. Two had returned to normal sinus rhythm
postoperatively and another two showed AAI- and DDD- Variables Group I, Group II, P-value
type pacing rhythm without atrial arrhythmia. One of the NYHA I NYHA $II
ðn ¼ 43Þ ðn ¼ 14Þ
two patients without arrhythmia surgery had persistent atrial
fibrillation with ventricular rate control and the other was a TOF repair age (years) 2.6 ^ 2.3 12.5 ^ 10.7 0.003
mortality case. New-onset atrial flutter developed in one PVR age (years) 10.1 ^ 5.0 23.2 ^ 12.8 0.001
patient with severe PR, TR, severe right atrial enlargement, Interval (years) 7.5 ^ 4.2 10.6 ^ 7.0 0.03
and right heart failure. Right atrial reduction plasty was Pre-PVR NYHA FC 1.4 ^ 0.8 2.4 ^ 0.5 ,0.001
Pre-PVR CTR (%) 59.5 ^ 6.9 64.8 ^ 7.2 0.04
done without prophylactic cryoablation. She was on anti-
Pre-PVR TR $ III (patients) 12 6 NS
arrhythmic therapy now. In one of the two patients who Pre-PVR RVEDP (mmHg) 10.2 ^ 2.8 9.3 ^ 4.5 NS
have had ventricular tachycardia preoperatively, sustained Pre-PVR RVP (mmHg) 57.1 ^ 18.7 49.2 ^ 15.3 NS
monomorphic ventricular tachycardia was recurred a year Pre-PVR p(RV/Ao) 0.54 ^ 0.18 0.42 ^ 0.14 0.047
after operation and well controlled with b-blocker. The Pre-PVR major arrhythmia 2 6 0.003
(patients)
other was free of arrhythmia 18 months after the operation. Hospital stay (days) 18.9 ^ 11.4 30.0 ^ 14.2 0.004
On follow-up electrocardiogram, the mean duration of QRS
complex did not change (142 ^ 30 to 144 ^ 30 ms, PVR, pulmonary valve replacement; NYHA FC, New York Heart
Association functional class; TOF, tetralogy of Fallot; CTR, cardiothoracic
P ¼ 0:05). ratio; TR, tricuspid valve regurgitation; RVEDP, right ventricular end-
Redo-PVR was performed in six patients during post- diastolic pressure; RVP, right ventricular systolic pressure; p(RV/Ao), the
operative follow-up. Mean duration of PVR to redo-PVR ratio of systolic right ventricular to aortic pressure.
732 C. Lim et al. / European Journal of Cardio-thoracic Surgery 25 (2004) 728–734

pressure parameters on cardiac catheterization were not methods measuring the RV volume and function [9]. But
different between groups. another explanation is that the ages at TOF repair or PVR
are different among groups. The patient population reported
by Bove et al. [7] was significantly younger at the time of
4. Discussion PVR (mean of 14.6 vs 33.9 years, respectively) than those of
Therrien et al. [9]. This implies that the potential for
Our study primarily focused on determining the potential contractile recovery and ability to undergo remodeling after
risk factors contributing to poor late outcome after PVR in PVR may diminish over time and earlier PVR should be
patients with significant PR after repair of TOF. After PVR, considered in adult population. The age at initial TOF repair

Downloaded from https://academic.oup.com/ejcts/article-abstract/25/5/728/456208 by guest on 06 September 2019

subjective symptomatic improvement was marked. Opera- also may have influence on the late outcome. The mean ages
tive mortality and morbidity were acceptable. Older age at at the time of PVR in the patients reported by Therrien et al.
TOF repair and PVR, longer interval from repair to PVR, [9] and Vliegen et al. [14] were similar (< 30 years), but the
and poor preoperative validity class were risk factors to poor mean ages at initial TOF were different (mean of 5.7 vs
late outcome. To our knowledge, this is the first study to 12.1 years, respectively). These findings are consistent with
show the risk factors to poor late outcome after PVR in our study, showing that older age at PVR and at initial TOF
patients with repaired TOF. repair caused adverse effect on postoperative outcomes.
Although long-term functional results of patients after Prolonged exposure to cyanosis and pressure overloading
TOF repair were excellent, the negative effects of chronic before total correction, and inadequate myocardial protec-
PR as a result of RV outflow track reconstruction on RV tion during cardiopulmonary bypass and direct myocardial
function, cardiac electrophysiology, and life expectancy injury at the time of initial repair may impair the ability to
were well recognized [1 –4]. Previous reports demonstrated recover after PVR in aged RV [9].
that PVR had beneficial effects on symptoms, RV function, The longer time interval from the initial repair to PVR
and arrhythmia propensity in patients with repaired TOF and preoperative poor functional class were other risk
[6 –8,17]. These findings were consistent with those of our factors to poor outcomes in our study. Conte et al. [20] also
study. Cardiothoracic ratio was decreased and RV dilatation found that the patients who did not have major benefits
and dysfunction on echocardiography improved substan- from PVR had been previously exposed to PR for a
tially. Symptomatic improvement was marked. considerably longer time than the others (mean interval
However, optimal timing of PVR is still a subject of between initial repair and PVR, 18.7 vs 12.6 years,
debate. Exercise intolerance or symptoms of right heart P ¼ 0:01). Moreover, the patients who underwent PVR
failure, significant arrhythmia, evidence of RV dysfunction, more than 15 years after initial repair had only mild
and development and progression of TR have been reduction in RV dilatation after PVR.
generally accepted as indications for PVR, as were in our Ilbawi et al. [21] have reported complete recovery of RV
study [8]. But precise evaluation of exercise tolerance is function and work performance only in patients who had
somewhat complex because of the lack of uniform standards PVR within the first 2 years after TOF repair, while in all the
for exercise testing in patients with congenital heart disease other patients who had PVR at a later time (2 – 13 years after
[18,19]. And the assessment of RV function is also complex TOF repair), RV function remained abnormal and exercise
because of its complex structure and altered loading tolerance did not improve significantly. They explained these
conditions in repaired TOF [14]. So, the results are variable findings with already existing irreversible myocardial
between study groups [7,9,14,20]. damage before PVR. They suggested early recognition of
Bove et al. [7] found a reduction in RV volume patients at risk of developing RV failure to proceed with PVR
(measures with M-mode echocardiography) and improved in time to have greater benefits and to prevent irreversible
RV ejection fraction (measured with radionuclide angio- deterioration in RV function. Therefore, waiting for
gram) after PVR in a group of patients with mean age of symptoms to appear may allow irreversible RV dysfunction
14.6 years. Subjective improvement in exercise tolerance to occur and result in minimal benefits from PVR.
was also recorded. On the contrary, Therrien et al. [9] found The exact timing of PVR would not be defined yet in our
no reduction in RV volumes after PVR using radionuclide study because of the subjective nature of diagnostic criteria.
angiogram and no improvement of exercise capacity in a Actually we cannot define the objective criteria yet.
group of adults with a mean age of 33.9 years, despite However, according to our results, older age at PVR and
substantial improvement of functional class. They suggested longer duration from TOF repair to PVR were the risk
that earlier PVR should be considered before RV function factors of poor outcome. Other report regarding PVR in the
deteriorated. In contrast, recent report on a group of patients adult age [9] also showed that recovery of RV dysfunction
with a mean age of 29.2 years, dramatic improvements were was impaired. So we think that PVR should be considered at
noted in MRI follow-up [14]. That showed reduction of RV least in the patient of late adolescence or early adulthood.
volume and improvement of RV ejection fraction corrected Arrhythmia including ventricular tachycardia and atrial
for regurgitation and RV end-systolic volume. These flutter or fibrillation may cause decreased cardiac output,
discrepancies may be basically due to the differences in increased exercise intolerance, and sudden cardiac death.
 C. Lim et al. / European Journal of Cardio-thoracic Surgery 25 (2004) 728–734 733

Gatzoulis et al. [3] described in their multicenter trial that Recently, we are performing cardiac MRI with cine-MRI to
the risk factors for arrhythmia and sudden cardiac death evaluate the RV size and function objectively in the
were QRS prolongation, older age at repair, pulmonary prospective manner. But the study period is still short and
valve regurgitation, and TR. They suggested that preser- more data collection will be needed to support our policy in
vation or restoration of pulmonary valve function might earlier PVR. Although uniform standard for exercise testing
reduce the risk of sudden death and electrocardiographic in patients with congenital heart disease is lacking, exercise
markers could help to identify patients at risk. Toronto test with maximal oxygen consumption rate is desirable
group [17] also reported that PVR led to stabilization of for a more complete assessment of objective exercise
QRS duration and, in conjunction with intraoperative capacity [18,19].

Downloaded from https://academic.oup.com/ejcts/article-abstract/25/5/728/456208 by guest on 06 September 2019

cryoablation, to a decrease in the incidence of preexisting In conclusion, PVR after repair of TOF can be performed
atrial or ventricular tachyarrhythmia. In our study, the with low mortality and morbidity and it can improve the RV
stabilization of QRS duration was also recorded. Eight function, functional class and patient’s physical activity.
patients had significant arrhythmia preoperatively and four Also it can reduce the possibility of life threatening
of them received anti-arrhythmia surgery including cryo- arrhythmia and sudden death. Risk factors that may cause
ablation and right-side maze procedure. Postoperatively, poor late outcomes are older age at initial TOF repair, older
atrial arrhythmias disappeared and all four patients are age at PVR, longer interval from TOF repair to PVR, poorer
doing well in a recent follow-up. But in one adult patient preoperative functional class, larger cardiothoracic ratio,
who had severe TR and severe right atrial enlargement but and presence of significant arrhythmia. Therefore, earlier
no preoperative arrhythmia, new atrial flutter developed, replacement of chronically regurgitant pulmonary valve
although TR was well corrected and right atrial reduction after repair of TOF should be considered before deterio-
plasty was done. We think that in patients with prolonged ration of RV function and functional class even in
severe TR and right atrial enlargement, prophylactic right asymptomatic patient, especially in the adult population.
cryoablation should be considered. Right atrial dilatation
from volume or pressure load prolongs atrial refractoriness
in a heterogeneous manner. This dilatation, together with Acknowledgements
right atrial scarring from previous surgery, makes the atria
susceptible to reentrant arrhythmia, even after PVR and TR Institutional Review Board of our hospital approved this
corrections [3,17]. In our opinion, patients with atrial flutter study.
or fibrillation should receive both cryoablation and right
side maze procedure. Also, in a patient with ventricular
tachycardia, both electrophysiologic study and cryoablation
References
should be considered. It would be the best option to improve
post-PVR outcome and to reduce the potential risk of [1] Nollert G, Fischlein T, Bouterwek S, Böhmer C, Klinner W, Reichart
sudden cardiac death. B. Long-term survival in patients with repair of tetralogy of Fallot: 36-
The mean age at operation was 5.2 years in our study year follow-up of 490 survivors of the first year after surgical repair.
with an incidence of transannular patching of 84.5%. The J Am Coll Cardiol 1997;30:1374–83.
state of the art now is to correct earlier using less a [2] Oechslin EN, Harrison DA, Harris L, Downar E, Webb GD, Siu SC,
Williams WG. Reoperation in adults with repair of tetralogy of Fallot:
transannular approach. A recent trend in our institution is indications and outcomes. J Thorac Cardiovasc Surg 1999;118:
early repair of TOF (less than 12 months of age). We are 245–51.
trying to save the pulmonary valve as long as we can. We [3] Gatzoulis MA, Balaji S, Webber SA, Siu SC, Hokanson JS, Poile C,
hope that this will decrease the incidence of late RV Rosenthal M, Nakazawa M, Moller JH, Gillette PC, Webb GD,
dysfunction. Redington AN. Risk factors for arrhythmia and sudden cardiac death
late after repair of tetralogy of Fallot: a multicentre study. Lancet
Limitation of our study includes rather subjective
2000;356:975 –81.
evaluation of postoperative changes, such as symptoms, [4] Harrison DA, Harris L, Siu SC, MacLoghlin CJ, Connelly MS, Webb
exercise tolerance and RV function on echocardiography. GD, Downar E, McLaughlin PR, Williams WG. Sustained ventricular
Until recently, there is no gold standard to evaluate the RV tachycardia in adult patients late after repair of tetralogy of Fallot.
functional reserve objectively and quantitatively, because of J Am Coll Cardiol 1997;30:1368–73.
[5] Kirklin JK, Kirklin JW, Blackstone EH, Milano A, Pacifico AD.
the complex geometry of RV. Assessment of RV function
Effect of transannular patching on outcome after repair of tetralogy of
by M-mode or 2D echocardiography has fundamental Fallot. Ann Thorac Surg 1989;48:783–91.
drawbacks. But, this modality is rather simple, and even [6] Yemets IM, Williams WG, Webb GD, Harrison DA, McLaughlin PR,
though not perfect, easy to apply to routine and regular Trusler GA, Coles JG, Rebeyka IM, Freedom RM. Pulmonary valve
follow-up. More objective measures to evaluate the RV replacement late after repair of tetralogy of Fallot. Ann Thorac Surg
1997;64:526 –30.
functional reserve such as dobutamine stress MRI, brain
[7] Bove EL, Kavey RE, Byrum CJ, Sondheimer HM, Blackman MS,
natriuretic peptide level or radionuclide study will be Thomas FD. Improved right ventricular function following late
needed in the future studies [9,13,14]. MRI is now emerging pulmonary valve replacement for residual pulmonary insufficiency or
as a gold standard technique to assess RV size and function. stenosis. J Thorac Cardiovasc Surg 1985;90:50–5.
734 C. Lim et al. / European Journal of Cardio-thoracic Surgery 25 (2004) 728–734

[8] Kanter KR, Budde JM, Parks WJ, Tam VKH, Sharma S, Williams Appendix A. Conference discussion
WH, Fyfe DA. One-hundred pulmonary valve replacements in
children after relief of right ventricular outflow tract obstruction.
Ann Thorac Surg 2002;73:1801–7. Dr T. Ebels (Groningen, The Netherlands): I noticed that the shortest
[9] Therrien J, Siu SC, McLaughlin PR, Liu PP, Williams WG, Webb interval between the Fallot repair and the valve replacement was 4
GD. Pulmonary valve replacement in adults late after repair of months. Now, what, in the end, is your recommendation? When should
tetralogy of fallot: are we operating too late? J Am Coll Cardiol 2000; we replace the valve? Because that was one of the questions you wanted
36:1670–5. to answer.
[10] d’Udekem Y. Failure of right ventricular recovery of Fallot patients Dr Lim: We have 6 reoperations in this cohort. But in the case of a
after pulmonary valve replacement: delay of reoperation or surgical mechanical valve we used, 2 or 3 of the mechanical valve have thrombosis,

Downloaded from https://academic.oup.com/ejcts/article-abstract/25/5/728/456208 by guest on 06 September 2019

technique? J Am Coll Cardiol 2001;37:2008 –9. so we do not prefer this type and 1 patient with homograft graft with 14 mm
[11] Hanley FL. Editorial: management of the congenitally abnormal right of annulus failed due to the calcification of homograft.
ventricular outflow tract. What is the right approach? J Thorac Dr Ebels: My question was, when do you want to operate? Not what
Cardiovasc Surg 2000;119:1– 3. valve, but when?
[12] Hazekamp MG, Kurvers MMJ, Schoof PH, Vliegen HW, Mulder BM, Dr Lim: The focus of our study is the timing. So we generally
Roest AAW, Ottenkamp J, Dion RAE. Pulmonary valve insertion late recommend that if the patient has symptoms, we recommend immediate
after repair of Fallot’s tetralogy. Eur J Cardiothorac Surg 2001;19: replacement.
667–70. But more importantly, in asymptomatic patients, we recommend they
[13] Tulevski II, Hirsch A, Dodge-Khatami A, Stoker J, van der Wall EE, come in to our outpatient department and we thoroughly check the chest X-
Mulder BJ. Effect of pulmonary valve regurgitation on right ray or echocardiography. And if any sign of like tricuspid regurgitation or
ventricular function in patients with chronic right ventricular pressure progressive increase in RV dilatation, we generally recommend operation.
overload. Am J Cardiol 2003;92:113–6. In my opinion, the proper timing is about no longer than 10 years after the
[14] Vliegen HW, van Straten A, de Roos A, Roest AAW, Schoof PH, initial repair.
Zwinderman AH, Ottenkamp J, van der Wall EE, Hazekamp MG. Dr G. Sarris (Athens, Greece): I have two questions. One is similar to
Magnetic resonance imaging to access the hemodynamic effects of Dr Ebels’ question. I think most people would agree that the symptomatic
pulmonary valve replacement in adults late after repair of tetralogy of
patient—and I notice 50% of your patients were symptomatic—should be
Fallot. Circulation 2002;106:1703–7.
offered pulmonary valve replacement.
[15] Goldberg SJ, Allen HD. Quantitative assessment by Doppler
In the asymptomatic patient group, your criteria were not well
echocardiography of pulmonary or aortic regurgitation. Am J Cardiol
delineated. You just alluded to serial echocardiographic findings that may
1985;56:131–5.
be useful. Have you used any more detailed studies of right ventricular
[16] Theodoro DA, Danielson GK, Porter CJ, Warnes CA. Right-sided
volume or function serially, such as CMR, or any provocative tests, such as
maze procedure for right atrial arrhythmias in congenital heart
exercise testing, trying to identify a subpopulation of otherwise asympto-
disease. Ann Thorac Surg 1998;65:149–54.
matic patients with significant PI and select them out for pulmonary valve
[17] Therrien J, Siu SC, Harris L, Dore A, Niwa K, Janousek J, Williams
WG, Webb G, Gatzoulis MA. Impact of pulmonary valve replacement replacement?
on arrhythmia propensity late after repair of tetralogy of Fallot. And the second question is, since prevention may be even more
Circulation 2001;103:2489–94. important, whether your technique of tetralogy repair initially is a
[18] Eyskens B, Reybrouck T, Bogaert J, Dymarkowsky S, Daenen W, transventricular or a transatrial technique, since the latter may preserve
Dumoulin M, Gewillig M. Homograft insertion for pulmonary pulmonary valve function better.
regurgitation after repair of tetralogy of Fallot improves cardior- Dr Lim: I think that the objective criteria is not known yet. So we try to
espiratory exercise performance. Am J Cardiol 2000;85:221–5. find the objective criteria and get objective studies like MRI study. But the
[19] Wessel HU, Paul MH. Exercise studies in tetralogy of Fallot: a review. study result is not ending yet, because the time period is very short.
Pediatr Cardiol 1999;20:39 –47. But I think usually a patient without symptoms restricts his activity,
[20] Conte S, Jashari R, Eyskens B, Gewillig M, Dumoulin M, Daenen W. so he doesn’t want to play together with his friends and the objective
Homograft valve insertion for pulmonary regurgitation late after symptom is maybe masked. So in outpatient department we carefully
valveless repair of right ventricular outflow tract obstruction. Eur J examined through the questionnaire that, Are you okay? But in
Cardiothorac Surg 1999;15:143–9. asymptomatic patient, he generally restricts his activity. So the functional
[21] Ilbawi MN, Idriss FS, DeLeon SY, Muster AJ, Gidding SS, Berry TE, class is underestimated.
Paul MH. Factors that exaggerate the deleterious effects of pulmonary So we generally recommend the PVR in asymptomatic patient is rather
insufficiency on the right ventricle after tetralogy repair. Surgical subjective, and we don’t know how to correctly indicate the indication of
implications. J Thorac Cardiovasc Surg 1987;93:36–44. PVR right now.