Annisa Maharani

111 2016 2099

 Tetralogy of Fallot (TOF) merupakan penyakit jantung kongenital
sianotik yang paling banyak disemua kelompok umur
Dideskripsikan pertama kali oleh Louis Arthur Etienne Fallot pada
1888, yang mendeskripsikan dengan kalimat La Maladie Bleu, yang
artinya penyakit jantung kongenital sianosis
Kejadian Tetralogy of fallot sekitar 8%-10% dari penyakit kelainan
jantung secara keseluruhan
Angka kejadiannya sama pada laki-laki maupun perempuan
ETIOLOGI
Faktor Endogen
- Kelainan kromosom: down
syndrome, dll
- Anak yang lahir sebelumnya
menderita PJB: VSD, stenosis
pulmonal, overriding aorta
- Penyakit keluarga: DM, hipertensi,
kolesterol tinggi, PJB
- Ibu di atas 40 tahun.
Faktor Eksogen
- Riwayat kehamilan ibu: program
KB/suntik, obat-obatan
- Ibu terinfeksi rubella
- Efek radiologi
- Ibu mengkonsumsi rokok dan
alkohol saat mengandung
- Gizi buruk saat hamil
DEFINISI
Tetralogy of Fallot ditandai dengan adanya 4
kelainan anatomi :
1. Defek septum ventrikel
2. Stenosis pulmonal (right ventricular outflow
obstruction)
3. Dekstroposisi aorta (overriding aorta)
4. Hipertrofi ventrikel kanan (RVH)
PATOFISIOLOGI
 INITIAL RIGHT-to-LEFT SHUNT (cyanotic)

TETRALOGY OF FALLOT
the most common cyanotic CHD
Classical Diagnostic Symptoms,
4 components:
1. Pulmonary Artery Stenosis: The principle
etiology responsible for the pulmonary
hypertension and right-to-left shunt.
2. Ventricular Septal Defect
3. Dextroposition of Aorta (overriding):
Aorta originates from the septal area,
such that it receives blood originating
from both right and left ventricle.
4. Right Ventricular Hypertrophy
 Condition Description

A narrowing of the right ventricular outflow tract and can

occur at the pulmonary valve (valvular stenosis) or just
below the pulmonary valve (infundibular stenosis).
Infundibular pulmonic stenosis is mostly caused by
overgrowth of the heart muscle wall (hypertrophy of the
septoparietal trabeculae), however the events leading to the
formation of the overriding aorta are also believed to be a
A: Pulmonary stenosis
cause. The pulmonic stenosis is the major cause of the
malformations, with the other associated malformations
acting as compensatory mechanisms to the pulmonic
stenosis. The degree of stenosis varies between individuals
with TOF, and is the primary determinant of symptoms and
severity. This malformation is infrequently described as sub-
pulmonary stenosis or subpulmonary obstruction.
An aortic valve with biventricular connection, that is, it is
situated above the ventricular septal defect and connected to
both the right and the left ventricle. The degree to which the
aorta is attached to the right ventricle is referred to as its
degree of "override." The aortic root can be displaced
B: Overriding aorta
toward the front (anteriorly) or directly above the septal
defect, but it is always abnormally located to the right of the
root of the pulmonary artery. The degree of override is quite
variable, with 5-95% of the valve being connected to the
right ventricle.
 A hole between the two bottom chambers
(ventricles) of the heart. The defect is
centered around the most superior aspect of
the ventricular septum (the outlet septum),
C: ventricular septal defect (VSD)
and in the majority of cases is single and
large. In some cases thickening of the septum
(septal hypertrophy) can narrow the margins
of the defect.
The right ventricle is more muscular than
normal, causing a characteristic boot-shaped
(coeur-en-sabot) appearance as seen by chest
X-ray. Due to the misarrangement of the
external ventricular septum, the right
D: Right ventricular hypertrophy
ventricular wall increases in size to deal with
the increased obstruction to the right outflow
tract. This feature is now generally agreed to
be a secondary anomaly, as the level of
hypertrophy generally increases with age
Patent Ductus Arteriosus: Often co-occurs with
Tetralogy, although it isn't part of the
syndrome. It is helpful in Tetralogy, as it
provides a channel for shunted blood to get
back into the pulmonary circulation where it
belongs.

(Right Atrium ------> Right Ventricle ------> VSD -

-----> Left Ventricle ------> Aorta ------> through
the Ductus Arteriosus ------> Pulmonary
Arteries)
Normal Heart Heart with Tetralogy of
Fallot
Tetralogy of Fallot results in low oxygenation of blood due to the mixing of
oxygenated and deoxygenated blood in the left ventricle via the VSD and
preferential flow of the mixed blood from both ventricles through the aorta
because of the obstruction to flow through the pulmonary valve. This is known
as a right-to-left shunt. The primary symptom is low blood oxygen saturation
with or without cyanosis from birth or developing in the first year of life. If the
baby is not cyanotic then it is sometimes referred to as a "pink tet". Other
symptoms include a heart murmur which may range from almost imperceptible
to very loud, difficulty in feeding, failure to gain weight, retarded growth and
physical development, dyspnea on exertion, clubbing of the fingers and toes,
and polycythemia.
Children with tetralogy of Fallot may develop "tet spells". The precise
mechanism of these episodes is in doubt, but presumably results from a
transient increase in resistance to blood flow to the lungs with increased
preferential flow of desaturated blood to the body. Tet spells are characterized
by a sudden, marked increase in cyanosis followed by syncope, and may result
in hypoxic brain injury and death. Older children will often squat during a tet
spell, which cuts off circulation to the legs and therefore improves blood flow to
the brain and vital organs.
Activity/Rest
Inability to carry on normal activities
Nocturnal/exertion-related dyspnea; orthopnea

Circulation
Tachycardia, palpitations; severe dysrhythmia.
History of congenital/organic heart disease, rheumatic fever.
Upward displacement of the diaphragm and heart proportionate to uterine
size.
May have a continuous diastolic or presystolic murmur; cardiac enlargement;
loud systolic
murmur, associated with a thrill.
BP may be elevated or may be decreased with decreased vascular resistance.
Clubbing of toes and fingers may be present, with symmetric cyanosis in
surgically untreated
tetralogy of Fallot.
Elimination
Urine output may be decreased.
Nocturia.

Food/Fluid
Obesity (risk factor)
May have edema of the lower extremities
Pain/Discomfort
May report chest pain with/without activity

Respiration
Cough; may or may not be productive.
Hemoptysis.
Respiratory rate may be increased.
Dyspnea/shortness of breath, orthopnea may be reported.
Rales may be present.
1. SIZE OF THE VENTICULAR SEPTAL DEFECT
AND THE DEGREE OF RIGHT VENTICULAR
OUTFLOW OBSTRUCTION.

2. CYANOSIS
a.) The shunt through the venticular
septal defect may be from left to right. Many
infants with this defedt are not cyanotic at birth,
but they develop cyanosis as they grow and as
the stenosis becomes relatively more severe.

b.) Cyanosis may at first be observed

only with exertion and crying, but during the
first few years of life, the child may become
cyanotic even at rest.
 c.) Infundibular stenosis may be minimal so
that cyanosis never develops (pink tetralogy)

3. CLUBBING OF THE FINGERS AND TOES

4. SQUATTING (a posture characteristically
assumed by children with this defect once they
have reached the walking stage)
5. SLOW WEIGHT GAIN
6. DYSPNEA ON EXERTION
7. HYPOXIC SPELLS, TRANSIENT CEREBAL
ISCHEMIA
Possible clinical features include:
The warm mucous membranes are blue, for example the
tongue, the inside of the lips
Central cyanosis increases immediately on exercise
which is not the case for peripheral cyanosis
Often there is polycythaemia with an abnormally high
haemoglobin and haematocrit; this must not be confused
with neonatal polycythaemia which may mimic cyanosis
Clubbing is often seen in patients with central cyanosis
Note that the absolute discriminating feature between
central and peripheral cyanosis is obtained from testing the
oxygen saturation of arterial blood.
Chronic cyanosis causes clubbing of the digits
Clubbing in Tetralogy of Fallot
DIAGNOSTIC STUDIES
White Blood Cell (WBC) Count: Leukocytosis indicative of generalized
infection, primarily
streptococcal.
Hemoglobin (Hg)/Hematocrit (Hct): Reveals actual versus physiological
anemia;
polycythemia.
Maternal Arterial Blood Gases: Provide secondary assessment of potential
fetal
compromise due to maternal respiratory involvement.
Sedimentation Rate: Elevated in the presence of cardiac inflammation.
Maternal Electrocardiogram (ECG): Demonstrates patterns associated
with specific cardiac
disorders, dysrhythmias.
Echocardiography: Diagnoses mitral valve prolapse or Marfans
syndrome.
Radionuclide Cardiac Imaging: Evaluates suspected atrial or ventricular
septal defects,
patent ductus arteriosus, or intracardiac shunts.
Serial Ultrasonography: Detects gestational age of fetus and possible
IUGR.
1. AUSCULTATION
a.) Single second sound(aortic component)
b.) Systolic Injection murmur at the second
and third interspaces to the left of the
sternum.
c.)Prominent ejection click heard
immediately after the first heart sound
2. CHEST X-RAY
a.) Heart size normal
b.) Pulmonary segment small and concave
(boot-shaped heart)
c.) Diminished pulmonary vascular
markings
3. ELECTROCARDIOGRAM- right axis
deviation: right venticular hypertrophy
4. CARDIAC CATHETIRIZATION
5. ANGIOCARDIOGRAPHY
6. LABORATORY DATA
a. Polycythemia
b. Increased Hematocrit
Typical boot-shaped
heart secondary to RVH
and small main
pulmonary artery
segment

Pulmonary vascular
markings are decreased
Epinephrine 10mcg/kg
Atropine 20mcg/kg
Calcium 10mg/kg
Phenylephrine 2-10mcg/kg
Propanolol 10-50mcg/kg
Volume 5% albumin 10-20ml/kg
1. Congestive heart failure- may occur
in newborn but is uncommon beyond
infancy.
2. Infective endocardins
3. Cerebral vascular accident (due to
thrombosis or severe hypoxia)
4. Brain absces
5. Iron defeciency anemia
Improve oxygenation of arterial blood.

1. Palliative
a) Blalock- Taussig shunt- anastomosis
between the right or left subclavian artery
and the right pulmonary artery
b) Watersion Shunt- anastomosis between the
posterior lateral aspect of the ascending
aorta and the right pulmonary artery.
2. Total Correction
a) Removal of shunt if previously performed
b) Total correction is increasingly being
advocated for all infants in whom pulmonary
arteries are sufficient size.
NURSING PRIORITIES
1. Monitor degree/progression of symptoms.
2. Promote client involvement in control of condition and
self-care.
3. Monitor fetal well-being.
4. Support client/couple toward culmination of a safe
delivery.

Teaching/Learning
Possible history of valve replacement/prosthetic
device, mitral valve prolapse, Marfans
syndrome, surgically treated/untreated (rare)
tetralogy of Fallot