ORIGINAL ARTICLE – CONGENITAL
Interactive CardioVascular and Thoracic Surgery 23 (2016) 240–246
doi:10.1093/icvts/ivw102 Advance Access publication 1 May 2016
Cite this article as: Baruteau A-E, Vergnat M, Kalfa D, Delpey J-G, Ly M, Capderou A et al. Long-term outcomes of the arterial switch operation for transposition of
the great arteries and ventricular septal defect and/or aortic arch obstruction. Interact CardioVasc Thorac Surg 2016;23:240–6.
Long-term outcomes of the arterial switch operation for
transposition of the great arteries and ventricular
septal defect and/or aortic arch obstruction
Alban-Elouen Baruteaua,b,*, Mathieu Vergnata, David Kalfab, Jean-Guillaume Delpeya, Mohamed Lya,
André Capderoua, Virginie Lamberta and Emre Bellia
a
b
Department of Pediatric Cardiac Surgery, Marie-Lannelongue Hospital, Paris-Sud University, Paris, France
Department of Pediatric Cardiac Surgery, New York Presbyterian Hospital, Columbia University, New York, NY, USA
* Corresponding author. Department of Pediatric Cardiac Surgery, Marie-Lannelongue Hospital, 133 Avenue de la Résistance, 92350 Le-Plessis-Robinson, France.
Tel: +33-1-40942800; fax: +33-1-40948800; e-mail: [email protected] (A.-E. Baruteau).
Received 16 September 2015; received in revised form 11 December 2015; accepted 8 January 2016
Abstract
OBJECTIVES: Long-term outcomes after the arterial switch operation (ASO) for complex transposition of the great arteries (TGA) should be
clarified.
METHODS: A retrospective study was conducted in patients operated on between 1982 and 1998. Overall 220 postoperative survivors,
79.1% with a ventricular septal defect, 13.2% with multiple ventricular septal defects, and 29.1% with aortic arch obstruction, were followed
for 17 years (0–28 years).
RESULTS: The conditional survival rate was 96.7% [95% confidence interval (CI): 94.4–99.1] at 25 years. Late sudden death occurred in 2
asymptomatic patients. The cumulative incidence rate of death or reinterventions was 3.8% (95% CI: 2.9–4.8) at 25 years, with age at ASO
<10 days and aortic regurgitation at discharge identified as independent risk factors. The cumulative incidence rate of neoaortic regurgitation was 41.6% (95% CI: 20.5–62.8) at 25 years with an aorto-pulmonary diameter mismatch at the time of the ASO, age at ASO <10 days
and aortic regurgitation at discharge identified as independent risk factors. At the last follow-up, 53 patients (24.1%) had neoaortic root
dilatation with an aortic sinus z-score ≥3 and 6 of them had a Bentall operation at a median delay of 14.1 years since the ASO. The only independent factors for neoaortic root dilatation were male sex and an aorto-pulmonary diameter mismatch at the time of the ASO.
CONCLUSIONS: Despite a continual rate of reinterventions, long-term survival and cardiovascular outcome are excellent after ASO for
complex TGA. Dilatation of the neoaortic root and neoaortic regurgitation may be observed with time and 2 late sudden deaths occurred,
justifying a close follow-up in all patients.
Keywords: Aortic root • Aortic valve • Replacement • Congenital heart disease • Great vessel anomalies • Transposition • Arterial switch •
Outcomes
INTRODUCTION
METHODS
The arterial switch operation (ASO) is the procedure of choice for
treatment of most forms of transposition of the great arteries (TGA)
[1]. Recent studies reported convincing results of the ASO for TGA
with an intact ventricular septum in the long term [2–4]. However,
the ASO for some complex TGA still remains a challenging procedure
whose long-term outcomes remain poorly defined [5]. Concerns
have arisen about the post-ASO risk of neoaortic root dilatation,
neoaortic regurgitation, coronary complications or pulmonary stenosis [1, 6–9]. Our group previously published short-term results of the
ASO for a complex TGA [10] and long-term outcomes after the ASO
for a simple TGA [6] and for Taussig-Bing anomaly [11]. The present
study aims to report the long-term outcomes after anatomical repair
of a complex TGA by the ASO.
We conducted a retrospective study at the Marie-Lannelongue
Hospital. All patients who underwent an ASO for complex TGA
from 1982 to 1998 were included in the database to allow a followup of at least 15 years. Complex TGA was defined as a TGA with
(i) unrestrictive ventricular septal defect (VSD) and/or (ii) aortic arch
obstruction and/or (iii) left ventricular outflow tract obstruction.
TGA patients with an isolated perimembranous VSD were not
included in the study, not being considered complex cases. Patients
with the Taussig-Bing anomaly, defined using the Van Praagh criteria [12], were not included, as this congenital heart malformation
is a distinct anatomical entity whose post-ASO long-term outcomes
have already been described [11]. Aorto (Ao)-pulmonary (PA) diameter mismatch was defined as a PA/Ao size ≥1.5 [7]. The aortic
© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
A.-E. Baruteau et al. / Interactive CardioVascular and Thoracic Surgery
from the identified variables with P < 0.05. The odds ratio (OR)
and 95% CI were reported for significant multivariable risk factors.
P < 0.05 was considered statistically significant.
RESULTS
Long-term cardiovascular outcomes were described from a singleinstitution series of 220 consecutive patients with complex TGA
who underwent an ASO for anatomical repair more than 15 years
before this evaluation.
Baseline characteristics
During the study period, 308 ASOs were performed for complex
TGA at our institution. Forty-two of them (13.6%) died during the
first postoperative month or before hospital discharge, and 46
(14.9%) were overseas patients with an unachievable follow-up,
leaving 220 patients in the analysis. Anatomical characteristics are
given in Table 1. One-stage ASO was performed in 190 (85.6%) of
them [median age: 19 days (range: 1–305 days), median weight:
3.4 kg (range: 2.0–7.0 kg)], whereas 32 (14.5%) patients had a
2-stage ASO [median age: 19 days (range: 1–305 days), median
weight: 4.6 kg (range: 2.9–30.0 kg)]. In the latter patients’ group,
pre-ASO surgery was a pulmonary artery banding in 28 (12.6%),
an aortic arch repair in 17 (7.7%) and a systemic-to-pulmonary
artery shunt in 2 (0.9%). ASO was performed after a median delay
of 3.3 months (range 7 days to 10.7 years) after the initial surgery.
Late mortality
During a median follow-up of 17 years (range: 0–28 years), late
deaths occurred in 7 of 220 (3.2%, 95% CI: 1.0–6.0) postoperative
survivors, yielding a conditional survival rate of 97.7% (95% CI:
Table 1:
Anatomical characteristics (n = 220)
Statistical analysis
R version 3.1.1 statistical software was used for data analysis.
Categorical variables are expressed as frequency ( percentage),
and continuous variables are expressed as median (min–max).
Percentages are presented with 95% confidence intervals (CIs).
Studied end-points were late mortality, reoperations, neoaortic
regurgitation and neoaortic root dilatation. Survival was estimated
by means of the Kaplan–Meier method. The hazard rate was
obtained by the ratio of the number of events during the interval
of time to the number of subjects at risk for this interval. Risk
factors for time-related outcomes (mortality or reoperation, and
neoaortic regurgitation) were tested using Cox regression analysis.
Univariate analysis identified variables with P < 0.05 that were
then entered in a stepwise fashion into a multivariate Cox proportional hazards regression model to determine the independent
predictors of outcomes. The hazard ratio (HR) and 95% CI were
reported for significant multivariable risk factors. The neoaortic
root dilatation was only determined at the end of follow-up and
the risk factors were studied using univariate analysis by the
Mann–Whitney U-test for continuous variables and the χ 2 test for
categorical variables, and a stepwise multivariate logistic regression
Characteristics
n (%)
Isolated ventricular septal defect
Multiple ventricular septal defects
Aortic arch obstruction
Hypoplastic arch ± coarctation
Interrupted aortic arch
Isolated coarctation
Aorto-pulmonary diameter mismatch
Side-by-side vessels
Coronary artery pattern
Type 1
Type 2
Type 3
Type 4
Intramural coronary course
Other cardiac anomalies
Situs inversus
Dextrocardia
Supero-inferior ventricles
Straddling/overriding atrioventricular valve
Cleft mitral valve
Left ventricular outflow tract obstruction
Right ventricular outflow tract obstruction
174 (79.1)
29 (13.2)
64 (29.1)
11 (5.0)
4 (1.8)
49 (22.3)
62 (28.2)
20 (9.1)
159 (72.3)
3 (1.4)
57 (25.9)
1 (0.4)
2 (0.9)
2 (0.9)
3 (1.4)
4 (1.8)
9 (4.1)
2 (0.9)
3 (1.4)
6 (2.7)
ORIGINAL ARTICLE
regurgitation (AR) quantification was evaluated by colour Doppler
flow mapping and graded as none, trivial, mild, moderate or severe
as previously published [7]. Coronary artery patterns were described
according to the classification published by our institution, which
takes into account the origin and initial course of the coronary arteries [13]. The surgical technique for ASO was standard and has been
previously described [5, 10, 13], and all patients had the LeCompte
manoeuvre. Late mortality included all deaths that occurred after the
first postoperative month or after hospital discharge. Fifteen patients
(6.8%, 95% CI: 4.0–11.0) were considered to be lost to follow-up, as
their last visit was more than 5 years ago. We asked their birth town
council if they are dead or alive, so that late mortality can be assessed
in the whole studied population, including lost to follow-up patients.
The hospital files of all patients were reviewed for details of preoperative assessment, operative records, major postoperative events and
hospital course. Follow-up data were collected with special attention
to clinical status, echocardiographic data, reinterventions and mortality. Since 1982, it was prospectively determined that all survivors
have an annual examination by the referring cardiologist including
clinical assessment, 12-lead electrocardiogram and echocardiogram.
An exercise test or myocardial scintigraphy was done if deemed
necessary. From their 5th year, all patients had every 5 years a catheterization with coronary arteriography until the year 2000 and a
coronary computed tomography (CT) scan after 2000. Coronary
stenosis was defined as ≥50% reduction of vessel diameter on coronary angiography [6]. All data were regularly transmitted to our centre.
Long-term outcomes were assessed by telephone interviews of all
patients and all referring cardiologists to obtain follow-up missing
data and information on cardiac status, data of last visit and any
delayed complication. Neoaortic root dimension was assessed at the
last visit by the measurement of the sinus of Valsalva diameter, which
was gathered for all patients either by echocardiography, cardiac CT
scan or cardiac magnetic resonance imaging, and compared with
adjusted normal values [14]. Post-ASO patients with AR, aortic root
dilatation (ARD), coronary artery stenosis or pulmonary stenosis were
considered for reintervention according to the ESC guidelines [15].
Our institutional review board approved the study and all patients
gave their informed consent for inclusion.
241
10.6
11.6
0.17
2.5
5.5
ASO: arterial switch operation; TGA-VSD: transposition of the great arteries with ventricular septal defect.
1993
1993
TGA-VSD and coarctation
TGA-VSD
7
19
26
30
21
1997
1987
1994
2.5
2.9
Type 1
Type 1
Aortic valve replacement (5.6 years)
No
Myocardial infarction
Pulmonary hypertension
Mechanic aortic valve
thrombosis with
embolic stroke
Sudden death
Sudden death
No
No
Aortic valve repair (4.4 years)
Aortic valve replacement (5.4 years)
Type 1
Type 3
Type 1
Pulmonary vein stenosis
Myocardial infarction
Pulmonary stenosis relief (7 months)
No
7
36
1995
1988
TGA-VSD and coarctation
TGA-VSD with supero-inferior ventricles
and overriding of the tricuspid valve
TGA-VSD, multiple VSDs and coarctation
TGA-VSD and coarctation
TGA-VSD
4.1
3.6
3.4
Operative
weight (kg)
Operative
age (days)
Year of
ASO
At the last follow-up or just before reoperation for AR, 28 patients
(12.7%, 95% CI: 9.0–18.0) had at least moderate AR. The cumulative
Cardiac anatomy
Neoaortic regurgitation
Table 2: Late mortality: patients’ characteristics (n = 7)
After hospital discharge 106 surgical or transcatheter reinterventions
were required in 65 postoperative survivors (29.5%, 95% CI: 24.0–
36.0), with a median time of 3.0 months (range: 1 month to 17.6
years) to the first reintervention (Table 3). Six of them experienced
late death. The follow-up was not different between patients without
reintervention (Group 1: n = 154 patients, median follow-up: 204
months) or those who had 1 reintervention (Group 2: n = 38 patients,
median follow-up: 216 months), 2 reinterventions (Group 3: n = 12
patients, median follow-up: 204 months) or 3 reinterventions (Group
4: 3 reinterventions, n = 7 patients, median follow-up: 204 months)
(Kruskal–Wallis test: P = 0.89). Reoperations were mainly represented
by aortic valve repair or replacement (n = 15, 6 out of them had a
Bentall procedure), pulmonary arterioplasty (n = 15) and recoarctation repair (n = 15). Of the 15 patients who required recoarctation
repair, 6 had undergone unsuccessful aortic arch balloon angioplasty. The cumulative incidence rate of death or reinterventions was
2.3% (95% CI: 1.6–2.9), 3.5% (95% CI: 2.6–4.3) and 3.8% (95% CI: 2.9–
4.8), respectively, 5, 15 and 25 years after the ASO (Fig. 2A).
Univariate and multivariate risk analysis of death or reoperation is
presented in Table 4. Male sex, VSD, aortic arch obstruction, age at
ASO <10 days, bypass time >180 min, previous aortic arch repair and
AR at discharge were significantly associated with an increased risk of
death or reoperation. The independent risk factors were an age at
ASO <10 days (HR = 1.92, 95% CI: 1.12–3.32, P = 0.018) and AR at discharge (HR = 4.58, 95% CI: 2.25–9.31, P < 0.001).
Coronary
pattern
Reinterventions
Type 1
Type 3
Reoperation
95.7–99.7) at 10 years and of 96.7% (95% CI: 94.4–99.1) at 20 and
25 years (Fig. 1). Late death occurred at a median delay of 2.5
years (range: 1.3–11.6 years) since the ASO. Patients’ characteristics are summarized in Table 2. Late sudden death occurred in 2
asymptomatic patients with normal coronary pattern and uneventful follow-up, respectively, 10.6 and 11.6 years after the ASO.
2.7
3.6
Cause of death
Figure 1: Post-ASO survival distribution function for the 220 patients with
complex TGA solid line: Kaplan–Meier estimate by dividing time in months;
dashed lines: 95% CI. ASO: arterial switch operation; TGA: transposition of the
great arteries.
0.64
0.11
A.-E. Baruteau et al. / Interactive CardioVascular and Thoracic Surgery
Time to
death (years)
242
A.-E. Baruteau et al. / Interactive CardioVascular and Thoracic Surgery
Table 3: Reinterventions after hospital discharge in 65
postoperative survivors
Reintervention
n (%)
No. of
procedures
Any reintervention
Transcatheter reintervention
Pulmonary artery balloon angioplasty
Aortic arch balloon angioplasty
Surgical reintervention
Aortic valve repair or replacement
Pulmonary arterioplasty
Recoarctation repair
Residual ventricular septal defect closure
Epicardic permanent pacemaker
implantation
Bentall procedure
Mediastinitis
Coronary artery bypass grafting
Pericardial effusion drainage
Hemidiaphragm plication
Mitral valvuloplasty
Relief of bronchus compression
Atrial septal defect closure
Superior vena cava thrombosis
65
11 (16.9)
106
12 (11.3)
5 (4.7)
7 (6.6)
94 (88.7)
15 (14.1)
15 (14.1)
15 (14.1)
8 (7.6)
8 (7.6)
65 (100)
6 (5.7)
6 (5.7)
4 (3.8)
4 (3.8)
4 (3.8)
3 (2.8)
3 (2.8)
2 (1.9)
1 (0.9)
Neoaortic root dilatation
At the last visit, after a median follow-up of 17 years (range: 0–28
years), 53 patients (24.1%, 95% CI: 19.0–30.0) had neoaortic root dilatation, defined as an aortic sinus z-score ≥3. However, severe ARD, with
an aortic sinus z-score ≥5, was observed only in 19 (8.6%) patients
[median z-score = 6.32 (range: 5.01–8.32), median diameter = 45 mm
(range: 40–72)], 6 of whom had undergone a Bentall operation with a
median time of 14.1 years (range: 7.4–20 years) to the Bentall [median
z-score = 8.2 (range: 7.95–8.32), median diameter = 51 mm (range: 42–
72)]. Male sex, aortic arch obstruction, an aorto-pulmonary diameter
mismatch and previous aortic arch repair were associated with ARD
(Table 6). The only multivariate predictors were male sex (OR = 2.35,
95% CI: 1.14–4.85, P = 0.021) and an aorto-pulmonary diameter mismatch at the time of the ASO (OR = 2.13, 95% CI: 1.10–4.16, P = 0.026).
No acute complication of ARD such as aortic rupture or dissection was
observed.
Cardiac status in the long term
At the last visit, after a 17-year median follow-up (range: 0–28 years),
211 of the 213 remaining patients (99.1%) were in New York Heart
Association functional class I, the remaining 2 being in Class II.
Echocardiogram demonstrated normal biventricular systolic function
in 210 (98.6%) patients and no patient had an ejection fraction less
than 40%. Sinus rhythm was maintained in 201 (94.4%) patients.
Nine patients were paced and 3 had experienced atrial flutter (2
medically controlled, 1 with successful ablation). Four patients (2.0%)
had undergone a coronary artery bypass grafting (1 for left main coronary artery occlusion, 1 for left main coronary artery stenosis and 2
for right ostial stenosis) at a median of 9.7 years (range: 8.9–14.9
years) from the ASO. All other patients had normal coronary arteries
or <50% coronary stenosis and normal exercise test or myocardial
scintigraphy. Twenty-five patients (11.7%) received a β-blocker agent,
60 (28.2%) an angiotensin enzyme inhibitor and 128 (60.1%) had no
treatment.
Figure 2: Actuarial survival. (A) Actuarial survival free from death or reoperation; (B) Actuarial survival free from neoaortic regurgitation.
ORIGINAL ARTICLE
incidence rate of at least moderate AR was 0.5% (95% CI: 0.0–1.4),
5.6% (95% CI: 2.3–8.9) and 41.6% (95% CI: 20.5–62.8) respectively 5,
15 and 25 years after the ASO (Fig. 2B). Univariate factors associated
with AR were an aortic arch obstruction, an aorto-pulmonary diameter mismatch, an age at ASO <10 days, circulatory arrest and AR at
discharge (Table 5). On multivariate analysis, an aorto-pulmonary
diameter mismatch (HR = 0.81, 95% CI: 0.41–2.0, P = 0.046), age at
ASO <10 days (HR = 5.11, 95% CI: 1.80–14.53, P = 0.002) and AR at
discharge (HR = 26.73, 95% CI: 9.07–78.79, P < 0.001) were identified as independent risk factors for AR.
243
A.-E. Baruteau et al. / Interactive CardioVascular and Thoracic Surgery
244
Table 4:
Risk analysis of death or reoperation (n = 220)
Factor
Preoperative factors
Male
Rashkind manoeuvre
Anatomic factors
Isolated or multiple VSDs
Multiple VSDs
Aortic arch obstruction
Coronary anomaly
Aorto-pulmonary diameter mismatch
Side-by-side vessels
Operative factors
Age at ASO <10 days
Operative weight <3 kg
Bypass time >180 min
Cross-clamp >90 min
Circulatory arrest
2-stage operation
Pulmonary artery banding (2 stage)
Aortic arch repair (2 stage)
Postoperative factors
Aortic regurgitation at discharge
Death or reoperation
(n = 66/220)
Univariate,
P-value
Multivariate,
P-value (HR, 95% CI)
50/142
22/78
0.033
0.873
57/203
7/29
33/64
22/61
23/62
5/20
0.020
0.568
<0.001
0.139
0.189
0.777
18/38
13/49
31/75
35/101
14/32
13/32
9/28
10/17
0.013
0.526
0.020
0.205
0.120
0.072
0.616
0.001
0.018 (HR = 1.92, CI: 1.12–3.32)
9/11
<0.001
<0.001 (HR = 4.58, CI: 2.25–9.31)
ASO: arterial switch operation; VSD: ventricular septal defect; OR: hazard ratio; CI: confidence interval.
Table 5: Risk analysis of neoaortic regurgitation (n = 220)
Factor
Preoperative factor
Male
Anatomic factors
Isolated or multiple VSDs
Multiple VSDs
Aortic arch obstruction
Coronary anomaly
Aorto-pulmonary diameter
mismatch
Side-by-side vessels
Operative factors
Age at ASO <10 days
Operative weight <3 kg
Bypass time >180 min
Cross-clamp >90 min
Circulatory arrest
2-stage operation
Pulmonary artery banding (2 stage)
Aortic arch repair (2 stage)
Postoperative factors
Aortic regurgitation at discharge
Neoaortic regurgitation
(n = 28/220)
Univariate,
P-value
21/142
0.264
26/203
0/29
12/64
7/61
16/62
0.928
0.997
0.035
0.605
0.013
2/20
0.794
6/38
3/49
15/75
13/101
6/32
5/32
4/28
5/17
0.025
0.292
0.130
0.200
0.026
0.835
0.828
0.153
7/11
<0.001
Multivariate,
P-value (HR, 95% CI)
0.046 (HR = 2.25, CI: 1.02–4.99)
0.002 (HR = 5.11, CI: 1.80–14.53)
<0.001 (HR = 26.73, CI: 9.07–78.79)
ASO: arterial switch operation; VSD: ventricular septal defect; HR: hazard ratio; CI: confidence interval.
COMMENT
Late mortality
To our knowledge, the present study describes the largest
reported cohort of consecutive patients with complex TGA to
undergo anatomical repair with the ASO. Our long-term follow-up
data from a 32-year experience make our results highly interesting
for addressing late mortality and the reoperation rate, as well as
late aortic valve function and neoaortic root dimensions.
Late mortality in our cohort was encouraging with a 96.7% survival
rate at 25 years. As previously reported, late deaths mainly occurred in the first postoperative months, some of them being attributable to myocardial ischaemic complications [1, 6, 11]. We
found that AR at discharge and age at ASO less than 10 days predispose the patients to death or reoperation. It has been shown
A.-E. Baruteau et al. / Interactive CardioVascular and Thoracic Surgery
245
Factor
Preoperative factor
Male
Anatomic factors
Isolated or multiple VSD repair
Multiple VSDs
Aortic arch obstruction
Coronary anomaly
Aorto-pulmonary diameter mismatch
Side-by-side vessels
Operative factors
Age at ASO <10 days
Operative weight <3 kg
Bypass time >180 min
Cross-clamp >90 min
Circulatory arrest
2-stage operation
Pulmonary artery banding (2 stage)
Aortic arch repair (2 stage)
Postoperative factors
Aortic regurgitation at discharge
Neoaortic root dilatation
(n = 53/220)
Univariate
P-value
Multivariate
P-value (OR, 95% CI)
41/142
0.025
0.021 (OR = 2.35, CI: 1.14–4.85)
50/203
6/29
22/64
15/61
21/62
4/20
0.518
0.646
0.022
0.915
0.034
0.654
7/38
8/49
22/75
25/101
12/32
10/32
8/28
8/17
0.369
0.149
0.201
0.860
0.055
0.305
0.553
0.021
3/11
0.800
0.026 (OR = 2.14, CI: 1.10–4.16)
ASO: arterial switch operation; VSD: ventricular septal defect; OR: odds ratio; CI: confidence interval.
that mild AR at discharge was a significant risk factor for later development of significant AR [15]. One can wonder whether intervention was too late in these patients, as 3 of our 7 late mortalities
had aortic valve intervention late after ASO. Age at ASO less than
10 days was not associated to aortic arch obstruction, prostaglandin infusion, coronary anomaly or low operative weight. We have
no clear explanation for this assertion/statement, unless incomplete organ maturity implied a delayed vascular tissue maturation
in neonates aged less than 10 days, even if born at term [16]. This
finding suggests that ASO should be delayed after the tenth day in
neonates with TGA and a VSD without aortic arch obstruction
if the VSD is large enough to maintain systemic left ventricular
pressure. We also recommend a closer follow-up in post-ASO
patients with mild AR at discharge and those who were operated
on before 10 days. But the major concerns are due to the 2
late sudden deaths in asymptomatic teenagers with normal left
ventricular systolic function, normal coronary anatomy and no
previously documented arrhythmias. Similar events have been
recently described with an incidence rate <0.1% per year [4].
These data shed light on the long-term risk of unexpected, presumed arrhythmic, sudden death in asymptomatic post-ASO
patients, whereas until now it was thought that excess mortality
concerned either symptomatic patients or those with residual
lesions [2]. Both a close rhythmological follow-up and a risk stratification score in this growing patient population may be useful in
the future.
Aortic regurgitation and aortic root dilatation
The cumulative incidence rate of AR was 5.6 and 41.6%, respectively, at 15 and 25 years after the ASO. This is not explained by an
irregular follow-up, as our patients had an annual examination. Lo
Rito et al. reported a 20% incidence of significant AR at 20 years of
follow-up. For patients discharged with mild AR, freedom from
significant AR was 50% at 20 years [15]. Although aortic functional
status seems to be relatively stable in the first 15 years, progression
of neoaortic valve regurgitation occurs between 15 and 25 years
of follow-up. This worrying finding warrants careful monitoring of
these patients in the long term. In our population, AR is one of the
leading causes of reoperation. At the last follow-up, 24.1% of our
patients had ARD. However, reintervention rates on the valve or
root remain low [9, 17–19]. Schwartz et al. reported a 51% probability of freedom from ARD at 10 years [8]. In another report, the
root diameter z-score increased at an average rate of 0.08 per
year over time after the ASO [9]. High-volume institutions have
observed that no further increase of the Valsalva sinus occurs after
some years of follow-up [1, 9]. This finding is debatable since
another group recently described persistence of neoaortic linear
growth in early adulthood [17]. In our experience, severe ARD is
rare and no neoaortic dissection or rupture was observed. Only six
Bentall operations were performed over time. Our indications for
intervention upon ARD were: (i) aortic sinus diameter of greater
than 5.5 cm; or (ii) ARD growth rate of more than 0.5 cm/year in
an aorta that is less than 5.5 cm in diameter; or (iii) patients who
had an indication for aortic valve repair/replacement and who
had an ARD of greater than 4.5 cm [18, 19]. Previous studies suggested that ARD has a relationship to AR [8, 9]. In our series, the 6
patients who underwent a Bentall operation had both severe AR
and severe ARD. However, the only multivariate predictors for
ARD were male sex and an aorto-pulmonary diameter mismatch
at the time of the ASO. Although the mechanism of the association of gender with ARD is unclear, our results suggest that a hormonal factor may play a role. Indeed, recent data have emerged
regarding the evidence of gender differences in cardiovascular
function and remodelling, suggesting a different impact of sex
hormones on cardiovascular physiology [20]. These new insights,
while still incompletely understood, may be a possible explanation
of male sex being a risk factor of ARD. On the contrary, some risk
factors for AR or ARD identified in older studies with smaller
populations, such as previous pulmonary artery banding, did not
reach statistical significance in our study.
ORIGINAL ARTICLE
Table 6: Risk analysis of neoaortic root dilatation, z-score ≥3 (n = 220)
246
A.-E. Baruteau et al. / Interactive CardioVascular and Thoracic Surgery
Limitations
The retrospective nature of our study implies inherent limitations
due to observational investigations. Prevalence of coronary artery
stenosis may be underestimated by the lack of routine invasive
testing. Details about the progression of ARD over time were unavailable and would be beneficial to this study.
Conflict of interest: none declared.
REFERENCES
[1] Villafane J, Lantin-Hermoso MR, Bhatt AB, Tweddell JS, Geva T, Nathan M
et al. D-transposition of the great arteries: the current era of the arterial
switch operation. J Am Coll Cardiol 2014;64:498–511.
[2] Kempny A, Wustmann K, Borgia F, Dimopoulos K, Uebing A, Li W et al.
Outcome in adult patients after arterial switch operation for transposition
of the great arteries. Int J Cardiol 2013;167:2588–93.
[3] Fricke TA, d’Udekem Y, Richardson M, Thuys C, Dronavalli M, Ramsay JM
et al. Outcomes of the arterial switch operation for transposition of the
great arteries: 25 years of experience. Ann Thorac Surg 2012;94:139–45.
[4] Khairy P, Clair M, Fernandes SM, Blume ED, Powell AJ, Newburger JW
et al. Cardiovascular outcomes after the arterial switch operation for
D-transposition of the great arteries. Circulation 2013;127:331–9.
[5] Lacour-Gayet F. Arterial switch operation with ventricular septal defect
repair and aortic arch reconstruction. Semin Thorac Cardiovasc Surg 2007;
19:245–8.
[6] Legendre A, Losay J, Touchot-Kone A, Serraf A, Belli E, Piot JD et al.
Coronary events after arterial switch operation for transposition of the
great arteries. Circulation 2003;108(S1):II186–90.
[7] Losay J, Touchot A, Capderou A, Piot JD, Belli E, Planché C et al. Aortic
valve regurgitation after arterial switch operation for transposition of the
great arteries: incidence, risk factors, and outcome. J Am Coll Cardiol
2006;47:2057–62.
[8] Schwartz ML, Gauvreau K, del Nido P, Mayer JE, Colan SD. Long-term predictors of aortic root dilation and aortic regurgitation after arterial switch
operation. Circulation 2004;110:II128–32.
[9] Co-Vu JG, Ginde S, Bartz PJ, Frommelt PC, Tweddell JS, Earing MG.
Long-term outcomes of the neoaorta after arterial switch operation for
transposition of the great arteries. Ann Thorac Surg 2013;95:1654–9.
[10] Mohammadi S, Serraf A, Belli E, Aupecle B, Capderou A, Lacour-Gayet F
et al. Left-sided lesions after anatomic repair of transposition of the great
arteries, ventricular septal defect, and coarctation: surgical factors.
J Thorac Cardiovasc Surg 2004;128:44–52.
[11] Vergnat M, Baruteau AE, Houyel L, Ly M, Roussin R, Capderou A et al. Late
outcomes after arterial switch operation for Taussig-Bing anomaly.
J Thorac Cardiovasc Surg 2015;149:1124–30.
[12] Van Praagh R. What is the Taussig-Bing malformation? Circulation 1968;
38:445–9.
[13] Serraf A, Lacour-Gayet F, Bruniaux J, Touchot A, Losay J, Comas J et al.
Anatomic correction of transposition of the great arteries in neonates.
J Am Coll Cardiol 1993;22:193–200.
[14] Nistri S, Sorbo MD, Marin M, Palisi M, Scognamiglio R, Thiene G. Aortic
root dilatation in young men with normally functioning bicuspid aortic
valves. Heart 1999;82:19–22.
[15] Lo Rito M, Fittipaldi M, Haththotuwa R, Jones TJ, Khan N, Clift P et al.
Long-term fate of the aortic valve after an arterial switch operation.
J ThoracCardiovasc Surg 2015;149:1089–94.
[16] Pees C, Laufer G, Michel-Behnke I. Similarities and differences of the
aortic root after arterial switch and Ross operation in children. Am J
Cardiol 2013;111:125–30.
[17] Van der Bom T, van der Palen RL, Bouma BJ, van Veldhuisen SL, Vliegen HW,
Konings TC et al. Persistent neo-aortic growth during adulthood in patients
after an arterial switch operation. Heart 2014;100:1360–5.
[18] Hiratzka LF, Bakris GL, Beckman JA, Bersin RM, Carr VF, Casey DE Jr et al.
2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM guidelines for
the diagnosis and management of patients with thoracic aortic disease.
Circulation 2010;121:e266–369.
[19] Cameron D. Surgery for congenital diseases of the aorta. J Thorac
Cardiovasc Surg 2015;149:S14–7.
[20] Masjedi S, Ferdous Z. Understanding the role of sex in heart valve and
major vascular diseases. Cardiovasc Eng Technol 2015;6:209–19.