INTRODUCTION

Double-outlet right ventricle is a heart condition present at birth. That means it's a
congenital heart defect. In this condition, the body's main artery and the lung artery do not
connect to the usual areas in the heart. The body's main artery is called the aorta. The lung
artery is called the pulmonary artery. Sometimes these blood vessels also are reversed from
their usual positions.
In a typical heart, the aorta connects to the left lower heart chamber. The pulmonary
artery connects to the right lower heart chamber. In babies with double-outlet right ventricle,
both the aorta and the pulmonary artery connect partially or completely to the right lower
heart chamber.
Babies with double-outlet right ventricle also have a hole between the lower heart
chambers. The lower heart chambers are called the ventricles. The hole is called a ventricular
septal defect. The hole causes oxygen-rich blood to mix with oxygen-poor blood. Babies with
this condition may not get enough oxygen in the bloodstream. Their skin may appear gray or
blue. Double-outlet right ventricle may occur with other heart problems present at birth.
These problems may include other holes in the heart, heart valve problems or blood vessel
problems.
DEFINITION
Double outlet right ventricle (DORV) is a controversial and fascinating cardiac
malformation encompassing a wide spectrum of anatomic arrangements and
pathophysiological disturbances.
Kirklin and Barrott-Boyes defined DORV as a congenital cardiac anomaly in which
both great arteries (aorta and pulmonary artery) arise wholly or in large part from the right
ventricle (RV). Within this broad definition there exist several distinct subtypes needing
precise description and management.
HISTORY
The earliest report of DORV was published in French literature in 1703, but it was
unnoticed for 90 years. Double outlet right ventricle like condition for the first time was
described in 1793 by Abernethy of St Bartholomer's hospital. But the name DORV was
introduced by Witham in 1957. Prior to this nomenclature Verordt in 1898 named this
anomaly known as "partial transposition." Pulmonary artery banding was first described by
Muller and Dammon in 1952.
INCIDENCE
The estimated incidence is 0.09 per 1000 live births and 4.5% of necropsy cases of
congenital heart diseases. Chromosomal abnormalities are often associated with DORV.
There is a higher incidence of DORV in patients with trisomy 13 and trisomy 18. Male to
female ratio is approximately 1.7 to 1.
PREVALENCE
Double-outlet right ventricle (DORV) occurs in fewer than 1% of all CHDS DORV
occurs frequently in patients with heterotaxy in association with other complex cardiac
defects.
EMBRYOLOGY
The exact embryogenesis of DORV is poorly understood. It is an abnormal
ventriculoarterial connection occurring in the early stages of ventricular loop formation and
infundibular development. During Streeter horizon 15, bulboventricular malformations give
rise to DORV. Variability in the size of the ventricular inflow and outlet septum decides the
location of ventricular septal defect (VSD) and relation to the great arteries. Defective
absorption of the subaortic conal free wall gives rise to aortic-mitral discontinuity. Another
view is that discordant conal development relative to the ventricular development results in
this anomaly. It is a spectrum of transposition complex.
ABNORMAL ANATOMY
Double outlet right ventricle is almost always associated with visceral situs solitus and
atrioventricular (AV) concordance. As the name suggests both the great arteries arise entirely
or predominantly from a well-developed RV. Invariably a well- developed left ventricle (LV)
is also present. To assign both the great arteries to any one ventricle, one great artery and at
least half of the other great artery have to be related to that ventricle. The three essential
components in this complex condition that need elaboration are (a) the relationship of the 1
great arteries to the ventricles, (b) the relationship of the VSD to the great arteries and (c) the
presence or absence of right ventricular outflow obstruction. Besides, other associated
anomalies should also be identified

There are four types of relationship of the great arteries to the ventricle: (i) aorta to the right
and posterior to the pulmonary trunk (normal arrangement), (ii) side-by-side great arteries
with aorta to the right of pulmonary artery, (iii) aorta to the right and anterior to the
pulmonary artery (d-malposition), (iv) aorta to the left and anterior to the pulmonary artery
(I-malposition). The second arrangement is the most common one. The VSD can be located
at four possible locations with respect to the great arteries, namely subaortic (50%),
subpulmonic (35%), doubly committed (10%) and noncommitted (5%). The noncommitted
VSD is found either in the muscular or in the inlet septum. Ventricular septal defect is
generally nonrestrictive and in a few cases multiple VSDs are observed. Taking the above 2
variables, 16 possible subtypes of DORV can be expected; the most frequent one being
DORV with a subaortic VSD and side- by-side relationship of the great arteries with the aorta
directly to the right of pulmonary artery.
Subaortic obstruction is seen in one-third of the cases. Pulmonic stenosis can be
observed in half to two-thirds of the patients and the valve is usually bicuspid. The
obstruction may be subpulmonic, valvar or both. The underdevelopment of respective conus
with malalignment of the infundibular septum leads to subaortic/subpulmonic obstruction.
The actual obstruction to the outflow may be at the VSD that is the only outlet to LV.
Decrease in size of this defect may end up in complete closure in a few cases. Congenitally
intact septum resulting in a hypoplastic LV is an extremely rare occurrence.
Presence of bilateral infundibuli (double conus), more than 50% aortic override and
aortic-mitral discontinuity are important morphological clues that help in differentiating
DORV from its close mimics; however they are not absolute prerequisites for the diagnosis.
Associated anomalies like hypoplasia of aortic arch, isthmic hypoplasia, patent ductus
arteriosus (PDA), interruption of aortic arch, juxtaposition of the atrial appendages, atrial
septal defect, variation in coronary artery course and AV valve abnormalities may coexist and
influence the hemodynamics and the prognosis.
Double outlet left ventricle (DOLV) is one of the rarest of abnormal ventriculoarterial
alignments and is the converse of DORV, that is, both great arteries arise entirely or
predominantly from the LV. The VSD may be subaortic, subpulmonic or doubly committed.
Obstruction to the ventricular outflow may or may not be present. Clinical picture varies
widely from high flow to decreased flow.
CLASSIFICATION
Different authors have postulated different classifications of DORV.
1. Neufeld postulated a physiological classification based on the presence of pulmonary
stenosis (PS) and position of VSD in 1961.
2. McGoon postulated a surgical classification in 1968.
3. Lev and Bharati proposed a clinical classification of DORV in 1972, which is widely
accepted due to its simplicity and functional utility. It is as follows:
A. Subaortic VSD (Commonest type)
1. Without pulmonic stenosis
a. Low pulmonary vascular resistance (PVR)
b. High PVR.
2. With pulmonic stenosis
B. Subpulmonic VSD
1. Without pulmonic stenosis (Taussig-Bing anomaly)
a. Low PVR
b. High PVR
2. With arch anomalies and subaortic stenosis.
C. Doubly committed VSD (Sub-aortic and sub-pulmonary type, close to the semilunar
valves).
D. Noncommitted VSD (Remote type distant from both semilunar valves).
E. Intact ventricular septum/closed VSD.
HEMODYNAMICS
The major hemodynamic determinants are intracardiac mixing at the location of the VSD,
presence or absence of pulmonic stenosis, PVR. Additional defects like ASD, sub aortic
stenosis and AV septal defect also influence the ultimate hemodynamics. The physiological
consequences fall into one of the four clinical patterns.
1. In those with subaortic VSD, no pulmonic stenosis and low PVR resemble a
nonrestrictive perimembranous VSD allowing the left ventricular blood streaming into the
aorta with adequate or increased pulmonary blood flow (PBF). The arterial saturation is
normal
2. Subsequently with significant rise in PVR, PBF falls and arterial saturation decreases.
This clinical form resembles Eisenmenger's complex.
3. When pulmonic stenosis present clinical pattern resembles tetralogy of Fallot (TOF),
However, anatomically VSD in this type of DORV forms a part of the LV outflow (a
different entity for the surgeon).
4. When subpulmonic VSD exists with no pulmonic stenosis, the physiology resembles
complete transposition of the great arteries (d-TGA) known as Taussig-Binganomaly.
There is admixture of pulmonary and systemic venous blood flow with increase in
saturation and pulmonary blood flow. Systemic desaturation occurs because the systemic
venous inflow is directed to the aorta and LV outflow is directed to the pulmonary trunk
in the setting of Taussig-Bing anomaly. Systemic pressures in LV, RV and main
pulmonary artery (MPA) are equal in absence of PS. With this pattern, coarctation of
aorta and aortic arch anomalies may be associated further augmenting the PBF leading to
LV volume overload. Subsequently, a significant rise in PVR occurs, which diverts RV
blood into aorta enhancing systemic desaturation.
PATHOLOGY
1. Both the aorta and the PA arise from the RV. The only outlet from the LV is a large VSD
2. The great arteries usually lie side by side. The aorta is usually to the right of the PA,
although one of the great arteries may be more anterior than the other. The aortic and
pulmonary valves are at the same level. Conus septum is present between the aorta and
the PA. The subaortic and sabpulmonary coni separate the aortic and pulmonary valves
from the tricuspid and mitral valves, respectively. This means that there is no fibrous
continuity between the semilunar valves and the AV valves. In a normal heart, the aortic
valve is lower than the pulmonary valve, and the aortic valve is in fibrous continuity with
the mitral valve
3. The position of the VSD and the presence or absence of PS (or RVOT obstruction)
influence hemodynamic alterations and form the basis for dividing the defect into the
following types of DORV
a. Subaortic VSD. The VSD is closer to the aortic valve than to the pulmonary valve and
lies to the right of the conus septum. This is the most common type, occurring in 55% to
70% of cases
b. Fallot . about 50% of patients with subaortic VSD, RVOT obstruction occurs RVOT
obstruction is most commonly caused by infundibular stenosis, but rarely pure valvular
PS can occur with small annulus.
c. Subpulmonary VSD (Le., Taussig-Bing anomaly). The VSD is closer to the pulmonary
valve than to the aortic valve, and it usually lies above the crista supraventricularis and
to the left of the conus septum. This type accounts for approximately 10% to 30% of
cases.
d. Doubly committed VSD. The VSD is closely related to both semilurar valves and is
usually above the crista supraventricularis (<5% of cases)
e. Noncommitted (or remote) VSD. The VSD is clearly away from the semilunar valves
(10% of cases) It most commonly represents the AV canal-type VSD and occasion- ally
an isolated muscular VSD. Atrial isomerism is commonly seen with this type
4. Sometimes surgeons and pathologists definitions of DORV are different and are the
source of confusion. Some cases of TOF with marked overriding of the aorta may be
called DORV by surgeons because the nutral-aortic fibrous continuity is not always clear
 in the operating room. Surgeons use the so-called 50% rule when the aortic annulus
overhes the RV by at least 50%, it is called DORV

PATHOPHYSIOLOGY AND CLINICAL MANIFESTATIONS

The pathophysiology and clinical manifestations of DORV are determined primarily by the
position of the VSD and the presence or absence of PS. Each type is presented separately
1. Subaortic VSD without PS. In subaortic VSD, oxygenated blood from the IV is directed
to the aorta, and desaturated systemic venous blood is directed to the PA, thereby
producing mild or no cyanosis. The PBF increases in the absence of PS, and CHF may
result. Therefore, the clinical pictures of this type resemble those of a large VSD with
pulmonary hypertension and CHF.
a. Growth retardation, tachypnea, and other signs of CHF are usually present. A hyper
active precordium, a loud S2, and a VSD-type (holosystolic or early systolic) marmur
are present. An apical diastolic rumble may be audible
b. The ECG often resembles that of complete endocardial cushion defect (ECD). "Superior
QRS axis (ie.. -30 to-170 degrees) may be found in this type. RVH or BVH as well as
LAH, is common. Occasionally, first-degree AV block is present
c. Chest radiography shows cardiomegaly with increased pulmonary vascular mark ings
and a prominent PA segment
2. Subaortic VSD with PS (Fallot type). Even though the VSD is subaoric, in the presence
of PS (or RVOT obstruction), some desaturated blood goes to the aorta. This causes
cyanosis and a decrease in PBF. The clinical pictures resemble those of TOF.
a. Growth retardation and cyanosis are common. The 52 is loud and single. A grade 2 to 4
of 6 midsystolic (ejection) murmur along the left sternal border is present. either with or
without a systolic thrill
b. The ECG shows RAD, RAH, RVH, or RBBB First-degree AV block is frequent
c. Chest radiography shows normal heart size with an upturned apex Pulmonary vas
cularity is decreased
3. Subpulmonary VSD (Taussig-Bing malformation). In subpulmonary VSD, or Taussig
Hing malformanon, coxygenated blood from the IV as dovcted to the PA, and desasard
blood from the systeme vein is directed to the aorta. This results in severe cyanosis. The
PBF increases with the fall of the PVR. Clinical pictures resenible those of complete
TGA
a. Growth retardation and severe cyanosis with or without clubbing ate common findings.
The S2 is loud, and a grade 2 to 3 of 6 systolic murmur is audible at the upper left
sternal border. An ejection click and an occasional PR murmur (as a result of
pulmonary hypertension) may be audible
b. The ECG shows RAD, RAH, and RVH LVH may be seen during infancy
c. Chest radiography shows cardiomegaly with increased pulmonary vascular mek ings
and a prominent PA segment
4. Doubly commuted or noncommitted VSD With the VSD close to both semilunar valves
(called doubly committed VSD) or remotely located from these valves noncommitted
VSD), cyanosis of a mild degree is present, and the PBF increases.

ECHOCARDIOGRAPHY
Three diagnostic signs of DORV are the origin of both great arteries from the anterior RV the
absence of LV outflow other than the VSD, and the discontinuity of the mitral and semilunar
valves
1. In the parasternal long-axis view, all three diagnostic features of DORV are imaged.
Typical subaortic or subpulmonary VSD can be demonstrated in this view for most patients.
No great artery is seen to arise from the posterior ventricle. The great arteries arising from the
anterior ventricle are seen in parallel orientation. In addition, a mass of echocardiography
positive tissue, usually larger than 3 mm in length, is present between the mitral valve
annulus and the semilunar valve (ie, mitral-semilunar discontinuity)
2. In the parasternal short-axis view, a double circle, rather than the normal circle and sausage
appearance of the great arteries, may be seen. Either the great arteries are side by side with
the aorta to the right or the aorta is anterior and slightly to the right of the PA
3. The size and position of the VSD should be determined in relation to the great arteries
a. Typical subpulmonary or subaortic VSD can be demonstrated by parasternal long axis
scanning in most patients
b. In the subcostal four-chamber view, the subaortic VSD is located to the right of the
conus septum just beneath the aortic valve. The subpulmonary VSD is located to the left
of the conus septum just beneath the pulmonary valve
c. Doubly communed VSD is recognized in the parasternal or the apical long-aos view
d. Noncommuted (remote) VSDs, either endocardial cushion type of apical muscular
VSD, are best recognized in the apical four-chamber view
4. Associated anomalies such as valvular or subvalvular PS and other left-to-right shunt
lesions (eg, ASD, PDA ) should be looked for.
5. Occasionally, differentiation of DORV from TOF with a marked overriding of the aorta
or from TGA is necessary There is mitral-semilunar continuity in TOF and TGA (De
mitral-aortic continuity in TOF and mitral-pulmonary continuity in TGA), but no mitral-
semilunar continuity is present in DORV
OTHER STUDIES
Cardiac catheterization and angiocardiography are not necessary for initial surgical
management of the condition with PA banding or BT shunt. They are indicated to perform
atrial septostomy in patients with Taussig-Bing malformation with inadequate atrial septal
mixing. They are usually indicated before later stage operations
NATURAL HISTORY
1. Infants without PS may develop severe CHF and later pulmonary vascular obstructive
disease if left untreated Spontaneous closure of VSD, which is fatal, is rare
2. When PS is present, complications common to cyanotic CHDs (eg, polycythemia,
cerebrovascular accident) may develop.
3. In patients with the Taussig-Bing malformation, severe pulmonary vascular obstructive
disease develops early in life, as seen in patients with D-TGA
4. Associated anomalies (e g.. COA, LV hypoplasia) also contribute to the poor prognosis
MANAGEMENT
Medical
Treatment of CHF with diuretics, ACE inhibitors, and digoxin is indicated
Surgical
Palliative Procedures
1. PA banding for symptomatic infants with increased PBF and CHF is occasionally per-
formed in infants with multiple muscular VSD or a remote VSD. However, this proce-
dure is not recommended for infants with subaortic VSD or doubly committed VSD
Primary repair is a better choice.
2. For infants with the Taussig-Bing type, enlarging the interatrial communication is
important for better mixing and for decompressing the LA, which causes pulmonary
venous congestion. Balloon or blade atrial septostomy should be considered
3. In infants with PS and decreased PBF with cyanosis, a systemic-to-PA shunt procedure is
occasionally indicated
Definitive Surgeries
1. Suhaortic or doubly committed VSD. An intraventricular tunnel between the VSD and the
subaortic outflow tract is created by means of a Dacron patch. This procedure is
performed early in life, preferably during the neonatal period or at least in early infancy,
without preliminary PA banding. Sometimes the RVOT may have to be augmented with
an outflow patch if the VSD-AO tunnel obstructs the RV outflow tract. The mortality rate
is less than 5% for simple subaortic VSD, it is slightly higher for doubly committed VSD
2. Fallot type. There are three surgical options. Surgical repair is generally advised by 6
months of age, preferably during the neonatal period. However, if the patient's condition
is poor or if there are major associated noncardiac anomalies, an initial shunt operation is
an option.
a. Tunnel VSD closure + Rastelli operation. An intraventricular tunnel between the
VSD and the aorta is established, and a Rastelli operation is performed to relieve PS
using either a pulmonary or aortic homograft conduit
b. REV procedure, So-called reparation a l'etage ventriculare (REV), which is similar
to ASO, may be performed. In the REV procedure, the proximal ascending aorta and
main PA are transected, and the proximal stump of the PA is oversewn, which is
performed for D-TGA VSD PS, a situation very similar to this one). The pulmonary
 arteries are translocated anterior to the aorta (Lecompte maneuver), and the ascending
aorta is reconnected. The distal PA is anastomosed directly to the upper margin of the
infundibular incision. Autologous pericardium forms the ante nor portion of the
pathway. The hospital mortality rate is 18%.
c. Nikaidoh procedure. This combines the principle of the Ross procedure and the
Konno operation. The aortic root including the aortic valve is detached in the same
manner as done to the pulmonary root in the Ross procedure. The PA is divided, and
the pulmonary valve is excised. The pulmonary root is divided, and the conal septum
above the VSD is excised, which creates a large opening to the LV cavity. The aortic
root is translocated posteriorly and sutured to the open orifice of the pulmonary
annulus. A pericardial patch is used to connect the lower margin of the VSD and the
anterior circumference of the harvested aortic root, completing the LV to AO
connection. A pericardial gusset completes the connection of the RV and the distal
end of the MPA, which has been described for D-TGA VSD PS)
3. Taussig-Bing anomaly (subpulmonary VSD). There are four possible surgical approaches
These operations should be carried out by 3 to 4 months of age or sooner because of the
rapid development of pulmonary vascular obstructive disease in this subtype
a. The procedure of choice is the creation of an intraventricular tunnel between the VSD
and the PA (resulting in TGA), which is then corrected by the ASO. The mortality rate
is between 5% and 15%
b. Creation of an intraventricular tunnel between the VSD and the PA is followed by the
Senning operation. This is a less desirable approach because of a high mortal ity rate
(>40%) and a higher late complication rate associated with the Senning procedure.
c. An intraventricular tunnel herween the VSD and the aorta is desirable but often
technically impossible. The surgical mortality rate is about 15%.
d. Creation of a VSD-to-PA tunnel followed by the Damus-Kaye-Stansel operation and
RV-to-PA conduit is another possibility.
4. Noncommuted VSD When possible, an intraventricular tunnel procedure between the AV
canal-type VSD and the aorta is performed, but the mortality rate is high (30%- 40%). PA
banding is usually needed in infancy to control CHF, and the surgery may be delayed
until 2 to 3 years of age.
POSTOPERATIVE FOLLOW-UP
Long-term, regular follow-up at 6-to 12-month intervals is necessary to detect and manage
late complications of surgery
1. In general, patients who had subaortic VSD without PS have an excellent long-term
outlook
2. Ventricular arrhythmia should be treated because it may cause sudden death.
3. About 20% of patients require reoperation of the intraventricular tunnel.
TETRALOGY OF FALLOT LIKE PRESENTATION
Clinical Features
The clinical features depend on the severity of the PS. The cyanosis can be present at
birth when PS is severe or is delayed for some weeks to months and then becomes
progressive. At later age, squatting and cyanotic spells are seen in some cases. Polycythemia
and failure to thrive are other important presenting features in young children. Arterial and
jugular venous pulses are normal. The precordial impulse is quiet and RV type. At times, a
systolic thrill is appreciated along with a pulmonic systolic murmur whose duration varies
inversely with the degree of stenosis
Less commonly when the pulmonic stenosis is very mild the child is acyanotic with a
holosystolic murmur at the left lower sternal horder. Such patients may show a delayed
pulmonary component of second heart sound and an apical mid-diastolic rumble. On the
other extreme if pulmonary atresia is present, an aortic ejection click, a soft aortic mid
systolic murmur and a loud aortic component of second heart sound may be heard. In
exceptional cases, a long decrescendo systolic murmur is heard at the left lower sternal
border due to the obligatory shunt via a restrictive VSD. In this setting, a fourth heart sound
is commonly audible.
Investigations
Electrocardiography
Electrocardiographic features of DORV with PS are peaked P in lead II (right atrial
enlargement), right axis deviation and right ventricular hypertrophy (RVH) resembling TOF
But prolonged pulmonary regurgitation (PR) interval counterclockwise loop, RVH having q8
pattern in VV q in I and aV2, and broad and slurred S in V, V, are very characteristic features
of DORV with TOF like presentation that differentiate it from classical TOF (Fig. 30.2),
Radiography
Chest X-ray shows a normal-sized heart with rounded or boot shaped apex (Fig. 30.3).
The pulmonary arterial vascularity may be normal or reduced. The ascending aorta is dilated,
but MPA is inconspicuous
Echocardiography
Two-dimensional (2D) echocardiogram with Doppler flow imaging is the most vital
investigation for confirming the diagnosis and delineating the anatomical structures which are
helpful to the surgeons. Subxiphoid view helps in visualization of both great arteries arising
from RV and the type and severity of PS. Short and long-axis views delineate pulmonary
valve, aortic valve and the VSD. It also helps to determine the great arterial relation and type
of VSD. Mitral- aortic discontinuity, which is an important feature of DORY, is well
visualized in long-axis view (Fig. 30.4). Doppler interrogation is useful in estimating severity
of PS. The echocardiographic measurements of the distance between the pulmonary and
tricuspid valves, aortic diameter and coronary artery anatomy are necessary for planning
surgical correction. Transesophgeal echo (TEE) delineate more clearly the position of VSD
and its relationship to great arteries along with extra cardiac anomalies. Real-time three-
dimensional (3D) echocardiogram is a better mode for diagnosis when available.
Cardiac Catheterization
Although DORV is an admisture lesion, due to variable streaming of blood, systemic
saturation may be misleading It warrants and invasive study to calculate PBF and resistance
accurately. The standard protocol of cardiac catheterization is from femoral approach. The
catheter is passed from RA to RV and from RV to aorta and pulmonary artery. In presence of
PS, the PA pressures are reduced and a pressure gradient is demonstrated across the stenosis.
Simultaneous opacification of both great arteries and commitment of aorta to RV (rather than
TOF like aortic override of interventricular septum) can often be demonstrated on RV
angiogram. In this RV angiogram, the location of VSD and type of pulmonic stenosis can be
delineated. Sometimes, RV angiogram in frontal projection may show a well-developed
conus or an infundibular outlet septum, which is abnormally oriented giving rise to teardrop
appearance. Left ventricular angiogram in axial projection also delineates the origin of great
arteries. Bilateral muscular infundibuli are seen characteristically in this condition. Double-
chambered RV is seen in some cases.
CT and MRI
Multidetector cardiac computed tomography (CT) and cardiac magnetic resonance
imaging (MRI) are very accurate in diagnosis of DORV, relationship of great arteries to VSD
and associated anomalies.
Diagnosis
A cyanotic infant with features sof RV dominance, normally split second sound and a
long systolic murmur at lower sternal border suggests DORV. The characteristic electro-
cardiography (ECG) findings like prolonged PR interval and counterclockwise initial forces
(Q waves in leads I and aVL) in spite of a vertical or rightward axis and prolonged terminal
forces (slurred S waves in leads I, aVL and V V with a broad R' in aVR) are suggestive of
DORV. Echocardiogram and angiogram confirm the diagnosis.
Differential Diagnosis
Anomalies having cyanosis from infancy but without any signs of congestive heart
failure (CHF) can be included in the differential diagnosis of this type of DORV. They are
mainly TOF, single ventricle with PS, tricuspid atresia and d-TGA-VSD- PS. Besides clinical
signs and symptoms, echocardiogram and angiogram are necessary for final diagnosis.
"TAUSSIG-BING ANOMALY" PRESENTATION
The anatomic, physiologic and clinical features were descri- bed in detail for the first
time by Taussig and Bing in 1949, This anomaly resembles complete TGA with
nonrestrictive VSD. Nonrestrictive subpulmonic VSD and great arteries side by side with
aorta slightly anterior and right to pulmonary artery is common. Absence of pulmonic
stenosis is almost uniform. Coarctation of aorta, subaortic stenosis and a PDA are frequent
associations. Van Praagh considered Taussig- Bing malformation a form of DORV with a
subpulmonic VSD and bilateral muscular infundibuli.
Clinical Features
Cyanosis is often present from birth. It varies from mild to severe de machyphens
suffer from repeated chest infections and CHF. and grunting are common. The infants are
underweight and development is delayed. At a later stage with the development of pulmonary
vascular disease some of them become apparently asymptomatic but cyanosis and clubbing
may be prominent. In rare cases a typical reverse differential cyanosis (fingers thore cyanotic
than toes) may be seen when associated with a patent ductus. It is pathognomic when present
and it occurs due to suprasystemic PVR that reverses the ductal shunt (oxygenated LV blood
goes to pulmonary artery, then ductus and to descending aorta). If coarctation is present early
congestive failure occurs in the first week of life and the neonate becomes very sick.
Peripheral pulses are normally felt. Femoral pulses may be diminished in presence of
coarctation of aorta. A large ductus distal to coarctation may mask this sign. Jugular venous
pressure wave pattern is not clearly appreciated in neonates and young infants because of
tachycardia and short neck. Precordium is pulsatile, first heart sound is normally heard, S, is
closely split (P, loud) and ejection click over left precordium (pulmonary. due to dilatation of
MPA) is audible. Ejection systolic murmur grade 2-3/6 over upper sternal border (pulmonary
flow murmur), pansystolic murmur (PSM) (due to VSD) over the second and the third sternal
border and in occasional cases a mid-diastolic murmur over apex (flow murmur) is audible.
Subsequently when the infant grows and attains late childhood these signs of high flow
pulmonary circulation changes to decreased flow group, because PVR increases. With the
development of pulmonary vascular disease, the failure ameliorates; apical impulse is less
conspicuous, thrill disappears and RV impulse becomes more gentle. Second heart sound
becomes single and loud. Graham-Steell murmur may be heard.
Investigations
Electrocardiography
Clockwise loop with vertical or right axis of frontal QRS is common (not left axis
deviation that is common with DORV and subaortic VSD). Pulmonary regurgitation interval
is often normal. Tall peaked right atrial P waves and RVH, evident by tall R waves in leads
V, and deep S waves in left precordial leads are common.
Radiography
X-ray chest in infants reveals cardiomegaly, increased vascularity and a narrow
vascular pedicle if pulmonary artery is not forming the border (egg lying on its side
appearance as seen in d-TGA). However, a prominent pulmonary trunk (MPA) is
characteristically seen unlike cases ofd-TGA. Thymic shadow is commonly present in
Taussig-Bing anomaly (not in d-TGA). With the onset of pulmonary vascular disease. the
heart size becomes near normal with persistence of prominent central pulmonary arteries.
Echocardiography
Cross-sectional echocardiography plays an important role in the diagnosis of Taussig-
Bing type of DORV, In 2D parasternal short-axis view, both great arteries appear as double
circle, aorta being on the right side and slightly ante- rior to pulmonary artery. The other
characteristic pictures are subpulmonic VSD and semilunar-mitral discontinuity. Frequently
coarctation of aorta and PDA are associated echocardiographic findings.
CT and MRI
 Multidetector cardiac CT and cardiac MRI are most helpful in demonstrating clearly
relationship of great arteries and also position of VSD with that of great arteries. It also
delineates associated anomalies like coarctation and functional status of ventricles.
Cardiac Catheterization
The systolic pressures in LV, RV, aorta and pulmonary artery are equal. When
catheter easily enters pulmonary artery and aorta from LV, DORV is a strong possibility.
Oxygen saturation in pulmonary artery in Taussig-Bing anomaly (as in d-TGA) is higher than
aorta and systemic arteries. In other types, PA saturation is higher than aorta but less than
systemic arteries. Angiography shows a high VSD related directly to the pulmonary valve.
Presence of conus between pulmonary valve and anterior mitral leaflet as well as the absence
of conus between VSD and pulmonary valve are considered important angiographic features
of this entity. Aortography including root injections is done to study aortic arch anomalies as
well as coronary arteries.
Diagnosis
With clinical findings like cyanosis from infancy, RV dominance, ejection click
(pulmonary), short ejection systolic murmur over upper and a PSM over lower sternal
bonder, ECG features of right axis and RVH and radiological findings of prominent MPA,
the clinician can consider Taussig-Bing anomaly as a possibility. Early cyanosis with
increased PBF in the presence of coarctation or presence of typical reversed differential
cyanosis (fingers more cyanotic than toes) give a positive clinical clue, for the diagnosis.
Echocardiography and cardiac catheterization with RV angiography confirm the diagnosis.
Differential Diagnosis
The anomalies having cyanosis from infancy, features of RV dominance and ECG
findings of RVH come under differential diagnosis. Common conditions are d-TGA, TOF,
persistent truncus, single ventricle and total anomalous pulmonary venous communication.
Clinical features of Taussig-Bing anomaly and TGA may be similar but radiological features
of prominent pulmonary artery are very suggestive of the former. Persistent truncus may have
a right arch, a bulge in the area of MPA and during cardiac catheterization PA is not easily
entered. In TOF, the MPA is not prominent and pulmonary oligemia and typical cardiac
silhouette suggest the diagnosis Total anomalous pulmonary venous communication is
differentiated by the prominent pulmonary murmur, wide splitting of S, and characteristic. X-
ray finding of figure of "8" appearance.
"NONRESTRICTIVE VSD" LIKE PRESENTATION
Clinical Features
Cyanosis is absent or minimal in these infants with DORV with large VSD as the
saturated LV blood is preferentially directed to aorta and unsaturated RV blood to pulmonary
artery. Most of these infants develop repeated respiratory infections and heart failure
(Tachypnea, tachycardia and hepatomegaly). Failure to thrive may be a dominant feature. A
bulging hyperdynamic precordium, RV apical impulse. palpable dilated pulmonary artery
with a loud second sound (pulmonic) are common presentations. First heart sound is normal
or soft (due to prolonged PR interval). A typical PSM (due to the VSD) is heard over the left
third and the fourth sternal border. At apex a mid-diastolic flow rumble is heard. Even with
the development of pulmonary vascular disease the pulmonary arterial flow is still increased
due to obligatory shunt. With the onset of pulmonary vascular disease, gradually cyanosis
and clubbing appear. Apical impulse is more sustained with quiet precordium. The systolic
murmur becomes less prominent. Pulmonic ejection click, a decrescendo early diastolic
murmur (Graham-Steell murmur) may be audible. In rare cases, pulmonary vascular disease
develops as early as second month of life.

Investigations
Electrocardiography
Sinus rhythm and prolonged PR interval are common findings. Left axis deviation
with counterclockwise loop and absence of splintering of the S waves in inferior leads are
characteristic features of this anomaly. Right ventricular hypertrophy (tall I waves about 15
mm in leads V, and aVR and deep S in left precordial leads) and IV volume overload pattern
(large RS complexes in mid precordial leads and tall R waves in leads V_{5} and V) are
consistent features. Right atrial enlargement is seen in majority of cases. Rise in PVR does
not affect the frontal QRS axis but the precordial leads show pure RVH. Left axis deviation
with counterclockwise loop is a useful clue to distinguish it from Eisenmenger's complex.
Radiography
Cardiomegaly, convex right border (RA enlargement), left atrial and left ventricular
enlargement, prominent MPA and increased PBF pattern are usual radiological features (Fig.
30.6). When pulmonary vascular disease sets in, the lung fields become oligemic, the cardiac
silhouette becomes normal or near normal, the proximal pulmonary arteries are grossly
dilated. In DORV, pulmonary plethora persists with cyanosis, but in Eisenmenger's complex
cyanosis is seen with pulmonary pruning and oligemia.
Echocardiography
Transthoracic echocardiography (TTE, particularly high resolution, 7.5 MHz multi-
format) is very informative in DORV with associated anomalies. Two-dimensional echo-
cardiogram with color Doppler delineates the anatomical details like the visceral and atrial
situs, atrial and ventricular size, their morphology, location and size of VSD, aortic root, its
override, presence or absence of aortic-mitral continuity, presence and absence of either
conus, the origin and spatial relationship of the two great arteries, subaortic/subpulmonic
stenosis, AV valve morphology and straddling and any other associated anomalies.
Particularly the parasternal long-and short-axis views help in visualization of all important
echocardiographic features of DORV like (1) the typical subaortic VSD that acts as the only
outlet for LV, (ii) the double circle appearance of great arteries arising from anteriorly placed
RV and (iii) mitral semilunar discontinuity (better seen in parasternal long-axis view). Real-
time 3D echocardiogram is also helpful in expert hands. Fetal echocardiography in four-
chamber view gives information about presence of VSD and origin of great arteries
Cardiac Catheterization
The recent trend in many advanced centers is to obtain most of the anatomical and
physiological information from echocardiographic examination so as to minimize or avoid
invasive studies in sick infants. Both the ventricles, PA and aorta show equal systolic
pressure. Calculation of PVR index, relative and absolute ventricular volumes and any
gradient across the VSD are important for surgical planning. The side-by-side great arteries
with aorta to the right of pulmonary artery are the most common pattern of great artery
relationship in this type of DORV. The size and location of VSD can be assessed by left
ventriculogram (Fig. 30.8). Selective IV angiogram establishes the diagnosis of DORY. The
characteristic angiographic findings of DORV are (a) opacification of both great arteries from
IIV (PA is usually better opacified), (b) both semilunar valves at the same level, (c) malposed
aorta in the lateral view, (d) tongue-like filling defect in frontal view and (e) aortic mitral
discontinuity. Delineation of coronary arteries is another important indication for
catheterization in complex congenital defects. The origin and course of coronary arteries are
variable in different subtypes of DORV
MRI/CT Scan
Though echocardiogram and cineangiogram give adequate anatomical details needed
for the surgeon, occasionally CT/MR angiogram is done for better visualization of the
pulmonary arteries, ventricular morphology, location of VSD and great artery relationship.
Diagnosis
In infants with clinical findings of large VSD (cardiomegaly, CHE, loud P, and PSM
over lower sternal border) if minimal cyanosis is detected DORV-VSD is a possible
diagnosis. With these clinical findings early heart failure, poor growth and delayed
development and ECG findings of left axis deviation countercloclovise loop with
biventricular enlargement strengthen the diagnosis of DORV with VSD. Echocardiogram and
cardiac catheterization confirm the diagnosis.
Differential Diagnosis
The most important condition to be differentiated is a non- restrictive
perimembranous VSD and VSD associated with complete AV canal defect. When neonatal
cyanosis is present DORV-VSD or complete AV canal defect is suspected but not simple
VSD. After the development of pulmonary vascular disease it may be indistinguishable from
Eisenmenger's complex. Persistent increase of PBF in the presence of cyanosis may suggest
DORV-VSD with pulmonary arterial hypertension (PAH) rather than large VSD with PAH.
Left parasternal soft decrescendo systolic murmur and characteristic ECG with
counterclockwise loop and left axis deviation are additional clues to diagnosis of DORV-
VSD Echocardiogram and cine-angiogram confirm the diagnosis, irrespective of the presence
or absence of PAH.
Associated Anomalies
The common associated lesions with DORV include (1) P monic stenosis/atresia in
70% of cases, (ii) Sub-aortic stenos (ii) Coarctation of aorta, (iv) Interruption of aortic an (v)
AV septal defect, (vi) Cleft mitral valve and (vii) Multip VSDs. Besides these common
associations, DORV associated with dextrocardia and double-chambered RV
Complications
Heart faihire and development of pulmonary vascular diseas are the most important
complications, Deep cyanosis and subsequently polycythemia and hyperviscosity state leads
cerebrovascular accidents. Brain abscess due to right to left shunt is also a common
complication in the group presenting as TOF.

SALIENT FEATURES
1. ln DORV bath the great arteries arise wholly or in large part from IIV
2. Relations of great arteries and location of VSD form the basis of classification. The
comitestest type of abnoстай anatomy is both the sesaels go side Iry side with aortato the
right of PM and a subuonnic VSD
3. Important morphological features are:
I. Presence of double conus, a subaortic contus resids in aortic mitral discontinuity
II. More than 50% of aortic override over the VSD
4. Clinical presentations are susually of three types
I. Large VSD without PS and lose PVR: Mildly cyanotic infants with cardiomegaly,
CHF, loud P2, and PSM over left patasternal border (LPSB), Electrocardiography
shows left axis, counterclockwise loop with boventri cular hypertrophy.
Tichocardiography confirms the diagnonis
II. TOF type (larg VSD with PS) Cyanotic infants with ITV dominance, single S_{2}
and a long systolic murmur over LPSII suggest DORV Electrocardiography with
normal to right asds, q in I and ast, EVIT and starred S in V5, V6 supports the
diagnosis
III. Taussig Bing anomaly (large suhpulmonic VSD with no PS haring transposition like
physiology): Cyanotic infants with RV dominance, pulmonary ejection click and short
ejection systolic murmur over upper and PSM over lower LPSB with prominent MPA
on X suggest the diagnosis. Electrocardiography shows clockwise loop, right axos,
BA enlargement and RVH. Two-dimensional echocardiogram with Doppler confirms
the Taussig Bing type
5. When PVIt increases in cases without PS It gives rise to Eisenmenger's syndrome
6. Besides medical management, total corrective surgery is the definitive treatment
NURSING MANAGEMENT
Nursing Diagnosis
1. Decreased Cardiac Output related to poor perfusion to tissues and organs
2. Compromised Family Coping related to situational family crisis
3. Impaired Gas Exchange related to poor perfusion to pulmonary tissues
 4. Ineffective Tissue Perfusion related to insufficient oxygenated blood circulation
5. Risk for Infection related to compromised immune system

Nursing Interventions

 Managing Decrease in Cardiac Output

 Promoting Effective Family Coping
 Improving Tolerance to Activity
 Preventing Injury and Infection
 Administering Medications and Providing Pharmacological Interventions
 Providing Perioperative Nursing Care

1. Managing Decrease in Cardiac Output

 Assess heart rate and blood pressure.

 Note skin color, temperature, and moisture..
 Check for peripheral pulses, including capillary refill.
 Assess for reports of fatigue and reduced activity tolerance.
 Inspect fluid balance and weight gain. Weigh the patient regularly prior to breakfast

 Assess heart sounds for gallops (S3, S4).

 Monitor electrocardiogram (ECG) for rate, rhythm, and ectopy.
 Provide adequate rest periods
 Position the child in a semi-Fowler’s position.
 Administer oxygen therapy as prescribed.
 Administer medications as prescribed.

2. Promoting Effective Family Coping

 Observe for erratic behaviors (anger, tension, disorganization), and perception of a

crisis situation.
 Assess usual family coping methods and effectiveness.
 Assess the need for information and support.
 Encourage the expression of feelings and provide factual information about the
infant/child.
 Clarify any misinformation and answer questions regarding the disease process.
 Assist in identifying and using techniques to cope with and solve problems and gain
control over the situation.
 Encourage maintaining the health of family members and social contacts.
 Teach that overprotective behavior may hinder growth and development during infancy/
childhood.
 Suggest and reinforce appropriate coping behaviors, and support family decisions.
  Encourage parents to include ill infant/ child in family activities rather than family
revolving around the needs of the infant/child.
 Encourage to maintain consistent behavior limits and modification techniques.
 Instruct parents on nutritional and activity needs and/or limitations and approaches that
will assist in establishing an effective pattern
 Refer the family for additional support and counseling if indicated.

3. Improving Tolerance to Activity

 Assess the level of fatigue, ability to perform ADL, and other activities in relation to
the severity of the condition.
 Assess dyspnea on exertion, and skin color changes during rest and when active.
 Allow for rest periods between care; disturb only when necessary for care and
procedures.
 Avoid allowing the infant to cry for long periods of time, use a soft nipple for feeding;
cross-cut nipple; if unable for the infant to ingest sufficient calories by mouth,gavage-
feed the infant.
 Provide neutral environmental temperature; when bathing the infant, expose only the
area being bathed and keep the infant covered to prevent heat loss.
 Provide toys and games for quiet play and diversion appropriate for the age of the
child (specify), and allow limiting activities as much as possible.
 Assist parents to plan for care and rest schedules.
 Inform of activity or exercise restrictions and to set own limits for exercise and
activity.
 Explain to parents the need to conserve energy and encourage rest.
 Inform to request assistance when needed for daily activities.

4. Preventing Injury and Infection

 Assess for risk of drug toxicity, a cardiac complication of heart failure.

 Assess temperature, IV site if present, increased WBC, increased pulse and
respirations (specify when).
 Monitor orders for diagnostic tests and procedures.
 Assist and support the family’s feelings and decisions regarding surgery.
 Prepare parents and child (use play doll) for diagnostic procedures and/or surgery;
should be extensive, consistent, and comprehensive, including a surgical procedure to
be performed and expected results, prognosis, and whether corrective, palliative,
temporary, or permanent.
 Instruct in the administration of cardiotonic, taking the apical pulse, when to
withhold (less than 70-80 in a child and 90-100 in an infant), to notify the physician
of low pulse or irregular pulse, signs of toxicity.
  Teach actions to take if the child becomes cyanotic (knee-chest or squatting position,
elevating head and chest), and when to call the physician.
 Avoid allowing those with infections to have contact with the infant/ child.
 Provide adequate rest and nutritional needs for age.
 Wash hands before giving care.
 Use a sterile technique for IV maintenance if present.
 Administer antibiotics as ordered (specify drug, dose, route, and times).
 Instruct parents and child in personal hygiene and practices (rest, nutrition,
activity, bathroom for elimination, bathing)
 Inform the client to avoid contact with those in family or friends that have an
infection

5. Administering Medications and Providing Pharmacological Interventions

 Diuretics
 Beta-blockers
 ACE inhibitors
 Digoxin
 Anticoagulants

6. Providing Perioperative Nursing Care

 Assist in providing a thorough preoperative evaluation

 Discuss with patient and family on what to expect before, during, and after surgery.
 Review medications being taken by the patient.
 Ensure that patient is being given adequate nutrition and hydration.
 Provide psychological support.
 Ensure patient’s safety and well-being.
 Monitor vital signs, fluids, and electrolyte balance
 Assist in anesthesia management.
 Respond immediately to possible complications.
 Aid in pain management after surgery.
 Maintain proper hydration and electrolyte balance.
 Discuss with patient and family about rehabilitation and physical therapy.

CONCLUSION
DORV is a complex form of congenital heart disease that is best understood by
knowledge of the intraventricular anatomy. Awareness of the location of the VSD and
associated lesions helps one determine the physiology. The physiology of DORV varies with
subtypes having similarities to a simple VSD, tetralogy of Fallot, transposition, or single-
ventricle type physiology. Knowledge of the physiology allows appropriate
surgical treatment to be planned. The long-term outlook today is quite favorable for the
majority of patients with DORV, although the need for reoperation may be present.

BIBLIOGRAPHY

 Park. KM, Park’s Pediatric cardiology for practitioners, 6 th edition, 2015, Elsevier
publishers, New Delhi, Page no: 276-280
 Satpathy. M, Mishra RB, Clinical Diagnosis of Congenital Heart Diseases, 2 nd
edition, Elsevier Publishers, New Delhi, 2015, Page no: 285-291