FAQ's
THE AORTA
WHAT are the signs& symptoms?
Symptoms include weakness, fatigue, increased
heart rate, rapid breathing, poor feeding and low
oxygen levels.
WHEN dosymptomspresent?
Symptoms generally present at 1-2 days old.
HOW isit diagnosed?
IAA is diagnosed with an echo-cardiogram.
(Lumen Learning, n.d.)
WHAT isthe cause?
IAA is caused by improper development of the
aortic arch. This defect is often associated with a
chromosomal abnormality called DiGeorge
syndrome.
(Cincinatti Children's Medical Center, 2018)
Blood vessels in various sections of the
aorta supply blood to different parts of the
body. In IAA, vessels beyond the gap will
not receive blood from the left side of the
heart, and therefore will not be able to
transport oxygen to various parts of the
body. Lack of blood and oxygen to the
bodies organs can be life threatening.
Reparation of the gap is essential for
survival.
(Baffa, 2018)
INTERRUPTED
AORTIC ARCH
(IAA)
Information for Families
Healt hy Hear t
Int er r upt ed Aor t ic
Ar ch
What Is IAA?
IAA is a r ar e hear t condit ion that occurs
when the baby's aor t a does not for m
pr oper ly during fetal development.
The aor t a is an essent ial " pipeline" that
carries oxygen-rich blood from the heart
to the rest of the body.
A properly developed aorta is shaped like
an arch, however in IAA, par t of t he
ar ch is missing, or "interrupted" leaving
a gap.
The gap is closed off on each end,
meaning there is no blood flow bet ween
t he sect ions.
HOW isit treated?
THE SURGERY
The child must undergo ur gent sur ger y
(within the first few days of life) t o
r econnect t he aor t ic ar ch.
Open heart surgery is performed to
connect the two separate parts of the
aorta.
Sur vival is not possible wit hout sur ger y.
This surgery is of a higher risk if the baby
is unstable, extremely ill, or has a
significantly small portion of the aorta.
Treatment prior to surgery:
-
Given IV infusion of prostaglandin
to keep the blood vessels open
Insertion of a breathing tube
"Water pills" to get rid of excess
fluid
Medications to keep the heart
pumping
Close monitoring of oxygen, carbon
dioxide and electrolyte levels
Adequate nourishment
(Cincinatti Children's Medical Center, 2018)
There are 3 types of IAA, which are
diagnosed based on the location of the
(Baffa, 2018)
interruption.
(Wheeler & Jeanty, 2018)
Survival after a complete aortic repair is
approximately 90%.
Type A: Interruption occurs just beyond
left subclavian artery.
LONG TERM CONSIDERATIONS
Type B (Most Common): Interruption
occurs between left carotid artery and left
subclavian artery.
Only 10-20% of children require a second
surgery, however life-long surveillance
with a cardiologist is important.
(Cincinatti Children's Medical Center, 2018)
Type C (Least Common): Interruption
occurs between brachiocephalic artery
and left carotid artery.
(Cincinatti Children's Medical Center, 2018)
Refer ences
Cincinatti Children's Medical Center. (2018, September). Interrupted Aortic Arch - Ventricular Septic Defect. Cincinatti Children's.
https:/ / www.cincinnatichildrens.org/ patients/ child/ encyclopedia/ defects/ iaa
Lumen Learning. (n.d.). Circulatory Pathways. Lumen Learning. https:/ / courses.lumenlearning.com/ ap2/ chapter/ circulatory-pathways/
Gaffa, G. (2018, January). Interrupted Aortic Arch (IAA). Neumers KidsHealth. https:/ / kidshealth.org/ en/ parents/ aortic-arch.html