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Tetralogy of Fallot
Patricia O’Brien, MSN, CPNP-AC; Audrey C. Marshall, MD
nancy and are present at birth. They are tricles (pumping chambers) of the How Is TOF Diagnosed?
the most common birth defects, occur- heart. Many newborns with TOF will have
ring in about 1 in 125 births. Tetralogy 3. Overriding of the aorta. The lower measured oxygen levels than
of Fallot (TOF) is a common defect in major blood vessel from the heart
normal or may have visibly blue lips
which obstruction to the flow of blood to the body, the aorta, is posi-
or nail beds (called cyanosis). It is
from the heart to the lungs causes low tioned more rightward than nor-
now recommended that newborns get
oxygen levels in the blood. Open heart mal and sits above the ventricular
screened for heart disease by hav-
septal defect.
surgery to correct the heart defect is ing their oxygen levels measured by
4. Hypertrophy of the right ven-
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the only treatment that gives long-term a machine called a pulse oximeter
tricle. The right ventricle is more
survival. before going home. A heart murmur
muscular than normal as a result
of the right ventricular outflow (abnormal heart sounds caused by tur-
What Is TOF? tract obstruction and the need to bulent blood flow) may be heard with
TOF is a defect combining 4 abnormal- pump harder. a stethoscope. An echocardiogram, an
ities that are all related (Figure): ultrasound that gives detailed images
TOF can be seen with other heart of the heart, is necessary to confirm
defects, but this Cardiology Patient the diagnosis. Today, many infants
1. Right ventricular outflow tract
Page focuses on the most common are diagnosed with a heart defect
obstruction. The connection
between the right ventricle of the form, TOF with pulmonary stenosis. before birth by an ultrasound during
heart and the lungs is narrowed, TOF occurs equally in boys and girls pregnancy.
and the blood flow to the lungs and in all races and ethnic groups. It is Symptoms vary, depending on the
is reduced. The main area of nar- associated with several genetic condi- severity of obstruction to blood flow
rowing is under the pulmonary tions, including trisomy 21 (Down to the lungs. Some infants appear
valve, caused by thick muscle in syndrome), and with deletions on blue soon after birth because of
this area. The valve may also be chromosome 22 and may occur with severe obstruction, whereas others
narrow (stenotic), and the pul- other birth defects such as cleft lip and with less obstruction are a normal
monary artery may also be small palate. If a parent or sibling has TOF, pink color. The obstruction generally
(hypoplastic). other children in the family also have gets worse over time, so blue lips may
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.
first appear at several months of age. surgery planned several months later. which they become intensely blue with
Some infants with mild obstruction Premature infants or newborns with deeper and faster breathing. Most com-
may never be blue (“pink Tets”) and other major medical problems that mon between 2 and 4 months of age,
may have signs of too much blood put them at higher risk may do better spells occur more often in the morning
flow to the lungs (rapid breathing, avoiding a full repair and instead hav- with crying, feeding, or stooling or at
poor feeding, and slow weight gain), ing a temporary procedure if they have times of stress, or they can be triggered
much like an infant with only a ven- severe obstruction (Table 1). by dehydration. Treatment includes
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tricular septal defect. calming the baby and holding the baby
Most newborns with TOF are well, Hypercyanotic Spells in a position with knees up touching
with normal breathing and feeding, Infants with TOF may have hypercya- the chest. This changes the blood flow
and can be discharged home with notic spells (“Tet spells”), episodes in pattern in the heart and may relieve the
Additional Resources Sable C, Foster E, Uzark K, Bjornsen K, Canobbio from the American Heart Association.
MM, Connolly HM, Graham TP, Gurvitz Circulation. 2011;123:1454–1485.
Marino BS, Lipkin PH, Newburger JW, Peacock G,
Gerdes M, Gaynor JW, Mussatto KA, Uzark MZ, Kovics A, Meadows AK, Reid GT, Reiss
K, Goldberg CS, Johnson WH, Li J, Smith SE, JG, Rosenbaum KN, Sagerman PJ, Saidi A, Adult Congenital Heart Association Web site.
Bellinger DC, Mahle WT. Neurodevelopmental Schonberg R, Shah S, Tong E, Williams RG. www.achaheart.org.
outcomes in children with congenital heart dis- Best practices in managing transition to adult- American Heart Association Web site. www.
ease: evaluation and management: Scientific hood for adolescents with congenital heart heart.org.
Statement from the American Heart Association. disease: the transition process and medical and Boston Children’s Hospital Web site. www.child-
Circulation. 2012;126:1143–1172. psychosocial issues: a Scientific Statement renshospital.org.
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