From the Division of Pediatric Cardiology, Department of Pediatrics,
College of Physicians and Surgeons of Columbia University, New York, NY
(L.R.P., A.J.H., M.S.R., W.M.G.), and the Department of Biostatistics and
Epidemiology, Cleveland Clinic Foundation, Cleveland, Ohio (M.S.).
Correspondence to Lourdes R. Prieto, MD, The Cleveland Clinic Foundation, Department of Pediatric Cardiology/M41, 9500 Euclid Ave, Cleveland, OH 44195. E-mail prietol{at}cesmtp.ccf.org
Methods and Results—Since 1958, 40 patients with CTGA were
studied to determine risk factors for poor outcome, including age, open
heart surgery, tricuspid insufficiency (TI), cardiac rhythm,
pulmonary overcirculation, and RV dysfunction. Follow-up ranged
from 7 to 36 years (mean 20 years). Intracardiac repair was performed
in 21 patients; 19 were unoperated or had closed heart procedures. For
the purposes of this study the designation TIs refers to at
least moderately severe TI as delineated by echocardiogram and/or
angiography. TIs was the only independently significant
factor for death (P=0.01), and in turn, only the
presence of a morphologically abnormal TV predicted TIs
(P=0.03). Twenty-year survival without TIs
was 93%, but only 49% with TIs. Poor long-term
postoperative outcome was due to TIs in all but 1 patient;
20-year survival rates for operated patients with and without
TIs were 34% and 90%, respectively
(P=0.002). Similarly, 20-year survival rates for
unoperated patients with and without TIs were 60% and
100%, respectively, whether or not attempts to repair the TI were made
(P=0.08).
Conclusions—TIs represents the major risk
factor for CTGA patients; RV dysfunction appears to be almost always
secondary to long-standing TI. Decisions related to surgical
interventions with or without associated lesions must be strongly
influenced by the status of the tricuspid valve.
No series to date has carefully defined the progression of TV disease
in operated as well as unoperated patients in order to establish the
role of open heart surgery and other potential risk factors in the
natural history of this disease. This study examines the long-term
course and outcome of patients with CTGA with and without open heart
surgery with particular attention to the status of the TV. It addresses
the important and controversial question as to whether TI or intrinsic
right ventricular (RV) dysfunction causes deterioration of
the cardiovascular status of patients with CTGA.
TIs refers to at least moderately severe
tricuspid insufficiency, as defined below.
Evaluation of Tricuspid Valve Function
Of the 40 patients, 20 had echocardiograms performed from the mid 1980s
to the present, during which time echocardiographic
assessment of atrioventricular valve insufficiency
severity was possible. The degree of TI was determined qualitatively by
taking into account the TI jet area and distance the jet extended into
the left atrium. If there was evidence of systolic flow
reversal in the pulmonary veins the TI was graded as severe.
When there was disagreement between the angiographic and
echocardiographic assessment (which was no more than 1
grade for any patient), the angiographic grade was used. If more than 1
year had elapsed between angiography and
echocardiography, the degree of TI was determined
from the more recent of the two. Thus TI severity at last follow-up was
graded by echocardiography in 10 patients in whom
angiography had been performed at least 1 year before the most recent
echocardiogram.
Evaluation of RV Function
Patient Population
Risk Factors for Death
Risk Factors for Tricuspid Insufficiency
To examine pulmonary overcirculation as a risk factor, the
clinical course and catheterization data of each
patient were reviewed to determine whether their physiology was one of
increased, decreased, or normal pulmonary flow. Two sets of
comparisons were made: (1) patients whose pulmonary flow was
increased throughout the entire period of observation were compared
with those whose pulmonary flow was either decreased, normal,
or increased only for a limited time, (2) patients whose
pulmonary flow was either always increased or increased for a
limited time were compared with patients whose flow was always either
normal or decreased.
Statistical Methods
Next, the total group was divided into 2 subgroups, OHS and NOHS
patients. The Kaplan-Meier procedure and Cox proportional hazards
regression analysis techniques were used to examine whether
TIs was a risk factor for death within each of
these subgroups.
Finally, the associations between TIs and
potential risk factors for its development were examined with Fisher's
exact tests.
Survival Analysis
Age at last follow-up was not associated with death. Mean age at death
or heart transplantation (24.9±16.5 years) for the 12 patients who
died or were given transplantation was similar to the age at last
follow-up for the 28 patients in New York Heart Association class
No difference was found in the long-term survival of patients who
underwent OHS and those who did not (Figure 2
The clinical course of patients with TIs
was one of rapid deterioration whether or not TV surgery was
undertaken. Survival 1 year after the diagnosis of
TIs was 70% and by 10 years 38% (Figure 4A
Risk Factors for Tricuspid Insufficiency
Open Heart Surgery
TIs developed in 10 (53%) of 19 NOHS patients at
a median age of 19 years (range 7 to 68 years). Six of the 10 patients
required TV replacement and 1 tricuspid valvuloplasty, 1 had
deterioration of RV function after severe TI and underwent heart
transplantation, and 2 are moderately symptomatic with
congestive heart failure. There was no difference in the incidence of
TIs in the operated and unoperated patients
(P=0.34).
VSD closure alone (as opposed to any type of OHS) was also not
significantly associated with development of TIs,
which occurred in 6 (33%) of 18 patients who underwent VSD closure and
in 11 (50%) of 22 patients who did not undergo VSD closure
(P=0.35).
Tricuspid Valve Morphology
Cardiac Rhythm
Pulmonary Overcirculation
Right Ventricular Dysfunction
Several investigators have reported that TV function in patients with
CTGA appears to be adversely affected by surgical repair of associated
lesions.2 3 7 8 Anatomic distortion of a
structurally abnormal TV by VSD closure has been
implicated,7 as has annular dilation after
cardiopulmonary bypass– induced RV dysfunction or large
preoperative left-to-right shunts.3 In this
series, however, multivariable analysis
indicates that in general, the long-term incidence of TV failure was
not affected by intracardiac repair of associated lesions, although 3
patients were identified in whom onset of TIs was
temporally related to OHS. It should also be noted that the diagnosis
of TIs was made at a younger median age in the
operated patients (14 years) than in the unoperated patients (19
years). Therefore it is reasonable to speculate that OHS can hasten the
development of TV failure in that fraction of patients with CTGA
already predisposed to the development of TV disease. However, at this
time, data are insufficient to validate this concept.
This study indicates that patients who have morphologic abnormalities
of the TV identified early in life are at increased risk for developing
TIs. In some cases, cardiologists' ability to
diagnose mild TV abnormalities is limited because autopsy
series1 have demonstrated a much higher incidence
(91%) than among previously reported clinical
cases2 3 5 6 7 or in our series (70%). The
autopsy/clinical findings may become more similar as
diagnostic imaging techniques improve and the presence of
milder forms of TV deformity are identified during life. No factors
examined other than TV anatomy, including complete heart block,
pulmonary overcirculation, or primary RV dysfunction were
associated with TV dysfunction.
The interplay between TI and RV dysfunction observed in this group of
patients with CTGA is of interest, and the "which is the
cart-or-horse" question as to whether TI most often leads to RV
dysfunction or vice versa is often raised. Several studies have
documented normal RV function at rest and normal increase in ejection
fraction with exercise in patients with CTGA into adulthood, even in
the presence of hemodynamically significant lesions not
including TI.14 16 A number of patients with CTGA
and no hemodynamically significant lesions have been
described who are doing well past age 50 years,17
attesting to the ability of the systemic RV to sustain long life in the
presence of normal hemodynamics. In our group, 2
elderly patients were seen, one with no intracardiac lesions who was
seen at age 62 years in atrial fibrillation and is now in NYHA class II
at age 69; and another who underwent corrective surgery for atrial
septal defect and PS at age 60 years and died at age 69 with severe TI.
A tendency toward deterioration of RV function with
age13 and the lack of increase in ejection
fraction with exercise15 have been reported in
patients with CTGA and minimal hemodynamic
abnormalities. Because of the very small number of such patients with
no significant associated lesions, it has been difficult to determine
whether the commonly observed deterioration of RV function in patients
with CTGA is a primary process, the result of long-standing associated
lesions, related to previous surgery, or secondary to long-standing TI.
The data from this series appears to help answer this question. Of 23
OHS and NOHS patients without TIs, 22 continue to
have normal RV function at last follow-up. There were only 2 patients
in whom RV dysfunction was clearly not associated with TV failure, and
other explanations for RV deterioration were obvious in one of the two
instances. It can be concluded that RV failure as a primary process
leading to TI is uncommon but is a frequent sequelae after an abnormal
TV fails.
The mean time from onset of TIs to documentation
of RV failure in our patients was 5 years. This time frame is in
contrast to patients with atrioventricular concordance,
who often tolerate a significant degree of mitral insufficiency for
decades before left ventricular failure
ensues.18 In CTGA, the usual vicious circle
occurs whereby the inability of the RV to cope with significant TI
leads to decreased contractility and annular dilation
that in turn exacerbate the degree of TI, but this occurs at an
accelerated rate. Because the systemic RV in CTGA appears to be less
tolerant than an anatomic left ventricle of similar degrees of
valvular incompetence, the progression of TI and subsequent RV
deterioration is often more rapid than in patients with mitral valve
disease. The reason for this distinction between the right and left
ventricles is not immediately apparent but is most likely related to
geometric differences between the two ventricles and the design of the
respective atrioventricular
valves.19
Repair or replacement of the TV has met with little success. In this
series, survival 10 years after surgical intervention on the TV was
only 14%. Similarly, Van Son et al20 reported
40% survival at a median follow-up of 4.7 years for 15 patients with
CTGA undergoing TV repair or replacement before 1981. It would seem,
therefore, that the high mortality rate for TV replacement for CTGA
patients with severe TI and RV dysfunction would argue for different
types of management. One approach would be to replace the TV earlier,
before a significant depression of RV function occurs. Although this
approach is theoretically attractive, most cardiologists are reluctant
to recommend valve replacement in asymptomatic patients
with abnormal but not severely regurgitant tricuspid valves without
absolute proof of benefit. Furthermore, in the absence of obvious
extreme RV failure, assessment of mild to moderate dysfunction by
various imaging techniques hitherto has not been useful. There remain
limitations in the quantitative assessment of RV function, especially
in the setting of abnormal preload and afterload conditions that
accompany systemic valvular
insufficiency.21 22
A "double switch" operation has been advocated for patients whose
tricuspid valves are severely insufficient. An atrial switch, combined
with an arterial switch in the absence of left
ventricular outflow obstruction or with a Rastelli
procedure have been successfully achieved. After surgery, the left
ventricle and mitral valve are restored to the systemic
circulation.23 24 25 Improvement in TV function in
a low-pressure RV has been documented after these
operations.24 25 This procedure carries
significant risk, and late complications relating to the atrial switch
component (baffle obstruction, sick sinus syndrome, and so forth) are
of additional concern. Heart transplantation remains the final option
for patients with CTGA, intractable TI, and RV failure.
Although the type of surgical treatment and timing for intervention for
patients with CTGA remain difficult problems, the focus on TV
anatomy and function as the major consideration in the
decision-making process should allow rational management concepts to
evolve. As surgical techniques improve and evaluation of RV function by
new diagnostic imaging methods emerge, earlier and more
effective interventions may become plausible in the not-too-distant
future.
Study Limitations
Similarly, RV function was determined qualitatively, there being no
simple way to determine it quantitatively by the available methods.
Such qualitative (or even quantitative) assessment can underestimate
dysfunction in the presence of decreased afterload offered by a
regurgitant atrioventricular valve. This problem was
minimized in the assessment of RV function as a risk factor for
development of TIs, as only those patients in
whom RV function was documented, albeit qualitatively, before the onset
of TIs were analyzed. However, the number
of patients with RV dysfunction at last follow-up may be underestimated
because many had TIs at this late time, and RV
unloading could have masked dysfunction. Finally, the effect of older
age on RV performance with or without TI may not be evident in
this study because only 5 patients were followed beyond 40 years of
age.
Several risk factors for development of TIs were
independently analyzed in this relatively small series from a
single institution. Accurate times to development of
TIs and larger numbers of patients would allow
more extensive analysis not only of individual risk factors but
of combinations of risk factors. For example, although neither VSD
closure nor the presence of complete heart block individually were
associated with TIs, patients who undergo VSD
closure complicated by complete heart block may be at higher risk.
Because only 3 patients in this study had VSD closure and postoperative
complete heart block, larger numbers would be needed to answer this
question.
Received March 13, 1998;
revision received April 28, 1998;
accepted April 28, 1998.
2.
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© 1998 American Heart Association, Inc.
Clinical Investigation and Reports
Progressive Tricuspid Valve Disease in Patients With Congenitally Corrected Transposition of the Great Arteries
Abstract
Top
Abstract
Introduction
Methods
Results
Discussion
References
Background—The outcome of patients
with corrected transposition of the great arteries (CTGA) is variably
affected by associated intracardiac defects, tricuspid valve
competence, and systemic right ventricular (RV) function.
The relative importance of these factors in long-term outcome has not
been evaluated.
Key Words: transposition of great vessels • valves • survival • pediatrics
Introduction
Top
Abstract
Introduction
Methods
Results
Discussion
References
Morphologic
abnormalities of the tricuspid valve (TV) are frequently found in
patients with congenitally corrected transposition of the great
arteries (CTGA).1 2 3 Autopsy studies of patients
with this lesion have documented TV abnormalities in 91% of cases,
most commonly an Ebstein-like anomaly with short, thickened chordae
tendineae.1 4 Clinically significant tricuspid
insufficiency (TI) has been reported in 20% to 50% of patients with
CTGA.2 3 5 6 7 In addition to the severity of the
anatomic abnormality of the valve, other potential predisposing factors
for the development of important TI have been proposed but not well
documented. Several investigators have noted the development or
exacerbation of TI shortly after open heart surgery for closure of a
ventricular septal defect (VSD) with or without pulmonic
stenosis (PS),2 3 7 8 whereas others have
not found such a relation.6 9
Methods
Top
Abstract
Introduction
Methods
Results
Discussion
References
Definitions
Open heart surgery (OHS) is defined as repair of any
intracardiac lesion other than TV disease and is distinguished from
repair or replacement of the TV, which is referred to as TV surgery.
Patients were categorized as non–open heart surgical (NOHS) if they
had not had open heart surgery for associated lesions before the
development of significant TI. Patients in this group either had no
significant intracardiac lesions, were well balanced with their
existing lesions, or had been palliated with closed heart procedures.
Patients undergoing repair of other intracardiac lesions at the time of
TV surgery were included in the NOHS group because they had developed
significant TV disease before undergoing open heart surgery for
other defects.
The status of the TV was determined from clinical information,
echocardiogram, catheterization, and angiographic data
at the time of presentation and during follow-up. All
patients underwent cardiac catheterization before open
heart surgery (OHS), 20 of 21 after OHS, and 12 of 13 before TV
surgery. Catheterization was performed in 18 of 19
patients who did not undergo OHS, either for diagnostic
purposes, before closed heart palliation, or because of TV disease.
Thus 39 (98%) of 40 patients were catheterized, with 28 of 39 having
had multiple studies. The severity of TI was graded angiographically as
follows10: mild (1+): contrast clears with each
beat and never opacifies the entire left atrium; moderate (2+):
contrast does not completely clear with 1 beat, may opacify the entire
left atrium after several beats, but not as densely as the "left"
ventricle (morphologic RV in these patients); moderately severe (3+):
the left atrium is completely opacified as densely as the "left"
ventricle; and severe (4+): the left atrium is completely opacified
with 1 beat, the opacification becomes more dense with subsequent
beats, and contrast can be seen refluxing into the pulmonary
veins.
RV function was evaluated qualitatively from angiography and/or
echocardiography as either normal or significantly
decreased. All except 1 of the 40 patients were catheterized, and in 30
of those RV function was evaluated by angiography. Ten patients had
echocardiograms performed at least 1 year after the most recent
catheterization, in which case RV function was assessed
qualitatively by echocardiography with 2 or more
observers.
Since 1958, 40 consecutive patients with CTGA and 2 normal-sized
ventricles have been followed at Columbia-Presbyterian Medical Center.
There were 13 female and 27 male patients. Clinical records,
including cardiac catheterization, angiographic,
echocardiographic, operative, and autopsy reports were
retrospectively reviewed. Of the 40 patients, 37 had associated
intracardiac anomalies, of which 30 were
hemodynamically significant (most commonly VSD and PS),
whereas 3 had no intracardiac lesions (Table 1
). Twenty-one of the 40 patients
underwent OHS between 1965 and 1990, at a median age of 11 years (range
2 to 60 years). Of those 21 patients, 10 (47.6%) underwent
intracardiac repair of VSD and PS, 4 (19.0%) VSD, 3 (14.3%) VSD and
coarctation, 2 (9.5%) atrial septal defect and PS, 1 (4.8%) VSD and
pulmonary atresia (PA), and 1 (4.8%) PS. Nineteen of the 40
patients were categorized as NOHS; 2 of 19 were palliated with
Blalock-Taussig shunts, 4 were well balanced with VSD and PS, 1 had
severe PS but refused surgery, 2 had TV surgery at the same time as
repair of VSD, PS and VSD, PA, and 10 had no clinically significant
lesions associated with CTGA.
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Table 1. Patient
Characteristics
The impact on survival of the following variables was
examined: age at presentation and at last follow-up, OHS,
TIs, cardiac rhythm, pulmonary
overcirculation, and RV dysfunction. For the purpose of survival
analysis, patients were classified as having RV dysfunction if
it occurred at any time during the follow-up, whether or not preceded
by TIs. Survival of the 21 OHS patients was
compared with that of the 19 NOHS patients, and the impact of
TIs on survival for these 2 subgroups was
examined.
A number of potential risk factors for the development of
TIs were evaluated, including OHS, morphology of
the TV, degree of preoperative TI in patients who underwent OHS,
cardiac rhythm, RV volume overload caused by increased
pulmonary blood flow (referred to below as pulmonary
overcirculation), and RV function. Patients who had
TIs at presentation with no prior
documentation of RV function were not included in the analysis
of RV function as a risk factor. The TV was classified as
morphologically abnormal if there was evidence from angiography,
echocardiography, direct intraoperative
visualization or autopsy of a structural abnormality such as an
Ebstein-like malformation, TV prolapse, or dysplastic features (eg,
thickened or redundant leaflets).
Descriptive statistics were presented as means, medians,
standard deviations, frequencies, percentages, and confidence
intervals, as appropriate. Group comparisons were completed with the
use of t tests, 
2 tests, or
Fisher's exact tests, as appropriate. Univariate Cox
proportional hazards regression analysis techniques were used
to examine the potential risk factors for death. Linearity and
proportionality assumptions were checked before modeling. The
Kaplan-Meier procedure was used to estimate event rates throughout the
study period and associated curves were graphed to support the
findings. After checking for multicollinearity with Fisher's exact
tests on the univariately significant risk factors, these
factors were considered for the multivariable Cox proportional
hazards regression model.
Results
Top
Abstract
Introduction
Methods
Results
Discussion
References
The mean±SD age at presentation of the 40 patients
was 5.3±12.9 years (range birth to 62 years). The median age was 3
months. Mean±SD and median age at last follow-up were 25±14 and 23
years, respectively (range 7 to 69 years). Follow-up from the time of
presentation to the time of last visit, heart
transplantation, or death ranged from 7 to 36 years (mean=20 years,
median=19 years). Seventeen subjects (42.5%) had
TIs during their clinical course. Twelve subjects
(30%) had RV dysfunction at last follow-up, 11 of whom also had
TIs. Pulmonary overcirculation occurred
in 14 (35%) patients, either from associated lesions or after surgical
palliation, and 13 (32.5%) patients had complete heart block.
Considering death and heart transplantation as the same outcome,
the Kaplan-Meier estimate of survival for this group of 40 patients
with CTGA was 74% at 20 years of follow-up. The results of
univariate survival analysis of risk factors
including age at presentation, TIs,
RV dysfunction, OHS, complete heart block, and pulmonary
overcirculation are shown in Table 2.
Both TIs (relative risk [RR]=14.8, 95%
confidence interval [CI]=1.9 to 117.1, P=0.01) and RV
dysfunction (RR=5.5, 95% CI=1.4 to 21.4, P=0.01) were
significantly related to increased mortality rates. Those with
TIs were 14.8 times more likely to die than those
without TIs, and those with RV dysfunction were
5.5 times more likely to die than those without RV dysfunction.
TIs and RV dysfunction were significantly
associated with each other (P=0.001); 11 of the 17 (65%)
patients with TIs had RV dysfunction, and 22 of
the 23 (96%) patients without TIs had normal RV
function. By multivariable analysis,
TIs was the only independent risk factor for
death among the factors considered in this analysis.
Kaplan-Meier estimates of survival for the 23 patients free of
TIs yielded a 93% survival rate at 20 years of
follow-up, whereas the 17 patients with TIs had a
20-year survival of 49% (Figure 1).
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Table 2. Risk Factors for Death
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Figure 1. Kaplan-Meier estimate of survival for all 40
patients with CTGA. The 40 patients are then broken down into 23
patients without TIs and 17 patients with TIs.
Note significantly different survival between the two groups
(*P=0.01). n indicates number of patients. 
III
(25.1±12.8 years) (P=0.96). ). Survival was 72% for OHS patients
and 77% for NOHS patients at 20 years of follow-up. When OHS patients
were divided into those who subsequently developed
TIs and those who did not, there was a marked
difference in survival: 90% 20-year survival for those who remained
free of TIs, in contrast to 34% for those who
developed TIs some time after surgical repair
(P=0.002) (Figure 3A). NOHS
patients who remained free of TIs during the
entire period of observation had a 100% 20-year survival rate, whereas
those who developed TIs at any time during the
follow-up period had a 20-year survival of 60%. This relation was not
statistically significant (P=0.08) (Figure 3B).
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Figure 2. Kaplan-Meier estimate of survival for 21 patients
with CTGA who underwent open heart surgery for associated lesions and
for 19 unoperated patients. Note similar survival for the two groups. n
indicates number of patients.
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Figure 3. Kaplan-Meier estimate of survival for A, 21
patients who underwent open heart surgery, and B, 19 unoperated
patients broken down into those without TIs and those with
TIs. Note significantly decreased survival for open heart
surgery patients with TIs (*P=0.002) and
trend toward decreased survival for unoperated patients with
TIs (P=0.08). n indicates number of
patients. ) despite surgical intervention on the
TV in 13 of the 17 patients. Outcome after TV surgery was poor; 14%
survival 10 years after TV replacement (n=12) or repair (n=1) (Figure 4B). Nine of the 12 patients whose tricuspid valves were replaced later
died (n=8) or were given transplantation (n=1). Of the 6 patients with
TIs still alive and not given transplantation at
last follow-up, 1 was in NYHA class III, 4 in class II, and only 1 in
class I. In contrast, there were no deaths in the 23 patients without
TIs, 19 were in NYHA class I at last follow-up, 3
in class II, and 1 was given transplantation because of RV failure.
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Figure 4. Kaplan-Meier estimate of survival A, from the time
of diagnosis of TIs in 17 patients with CTGA, and B, after
tricuspid valve surgery in the subset of patients with TIs
who underwent either repair or replacement of the tricuspid valve. Note
very high and continuing mortality rates within months of the diagnosis
of TIs and dismal survival rates after tricuspid valve
surgery.
Only the presence of a morphologically abnormal TV was
significantly associated with development of TIs
during follow-up. OHS, preoperative TI, complete heart block, RV
dysfunction, and pulmonary overcirculation were not significant
risk factors.
Of the 21 OHS patients 7 (33%) developed
TIs at a median age of 14 years (range 8 to 69
years), and 6 of the 7 required TV replacement a median of 8 years
after OHS. One patient died of congestive heart failure awaiting TV
surgery. Preoperative TI was insignificant in 19 and moderate in 2,
both of whom developed severe TI on long-term follow-up. Five of the 19
(26%) OHS patients without significant preoperative TI developed
TIs, ranging from immediately after surgery to 12
years later. Three of the 7 OHS patients with TIs
after surgery developed it within 2 months of OHS despite having no
significant TI before surgery.
The morphology of the TV was ascertained in 27 of the 40 patients.
The remaining 13 patients were not entered in the analysis. The
valve was abnormal in 19 (70%) of the 27, with an Ebstein-like anomaly
in 13, dysplasia in 5, and slight hypoplasia in 1. Twelve (63%) of the
19 developed TIs. The valve appeared normal in 8
of the 27 patients, and 1 (12.5%) of the 8 developed
TIs (P=0.03).
Complete heart block (CHB) occurred in 13 patients; 2 were
diagnosed at birth, 7 developed it spontaneously at a mean of 12 years
and 4 after OHS. Eight of the 13 (62%) subsequently developed
TIs. Time from diagnosis of CHB to onset of
TIs ranged from 1 month to 28 years (mean 13
years). In 2 patients the 2 events were closely linked temporally, CHB
preceding TIs by 1 month and 1.5 months,
respectively. Nine of 27 (33%) patients in sinus rhythm developed
TIs. There was no significant difference in
TIs between patients with CHB and sinus rhythm
(P=0.13).
Of the 40 patients, 5 had increased pulmonary blood flow
during the entire follow-up time, whereas in 35 pulmonary blood
flow was either never or only temporarily increased. One of the 5
patients with chronic pulmonary overcirculation developed
TIs, as compared with 16 of the 35 (46%) whose
pulmonary flow was either never or only temporarily increased
(P=0.37). Pulmonary blood flow was increased during
part or all of the follow-up time in 14 patients, 6 of whom (43%)
developed TIs. Twenty-six patients always had
either normal or decreased pulmonary flow, and 11 of the 26
(42%) developed TIs (P=1.0).
Thirty six of the 40 patients had documentation of RV function by
echocardiogram and/or angiography before onset of
TIs, and 2 of the 36 had decreased function. One
of these 2 patients had RV dysfunction, subaortic obstruction, and mild
TI documented angiographically 3 years after VSD closure and
coarctation repair. He had heart transplantation 8 years later, at
which time he still had only mild TI and severe RV dysfunction. The
second patient had deterioration of RV function with no obvious
preceding event and later developed TIs. Of the
34 patients with normal RV function, 12 developed
TIs, and 6 of those 12 went on to develop late RV
dysfunction. The TV remained competent in 22 of the 34 patients, and
all had normal RV function at last follow-up.
Discussion
Top
Abstract
Introduction
Methods
Results
Discussion
References
The natural history and postoperative outcome of patients with
CTGA and the commonly associated lesions of VSD and PS are known to be
less satisfactory than those of patients with normal
atrioventricular connections and similar intracardiac
lesions.11 Several features render these patients
more fragile than their counterparts with
atrioventricular concordance. Among these features is
the propensity for atrioventricular conduction
abnormalities12 and for TV
dysfunction1 2 3 as well as the much-debated
capability of the RV to function adequately in the systemic circulation
over the course of a normal life span, regardless of the state of the
TV.13 14 15 16
The determination of TI severity by either angiography or
echocardiography was carried out with the use of
standard qualitative techniques. No attempt was made to calculate
regurgitant fraction because there is to date no gold standard for
grading atrioventricular valve insufficiency. The error
intrinsic in the angiographic calculation of regurgitant fraction is
quite significant,10 limiting its accuracy.
Although recent echocardiographic
methods26 may prove superior, the majority of our
patients were studied before these technical developments. The rapidly
progressive nature of TI in these patients, once it became more than
mild, made for a clear delineation on clinical grounds between those
with little TI and those with TIs. This argues
against a significant methodological error in the serial assessment of
TI severity.
References
Top
Abstract
Introduction
Methods
Results
Discussion
References
1.
Allwork SP, Bentall HH, Becker AE, Cameron H,
Gerlis LM, Wilkinson JL, Anderson RH. Congenitally corrected
transposition of the great arteries: morphologic study of 32 cases.
Am J Cardiol. 1979;38:910–923.
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