Double outlet right ventricle

Double outlet right ventricle is a group of complex congenital (present at birth) heart
abnormalities. In double-outlet right ventricle, the aorta and pulmonary artery connect
partially or completely to the right ventricle 1). The position and size of the great arteries
vary and other cardiac malformations are commonly present and include pulmonary
outflow or left ventricular outflow obstruction, and major atrioventricular valve
abnormalities. The aorta may be on the right or left and may be anterior, posterior, or
lateral. In addition double outlet right ventricle is commonly associated with syndromes
and chromosomal abnormalities. A hole also exists between the two ventricles
(ventricular septal defect). In a normal heart, as shown on the left, the pulmonary
artery connects to the right ventricle and the aorta connects to the left ventricle (Figure
1). Double outlet right ventricle accounts for less than 1% of all congenital heart
defects 2). The mean incidence is 4 to 1,000 to 50 to 1,000 live births 3).
In double-outlet right ventricle a heart condition present at birth (congenital) — the
main artery that carries blood from the heart to the body (aorta) and the artery that
directs blood from the heart to the lungs (pulmonary artery) are partially or completely
connected to the lower right heart chamber (right ventricle). Sometimes these blood
vessels are also reversed from their normal positions (transposed). In a normal heart,
the pulmonary artery is connected to the right ventricle and the aorta is connected to
the left ventricle.

In people with double-outlet right ventricle, there is also a hole between the lower heart
chambers (ventricles), called a ventricular septal defect (VSD), which can be located in
several places in the wall between the ventricles. This causes oxygen-rich blood to flow
from the left ventricle to the right ventricle and mix with oxygen-poor blood. Due to this
mixing, children born with double-outlet right ventricle can have oxygen saturations that
are lower than normal.
Too much blood flow may then be delivered through the pulmonary artery to the lungs,
causing heart failure and poor growth over time. In other cases, blood flow through the
pulmonary artery may be reduced, which can cause your child’s skin to turn a bluish
color (cyanosis).

Double outlet right ventricle is initially associated with excessive pulmonary blood flow
and frequent recurrent pulmonary infections 4). Cyanosis may be present soon after
birth. The onset of pulmonary hypertension often leads to a progressive reduction in
exercise capacity and fatigue resulting from impaired respiratory and heart function,
which cause stunted childhood development. The disorder is complex and is often
associated with other forms of intracardiac or extracardiac malformation. Pulmonary
hypotension usually arises early and eventually results in heart and lung dysfunction,
with a missed opportunity to performing surgery. It is generally considered that these
patients often develop significant congestive heart failure due to a large left-to-right
shunt, and surgical repair should be carried out in the first several months of life 5).

Note: Both the pulmonary artery (PA) and

aorta (AO) arise from the right ventricle in
double-outlet right ventricle (DORV). Blood
from the left ventricle must exit through a
large ventricular septal defect (VSD). Most
often the great arteries, PA and AO, are side
by side and separated by the conus septum.
In double-outlet right ventricle, the
pulmonary and aortic valves are at the same
level. Subtypes of double-outlet right
ventricle are based upon the position of the
VSD and presence or absence of pulmonary
stenosis or right ventricular outflow tract
obstruction (RVOTO).

Double outlet right ventricle types

These two great arteries – the aorta and pulmonary artery – may override the ventricular septum by
more than 50%, 90%, or both arteries may arise fully from the right ventricle (200%) 6). Double outlet
right ventricle is frequently associated with other intracardiac or extracardiac malformations.
The current double outlet right ventricle classification system is based on the international nomenclature
databases adopted by the Society of Thoracic Surgeons and the European Association of Cardiothoracic
Surgery 7). There are four types of double outlet right ventricle 8):

 Tetralogy of Fallot type,

 Transposition of the great arteries type,
 Ventricular septal defect [VSD] type, and
 Remote VSD type have been classified
These four types differ significantly in their surgical treatment, postoperative management, and prognosis.
Except for Tetralogy of Fallot type, which usually has pulmonary hypertension, the other three types
frequently induce pulmonary hypertension. Preoperative pulmonary hypertension was concerned as an
important risk factor for early deaths in the surgical treatment of double outlet right ventricle 9).

Double outlet right ventricle survival rate

In a series 10) of 61 patients (36 males and 25 females) aged 2 weeks to 26 years with double outlet
right ventricle (two great arteries overriding at least 50%) complicated by pulmonary hypertension who
underwent surgical treatment, 31 of 36 patients aged <1 year recovered uneventfully. However, 5 of 36
infants died (13.9%), 3 of the 36 infant patients underwent secondary surgery to correct postoperative
complications (two patients underwent successful closure of a residual shunt in the intracardiac channel,
and one patient was revised for a residual shunt in the intracardiac channel combined with enlarging an
right ventricle outflow tract stenosis) 11). In contrast to the infant group above, all operations were
successfully performed in the 22 patients aged between 1 and 14 years, including in 3 patients who had
undergone a previous banding procedure. No patients died or required further surgery. Senning procedure
and Mustard procedure were performed in three adult patients to reverse the features of hypoxia; one
died from low cardiac output syndrome 12). Although the average survival time after diagnosis of
pulmonary hypertension was estimated to be 5–7 years nowadays, the mortality was raised significantly
after 3 years old 13), 14), 15), 16). Therefore, according to that study observation 17), it was safe for
patients with double outlet right ventricle and pulmonary hypertension to receive surgical treatment
between 1 and 3 years old. Younger infants and adult patients may have higher mortality.

Double outlet right ventricle life expectancy

How well the baby does depends on:

 The size and location of the VSD

 The size of the pumping chambers
 The location of the aorta and pulmonary artery
 The presence of other complications (such as coarctation of the aorta and mitral valve problems)
 The baby’s overall health at the time of diagnosis
 Whether lung damage has occurred from too much blood flowing to the lungs for a long period of
time

Double outlet right ventricle possible complications

Complications from double outlet right ventricle may include:

 Heart failure
 High blood pressure in the lungs (pulmonary hypertension), which untreated can lead to
permanent lung damage
 Death
Double outlet right ventricle causes
In a normal heart structure, the aorta connects to the left ventricle. The pulmonary artery normally is
connected to the right ventricle. In double outlet right ventricle, both arteries flow out of the right
ventricle. This is a problem because the right ventricle carries oxygen-poor blood. This blood is then
circulated throughout the body.
Another defect called a ventricular septal defect (VSD) always occurs with double outlet right ventricle.
Other conditions that may be part of the defect are pulmonary valve stenosis and transposition of the
great arteries.

Oxygen-rich blood from the lungs flows from the left side of the heart, through the VSD opening and into
the right ventricle. This helps the infant with double outlet right ventricle by allowing oxygen-rich blood to
mix with oxygen-poor blood. Even with this mixture, the body may not get enough oxygen. This makes
the heart work harder to meet the body’s needs. There are several types of double outlet right ventricle.

The difference between these types is the location of the VSD as it relates to the location of the pulmonary
artery and aorta. The symptoms and severity of the problem will depend on the type of double outlet right
ventricle. The presence of pulmonary valve stenosis also affects the condition.

People with double outlet right ventricle often have other heart defects, such
as:

 Endocardial cushion defects (the walls separating all 4 chambers of the heart are poorly formed or
absent)
 Coarctation of the aorta (narrowing of the aorta)
 Mitral valve problems
 Pulmonary atresia (pulmonary valve does not form properly)
 Pulmonary valve stenosis (narrowing of the pulmonary valve)
 Right-sided aortic arch (aortic arch is on right instead of the left)
 Transposition of the great arteries (the aorta and pulmonary artery are switched)

Double outlet right ventricle symptoms

Common symptoms of double outlet right ventricle in babies include:

 rapid breathing
 rapid heartbeat
 sweating
 disinterest in feeding or tiring while feeding
 poor weight gain
 blue color of the skin, lips and nail beds (cyanosis)
 heart murmur (detected by doctor)
 In older children, symptoms may include:

 fatigue
 shortness of breath

Double outlet right ventricle diagnosis

To diagnose double-outlet right ventricle, doctors will generally perform an echocardiogram.
Echocardiograms use sound waves to produce an image of the heart. Sound waves bounce off the heart
and produce moving images that can be viewed on a video screen.

This test can help doctors to look closely at the heart, including the ventricles, aorta and
pulmonary arteries as well as the heart valves. Doctors often use this test to diagnose this
condition and any associated defects, and determine the appropriate treatment.
If more information is needed, doctors may conduct cardiac CT and MRI scans, or cardiac
catheterization. In cardiac catheterization, your child’s doctor inserts a thin, flexible tube
(catheter) into an artery or vein in the groin or neck and threads it into the heart. A dye is
injected through the catheter to make the heart structures more visible on X-ray pictures.
Cardiac catheterization also measures pressure and oxygen levels in the chambers of the heart
and in the blood vessels.

Double outlet right ventricle treatment

Several types of surgery may be performed, depending on the specific heart defect and any associated
defects.

Factors that determine the type and number of operations the baby needs include:

 The type of double outlet right ventricle

 The severity of the defect
 The presence of other problems in the heart
 The child’s overall condition

Depending on the type of defect, surgeons may:

 Create a tunnel (baffle) through the VSD to connect the left ventricle to the aorta
 Switch the aorta and pulmonary artery positions, if they are in reversed positions, in order to
connect the pulmonary artery to the right ventricle and the aorta to the left ventricle
 Insert a patch between the ventricles to close the hole between the ventricles (VSD)
 Insert a blood vessel to connect the right ventricle to the pulmonary artery to allow more blood
flow if the pulmonary artery is small or absent
 Widen the narrowed pulmonary artery to allow more blood flow
 Place an aorta to pulmonary artery shunt to provide more blood flow to the pulmonary arteries
 Conduct a series of other procedures to allow blood to move to the lungs and for the heart to
function with one ventricle, if the defect is complex
 Repair any other congenital heart defects, such as atrial septal defects or patent ductus arteriosus

Another procedure, called an arterial switch operation, is necessary if the aorta and the pulmonary artery
are reversed in relation to each other. For this procedure, a tunnel from the VSD to the pulmonary artery
is created, connecting the left ventricle to the pulmonary artery. Then, the vessels are disconnected and
reconnected so that the pulmonary artery becomes the aorta and the aortic valve is connected to the
pulmonary artery, and the holes between the left and right ventricles of the heart are closed.

In some infants with inadequate blood flow to their lungs at birth, a temporary procedure may be done to
insert a shunt between the aorta and the pulmonary artery. The shunt is then removed later in life during
your child’s definitive cardiac surgery. If your child has other heart conditions associated with double
outlet right ventricle, he or she may also need other types of surgery.

Adults who were born with double-outlet right ventricle will need lifelong care and regular follow-up
evaluations from cardiologists trained in evaluating and treating congenital heart conditions (adult
congenital cardiologists) to monitor for any changes in their condition. Surgery may need to be performed
later in life for valve disease such as narrowing or leakage of the heart valves. Some adults need close
monitoring of their aortas and pulmonary arteries, especially if they required surgery early in life. A small
group of adults may require medications for treatment of decreased function involving the right or left
ventricles.

Double outlet right ventricle surgery

Regardless of the double outlet right ventricle classification system used, the choice of surgical approach
depends on 3 factors:

 the relative positions of the great arteries,

 the relationship between the arteries and the ventricular septal defect (VSD), and
 the presence or absence of right ventricular outflow tract obstruction.

The four types of double outlet right ventricle :

 Tetralogy of Fallot type,

 Transposition of the great arteries type,
 Ventricular septal defect [VSD] type, and
 Remote VSD type

The first 2 types of double outlet right ventricle—the VSD type and the Tetralogy of Fallot type—have clear
surgical options. However, the choice of surgical treatment for double outlet right ventricle with
transposition of the great arteries is more difficult because right ventricular outflow tract obstruction may
also be present; the current classification system does not adequately account for this possibility. This is
especially problematic for patients with a noncommitted VSD and abnormal great arteries because they
might need arterial switch operations or double-root translocations 19).

Choice of surgical method and prevention of complications

Pathological changes in patients with double outlet right ventricle are varied, necessitating an
individualized surgical strategy to optimize success. The suture technique used in intracardiac channel
repair of VSD type double outlet right ventricle should be sufficient to prevent residual shunt formation. In
cases of restricted VSD, enlargement of the outflow defect should be carried out, with attention paid to
avoid outflow tract stenosis from both the left and right ventricular chambers. To avoid right ventricle
outflow tract stenosis, the tract should simultaneously be enlarged with a patch. Eight of our patients in
this group received right ventricle outflow tract patch widening. For patients with double outlet right
ventricle of remote VSD type, a total intracardiac conduit may be needed. For transposition of the great
arteries type double outlet right ventricle, an arterial switch operation remains the best choice. During the
operation, coronary anastomotic patency should be confirmed, and care taken to prevent distortion and
stenosis. Aortic and pulmonary valves should be protected. It is important to avoid stenosis of the
pulmonary artery and its branches during pulmonary artery anastomosis. If the aorta and pulmonary
artery are located in an anterior and posterior position and the VSD is below the pulmonary valve, arterial
switch surgery can be performed after establishing an intracardiac channel.

Mustard or Senning surgical techniques are generally safe because of their relative simplicity. Both
methods are useful for neglected cases with severe pulmonary hypertension, cyanosis, and hypoxia, in
which the best opportunity for surgery has been missed. These palliative procedures will improve hypoxia
and overall quality of life; however, the operative risk is high. During surgery, attention should be paid to
prevent lung injury and maintain cardiac function; consequently, VSD closure can be avoided. One patient
with mild pulmonary hypertension (<50 mmHg) was deemed suitable to undergo Rastelli surgery, with
careful paid to prevent distortion and stenosis of intracardiac and extracardiac channels that would
normally require reoperation. double outlet right ventricle is often associated with other intracardiac
malformations such as atrial septal defect (ASD), patent ductus arteriosus (PDA), and atrioventricular
valve regurgitation, which require concurrent correction.