Patent Ductus

Arteriosus
Congenital Heart Defect
Definition:
Patent ductus arteriosus , or
PDA, is a heart condition that is
normal but reverses soon after
birth. In a persistent PDA, there
is an irregular transmission of
blood between two of the most
important arteries in close
proximity to the heart. Although
the ductus arteriosus normally
seals off within a few days, in
PDA, the newborn's ductus
. PDA is common in neonates with
persistent respiratory problems
such as hypoxia, and has a high
occurrence in premature
children.
In hypoxic newborns, too little
oxygen reaches the lungs to
produce sufficient levels of
bradykinin. Bradykinin is
responsible for the vasodilation
of blood vessels, in this case it is
 Connection of the
aorta with the
pulmonary artery

Before birth, the two major arteries—the aorta



and the pulmonary artery—are connected by a

blood vessel called the ductus arteriosus. This
vessel is an essential part of fetal blood
circulation.
Causes:
A patent ductus arteriosus can
be idiopathic (i.e. without an
identifiable cause), or secondary
to another condition.
Some common contributing
factors in humans include:
vPremature infants
vCongenital rubella syndrome
vChromosomal abnormalities such
as Down Syndrome

Another cause of PDA is when
during the first several weeks
after birth there is a decrease
in the blood supply or
oxygen saturation of the baby
which could lead to the return of
the fetal type circulation in
the baby and induce the
reopening of the ductus
arteriosus.
Signs and Symptoms:
tachycardia or other arrhythmia
difficulty breathing because the
lungs are wet, congested, or fluid-
filled (congestive heart failure)
continuous machine-like murmur
enlarged heart
Left subclavicular thrill
Bounding pulse
Widened pulse pressure
excessive work load on heart that
interferes with breathing, feeding,
Risk Factors:
 Experiencing any of the following conditions
during pregnancy can increase your risk
of having a baby with a heart defect.
vRubella infection. Becoming infected with
rubella (German measles) while pregnant
can increase the risk of fetal heart defects.
The rubella virus crosses the placenta and
spreads through the fetus's circulatory
system damaging blood vessels and organs,
including the heart.
vPoorly controlled diabetes. Uncontrolled
diabetes in the mother in turn affects the
fetus's blood sugar causing various
damaging effects to the developing fetus.
vDrug or alcohol use or exposure to
Demographic Factors:
 Race/Ethnicity
◦ Studies of risk of PDA by race/ethnicity have been
inconsistent. While some studies have reported
higher rates of PDA among African Americans
than among whites, other investigations found
no such difference . PDA rates among Hispanics
tend to be lower than among whites and African
Americans (Fixler 1993, Chavez 1988).
 Sex
◦ PDA is more common among females than among
males, although one investigation reported 53%
of the PDA cases to be among males (Lary
2001).
 Parity
◦ One study noted isolated PDA not to be
associated with the mother’s number of
previous pregnancies. Another study reported
 Plurality
◦ Several investigations reported increased risk of PDA
among twins while a more recent study found no
association between isolated PDA risk and twins
(Ferencz 1997).
 Gestational Age and Birth Weight
◦ As noted previously, PDA is associated with preterm
delivery, where PDA generally is not considered to be
a birth defect. However, among term births isolated
PDA risk is associated with lower birth weight. PDA
risk is associated with small for gestational age
(intrauterine growth retardation). One study indicated
that there was an association between PDA and
hypothyroidism in the newborn. The defect corrected
itself after thyroid replacement therapy in most cases
(Allegaert 2004).
 Consanguinity
◦ One investigation reported no increased risk of PDA
 Parental Age
◦ Several studies observed decreased
risk of PDA with increasing
maternal age. However, another
study noted no association
between isolated PDA and
maternal or paternal age (Ferencz
1997).
◦

Pathophysiology:
 As a baby develops in the womb, a
vascular connection (ductus
arteriosus) between two major blood
vessels leading from the heart — the
aorta and pulmonary artery — is a
normal and necessary part of fetal
circulation.
 But, this duct is supposed to close within
two or three days after birth once the
newborn's heart adapts to life outside
the womb.
 In premature infants, the duct often
closes on its own within a few weeks of
Heart with PDA
Normal heart
Complications:
 Larger defects that are untreated can cause
high blood pressure in the lungs
(pulmonary hypertension), frequent
lung infections, irregular heartbeat
(arrhythmia) or heart failure, a chronic
condition in which the heart can't pump
effectively.
 People with structural heart problems, such
as a patent ductus arteriosus, are at a
higher risk of infectious endocarditis
than is the general population. Infectious
endocarditis is an inflammation of the
inner lining of the heart caused by a
bacterial infection.
Treatment:
 Medications
n premature infants, an intravenous (IV)
medication called indomethacin may help
close a patent ductus arteriosus.
Indomethacin is related to aspirin and
ibuprofen and works by stimulating the
muscles inside the PDA to constrict,
thereby closing the connection. Your
child's physician can answer any further
questions you may have about this
treatment.
◦ digoxin - a medicine that helps strengthen
the heart muscle, enabling it to pump more
efficiently.
 Treatment: (surgical)
 An incision may be made
through the breastbone
(sternum) and between
the lungs (mediastinum)
while the child is deep
asleep and pain-free
(under general
anesthesia). For some
heart defect repairs, the
incision is made on the
side of the chest,
between the ribs
(thoracotomy) instead
of through the
breastbone. Heart-lung
bypass may be needed.
Tubes are used to re-
route the blood through
a special pump that adds
oxygen to the blood and
 Treatment: (surgical)
 Most children need to stay
in the Intensive Care Unit
for 3 to 7 days and stay
in the hospital for 5 to 14
days. By the time the
child is transferred out of
the intensive care unit,
most of the tubes and
wires have been
removed and he is
encouraged to resume
many of his daily
activities. At the time of
discharge, the parents
are instructed on
activity, how to care for
the incision and how to
give medications their
child may need to take
such as Digoxin, Lasix,
Treatment: (conservative)
 Most infants with PDA eat and grow normally, but
premature infants or those infants with a large PDA
may become tired when feeding, and are not able to
eat enough to gain weight. Options that can be used
to ensure your baby will have adequate nutrition
include the following:
◦ high-calorie formula or breast milk
Special nutritional supplements may be added to
formula or pumped breast milk that increase the
number of calories in each ounce, thereby allowing
your baby to drink less and still consume enough
calories to grow properly.
◦ supplemental tube feedings
Feedings given through a small, flexible tube that
passes through the nose, down the esophagus, and
into the stomach, can either supplement or take the
place of bottle-feedings. Infants who can drink part of
their bottle, but not all, may be fed the remainder
through the feeding tube. Infants who are too tired to
Prevention:
 In most cases, you can't do anything to prevent having
a baby with a heart defect. However, it's important
to do everything possible to have a healthy
pregnancy. Here are the basics:
◦ Get early prenatal care, even before you're
pregnant. Quitting smoking, reducing stress,
stopping birth control — these are all things to talk to
your doctor about before you get pregnant. Also, be
sure you talk to your doctor about any medications
you're taking.
◦ Eat a well-balanced diet. Include a vitamin
supplement that contains folic acid. Also, limit
caffeine.
◦ Exercise regularly. Work with your doctor to develop
an exercise plan that's right for you.
◦ Avoid risks. These include harmful substances such as
alcohol, cigarettes and illicit drugs. Also, avoid X-rays,
hot tubs and saunas.
Tetralogy of
Fallot
Congenital Heart Defect
Definition:
Tetralogy of Fallot (TOF) is a
congenital heart defect which is
classically understood to involve
four anatomical abnormalities
(although only three of them are
always present). It is the most
common cyanotic heart defect,
representing 55-70%, and the
most common cause of blue
baby syndrome.
Heart with TOF
 Normal heart

Primary four malformations:
 A: Pulmonary stenosis
◦ 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 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
Primary four malformations:
B: Overriding aorta
◦ 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 toward the front
(anteriorly) or directly above the
septal defect, but it is always
abnormally located to the right of
Primary four malformations:
C: ventricular septal defect (VSD)
◦ 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), 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.
Primary four malformations:
D: Right ventricular hypertrophy
◦ 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
ventricular wall increases in size
to deal with the increased
obstruction to the right outflow
tract. This feature is now generally
Other Anomalies:
 There is anatomic variation between the hearts of
individuals with tetralogy of Fallot. Primarily, the
degree of right ventricular outflow tract obstruction
varies between patients and generally determines
clinical symptoms and disease progression.
In addition, tetralogy of Fallot may present with other

anatomical anomalies, including:

◦ stenosis of the left pulmonary artery, in 40% of patients
◦ a bicuspid pulmonary valve, in 40% of patients
◦ right-sided aortic arch, in 25% of patients
◦ coronary artery anomalies, in 10% of patients
◦ a foramen ovale or atrial septal defect, in which case
the syndrome is sometimes called a pentalogy of
Fallot
◦ an atrioventricular septal defect
◦ partially or totally anomalous pulmonary venous return
◦ forked ribs and scoliosis
Tetralogy of Fallot with
pulmonary atresia
(pseudotruncus arteriosus) is a
severe variant in which there is
complete obstruction (atresia) of
the right ventricular outflow tract,
causing an absence of the
pulmonary trunk during embryonic
development. In these individuals,
blood shunts completely from the
right ventricle to the left where it is
pumped only through the aorta.
Causes:
 Tetralogy of Fallot occurs in approximately 400 per
million live births.
 Its cause is thought to be due to environmental or
genetic factors or a combination. It is associated
with chromosome 22 deletions and diGeorge
syndrome.
 Specific genetic associations include:
◦ JAG1
◦ NKX2-5
◦ ZFPM2
◦ VEGF
 It occurs slightly more often in males than in females.
 Embryology studies show that it is a result of anterior
malalignment of the conal septum, resulting in the
clinical combination of a VSD, pulmonary stenosis,
and an overriding aorta. Right ventricular
Pathophysiology:
 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".
 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 desaturatedblood 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
Blood flow in TOF
Signs and Symptoms:
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.
Diagnosis:
The abnormal "coeur-en-sabot"
(boot-like) appearance of a heart
with tetralogy of Fallot is easily
visible via chest x-ray, and
before more sophisticated
techniques became available,
this was the definitive method of
diagnosis. Congenital heart
defects are now diagnosed with
echocardiography, which is
quick, involves no radiation, is
Treatment:
 Emergency management of tet spells
◦ Prior to corrective surgery, children with tetralogy
of Fallot may be prone to consequential acute
hypoxia (tet spells), characterized by sudden
cyanosis and syncope.
◦ These may be treated with beta-blockers such as
propranolol, but acute episodes may require
rapid intervention with morphine to reduce
ventilatory drive and a vasopressor such as
epinephrine, phenylephrine, or norepinephrine
to increase blood pressure.
◦ Oxygen is ineffective in treating hypoxic spells
because the underlying problem is lack of blood
flow through the lungs and not oxygenation
within the lungs.
◦ There are also simple procedures such as
squatting in the knee-chest position which
Treatment:
Surgical:
 The Blalock-Taussig shunt (also referred to as a Blalock-
Thomas-Taussig shunt) is a surgical procedure to give
palliation to cyanotic heart defects which are common
causes of blue baby syndrome. In modern surgery, this
procedure is temporarily used to direct blood flow to the
lungs and relieve cyanosis while the infant is waiting for
corrective or palliative surgery.
 One branch of the subclavian artery or carotid artery is
separated and connected with the pulmonary artery.
The lung receives more blood with low oxygenation from
the body. The first area of application was tetralogy of
Fallot.
 The procedure is no longer in use in its original form. Now a
length of artificial tubing, 3 to 4 millimeters in
diameter, is sewn between either the subclavian or the
carotid artery and the corresponding side branch of the
pulmonary artery, thus obviating the need to cut off blood

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