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ARTICLES

Anatomic Features and Surgical Strategies in Double-Outlet

Right Ventricle
S. Kleinert, T. Sano, R.G. Weintraub, R.B.B. Mee, T.R. Karl and J.L. Wilkinson

http://dx.doi.org/10.1161/01.CIR.96.4.1233
Circulation. 1997;96:1233-1239
Originally published August 19, 1997

Abstract
Background The objective of this study was to review anatomic features and surgical strategies in children with
double-outlet right ventricle (DORV) and to assess risk factors for early mortality.
Methods and Results Records were reviewed of all children with DORV undergoing surgery between 1978 and
1993. Noncomplex patients (group 1) had atrioventricular (AV) concordance, a single ventricular septal defect
(VSD), balanced ventricles, no straddling AV valves, and no major pulmonary artery anomaly. Group 2 (complex)
comprised all remaining patients. Independent risk factors analyzed included location of the main VSD, presence
of additional VSDs, coarctation, ventricular outflow obstruction, ventricular hypoplasia, age at operation, operation
before 1985, previous palliation, and type of definitive operation. Of 193 patients, 117 were in group 1 and 76 in
group 2. In 148 patients, biventricular repair was undertaken, including 111 of 117 group 1 patients and 37 of 76
group 2 patients. Early mortality was higher among group 2 patients undergoing biventricular repair than among
group 1 patients (8 of 37 versus 4 of 111, P<.005) and higher than group 2 patients undergoing a Fontan
procedure (none of 29, P<.01). Aortic arch obstruction, operation before 1985, and multiple VSDs were significant
risk factors for mortality. Age <1 month (P<.05) and multiple VSDs (P<.005) were independent risk factors after
definitive repair. Up-to-date follow-up is available on 144 surviving patients, with 127 (88%) in New York Heart
Association class I and the remaining 17 in class II. Overall 10-year survival probability was 81%, whereas
probability of survival, free from reoperation (after definitive surgery), was 65% at 10 years.
Conclusions Biventricular repair can be achieved in most patients with DORV with low risk. In complex DORV, a
Fontan procedure is associated with a lower surgical mortality.

ventricles

surgery

risk factors

Fontan procedure

By describing a ventriculoarterial connection rather than a specific congenital malformation, double-outlet right
ventricle (DORV) includes a broad spectrum of anatomic variants and associated malformations. In 1964, Kirklin
et al1 reported successful correction in a child with DORV, subaortic ventricular septal defect (VSD), and
concordant atrioventricular (AV) connections. Since then, complete correction through the use of a variety of
surgical techniques has been achieved in more complex forms of DORV. Modified Fontan procedures have been
advocated when biventricular DORV repair has been either impractical or extremely complex.2 With the improving
short-term and medium-term outcome for Fontan procedures in recent years,3 4 5 this approach might also be
extended to patients who are at increased operative risk with a conventional biventricular repair. This report
reviews the anatomic findings, surgical strategies, and results among patients with DORV presenting for surgery
in a single institution over a 15-year period.

Methods
Patient Selection
The study population comprised all patients with a diagnosis of DORV who did not have a univentricular AV
connection and who underwent a surgical procedure between 1978 and 1993. A diagnosis of DORV was made if
both great arteries originated predominantly from the right ventricle with application of the 50% rule, which
requires that one great artery arises completely and the second >50% from the right ventricle. The diagnosis and
anatomic findings were based on a combination of angiography, echocardiography, and surgical inspection.
Group 1 patients, with noncomplex anatomy, were defined as those with AV concordance, a single VSD, balanced
ventricles, no straddling AV valves, and no major pulmonary artery anomaly. Group 2 patients, with complex
anatomy, consisted of all the remaining patients. Complex anatomy was defined by the presence of atrial
isomerism, AV discordance, atrioventricular septal defect, multiple VSDs, hypoplastic right or left ventricles,
straddling AV valves, and major pulmonary artery anomalies including pulmonary atresia, pulmonary artery sling,
aorto-pulmonary window, or discontinuous branch pulmonary arteries. Early mortality was defined as death on the
same hospital admission as the operation, or within 30 days of operation for patients discharged from hospital.
Follow-up information was obtained from hospital files for locally followed patients and from referring cardiologists
for patients followed elsewhere.

Statistical Analysis
Outcome measures included early survival and freedom from reoperation. To assess potential risk factors for
early mortality and reoperation, univariate analysis with Fishers exact test was initially used for each variable.
Variables with a value of P<.1 were then entered into a stepwise logistic regression analysis. A commercially
available statistic program was used (StataCorp, 1995. Stata Statistical Software: Release 4.0). Variables were
considered statistically significant in the multivariate analysis at P<.05. Odds ratios and 95% confidence intervals
are stated. Survival and freedom from reoperation probabilities were estimated with the Kaplan-Meier method. t
tests were used to compare normally distributed continuous variables and Pearsons 2 analysis or Fishers exact
test to compare discrete variables. Values are expressed as meanSD unless otherwise stated.

Results
A total of 193 patients who underwent surgical procedures comprised the study population. Four types of
definitive repairs were performed: (1) intraventricular repair with a baffle from the left ventricle to the aorta (IVR),
(2) arterial switch operation with a baffle from the left ventricle to the pulmonary artery (ASO), (3) atrial inversion
using the Senning operation, and (4) cavopulmonary shunt/Fontan procedures including bidirectional
cavopulmonary connection, modified Fontan procedures, total cavopulmonary connection, and the Kawashima
operation. The mean age at definitive repair was 3.24.1 years and ranged from 1 day to 30.2 years, with a
median of 1.9 years.

Anatomic Features
Complex versus noncomplex anatomy and associated anomalies. Complex anatomic features among group
2 patients are listed in Table 1. Of the 76 (39%) patients in group 2, the most commonly present complex
anatomic feature was the presence of additional VSDs (24 patients), followed by hypoplastic right or left ventricle
(20 patients) and atrioventricular septal defect (18 patients). Associated noncomplex anomalies present in group
1 and 2 patients are listed in Table 2.

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Table 1.
Complex Anomalies in Group 2 Patients (n=76)
Anatomic Feature

No.

Multiple VSDs

24

Hypoplastic RV/LV

20

AVSD

18

Isomerism

18

AV discordance

Pulmonary atresia

Restrictive VSD

Criss-cross AV connection

Straddling AV valves

Pulmonary artery sling

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Anatomic Feature

No.

Aortopulmonary window

LPA from aorta

Ectopia cordis

AV indicates atrioventricular; AVSD, atrioventricular septal defect; LPA, left pulmonary artery; LV, left
ventricle; RV, right ventricle; and VSD, ventricular septal defect.

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Table 2.
Associated Anomalies in Patients With Noncomplex (Group 1) and Complex Anatomy (Group 2)
Anatomic Feature

Group 1

Group 2

Pulmonary stenosis1

53

35

(37 of these TOF)

(13 of these TOF)

Coarctation of aorta

12

Situs inversus

Subaortic stenosis

Aortic stenosis

Interrupted aortic arch

TAPVD

Double-chambered RV

Common atrium

Dextrocardia

RV indicates right ventricle; TAPVD, total anomalous pulmonary venous drainage; and TOF, tetralogy of
Fallot.
1 Not including pulmonary atresia.

VSD type. The location of the primary VSD and the presence of additional VSDs are shown in Table 3. Of the 90
patients in whom the main VSD was subaortic, 50 had tetralogy of Fallottype anatomy. A subpulmonary VSD
was present in 49 patients. The main VSD was not committed to either outflow tract in 31 patients
(noncommitted). This designation was used in all cases in which the VSD was anatomically distant from both
arterial valves and was not related more directly to one outflow tract than to the other. An additional 18 patients
had a complete atrioventricular septal defect. In 5 patients, all in group 1, the VSD was committed to both outflow
tracts. There was no statistical difference in the primary VSD location between patients in group 1 and group 2. In
three cases, all with subaortic VSDs, the VSD was restrictive.

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Table 3.
Location of Primary VSD
Group 1

Group 2

Multiple VSDs

Subaortic VSD

61

29

11

Subpulmonary VSD

32

17

11

Noncommitted VSD

19

12

AV septal defect

18

DCSA VSD

Total

117

76

VSD indicates ventricular septal defect; AV, atrioventicular; and DCSA, doubly committed subarterial.

Outflow tract obstruction. Pulmonary outflow tract obstruction (POTO) not associated with tetralogy of Fallot but
including pulmonary atresia was present in 45 patients and was most prevalent in the group with subaortic VSDs
(17 of the remaining 40 patients, 42%). Eleven of 31 (35%) of children with noncommitted VSD had POTO, as did
2 of 5 (40%) with doubly committed subarterial defects and 7 of 18 with atrioventricular septal defects (39%). Only
8 of 49 patients (16%) with subpulmonary VSD had valvar or infundibular pulmonary stenosis. The majority of

patients (64%) with pulmonary outflow obstruction belonged to group 2. Aortic or subaortic stenosis was seen in 6
patients (3%), of whom 2 belonged to group 1 and 4 to group 2. Of the 6 patients with left ventricular outflow
obstruction, 3 had subpulmonary VSDs, 2 had subaortic, and 1 a noncommitted VSD. Aortic arch obstruction
coexisted with left ventricular outflow tract obstruction in 3 patients. Overall, aortic arch obstruction (aortic
coarctation or interruption) occurred in 26 (13%) of patients, of whom 14 had subpulmonary, 7 had subaortic, and
5 had noncommitted VSDs. The majority (17 of 26, 65%) of patients with aortic arch obstruction had noncomplex
anatomy (group 1).

Surgical Treatment
Prior palliative procedures. Definitive repair was the primary procedure in 92 of 193 (48%) patients. Table 4
summarizes the details of prior palliative procedures undertaken in the remaining 101 patients. Of these, 77
patients required only one palliative procedure, 19 had two procedures, 4 patients had three, and 1 patient
required four palliative procedures. The proportion of group 2 patients requiring palliation before definitive surgery
(50 of 76 or 65.8%; CI, 54.0% to 76.3%) was significantly higher than that of group 1 patients (51 of 117 or 43.6%;
CI, 34.4% to 53.0%; P<.005).

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Table 4.
Previous Palliative Procedures in 101 Patients
Palliative Procedure

No.

PA to systemic shunts

60

RMBTS

35

LMBTS

R, LMBTS

Waterston shunt

Central shunt

RMBTS, central shunt*

R, LMBTS, central shunt

RMBTS, atrial septectomy

L, RMBTS, PA sling

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Palliative Procedure

No.

CoA repair, RMBTS

RMBTS+atrial septectomy

Other procedures

CoA repair only

CoA repair+atrial septectomy

Aortic valvotomy

ASW, BCPS

Norwood operation

PA banding

33

PA banding only

19

CoA/IAA repair+band

Band, RMBTS

Band, RMBTS+CoA repair

CoA repair+band

Band, tightening band

Band+CoA repair, rebanding

Band, atrial septectomy

CoA repair+band, rebanding, BCPS

ASW indicates arterial switch; BCPS, bidirectional cavopulmonary shunt; CoA, coarctation of the aorta;
IAA, interrupted aortic arch; LMBTS, left modified Blalock-Taussig shunt; PA, pulmonary artery; and
RMBTS, right modified Blalock-Taussig shunt.
Comma (,) indicates separate procedures; plus sign (+) indicates a single procedure.

Definitive surgery. One hundred seventy-nine patients have so far undergone definitive surgery. Of the
remaining 14 patients, 5 await definitive surgery and 9 died before repair could be undertaken. At definitive
surgery, a biventricular repair was attempted if considered possible. Table 5 summarizes the details of the
definitive surgical procedure according to the VSD site. In 148 patients a biventricular repair was undertaken, with
an intraventricular baffle in 107 cases, the arterial switch operation as a primary procedure in 31, and a Senning
procedure in 10 (all in the early part of the seriesbefore introduction of the arterial switch). In 4 of these Senning
patients conversion to an arterial switch operation was subsequently performed because of increasing effort
intolerance late after surgery because of either right ventricular dysfunction or tricuspid incompetence. Three of
these four patients are long-term survivors, two being in New York Heart Association class I and the third in NYHA
class II. In 3 of the 6 other patients undergoing a Senning procedure, the VSD was not closed because of the
presence of pulmonary vascular disease. One patient died after his Senning operation because of intractable
problems related to multiple VSDs (being converted to a Fontan shortly before he died). Excluding this latter
patient, 31 modified Fontan-type procedures were performed for definitive palliation. These included 29 group 2
patients who had anatomic features (eg, severely hypoplastic ventricle or straddling AV valve) precluding a
biventricular repair. Two patients in group 1, both with noncommitted VSDs, also underwent modified Fontan
procedures.

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Table 5.
Type of Operation by VSD Location
VSD Location

IVR

ASW

Senning

Fontan

Subaortic

80 (60/20)

4 (0/4)

Subpulmonary

2 (0/2)

27 (21/6)

101 (9/1)

6 (0/6)

Noncommitted

14 (12/2)

4 (4/0)

11 (2/9)

AVSD

6 (0/6)

10 (0/10)

Doubly committed

5 (5/0)

Total

107 (77/30)

35 (29/6)

6 (5/1)

31 (2/29)

VSD indicates ventricular septal defect; IVR, intraventricular baffle repair; ASW, arterial switch
operation; and AVSD, atrioventricular septal defect.
Proportions of group 1/group 2 patients are shown in parentheses.
1 Four of these patients later converted to arterial switch.

Children underwent the ASO at a significantly younger age than the intraventricular baffle operation (1.42.9
years versus 2.93.4 years; P<.05) and the modified Fontan procedures (5.75.8 years; P<.005).

Surgical Outcome
Early mortality. Table 6 summarizes the details of 18 (9.3%; CI, 5.6% to 14.4%) hospital deaths, 6 of which
occurred after a palliative procedure and 12 after definitive surgery. A significantly higher proportion of group 2
patients (8 of 37 or 21.6%; CI, 9.8% to 38.3%) died after a biventricular repair than did group 1 patients (4 of 111
or 3.6%; CI, 1.0% to 9.0%; P<.005). In contrast, there were no hospital deaths among the 29 group 2 or the two
group 1 patients undergoing a cavopulmonary shunt or modified Fontan procedure (0%; CI, 0% to 11.2%). Table
7 presents the results of univariate and multivariate risk factor analysis for early mortality. Of the potential risk
factors analyzed in all 193 patients (including those undergoing palliative procedures), only the presence of
multiple VSDs, aortic arch obstruction, and operation before 1985 reached near significance in univariate analysis
(Table 7, A) and were significant risk factors for early mortality in multivariate analysis (Table 7, B). In the
analysis of the 179 patients who survived to definitive repair, the presence of multiple VSDs and age at operation
<1 month were significantly associated with early mortality, both in univariate and multivariate analysis (Table 7).

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Table 6.
Hospital Deaths
Group

Age

Operation Type

Anatomy

Cause of Death

1d

IVR

SAVSD, ectopia

Sepsis

cordis
2

4d

RMBTS

AVSD, isomerism

Sepsis

22 d

ASW+ PA band

SPVSD, MVSD

Low output cardiac failure

2y

IVR

SAVSD, TOF,

Perforation of coronary artery by

MVSD

chest drain

15 y

IVR

SAVSD, MVSD

Low output cardiac failure

7y

Senning (later

SPVSD, PS, MVSD

Cardiac failure, AV valve

converted to Fontan)
2

2y

IVR

regurgitation
SPVSD, MVSD,

Cardiac failure, residual VSD

hypoplastic LV
2

2d

IVR + Kono+ CoA

SAVSD, CoA,

repair

hypoplastic LV

Unexplained

Group

Age

Operation Type

Anatomy

Cause of Death

CoA repair+ PA

SAVSD, CoA,

Low output cardiac failure

mo

band, reband

hypoplastic LV

Norwood

SAVSD, CoA,

mo
2

Pneumonia/pulmonary hemorrhage

hypoplastic LV
IVR

mo

NCVSD,

Pulmonary hypertension,

criss-cross AVC, SI

electromechanical dissociation

1y

IVR

NCVSD, IAA

Low output cardiac failure

2y

RMBTS, atrial

NCVSD, PS

Unexplained

CoA repair+ PA band

SAVSD, CoA

Cerebral damage (anoxia)

IVR

SAVSD, PS

Postoperative complete heart block,

septectomy
1

4
mo

7
mo

7.8

cardiac failure
IVR

y
1

2.5

SAVSD, PS,

Low output cardiac failure

TAPVD, SI
ASW

SPVSD

Low output cardiac failure

CoA repair+ atrial

SPVSD, CoA

Low output cardiac failure

mo

septectomy

y
1

ASW indicates arterial switch operation; AV, atrioventricular; AVC, atrioventricular connection; AVSD,
atrioventricular septal defect; CoA, coarctation of the aorta; IAA, interrupted aortic arch; IVR,
intraventricular baffle repair; LV, left ventricle; MVSD, multiple ventricular septal defects; NCVSD,
noncommitted ventricular septal defect; PA, pulmonary artery; PS, pulmonary stenosis; RMBTS, right
modified Blalock-Taussig shunt; SAVSD, subaortic ventricular septal defect; SI, situs inversus; SPVSD,
subpulmonary ventricular septal defect; TAPVD, total anomalous pulmonary venous drainage; TOF,
tetralogy of Fallot; and VSD, ventricular septal defect.

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Table 7.

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Risk Factor Analysis for Early Mortality in All Patients and in Patients Surviving to Definitive Repair

Late deaths. Table 8 summarizes the details of late deaths. There were 9 late deaths, 2 in group 1 and 7 in
group 2 at a mean follow-up time of 4.73.8 years. Three of the 9 patients had undergone only a palliative
procedure, 5 had undergone biventricular repair, and 1 patient a Fontan-type procedure. Fig 1 shows the overall
survival probability with 90% confidence intervals for the entire study cohort, with an estimated 10-year survival
probability of 81%.

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Figure 1.
Probability of survival with accompanying 95% confidence limits for the entire study cohort.

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Table 8.
Late Deaths

Reoperations and follow-up information. Follow-up information was available in 144 of 166 surviving patients.
During the period of follow-up, 25 patients underwent 29 reoperations at a mean time interval of 33.6 years
(range, 6 days to 14 years) after definitive repair. Of these, 14 patients belonged to group 1 and 11 to group 2.
The initial anatomic features and the type of reoperation are summarized in Table 9. Risk factor analysis for
reoperation is presented in Table 10. There were no statistically significant variables associated with reoperation,
with only the presence of left ventricular outflow tract obstruction present having a value of P<.10. The probability

of freedom from death and reoperation with 90% confidence intervals is depicted in Fig 2. The estimated
freedom from death and reoperation at 10 years was 65%.

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Figure 2.
Probability of freedom from death or reoperation with accompanying 95% confidence limits for the entire
study cohort.

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Table 9.
Reoperations and VSD Type

Table 10.
Risk Factor Analysis for Reoperation in All Survivors (n=166) by Univariate Analysis (Fishers Exact
Test)

Additionally, 10 patients underwent pacemaker implantation, 8 for postoperative complete heart block, 1 as
prophylaxis, and the remaining patient because of frequent episodes of atrial flutter after a previous Senning
operation and a subsequent arterial switch conversion.

Of the 144 patients available for long-term follow-up, 127 (88%) were in NYHA class I and 17 (12%) in class II.
Twenty-three had mild and 4 moderate residual right ventricular outflow tract obstruction. Residual left ventricular
outflow tract obstruction was present in 10 patients. In 7 of these it was classified as mild and in 3 as moderate.

Discussion
This study confirms that a biventricular repair can be achieved in the great majority of patients with DORV with an
acceptably low perioperative mortality. Previous reviews have reported hospital mortality figures of up to 27%,
especially in cases with noncommitted or subpulmonary VSDs.6 7 8 9 Since then, new surgical techniques
including the arterial switch operation10 11 have significantly improved the outcome in noncomplex forms of
DORV.8 11 12 13

VSD Site and Choice of Surgical Procedure

Although the classification of DORV by VSD site as introduced by Lev and coworkers14 remains useful for
surgical planning, the present study has not demonstrated a significantly higher risk in patients with
noncommitted, subpulmonary, or atrioventricular septal defects, as has been reported in earlier series.6 9 15 In our
institution, the arterial switch operation is now the procedure of choice for patients with subpulmonary VSD
without significant right ventricular outflow obstruction as well as in other cases in which baffling from the left
ventricle to the pulmonary artery is technically easier than baffling to the aorta. In the present series, the arterial
switch procedure for DORV with subpulmonary VSD was associated with a low hospital mortality. On the other
hand, an intraventricular baffle repair without arterial switch could only be performed in a small number of cases
with subpulmonary VSD. When technically possible, this approach has led to good results with low operative
mortality and no increased need for reoperation.13 16 17 The tricuspidtopulmonary annulus distance has been
shown to be a useful predictor for the feasibility of this type of repair.12 13 17
Among patients with noncomplex forms of DORV and noncommitted VSD, an intraventricular baffle repair was
still possible in the majority of cases. All VSDs in this group were anatomically rather than surgically
noncommitted,18 with no case in which interposed tension apparatus of the atrioventricular valves prevented
biventricular repair. In this series, none of the patients with complex forms of DORV who had an associated
atrioventricular septal defect died after definitive repair. However, in more than half of such cases a
cavopulmonary shunt or modified Fontan operation was chosen because of associated atrial isomerism with
some degree of ventricular imbalance.

Risk Factors for Hospital Mortality

Aortic arch obstruction. Aortic arch obstruction was a significant risk factor for hospital mortality in the analysis
of all patients combined and was an associated finding in more than half of patients who died after palliative
procedures. The majority of patients with aortic arch obstruction underwent surgical arch repair as a separate
palliative procedure. Recently, better survival probability has been reported in children with aortic arch obstruction
and associated complex intracardiac lesions if repair has been undertaken as a one-stage procedure19 20 21 ; this
is now our preferred approach in noncomplex forms of DORV with associated aortic arch obstruction that includes
hypoplasia of the aortic arch proximal to the left subclavian artery or complete interruption. When a localized
coarctation is present, this would normally be dealt with through a left thoracotomy as a separate procedure.
Multiple ventricular septal defects. By multivariate risk factor analysis of all patients surviving to definitive

surgery, the presence of multiple ventricular septal defects was the only factor related to early mortality. Similar
results were reported by Aoki et al13 in an analysis of patients with DORV undergoing biventricular repair. In most
cases the presence of additional VSDs does not preclude successful biventricular repair. However, complex forms
of DORV remain a challenge, and the optimal surgical approach needs to be individualized according to the
specific anatomic features present, including the number and site of any additional VSDs. The advent of newer
surgical and interventional catheter techniques22 23 may further facilitate biventricular repair and improve the
results in this group of patients.
Early surgical repair. Patients who underwent definitive repair before 1 month of age were also at significantly
higher risk in the present series, but this group tended to include patients with unfavorable anatomic variants.
Early definitive repair is seldom required in most patients with DORV and is probably best avoided in patients with
complicating anatomic features.

Role of the Modified Fontan Procedure

The use of a cavopulmonary shunt or modified Fontan procedure has previously been advocated as the surgical
procedure of choice in the presence of complex anatomic features such as straddling AV valves or ventricular
imbalance. In recent years, surgical modifications with staged procedures and atrial baffle fenestration have been
associated with a considerable improvement in the short-term and medium-term results for the modified Fontan
operation.3 4 5 In the present series there was no hospital mortality among any patients undergoing a
cavopulmonary shunt or modified Fontan procedure as the definitive surgical procedure for complex forms of
DORV. The difference in early mortality between patients with complicating anatomic features undergoing
biventricular repair and those subjected to some form of Fontan procedure strongly suggests that the latter is the
procedure of choice, particularly among those with surgically inaccessible multiple VSDs.

Late Results and Requirements for Reoperation

Arrhythmias have been recognized as a cause for late deaths in patients after DORV repair with increasing
surgical age, postoperative ventricular tachyarrhythmias, and complete heart block as significant risk factors.7
Indications for permanent cardiac pacing should therefore include the development of complete or prolonged
postoperative heart block, which is often poorly tolerated in the presence of even a minor residual hemodynamic
disturbance.
The 5- to 10-year survival and freedom from reoperation in the present series compare favorably with other
reported series in which patients with complex DORV have not always been included in the analysis.13 24 It has
been suggested that patients with subaortic and noncommitted VSD are at low and high risk, respectively, for
reoperation.13 In the present series there were no statistically significant associations with reoperation, including
the site of the primary ventricular septal defect and other complicating anatomic features.

Study Limitations
The classification of varying anatomic DORV subtypes into simple and complex forms has, to some extent, been
arbitrary. However, this had no bearing on risk factor analysis, in which anatomic variables were considered
separately, regardless of whether the anatomy was regarded as complex or noncomplex. This study has the
inherent limitations of a retrospective review in which patients were not randomly allocated to varying treatment
groups. Risk factor analysis is, by its nature, predicated on outcome, which may in turn be influenced by varying
treatment options. Despite this, the lower hospital mortality among patients with complex DORV who underwent a

modified Fontan procedure rather than an attempt at biventricular repair indicates that the latter is a worthwhile
option in this difficult subset of patients.

Conclusions
The surgical procedures in a cohort of consecutive patients with DORV and a wide range of anatomic features
have been examined. Early mortality was low among patients with noncomplex forms of DORV undergoing
biventricular repair but was higher in patients with complicating anatomic features undergoing similar surgery.
Risk factors for early mortality identified from multivariate analysis include aortic arch obstruction, the presence of
multiple VSDs, and definitive surgical repair before 30 days of age. A modified Fontan procedure is the treatment
of choice for many patients with complex forms of DORV, particularly in the presence of multiple ventricular septal
defects.

Acknowledgments
The authors wish to thank Dr John Carlin for assistance with statistical analysis.

Footnotes
Presented in part at the 44th Annual Scientific Session of the American College of Cardiology, New Orleans, La,
March 1995.
Received October 2, 1996.
Revision received March 12, 1997.
Accepted March 18, 1997.
Copyright 1997 by American Heart Association

References
1. Kirklin JW, Harp RA, McGoon DC. Surgical treatment of origin of both vessels from right ventricle, including cases of pulmonary
stenosis. J Thorac Cardiovasc Surg. 1964;48:1026-1036.
2. Russo P, Danielson GK, Puga FJ, McGoon DC, Humes R. Modified Fontan procedure for biventricular hearts with complex forms
of double-outlet right ventricle. Circulation. 1988;78(suppl III):III-20-III-25.
3. Pearl JM, Laks H, Drinkwater DC, Capouya ER, George BL, Williams RG. Modified Fontan procedure in patients less than 4 years
of age. Circulation. 1992;86(suppl II):II-100-II-105.
4. Bridges ND, Castaneda AR. The fenestrated Fontan procedure. Herz . 1992;17:242-245.
5. Jacobs ML, Norwood WI Jr. Fontan operation: influence of modifications on morbidity and mortality. Ann Thorac Surg.
1994;58:945-952.
6. Musumeci F, Shumway S, Lincoln C, Anderson RH. Surgical treatment for double-outlet right ventricle at the Brompton Hospital,
1973-1986. J Thorac Cardiovasc Surg. 1988;96:278-287.
7. Shen WK, Holmes DR Jr, Porter CJ, McGoon DC, Ilstrup DM. Sudden death after repair of double outlet right ventricle.

Circulation . 1990;81:128-136.
8. Vogt PR, Carrel T, Pasic M, Arbenz U, von Segesser LK, Turina MI. Early and late results after correction for double-outlet right
ventricle: uni- and multivariate analysis of risk factors. Eur J Cardiothorac Surg. 1994;8:301-307.
9. Kirklin JW, Pacifico AD, Blackstone EH, Kirklin JK, Bargeron LM Jr. Current risks and protocols for double-outlet right ventricle. J
Thorac Cardiovasc Surg. 1986;92:913-930.
10. Wernovsky G, Mayer JE Jr, Jonas RA, Hanley FL, Blackstone EH, Kirklin JW, Castaneda AR. Factors influencing early and late
outcome of the arterial switch operation for transposition of the great arteries. J Thorac Cardiovasc Surg. 1995;109:289-301.
11. Serraf A, Bruniaux J, Lacour-Gayet F, Sidi D, Kachaner J, Bouchart F, Planche C. Anatomic correction of transposition of the
great arteries with ventricular septal defect: experience with 118 cases. J Thorac Cardiovasc Surg . 1991;102:140-147.
12. Kirklin JK, Pacifico AD, Kirklin JW. Intraventricular tunnel repair of double outlet right ventricle. J Card Surg . 1987;2:231-245.
13. Aoki M, Forbess JM, Jonas RA, Mayer JE Jr, Castaneda AR. Result of biventricular repair for double outlet right ventricle. J
Thorac Cardiovasc Surg. 1994;107:338-350.
14. Lev M, Bharati S, Meng CCL, Liberthson RR, Paul MH, Idriss F. A concept of double-outlet right ventricle. J Thorac Cardiovasc
Surg. 1972;64:271-281.
15. Luber JM, Castaneda AR, Lang P, Norwood WI. Repair of double-outlet right ventricle: early and late results. Circulation.
1983;68(suppl II):II-144-II-147.
16. Kawashima Y, Matsuda H, Yagihara T, Shimazaki Y, Yamamoto F, Nishigaki K, Miura T, Vemura H. Intraventricular repair for
Taussig-Bing anomaly. J Thorac Cardiovasc Surg . 1993;105:591-596.
17. Sakata R, Lecompte Y, Batisse A, Borromee L, Durandy Y. Anatomic repair of ventriculoarterial connection associated with
ventricular septal defect, I: criteria of surgical decision. J Thorac Cardiovasc Surg. 1988;95:90-95.
18. Stellin G, Ho SY, Anderson RH, Zuberbuhler JR, Siewers RD. The surgical anatomy of double-outlet right ventricle with
concordant atrioventricular connection and noncommitted ventricular septal defect. J Thorac Cardiovasc Surg. 1991;102:849-855.
19. Planche C, Serraf A, Comas JV, Lacour-Gayet F, Bruniaux J, Touchot A. Anatomic repair of transposition of great arteries with
ventricular septal defect and aortic arch obstruction: one-stage versus two-stage procedure. J Thorac Cardiovasc Surg.
1993;105:925-933.
20. Conte S, Lacour-Gayet F, Serraf A, Sousa-Uva M, Bruniaux J, Touchot A, Planche C. Surgical management of neonatal
coarctation. J Thorac Cardiovasc Surg. 1995;109:663-674.
21. Karl TR, Sano S, Brawn W, Mee RB. Repair of hypoplastic or interrupted aortic arch via sternotomy. J Thorac Cardiovasc Surg .
1992;104:688-695.
22. Leca F, Karam J, Vouhe PR, Khoury W, Tamisier D, Bical O, DaCruz E, Thibert M. Surgical treatment of multiple ventricular
septal defects using a biological glue. J Thorac Cardiovasc Surg . 1994;107:96-102.
23. Bridges ND, Perry SB, Keane JF, Goldstein SA, Mandell V, Mayer JE Jr, Jonas RA, Castaneda AR, Lock JE. Preoperative
transcatheter closure of congenital muscular ventricular septal defects. N Engl J Med . 1991;324:1312-1317.
24. Serraf A, Lacour-Gayet F, Bruniaux J, Losay J, Petit J, Touchot-Kone A, Bouchart F, Planche C. Anatomic repair of Taussig-Bing
hearts. Circulation. 1991;84(suppl III):III-200-III-205.

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