Cardiology Patient Page

Tetralogy of Fallot
Patricia O’Brien, MSN, CPNP-AC; Audrey C. Marshall, MD
o

form, TOF with pulmonary stenosis.

heart and the lungs is narrowed,
r TOF occurs equally in boys and
g

girls and in all races and ethnic

b

and the blood flow to the lungs groups. It is associated with several

C genetic condi tions, including trisomy

y

ongenital heart defects result from o

21 (Down syndrome), and with
is reduced. The main area of nar deletions on chromosome 22 and may
abnormal changes in the structure n

occur with other birth defects such as

of the heart that occur early in preg J

rowing is under the pulmonary cleft lip and palate. If a parent or

nancy and are present at birth. They u

sibling has TOF, other children in the

are the most common birth defects, n

e
family also have
occur ring in about 1 in 125 births. valve, caused by thick muscle in
Tetralogy of Fallot (TOF) is a 1
a higher-than-average chance of having
common defect in which obstruction 0
this heart defect.
to the flow of blood from the heart to this area. The valve may also be
,

the lungs causes low oxygen levels in 2 How Is TOF Diagnosed?

the blood. Open heart surgery to 0

Many newborns with TOF will have

D
correct the heart defect is 2
narrow (stenotic), and the pul lower measured oxygen levels than
o
the only treatment that gives long-term 1
normal or may have visibly blue lips
w monary artery may also be small or nail beds (called cyanosis). It is now
n
survival. (hypoplastic). recommended that newborns get
l screened for heart disease by hav ing
o 2. Ventricular septal defect. There
a their oxygen levels measured by a
is a hole in the wall (septum)
d
machine called a pulse oximeter
e
between the right and left ven
d
before going home. A heart murmur
What Is TOF? tricles (pumping chambers) of
(abnormal heart sounds caused by tur
the heart.
f
bulent blood flow) may be heard with
r
3. Overriding of the aorta. The
o
a stethoscope. An echocardiogram, an
m
major blood vessel from the heart ultrasound that gives detailed images
TOF is a defect combining 4 abnormal to the body, the aorta, is posi
h of the heart, is necessary to confirm
tioned more rightward than nor the diagnosis. Today, many infants are
ities that are all related (Figure):
t mal and sits above the ventricular diagnosed with a heart defect before
t

p septal defect. birth by an ultrasound during

:

/
4. Hypertrophy of the right ven pregnancy.
a
tricle. The right ventricle is more Symptoms vary, depending on the
h

muscular than normal as a result severity of obstruction to blood flow to

1. Right ventricular outflow tract of the right ventricular outflow
a

the lungs. Some infants appear blue

tract obstruction and the need to
j

soon after birth because of severe

u
pump harder. obstruction, whereas others with less
obstruction. The connection
r

n
TOF can be seen with other heart obstruction are a normal pink color.
a

defects, but this Cardiology Patient The obstruction generally gets worse
between the right ventricle of the over time, so blue lips may
l

s
Page focuses on the most common
.

The information contained in this Circulation Cardiology Patient Page is not a substitute for medical advice, and the American Heart Association
recommends consultation with your doctor or healthcare professional.
From the Department of Nursing/Patient Services, Cardiovascular Program, Boston Children’s Hospital, Boston, MA (P.O.); and Department of
Pediatrics, Harvard Medical School, and Cardiac Catheterization Laboratory, Department of Cardiology, Boston Children’s Hospital, Boston, MA
(A.C.M.). Correspondence to Patricia O’Brien, MSN, CPNP-AC, Department of Nursing/Patient Services, Cardiovascular Program, Boston Children’s
Hospital. E-mail pat.obrien@cardio.chboston.org
 (Circulation. 2014;130:e26-e29.)
© 2014 American Heart Association, Inc.
Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIRCULATIONAHA.113.005547

e26
O’Brien and Marshall Tetralogy of Fallot e27

Figure. Diagram of normal heart contrasted with heart defects associated with Tetralogy of Fallot. From Boston Children’s Hospital Web
site.
d

Infants with TOF may have hypercya

first appear at several months of age. f

notic spells (“Tet spells”), episodes in

Some infants with mild obstruction and can be discharged home with
r which they become intensely blue with
may never be blue (“pink Tets”) and o

m deeper and faster breathing. Most com

may have signs of too much blood
flow to the lungs (rapid breathing,
poor feeding, and slow weight gain),
much like an infant with only a ven
D
o
w
h mon between 2 and 4 months of age,
t
spells occur more often in the morning
surgery planned several months later.
Premature infants or newborns with with crying, feeding, or stooling or at
times of stress, or they can be triggered
other major medical problems that put
them at higher risk may do better by dehydration. Treatment includes

tricular septal defect. avoiding a full repair and instead hav calming the baby and holding the baby
n

l
ing a temporary procedure if they have in a position with knees up touching
o

severe obstruction (Table 1). the chest. This changes the blood flow
Most newborns with TOF are well,
a
pattern in the heart and may relieve the
d

e
Hypercyanotic Spells
with normal breathing and feeding,

t
Table 1. Treatments for Tetralogy of Fallot With Pulmonary Stenosis
p

h
Age Characteristics Treatments
a

Newborn Severe cyanosis in newborns at higher risk for 3–6 mo Open heart surgery, heart-lung bypass
r n

J
a

complete repair:
u

n
l

.
Type of surgical repair depends on child’s
e

premature infants, infants with other birth 1

o

g
anatomy
0

defects, very ill infants ,

2
b
0
y
2

o 1
 sternum (breastbone), and the heart-lung bypass machine is used to
support the circulation during the operation (Table 1). Infants are
usu
ally in the hospital for about a week and fully recover in 4 to 6
weeks. Surgical results are excellent, with a less than 2% surgical
mortality and very good long-term outcomes.
After surgery, infants are in the intensive care unit for several days,
are on a ventilator to help them breathe, and receive medicines to
help the heart recover from surgery. A problem seen D

early after surgery can be fluid over

o

Adolescents and adults Progressive leaking of the pulmonary valve and w

dilation of the right ventricle load in the lungs and body because the
Narrow pulmonary valve or pulmonary artery n

TEMPORARY PROCEDURES:
l

SURGERY: Shunt procedure: Gore-Tex tube from an artery off the aorta right ventricle is not pumping effec
to the pulmonary artery to provide blood flow to the lungs a

CATHETERIZATION: dilation of the pulmonary valve

d

SURGERY: complete repair:

d

tively as it recovers. Medicines called

1. Close the VSD with a patch so that the aorta comes from the left f

ventricle Table 2. Future Care

2. Remove extra-thick muscle in the right ventricle below the valve
3. Repair narrow pulmonary valve
a. Valve-sparing technique for mild obstruction:
pulmonary valve is dilated
b. Transannular patch for severe obstruction:
larger patch going across the pulmonary valve extend ing from the right
ventricle to the pulmonary artery that destroys the pulmonary valve
4. Patch the right ventricle to relieve obstruction
In 5% of patients, an abnormal coronary artery (supplies blood to heart
muscle) crosses the area where the patch would go. In that case, repair
is done with a conduit (tube) from right ventricle to main pulmonary
artery
SURGERY: pulmonary valve replacement with tissue valve
Rhythm problems
CATHETERIZATION: balloon dilate pulmonary valve or pulmonary artery Ventricular tachycardia: dangerous fast rhythm
e28 Circulation July 22, 2014
spell. If there is no improvement in a few minutes, call 9-1-1.
Rarely, spells can cause babies to pass out or to have a seizure or a
stroke. If a spell occurs, surgery is performed soon to prevent
further spells.
Surgical Treatment
Most infants have a surgical repair at 3 to 6 months of age. Surgery
Most will achieve normal adult lifestyle (eg, work full-time, have children)
is done through an incision in the center of the chest along the
u
Few children have symptoms, and most may participate in normal
activities without restriction
Medical care involves regular (yearly) evaluations by the cardiologist with
electrocardiograms and echocardiograms to monitor for cardiac
concerns that may occur late after repair
Continued or progressive obstruction in the right ventricle
Continued or progressive obstruction across the pulmonary arteries
Leaking (regurgitation) of the pulmonary valve
Increasing dilation of the right ventricle and decreased ventricle function
Dilation of the aorta (rarely needs treatment)
Abnormal slow rhythms: need for pacemaker
Heart-healthy lifestyle is encouraged
Healthy diet and appropriate weight for age
Regular exercise
No smoking
Regular dental care is necessary
Higher incidence of learning and behavioral problems is observed
Attention deficit–hyperactivity disorder, learning disabilities, speech
delays, etc Early assessment and treatment
Adolescents are prepared to become more responsible for their own
health care Learn more about tetralogy of Fallot and long-term issues
Assume more responsibility for care over time

n
1
Long-Term Concerns
a
0

o
l
heart, which runs next to the ventricu ,

m
diuretics are used to help get rid of s

. During childhood, most children do

o 2

h
r
lar septal defect. Rarely, a pacemaker 0

extra fluid. Problems with abnormal g

t
very well, with normal growth and 1

p
may be needed if the rhythm does not
heartbeats (rhythm problems) may also
b
development, but need regular cardi
y
:

/
ology care (Table 2). However, even
o
/

a
return to normal. successful surgery does not result in a
totally normal heart. The right ven
h n

occur after surgery because of swelling

a
J

u
tricular outflow tract often has some
j

o
n

e
degree of obstruction, and the pulmo
or injury to the electric system of the
 nary valve does not function normally.
As a result of most surgical repairs,
the valve leaflets cannot close
completely, allowing blood to leak
back into the right ventricle (called
pulmonary regurgitation).
Pulmonary regurgitation is well
tolerated in children, but over many
years, the right ventricle may dilate
and stretch in some patients. The right
ventricle may pump less effectively,
which increases the risk of rhythm
problems and may decrease exercise
ability. Early signs of trouble from
pulmonary regurgitation are seen on
echocardiograms and are further evalu
ated with magnetic resonance imag
ing. Magnetic resonance imaging is an
imaging study done inside a magnet
that allows very detailed pictures and
from the American Heart Association.
measurements of the heart structures.
Surgical replacement of the pulmonary
valve with a tissue valve is considered
for patients with progressive right ven
tricular dilation. This is a low-risk
open heart procedure done on the
heart-lung bypass machine; the usual
hospital stay is less than a week; and
recovery is in 6 to 8 weeks.
Abnormal rhythm problems, espe
cially a very rapid heart rate called
ventricular tachycardia, which can be
life-threatening, can occur years after
TOF repair. More common in those
with significant obstruction in the right
ventricle, symptoms include dizziness,
fainting, or palpitations (irregular or
rapid heartbeat). Treatments may
include medications or further
procedures.
O’Brien and Marshall Tetralogy of Fallot e29
a
Summary
TOF is a congenital heart defect that
results in decreased blood flow to the
lungs and is successfully repaired by
surgery in infancy, allowing most
patients a normal lifestyle. Lifelong
follow-up is important to watch for
problems such as an abnormal heart
rhythm, leaking of the pulmonary
valve, or poor function of the right
ventricle. Continued research leading
to new knowledge and treatments for
congenital heart disease will improve
the care of children in the future.
Disclosures
None.

Circulation. 2011;123:1454–1485. s

Adult Congenital Heart Association Web site. g

www.achaheart.org. b

American Heart Association Web site. www. y

heart.org. o

Boston Children’s Hospital Web site. www.child n

renshospital.org. J

Additional Resources
Marino BS, Lipkin PH, Newburger JW, Peacock G,
Gerdes M, Gaynor JW, Mussatto KA, Uzark K,
Goldberg CS, Johnson WH, Li J, Smith SE, Bellinger
DC, Mahle WT. Neurodevelopmental outcomes in
children with congenital heart dis
ease: evaluation and management: Scientific Statement
from the American Heart Association. Circulation.
2012;126:1143–1172.
Sable C, Foster E, Uzark K, Bjornsen K, Canobbio MM,
Connolly HM, Graham TP, Gurvitz MZ, Kovics A,
Meadows AK, Reid GT, Reiss JG, Rosenbaum KN,
Sagerman PJ, Saidi A, Schonberg R, Shah S, Tong E,
D Williams RG. Best practices in managing transition to
o adult
w
hood for adolescents with congenital heart disease: the
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transition process and medical and psychosocial issues: a
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a
Scientific Statement
d