Truncus Arteriosus

Cara Guenther
Undergraduate Fellow
Redmond P. Burke
Chief, Division of Cardiovascular Surgery
The Congenital Heart Institute
Miami Children’s Hospital and Arnold Palmer Hospital
www.pediatricsurgery.com
 What is Truncus Arteriosus?
 In a child with Truncus Arteriosus (TA), the embryological
structure known as the truncus arteriosus does not divide
properly into the aorta and pulmonary artery.

 Normally, a child has two main blood vessels leaving the heart
– the pulmonary artery that supplies blood to the lungs and the
aorta that supplies blood to the body.

 A child with Truncus Arteriosus, however, has one large vessel,

or truncus, that leaves the heart and later splits to supply
both the lungs and body with blood. Usually this vessel only
has one large valve, known as the truncal valve.

 Children with Truncus Arteriosus also have a ventricle septal

defect (VSD), or a hole in the septum separating the left and
right ventricle. The child’s truncus sits on top of this opening.

Information from http://www.cincinnatichildrens.org/health/heart-encyclopedia/anomalies/truncus.htm

 Normal Heart vs. Heart with
Truncus Arteriosus
Normal Heart Heart with TA

*Please note the completely separate *Please note the single large
pulmonary artery and aorta as well as vessel (truncus) and the hole in
the continuous ventricular septum. the ventricular septum (VSD)

Images from www.americanheart.org

 What are the different types of
Truncus Arteriosus?
In 1965 van Praagh proposed four different types of
Truncus Arteriosus.
 Type A1: Single pulmonary trunk originates from the left
lateral aspect of the truncus, with branching of left and
right pulmonary arteries from the pulmonary trunk.
 Type A2: Left and right pulmonary artery branches have
separate origins on the lateral aspects of the truncus.
 Type A3: One branch of the pulmonary artery stems from
the truncus whereas the other branch originates from the
aortic arch
 Type A4: Defined by the coexistence of an interrupted
aortic arch.

Information from http://emedicine.medscape.com/article/892489-overview

 Diagram of different types of
Truncus Arteriosus

Image from http://emedicine.medscape.com/article/892489-overview

 How does Truncus Arteriosus affect
my baby’s heart and health?
All the blood that leaves the heart can now flow either
to the body or lungs. At a young age, more blood tends
to flow to the lungs which requires the heart to work a
lot harder. Overtime, the extra blood flow damages the
vessels in the lungs and causes pulmonary hypertension.
The truncal valve can also put extra stress on the heart
by leaking or blocking the blood’s path out of the heart.

Leaflets

Truncal valve
Image from www.irounds.mch.com
 What are the symptoms of
Truncus Arteriosus?
 Low oxygen levels, often causing
the child’s skin turning a light hue
of purple or blue, known as
cyanosis.
 On an X-ray, the heart appears
very large since the muscle
thickens from the extra work and
the lungs are hazy due to the
excess amount of blood flowing
into the lungs
 Rapid breathing
 Poor feeding

Information from http://www.cincinnatichildrens.org/health/heart-encyclopedia/anomalies/truncus.htmz

 What happens if the Truncus
Arteriosus is not fixed?
Over time, the lung’s arteries will become damaged,
causing the child to have pulmonary hypertension.
Congestive heart failure (when the heart pumps too
much blood to the lungs and they fill with fluid. This
makes it difficult for a child to breathe, so they breathe
rapidly. When a baby breathes rapidly, it can’t suck,
and therefore cannot feed. The baby loses weight, and
this is called “failure to thrive”).

Information from http://www.pted.org/?id=truncusarteriosus3

How does my team diagnose
Truncus Arteriosus?
The presence of one large vessel, one valve, and a VSD on an
esophageal echocardiogram illustrates that a child has Truncus
Arteriosus.
Nevertheless, this diagnosis must be discussed with my whole
team during a conference before the operation can take place.
Each patient is different, so every case is analyzed thoroughly
by multiple experts before any surgical procedures are done.
In conference, we refer to the results of our previous patients
and surgeries for clinical decision making. All of our outcomes
are reported on our website www.pediatricheartsurgery.com
 Our Mission
To successfully treat each patient with the
most effective and least traumatic approach
available!
 Surgery: Steps 1-3
Open chest, harvest pericardium, and place patient on
heart-lung bypass.

Venous cannula

Aortic cannula

This patient is now on heart-lung

bypass
IVC cannula

Image from www.irounds.mch.com

 Surgery: Step 4
Disconnect pulmonary artery from truncus.

Truncal transection

Image from www.irounds.mch.com

 Surgery: Step 5

Suture where pulmonary artery was disconnected from

truncus.

Pericardial patch sutured

to truncal defect

Truncus

Image from www.irounds.mch.com

 Surgery: Step 6

Close VSD either with a continuous suture line and a

patch cut from harvested pericardium.

VSD repair with running

suture line

Image from www.irounds.mch.com

 Surgery: Step 7

Connect the right ventricle to the lungs. Suture a

conduit (this one is a donor aortic valve from a patient
who died and donated their organs) to the distal
pulmonary arteries.
Conduit connecting
pulmonary artery and
right ventricle

Image from www.irounds.mch.com

 Surgery: Step 8, 9, 10, and 11

Re-warm the heart, separate from the bypass machine,

insert and secure draining tubes, and possibly close the
chest.

Sternal closure

Chest tube

Image from www.irounds.mch.com

 Day 1 (Day of Operation)

Images from https://irounds.mch.com/

 Postoperative Day 2

Images from https://irounds.mch.com/

 Postoperative Day 3

Images from https://irounds.mch.com/

 Postoperative Day 4

Images from https://irounds.mch.com/

 Postoperative Day 5

Images from https://irounds.mch.com/

 Postoperative Day 6

Images from https://irounds.mch.com/

 Postoperative Day 7

Images from https://irounds.mch.com/

 Postoperative Day 10

The baby is ready to go home on the 10th day after surgery!

 What are my main concerns
during surgery?
That the homograft is the
correct length; the conduit can
neither be stretched nor
kinked.
That the truncal valve
functions well, no leak and no
obstruction.
Why do I sometimes wait to close
the chest?
It is normal for the chest cavity to fill with fluid after an
open heart, bypass surgery. This is why we leave one to
three draining tubes in the child’s chest.
However, if I foresee a large build up of fluid or intense
swelling of the heart muscle, I wait to close the chest to
avoid any pressure on the heart.
If the chest remains open, antibiotics are given to
reduce risk of infection. The child’s chest then is usually
closed in the ICU in the first or second postoperative
day.
 Different types of grafts
 Homograft
 Pulmonary homograft– comes from a human donor
 Aortic homograft – comes from a human donor

 Zenograft
 Contegra – comes from bovine (Cow) donor.

 Selection of type of graft depends on size and availability

 My preference:
 Pulmonary homograft
 Contegra zenograft
 Aortic homograft
 Will my child have to take
immunosuppressive drugs if he/she
receives a graft from a donor?
No, not because of the graft. All three types of graft
(pulmonary, aortic, and contegra) are stripped of all
cell material before they are used in surgery.
Therefore, immunosuppressive drugs are unnecessary.
 Can my child outgrow his or her
conduit?
Yes, this is a possibility. However, only 29% of my
patients have needed their conduit to be replaced.
Current MCH Surgical Results for Truncus
Arteriosus

Total Cases (N) 29

Surgical Mortality (N) 0
Median Age (days) 15.5
Median Weight (kg) 3.1
Mean Post-op Stay (days) 7
Delayed Sternal Closure (%) 39%
If delayed closure, mean duration 2
of open chest (days)

This data represents all MCH cases since 2002.

Data found in https://irounds.mch.com
 More MCH Surgical Results for
Truncus Arteriosus
Type A1 (%) 71%
Type A2 (%) 21%
Type A3 (%) 8%
Pulmonary Homograft used (%) 48%
Aortic Homograft used (%) 48%
Contegra Zenograft used (%) 4%
Conduit Replacement (%) 29%
Median Age at Replacement (days) 958
Interrupted Aortic Arch (%) .07%
This data represents all MCH cases since 2002.
Data found in https://irounds.mch.com
 Images

My team and I take intra-operative images during

every surgery for two reasons:
To help you understand what we did in surgery,
your family and you will receive a copy of these
images. The images can also be accessed at
https://irounds.mch.com
We reference these images when discussing
subsequent procedures in conference.
 Conclusion
Surgical repair of the Truncus Arteriosus defect is very
effective and absolutely vital. We have been very
successful in repairing this defect (no surgical
mortalities since before 2002).
If your child has TA, expect him/her to be hospitalized
for at least a week after the surgery. Also, do not be
discouraged if we wait to close your child’s chest.