(Circulation. 1995;92:272-278.)
© 1995 American Heart Association, Inc.
Articles |
From the Department of Cardiology and Victorian Paediatric Cardiac Surgical Unit, Royal Children's Hospital, Melbourne, Australia.
Correspondence to Dr James L. Wilkinson, Department of Cardiology, Royal Children's Hospital, Melbourne, Flemington Rd, Parkville, Victoria 3052, Australia.
| Abstract |
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Methods and Results We retrospectively reviewed the intermediate-term follow-up of 28 patients, totaling 158 patient-years (median, 60 months), after intracardiac repair involving closure of a ventricular septal defect (VSD) with or without additional surgery. Seven patients had VSD closure alone, 5 had VSD repair with pulmonary stenosis relief, and 16 had VSD closure with conduit insertion between left ventricle and main pulmonary artery. Hospital mortality was 4% (1 of 28 patients; 70% confidence limits, 0.07% to 12%) and the 1-, 5-, and 10-year actuarial survival probabilities were 89%, 83%, and 83%, respectively. Twenty-one of 24 long-term survivors were in New York Heart Association functional class I and 3 were in class II. Sixteen of 24 patients showed increasing tricuspid regurgitation (TR) of more than moderate degree, which occurred within 3 years after surgery in 7 patients. Twelve of 22 patients showed deterioration of RV pump function, mainly (9 of 12 patients) within 3 years postoperatively. The pulmonary to systemic flow ratio at the preoperative cardiac catheter study was significantly (P<.05) higher in patients who developed RV dysfunction (2.3±1.0, mean±SD) than in those with well-maintained RV function (1.4±0.6).
Conclusions Intermediate-term results of intracardiac repair for AV and VA discordance were satisfactory in terms of survival and clinical functional status; however, there is concern about systemic RV dysfunction with development of TR relatively early after the operation. Alternative surgical approaches such as anatomic correction or Fontan repair for cases unsuitable for biventricular repair may improve the long-term results, including ventricular and valvular function.
Key Words: regurgitation prognosis transposition of great vessels myocardial contraction surgery
| Introduction |
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There has been significant concern regarding long-term prognosis after intracardiac repair for patients with AV and VA discordance. Although there are several studies regarding cardiac function and hemodynamics in this condition, with or without surgical interventions,6 7 8 9 10 the long-term fate of the anatomic RV as the systemic ventricle remains controversial. In the present study, we present the intermediate-term outcome of repair of ventricular septal defect and associated intracardiac defects, especially longitudinal follow-up of the systemic RV and tricuspid valve function, to elucidate risk factors affecting long-term surgical results in this cardiac malformation.
| Methods |
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Cardiac Anatomy
All patients had various associated
cardiovascular anomalies, with ventricular
septal defect in 28 patients (100%), pulmonary
stenosis or atresia in 21 (75%), and tricuspid valve
abnormality in 11 (39%) (Table 1
). The segmental
cardiac arrangement of the 28 patients consisted of situs solitus with
AV and VA discordant connection (S,L,L) in 24 and situs inversus with
AV and VA discordant connection (I,D,D) in 4 patients.
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Assessment of Tricuspid Regurgitation
TR was assessed by color
flow mapping, Doppler study, and RV
angiography. It was graded qualitatively as no TR, mild TR, moderate
TR, or severe regurgitation.
Echocardiographically, TR was considered mild if the
regurgitant jet crossed less than one third of the left atrium from the
tricuspid orifice, moderate if it projected from one third to two
thirds of the depth of the left atrium, or severe if it reached beyond
two thirds of the left atrium with a significantly wide jet.
Angiographically, TR was assessed by use of the criteria for definition
of mitral regurgitation described by
Grossman11 and was graded as mild (1/4 and 2/4), moderate
(3/4), or severe (4/4).
Assessment of RV Pump Function
RV pump function was assessed
by
echocardiography, cineangiography, and radionuclide
angiography. It was evaluated qualitatively from
echocardiography and cineangiography and graded as
good contraction, mild depression, moderate depression, or severe
depression. Quantitative assessment of RV function was obtained from
gated equilibrium radionuclide angiographic study in 10 patients, and
RV pump function was also graded as good function (RV ejection fraction
>50%), mild depression (45% to 50%), moderate depression (35% to
45%) or severe depression (<35%). Grades of good contraction or mild
depression were considered adequate RV function in data
analysis.
Statistical Analysis
Preoperative and postoperative changes
of parameters
were tested by use of paired Student's t test.
Variables were compared within subgroups by the unpaired Student's
t test. Risk factor analysis of postoperative RV
dysfunction was performed by use of univariate
analysis and then by use of multivariate
analysis (EGRET) to variables with a value of
P<.10. Time-related survival and freedom from
reoperation were estimated using the Kaplan-Meier estimator. Values of
P<.05 were considered significant.
| Results |
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Before the intracardiac repair, 21 of 28 patients (75%) were symptomatic, of whom 13 were cyanosed, 5 were in congestive heart failure, and the remaining 3 had a combination of cyanosis and heart failure.
The mean systemic oxygen saturation at the cardiac catheter study before the intracardiac repair measured 89% (SD, 9.2%) and mean pulmonary to systemic flow ratio was 2.0 (SD, 1.0). Preoperative cardiothoracic ratio, hemoglobin concentration, and hematocrit (mean±SD) were 57±6%, 15.8±2.8 g/dL, and 49±9%, respectively; after repair, these clinical parameters were 54±5%, (P<.002), 11.5±0.7 g/dL (P<.001), and 35±2%, (P<.001), respectively.
Intracardiac Repair
All 28 patients underwent intracardiac
repair, with age at
operation ranging from 1 to 13 years (median, 4 years 9 months).
Surgical procedures are summarized in Table 2
.
Intracardiac repair consisted of closure of ventricular
septal defect alone in 7 patients, with relief of pulmonary
stenosis in 5 and with valved conduit insertion between left
ventricle and pulmonary artery in 16 patients. Procedures to
relieve pulmonary stenosis consisted of
subpulmonary resection in 4 patients, including excision of
aneurysmal or accessory endocardial tissue in 3 and
pulmonary valvotomy with infundibulectomy in 1 patient.
Conduits used to relieve left ventricular outflow
obstruction consisted of Hancock valved conduits in 12 patients, Tascon
valved conduits in 3, and an aortic homograft in 1 patient. Additional
surgical procedures during the intracardiac repair included closure of
atrial septal defect in 9 patients and reconstruction of
stenotic main pulmonary artery and/or branch
pulmonary artery in 7 patients. Two patients with massive
tricuspid valve regurgitation preoperatively due to
Ebstein's anomaly or dysplastic valve underwent tricuspid valve
replacement with a St Jude valve. Division of an accessory conduction
pathway was undertaken in another 2 patients with Wolff-Parkinson-White
syndrome and recurrent supraventricular
tachycardia resistant to drug therapy.
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All but 4 patients had insertion of a permanent pacemaker 3 to 22 days (median, 7.5 days) after the intracardiac repair or simultaneously with the correction in 1 patient. Of 24 patients with implanted pacemakers, 15 implants were prophylactic against the risk of late development of heart block and another 9 were placed for treatment of heart block before or after the repair (5 with congenital and 4 with surgical heart block).
Early Results
All but one patient survived intracardiac
repair (hospital
mortality, 4%; 70% confidence limits, 0.07% to 12%). That patient
died of massive gastrointestinal bleeding and septicemia 45 days after
the operation. There were three late deaths: one was due to acute
myocarditis 6 months after the operation; another occurred at 4 months
in a child who suffered severe brain damage secondary to postoperative
cardiorespiratory arrest; and the third occurred after reoperation to
place a pulmonary artery band, 26 months after the intracardiac
repair, to "retrain" the left ventricle for double switch
conversion. All three patients also had underlying RV dysfunction with
development of TR early after surgery.
Intermediate-Term Prognosis
Follow-up in 24 survivors ranged
from 3 to 148 months (median,
70 months). At the last follow-up, 21 patients were in NYHA
functional class I and 3 were in class II. The 3 patients in NYHA class
II were all long-term survivors (from 112 to 131 months) and were
mildly symptomatic, with easy fatigability and dyspnea on
exertion. Two of the 3 had recurrent atrial flutter and fibrillation.
The 1-month and 1-, 5-, and 10-year actuarial survival after
intracardiac repair was 100% (70% confidence limits, 94% to 100%),
89% (78% to 96%), 85% (70% to 94%), and 85% (53% to
99%), respectively (Fig 1
).
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Reoperation
Excluding pacemaker revisions, seven patients
underwent one or
more reoperations 3 to 126 months after the initial surgery (Table
2
).
Two patients underwent pulmonary artery banding to
"train" the left ventricle as preparation for double switch
conversion because of progressive TR with or without compromised RV
pump function during follow-up after intracardiac repair. One of
these patients, with severe TR and moderate RV dysfunction, died
shortly after the placement of a pulmonary artery band, and the
other patient had an ineffective band, despite subsequent tightening of
the band. The double switch conversion of Senning operation and
arterial switch was successfully performed for one patient
with severe RV dysfunction 3 months after the conventional intracardiac
repair and tricuspid valve replacement, without prior banding in this
case. The 1-month and 1-, 5-, and 10-year freedom from reoperation
rates after intracardiac repair were 100% (70% confidence limits,
93% to 100%), 96% (86% to 100%), 80% (62% to 92%), and 72%
(36% to 95%), respectively. (Fig 2
).
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Tricuspid Regurgitation
Echocardiographic and/or angiographic
assessment
of TR was available in 25 patients preoperatively, 21 early (<3 years)
postoperatively, and 16 intermediate-term (>3 years)
postoperatively. Preoperatively, TR of moderate or severe degree was
documented in 9 (7 moderate and 2 severe) of 25 patients (36%).
Patients with tricuspid valve abnormalities showed a high incidence of
significant TR (6 of 11 patients) compared with patients with normal
tricuspid valve (3 of 14 patients). In contrast, TR was demonstrated
early postoperatively in 12 of 19 patients (63%), excluding 2 patients
who had undergone tricuspid replacement (10 with moderate and 2 with
severe TR). Similarly, 10 of 14 patients (71%) had significant TR at
late follow-up, 8 (57%) with moderate TR, and 2 (14%) with severe
TR. Increases in both frequency and severity of TR were demonstrated in
the short-term after intracardiac repair, although neither was
significant statistically.
Fig 3
shows the longitudinal
change in TR in 24 patients
who were assessed serially. Of 24 patients, only 6 (25%), excluding 2
who had tricuspid valve replacement, had competent tricuspid valves
during postoperative follow-up. The remaining 16 patients (67%)
showed clearly worsening TR of moderate or severe degree. Of these 16
patients, 7 developed TR within 3 years. Three of 5 patients with early
deterioration (within 1 year) had late deaths. All patients with
abnormal tricuspid valves showed deterioration in tricuspid function,
except the 2 who underwent tricuspid valve replacement.
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RV Pump Function
Qualitative assessment of pump function of
the morphological RV
was available in 25 patients preoperatively, 19 early postoperatively
(<3 years after intracardiac repair), and 15 at intermediate term (>3
years after repair). Preoperatively, all 25 patients were evaluated as
having adequate RV pump function (good or mildly depressed). In
contrast, 9 of the 19 (47%) patients evaluated early postoperatively
developed RV dysfunction postoperatively (7 moderate and 2 severe)
(P<.01 versus before surgery). Similarly, of 14 patients
evaluated later postoperatively, 7 (50%) showed RV dysfunction (6
moderate and 1 severe) (P<.01 versus before surgery).
Fig
4
shows the longitudinal change in RV pump function
before and after intracardiac repair in 22 patients in whom serial
assessment was available. Of these 22 patients, only 10 (45%) retained
adequate RV pump function during postoperative follow-up, and the
remaining 12 (55%) showed deterioration. Of these 12 patients, 9
showed deterioration in the early postoperative phase (within 3 years).
Particularly, 4 patients with significant deterioration within 1 year
postoperatively showed poor prognosis, that is, 2 died and 1 required
reoperation (double switch conversion).
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Several factors were analyzed
to compare the 10 patients who
retained adequate RV function with the 12 patients who developed RV
dysfunction (Table 3
). There was no significant
difference in operative factors, including operative age, bypass time,
or aortic cross-clamp time. There was also no difference in
preoperative arterial oxygen saturation, hemoglobin
concentration, or cardiothoracic ratio. However, the pulmonary
to systemic flow ratio was significantly (P<.05) higher in
patients who developed RV dysfunction postoperatively (2.3±1.0) than
in those with adequate RV function (1.4±0.6). In addition, logistic
regression analysis on postoperative RV dysfunction was
performed for variables including atrial situs, absence of
pulmonary outflow obstruction, abnormal tricuspid valve,
previous palliation, preoperative TR, symptomatic heart
failure, pulmonary to systemic flow ratio >2:1,
arterial saturation <90%, hemoglobin concentration >16
g/dL, operative age >5 years, conduit repair, additional operative
procedure (excluding closure of atrial septal defect), long
follow-up period, age >15 years, and postoperative TR. Although no
variables were significant predictors of postoperative RV
dysfunction, high pulmonary to systemic flow ratio
(P=.079 and odds ratio=6.0) and postoperative TR
(P=.074, odds ratio=6.3) were relatively correlated to
the
postoperative RV dysfunction.
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| Discussion |
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Heart Block
Perioperative or late-developing complete
heart block is one of the characteristic problems in patients with AV
and VA discordance and is also considered one of the major reasons for
lower survival rates in patients with this
condition.1 2
It has been suggested that some postoperative patients have died
suddenly as a result of the sudden appearance of complete heart block
with ventricular asystole or fibrillation.17
Additionally, postoperative tricuspid valve
regurgitation may be associated with the development of
complete heart block.4 The prevalence of complete heart
block after operation has been reported as 15% to
30%.2 4 5 16 In our study,
perioperative
complete heart block was seen in 9 of 28 patients (32%). However, 5 of
these 9 patients had heart block before surgery. Although positive
knowledge of the location of the cardiac conduction system in AV and VA
discordance18 may help reduce the incidence of complete
heart block as a complication of repair,19 the risk of
late development of block will never be completely eliminated because
pathological change in the conduction system is an acquired progressive
condition.20 Our strategy for this difficult problem,
therefore, has been to implant permanent pacemakers
prophylactically in patients undergoing repair, even if
sinus rhythm is preserved. In the present series, no patient had
postoperative sudden death.
Tricuspid Regurgitation
TR is recognized as another risk
factor for poor long-term
prognosis in patients with AV and VA discordance.15 It is
well known that significant TR develops in many patients with this
anomaly both during the natural history and after
surgery,2 3 4 15 especially
in patients with coexisting
tricuspid valve abnormalities.2 In the present study,
60% of postoperative patients displayed significant TR during
intermediate-term follow-up, and all patients with abnormal
tricuspid valves showed deterioration of TR during postoperative
follow-up, except for two who had early valve replacement. Of
greater importance, this deterioration of TR was demonstrated during
the relatively early postoperative period. Westerman et
al4 reported that TR has been apparent immediately after
repair in patients without preoperative TR and speculated that it might
be associated with the development of complete heart block. However,
our study showed that deterioration of TR occurred even in patients
without complete heart block. All patients in the present series
underwent closure of the ventricular septal defect through
the right atrium or via a left ventricular incision for
conduit anastomosis. Therefore, operative damage to the tricuspid valve
is unlikely to be responsible for the deteriorating TR. A major cause
of this phenomenon may be poor tolerance of systemic pressure by the
tricuspid valve itself, especially when the valve is dysplastic or
abnormal or with the changes in afterload that result from closure of
the ventricular septal defect. Additionally, changes in RV
geometry, especially dilatation after surgery, may contribute to
increased TR.
Huhta et al15 demonstrated that TR altered the long-term outcome of patients with AV and VA discordance and recommended tricuspid valve replacement in patients with hemodynamically significant TR. One patient who underwent tricuspid valve replacement in our series showed good long-term results with satisfactory RV function, although the other patient required reoperation for early progressive RV function (double switch conversion). Our results demonstrate that TR does not improve spontaneously, even when volume overload is eliminated by intracardiac repair.
RV Function
Long-term RV function has been a major concern in
patients
with AV and VA discordance or after atrial switch for transposition of
great arteries. Although there are several studies regarding RV
function in AV and VA discordance, many of them studied patients
without associated cardiac anomalies or before surgery, and few studies
have been made after intracardiac repair of coexisting cardiac
anomalies.6 7 8 9 10
Some reports have shown that the
morphological RV can function well over many decades in patients with
isolated AV and VA discordance or with minimal associated
anomalies.8 9 10 On the other hand,
Graham et
al7 demonstrated that RV function was maintained well
during childhood but that RV dysfunction was more common in adolescence
or adult life. Thus, systemic RV function during long-term
follow-up remains controversial. Our results showed that RV
function was well maintained in all patients before intracardiac
repair. However, 55% of patients developed RV dysfunction, even with
normalized hemodynamics, after intracardiac repair.
This finding is clearly different from previous studies of patients
with isolated AV and VA discordance and suggests that patients with AV
and VA discordance are at high risk of development of systemic RV
dysfunction after intracardiac repair of ventricular septal
defect with or without associated left ventricular outflow
obstruction.
The cause of this phenomenon is not evident. However, our data suggest that two factors are relevant: preoperative high pulmonary flow and postoperative development of TR. The pulmonary to systemic flow ratio was significantly higher in patients who developed RV dysfunction than in those who retained adequate RV function. Postoperative significant TR was also very frequent, with more than 80% of patients developing RV dysfunction. In contrast, there was no difference in the incidence of preoperative TR between patients who later developed RV dysfunction and those who did not. Decreased RV pump function with significant TR suggests that the systemic RV is functioning with afterload mismatch, that is, elevated afterload due to an inappropriate compensation against the volume overload, with or without impaired myocardial contractility. Sano et al21 reported that compensation for volume overload was not sufficient in the morphological RV, in which afterload mismatch occurred easily, compared with the morphological left ventricle in a study of patients with univentricular heart. In addition, Graham et al22 described the long-standing, volume overloadinduced impairment of myocardial contractility in patients with tricuspid atresia long term after systemic to pulmonary shunt. Preoperative excessive volume overload of high pulmonary flow may more easily induce afterload mismatch when the systemic RV is suffering from additional volume overload due to TR postoperatively.
Double Switch Operation
There are now a number of reports of
anatomic correction in AV
discordance with a combined atrial switch and Rastelli-type
repair12 13 14 or combined arterial and
atrial
switches (so-called "double switch"
procedure).14 Imai et al14 reported such
corrective procedures in 18 patients with AV discordance and documented
satisfactory postoperative biventricular function with
significant reduction of TR as well as excellent early operative
results. The double switch repair is likely to be a better surgical
option than conventional intracardiac repair, at least from the
viewpoint of long-term systemic ventricular and AV
valvular function, although it is a complex and
time-consuming procedure and the long-term results are still
unclear. One patient in the present series underwent combined
arterial and atrial switch procedure because of severe
systemic RV dysfunction shortly after conventional intracardiac repair
with tricuspid valve replacement. This patient remains well, with good
biventricular and AV valvular function after 5
years. An additional 6 patients have had a combined
arterial and atrial switch procedure as primary correction.
Follow-up on these patients is, as yet, too brief to allow
comparison with the main cohort. Combined arterial and
atrial switch should be most appropriate for AV and VA discordance with
unrestricted pulmonary flow, not only because the cardiac
anatomy is usually suitable but also because such patients
appear to be at increased risk of development of RV dysfunction after
conventional intracardiac repair. Unfortunately, only 25% of all
patients have no pulmonary outflow obstruction in our
experience, and hence the majority of patients are not suitable for
this form of repair. It is also unclear whether combined atrial switch
and Rastelli-type repair is feasible for the majority of patients
with AV and VA discordance associated with pulmonary outflow
obstruction.
Fontan Operation
An alternative procedure for discordant AV
and VA connection with
pulmonary obstruction, especially atresia, and favorable
pulmonary circulation is a Fontan operation. This may well be
the first choice when additional complex factors such as unbalanced
ventricular sizes, straddling AV valve, or abnormal venous
return coexist. Another four patients with AV and VA discordance have
had Fontan repair with no mortality during the same period. The
long-term follow-up on these patients has not been included
because of the small numbers.
In conclusion, the intermediate-term outcome of conventional intracardiac repair for patients with AV and VA discordance is acceptable in terms of survival and functional status. However, there is concern about progressive systemic RV dysfunction with development of significant TR after the operation. Preoperative unrestricted pulmonary flow and postoperative development of TR may be risk factors for progressive RV dysfunction after conventional intracardiac repair. Combined arterial and atrial switch procedure or, in complex cases that are unlikely to be amenable to biventricular repair, a Fontan-type repair may produce better long-term results, including better ventricular and AV valvular function, in such patients.
| Selected Abbreviations and Acronyms |
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| Acknowledgments |
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| References |
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