Cyanotic Heart Defects

1. CYANOTIC HEART CYANOTIC HEART
DEFECTSDEFECTS
Ma. Tosca Cybil A. Torres, RN

2. CYANOTIC HEART DEFECTS

●
Permit unoxygenated or desaturated blood to enter the systemic
circulation.

●
Infants with complex or mixing cyanotic heart lesions who are
dependent on having a PDA for all or the majority of their pulmonary
or systemic blood flow can become severely symptomatic within the
first few days of life as the ductus arteriosus begins to close. They
often need emergency management with medical or surgical
intervention to survive the neonatal period.

●
PGE1 is a potent vasodilating drug that is administered to prevent
closure of the ductus or to reopen the ductus arteriosus and restore
pulmonary or systemic blood flow. Continuous infusion of the drug
may improve arterial oxygen saturation and tissue perfusion,
allowing the infant to be stabilized in anticipation of further
diagnostic and treatment interventions. It is rapidly metabolized
through the pulmonary circulation and excreted through the renal
system. It must be infused by continuous IV administration. The
major side effect is apnea and infants frequently require intubation.

3. CYANOTIC HEART DEFECTS
● Cyanosis – bluish discoloration of the skin and nail beds and mucus membranes appears
when tissues are deprived of adequate amount of O2.

●
It becomes visible when hemoglobin, approximately 5 gms/dl blood circulates unbound
to O2 and the measured O2 saturation drops below 85%.

●
Cardiac lesions produce cyanosis when desaturated blood from the venous system
enters the saturated arterial system without passing through the lungs.

●
Cyanosis can occur when:
1. blood flow to the lungs is decreased or insufficient.
2. deoxygenated or desaturated blood is pumped to the body and a decreased, normal,
or increased amount of deoxygenated and oxygenated blood is pumped to the lungs.
These are due to:
1. right to left shunting lesions when blood flow to the lungs is decreased and there is a
communication between the intracardiac chambers or great vessels (ex. TOF).
2. Mixing lesions where blood flow to the lungs is increased or normal and there is a
connection between the great arteries (ex. Single ventricle without pulmonary stenosis
or tricuspid atresia)
3. Pulmonary hypertension or pulmonary vascular disease and a communication between
the intracardiac chambers or great vessels (Eisenmerger’s complex) 

4. CYANOTIC HEART DEFECTS
Clinical consequences of cyanosis:
1. Polycythemia
2. Anemia
3. Clotting abnormalities
4. Hypercyanotic episodes or tet spells ( rapid and
deep respirations, irritability and crying, peripheral
vasodilation, increased systemic venous return,
increasing cyanosis that can be very severe and
decreased systolic murmur reflecting decreased
pulmonary blood flow.
5. CNS injury caused by abscess or embolic events.
6. Pulmonary hypertension
7. Endocarditis

5. CYANOTIC HEART DEFECTS
 Hypercyanotic episode:

●
As the child becomes more cyanotic, the child experiences
increased tachypnea and hyperpnia and this increase the
degree of right to left shunting.

●
treatment of the episodes includes:
1. Calming the infant, placing the infant in the knee-chest
position, and administering O2.
2. Morphine SO4 is administered to suppress the
respiratory center and decrease the degree of hyperpnea.
3. Potent medication that causes vasoconstriction (ex.
Phenylephrine) may be needed to increase systemic
vascular resistance, decrease the degree of right to left
shunting, and force blood into the pulmonary system.

6. Tetralogy of Fallot (TOF)
•Incidence and Pathophysiology:

●
TOF accounts for 8%-10% of all CHDs, and is the most common
cyanotic lesion seen in the 1
st
year of life.

●
Misalignment of ventricular septum during fetal development results
in the constellation of three of the four characteristics of this lesion.
a. VSD
b. Pulmonary stenosis (infundibular/ subpulmonic, valvular, and
supravalvular)
c. Overriding of the aorta (into the right ventricular side instead of
the over the left ventricle)
d. RVH develops secondary to the pulmonary stenosis (also known
as right ventricular outflow tract obstruction)

7. Tetralogy of Fallot

8. Altered Hemodynamics:

●
The degree of pulmonary stenosis (infundibular,
valvular, supravalvular) determines the resistance to
blood flow out to the lungs through the pulmonary
artery.

●
The VSD is usually large and the pressures in both
ventricles are equal. So the saturated blood enters the
RV from the RA, it can flow into the coronary artery or
shunt right to left into the aorta (causing desaturated
blood to enter the systemic circulation),depending on
the relative resistance of the pulmonary versus the
aortic circulation.

9. Tetralogy of Fallot (TOF)
Manifestations:
1. The degree of pulmonary stenosis governs the onset and severity of the
symptoms. The more severe the pulmonary stenosis (primarily infundibular
stenosis), the less pulmonary blood flow, the greater the right to left shunting,
and the more cyanotic and desaturated the patient.
2. If pulmonary stenosis is mild, there is little or no right to left shunting. The
saturation can be normal or low normal. This is known as “pink tet”.
3. Some infants present as cyanotic newborns. When antegrade (forward)
pulmonary blood flow is severely impeded because of pulmonary stenosis,
blood flow to the lungs depends on a PDA. As the mixed saturated blood enters
the aorta, certain amount will shunt through the ductus arteriosus into the
pulmonary arteries, allowing it to be oxygenated. As this structure closes, the
NB becomes profoundly cyanotic.
4. Other infants become cyanotic over the 1
st
few months of life. Initially, they may
tire easily, especially with exertion, and may have difficulty feeding and gaining
weight before cyanosis develops. In time these infants may have hypercyanotic
episodes, as well as other clinical signs of chronic hypoxemia.
5. Auscultation reveals a harsh systolic murmur, often accompanied by a palpable
thrill.
6. The heart is “boot shaped” on chest x-ray because of poor development of the
pulmonary artery.

10. Tetralogy of Fallot (TOF)
Therapeutic Management:
a. Medical Management:
1. Continuous PGE1 infusion to maintain ductal patency for
symptomatic NB (severe desaturation related to decreased
pulmonary blood flow or frequent tet spells).
2. Close monitoring for signs and symptoms of worsening
hypoxemia for older infants.
3. Illnesses that put them at risk for dehydration must be
treated promptly.
4. Hemoglobin and hematocrit levels must be evaluated to
assess for anemia.
5. Close monitoring for hypercyanortic episodes may detect
some very subtle and often self limiting episodes lasting
10-15 minutes.

11. Tetralogy of Fallot (TOF)
b. Surgical Management:
1. Primary repair during infancy has become the
treatment of choice of many centers with surgery
scheduled at 2-4 months of age for asymptomatic
infants. This is to normalize the physiology sooner and
promote normal growth of the pulmonary arteries. It
requires CP bypass.

●
Postop complications include: rhythm disturbances
(ex. Narrow complex tachycardia, varying degrees of
heart block), residual VSD, low CO related to RV
dysfunction, residual RV outflow obstruction and
branch pulmonary artery stenosis.

●
Mortality rate is 2%-5%.

●
Decisions and considerations regarding palliative or
definitive repairs include institutional approach,
associated anatomic issues such as abnormal coronary
arteries, branch pulmonary artery size or stenosis,
infant size and whether pulmonary atresia is also
present.

●
Earlier surgical intervention is indicated for increasing
or severe cyanosis, significant polycythemia, or
hypercyanotic episodes.

12. 2. Modified Blalock – Taussig Procedure –
the most commonly performed in some
symptomatic NBs that are poor
candidates for primary repair. This is a
lower risk surgical procedure by creating
a systemic – pulmonary artery shunt to
increase the pulmonary blood flow. This
is usually not done with the child in CP
bypass.

●
Complications are the same as for
thoracotomy incision, including
pneumothorax, stridor (harsh vibrating
sound heard during respiration
obstructing air passage) from recurrent
laryngeal nerve injury, diaphragm
paresis from phrenic nerve injury, and
chylothorax from injury to the thoracic
duct. In addition, shunt failure because
of thrombosis or clot remains a
potential major problem.

13. Tricuspid Atresia
Incidence and Pathophysiology:

●
It represents approximately 2%-3% of all CHDs. It is the 3
rd
most
common cyanotic cardiac condition.

●
It is a complex lesion with many variations. In this lesion, the
tricuspid valve does not develop. An ASD or patent foramen ovale
must be present for the fetus or infant to survive.

●
The RV is hypoplastic (underdeveloped).The VSD can be of varying
size. The pulmonary artery may be in the normal position or
transpose with the aorta.

●
There may be pulmonary stenosis of varying degrees. The NB may
depend on the ductus arteriosus for pulmonary blood flow. The
degree of cyanosis and symptoms are related to these multiple
factors. 

14. Tricuspid Atresia

15. Altered Hemodynamics:

●
The desaturated blood enters the RA and is shunted right to
left through the patent foramen ovale/ASD into the LA,
since it cannot flow into the RV because the tricuspid valve
is atretic or absent. In the LA, the desaturated blood mixes
with the saturated blood (returning from the lungs) and
from the LA, it flows through the mitral valve into the LV

●
Some of the mixed saturated blood flows out of the aorta
and to systemic circulation. Some will flow through the VSD
and into the right ventricular chamber into the pulmonary
artery into the lungs to become oxygenated.

●
For children with severe pulmonary stenosis and no VSD or
other complex anatomy, the PDA is critical to ensure
pulmonary blood flow.

16. Tricuspid Atresia
Manifestations:
1. Profound cyanosis maybe present in the
neonate and is usually visible within the 1
st
few
hours of life in neonates with decreased
pulmonary blood flow. Infants with increased
pulmonary blood flow present with milder
cyanosis and increasing signs of CHF.
2. Single heart sound is present because there is no
closure of the tricuspid valve.
3. A systolic murmur of the VSD or a PDA murmur
may be heard (if patent)

17. Therapeutic Management:
a. Medical Management
1. PGE1 infusion is initiated for infants who depend on the
PDA for pulmonary blood flow; and the infant is stabilized
and readied for surgery.
b. Interventional Cardiac catheterization
1. Balloon atrial septostomy during cardiac catheterization to
allow blood to flow from the RA to the LA.

●
A catheter is inserted into the femoral vein and advanced
into the RA and across the foramen ovale/intraatrial
septum. A balloon in this catheter is then inflated, and this
balloon is pulled back through the foramen ovale, tearing
the septum. If the balloon procedure is not effective, and
can happen in infants beyond NB age, a catheter blade
septostomy can be performed to cut the septum.

18. Tricuspid Atresia
c. Surgical Management:

●
The goal of this staged palliative repair is to
separate the desaturated and saturated
blood, thereby eliminating cyanosis. The
equally important role is to optimize
ventricular function, by decreasing the
workload (volume overload) on the heart.
Norwood Procedure -is a series of three open-
heart surgeries that gradually improve
certain life-threatening forms of congenital
heart disease
1. Systemic to pulmonary artery shunt (Blalock
Taussig Shunt)– to provide adequate
pulmonary blood flow if significant
pulmonary stenosis is present. This is the 1
st
procedure in a three-stage effort to palliate
this defect.

19. 2. Hemi-Fontan (Bi-
directional Glenn
procedure) – is
performed at 4-6 months
of age, once the
pulmonary vascular
resistance has decreased
to normal pressures. In
this procedure, the SVC is
connected directly to the
pulmonary arteries,
thereby reducing left
ventricular volume load
by 1/3. This part of the
blood flow to the lungs is
now by passive flow,
since it is not pumped by
the RV to the lungs.

20. •Pulmonary hypertension must be prevented and
managed aggressively postop to ensure adequate
pulmonary blood flow. Position the child with the head
of the bed up to encourage blood flow to the lungs will
help decrease the degree of venous congestion in the
upper body.

●
Pleural effusions can develop as the body adjusts to the
flow and pressure changes. Occasionally, atrial
dysrythmias are seen. SVC syndrome may be present,
this is a condition in which SVC pressures are elevated
and the patients have upper body edema or plethora. It
can occur when there is an obstruction at the SVC to
branch pulmonary artery anastomosis, abnormal
pulmonary artery anatomy, or elevated pulmonary
vascular resistance

21. Tricuspid Atresia
3. Fontan Operation – the 3
rd
procedure, is usually performed
between ages 18 months and 6 years period. Desaturated blood is
directly channeled from the IVC to the pulmonary arteries.

22. The goals of the Fontan procedure are:
a. separation of the desaturated venous and saturated
arterial blood.
b. Volume unloading of the single ventricle. 

●
Sometimes, a small connection between the venous and
the arterial circulations is maintained called fenestration.
This is placed in case the pressures are slightly higher than
normal in the pulmonary arteries is that some
desaturated blood can shunt right to left to the systemic
circulation until the pulmonary arteries adjust to the new
flow and pressures. At a later time, the fenestration may
be closed. This can be done in the cardiac catheterization
laboratory if an adjustable tie has been placed at the time
of the fontan procedure.

●
Surgical mortality declined and is now reported to be
4%-8%.

23. Pulmonary Atresia with intact ventricular septum
Incidence and
Pathophysiology:

●
it accounts for approximately
3% of all CHDs. The causes of
this lesion are the failure of
the pulmonary valve to
develop, accompanied by
hypoplastic development of
the pulmonary artery and RV.
The tricuspid valve may also
be underdeveloped. The RV
pressures maybe extremely
high, and the coronary arteries
may also be abnormal.

24. Altered hemodynamics:

●
As the blood enters the RV, it cannot flow directly to the
pulmonary arteries because of atresia of the pulmonary
valve. The blood entering the RV is propelled back through
the tricuspid valve into the RA and shunted right to left
through the foramen ovale to the LA. The desaturated
blood and saturated blood mix in the LA and flow through
the mitral valve and into the LV, where the mix blood is
pumped to the aorta. From the aorta, this mixed saturated
blood flows to the body and brain. Oxygenation of the
blood occurs through persistent PDA into the pulmonary
arteries and to the lungs. 
Manifestations:
1. Profound cyanosis is seen during the early neonatal period.
Survival depends on the presence of PDA.
2. S2 single.
3. Soft or continuous systolic murmur if PDA is present. 

25. Pulmonary Atresia with intact ventricular septum
Therapeutic management:
a. Medical Management;
1. Continuous PGE1 infusion for NB to maintain ductal patency. 
b. Surgical management:

●
The primary treatment for this lesion.
1. Pulmonary Valvotomy or Transannular patch as the early surgical interventions and/or the
creation of a systemic to pulmonary artery shunt (Blalock-Taussig shunt).

●
Valvotomy may encourage growth of the RV. Over time, right to left shunting of the
atrial level may decrease as the RV increases the amount of blood it pumps to the
pulmonary system.

26. 2. Bidirectional Glenn Procedure or staging to the modified fontan
procedure is performed if the RV remains very small and cannot
pump an adequate amount of blood to the lungs.

●
Surgical mortality ranges from 19% after the 1
st
procedure to 36%
after both procedures.
Contraindications for pulmonary valvotomy:
1. Very high RV pressures and associated coronary artery
abnormalities because there would be inadequate coronary blood
flow, MI and ventricular muscle dysfunction.

●
These children are given a systemic to pulmonary artery shunt and
then staged as for a single ventricle. Sometimes, cardiac
transplantation may be indicated for this subgroup of children with
pulmonary atresia and intact ventricular septum. 

27. Truncus arteriosus
Incidence and Pathophysiology:

●
It accounts for 1%-4% of all CHDs.

●
It is marked by incomplete division of the common great vessels. The truncus arteriosus,
which normally divides into the pulmonary artery and pulmonary valve and the aorta
and aortic valve. This failure in division results in a single large vessel and single valve,
which gives rise to the pulmonary, systemic and coronary circulations.

●
The ventricular septum fails to develop at the same time, therefore VSD is present. The
common truncal arteriosus vessel overrides the VSD and receives both right and left
ventricles.

28. Altered Hemodynamics:

●
Desaturated blood enters the RA and flows through the
tricuspid valve into the RV. Saturated blood from the
LA flows through the mitral valve and into the LV. The
desaturated and saturated blood mixes in the
ventricles at the level of the VSD and common
ventricular outflow tract. The common great vessel
sends this mixed blood to the systemic, pulmonary,
and coronary circulations. O2 saturation depends on
the volume of pulmonary blood flow related to the
pulmonary vascular resistance; the greater this flow
the more symptoms of CHF, decreased CO, and
potential for coronary artery ischemia. The ventricles
are under pressure and volume overload.

29. Truncus Arteriosus
Manifestations:
1. The infant presents (often in the neonatal period),
with CHF and some degree of cyanosis. The volume of
pulmonary blood flow determines the severity of
symptoms. Unrestricted flow to the pulmonary artery
results in pulmonary congestion and severe CHF. If
pulmonic stenosis is present, pulmonary blood flow is
limited and cyanosis increases.
2. A harsh systolic murmur is heard and may be
accompanied by thrill. A diastolic murmur of truncal
valve insufficiency may be heard. The opening of the
single truncal valve may produce a click.
3. Bounding pulses and a widened pulse pressure
because of truncal insufficiency. 

30. Therapeutic
Management:
b.Medical Management:
aimed at reducing the
effects of CHF and
preventing
polycythemia
3.CHF is treated with
digoxin and diuretics. 
b. Surgical Management:
1. Pulmonary artery
banding for NBs who do
not respond to early
medical management.

31. 2. Rastelli repair- Total corrective repair includes
closing the VSD and placement of a conduit
from the RV to the pulmonary artery.

32. 3. Valvuloplasty of the truncal
valve which is the new aortic
valve may be performed to
improve valvular
competence. Blood flow
postop is normal.

●
Surgical mortality is about
5% - 10% in children who do
not have associated
malformations.
4.Conduit replacement is
necessary as the child grows
and a future truncal valve
repair or replacement may
be needed.

33. Hypoplastic Left Heart Syndrome
Incidence and pathophysiology:

●
Accounts for 1% of all CHDs. It is seen more frequently in
males than in females. Approximately 95% of all affected
infants who are untreated will die within the 1
st
months
of life.

●
Inadequate development of the left side of the heart
results in only one effective ventricle. The syndrome may
include aortic valve atresia, hypoplasia of the LV, atresia
or hypoplasia of the ascending aorta, and mitral valve
stenosis or atresia. Most infants have intact ventricular
septum.

34. Hypoplastic Left Heart Syndrome

35. Altered Hemodynamics:

●
Saturated pulmonary venous blood return is
unable to flow from the LA through the rest of the
left side of the heart. It is shunted left to right
through a patent foramen ovale into the RA,
where it mixes with desaturated blood. Mixed
blood travels through the RV to the main
pulmonary artery. A portion of blood flows to the
branch pulmonary artery through the PDA to the
descending aorta. From the aorta this mixed
saturated blood provides systemic and coronary
blood supply. The coronary blood supply is from
retrograde flow in the ascending aorta. 

36. Hypoplastic Left Heart Syndrome
Manifestations:
1. Most infants present (within the first few days of life) with tachypnea and
early CHF from increased pulmonary blood flow and as the ductus arteriosus
begins to close, systemic hypoperfusion and shock. The infant appears
grayish blue in color with dyspnea and hypotension. 
 Therapeutic Management:

●
Nearly all neonates with hypoplastic left heart syndrome will die within the
first month of life without surgical intervention. 
a. Medical Management:
6.Emergency management addresses correction of the acid-base and
electrolyte imbalances and reestablishment of ductal patency with PGE1.
b. Surgical Management
1. Cardiac transplantation as a single, definitive correction has been successful
with 85% operative survival rate and 81% 5-year survival rate.

●
The scarcity of neonatal donor heart, however, greatly limits the number of
infants who may receive transplant.
 

37. 2. Norwood Procedure – a three-step
palliative repair.
a. The stage 1 procedure provides
unobstructed blood flow from the RV to
the main pulmonary artery, which is
anastomosed to the ascending aorta to
make a “neoaorta” (often with patch
enlargement of the native aorta). The RV
acts as the systemic ventricle. Pulmonary
blood flow is supplied through a systemic-
to-pulmonary artery surgical shunt.
b. the 2
nd
stage, a bidirectional Glenn
procedure, is performed at approximately
6 months of age.
c. The palliation is completed usually by 6
yrs of age by a modified Fontan
procedure. The 4-year survival after
staged repair is greater than 50%; a 5-year
survival 50%-70% depending on the
associated factors.

●
Postop and long term complications
include hypoxemia, CHF, RV dysfunction,
pulmonary artery anomalies, systemic
venous hypertension, dysrythmia,
endocarditis, and developmental delays. 

38. Transposition of the Great Arteries
Transposition of the great arteries is a birth
defect causing a fatal condition in which there
is a reversal, or switch, in the truncal
connections of the two main (great) blood
vessels to the heart, the aorta and pulmonary
artery.

39. Transposition of the Great
Arteries

40. Manifestations:
•Blueness of the skin
•Shortness of breath
•Poor feeding
•Clubbing of the fingers or toes
Tests often include the following:
•Chest x-ray
•Cardiac catheterization
•ECG
•Echocardiogram (if done before birth, it is called a fetal
echocardiogram)
•Pulse oximetry (to check blood oxygen level)

41. Surgical Management
1. Mustard procedure or
the Senning procedure-
creates a tunnel (a
baffle) between the
atria. This redirects the
oxygen-rich blood to
the right ventricle and
aorta and the oxygen-
poor blood to the left
ventricle and the
pulmonary artery. This
operation is called an
atrial or venous switch.

42. 2. arterial switch
operation- The aorta
and pulmonary artery
are switched back to
their normal positions.
The aorta is connected
to the left ventricle, and
the pulmonary artery is
connected to the right
ventricle. The coronary
arteries, which carry the
oxygen-rich blood that
nourishes the heart
muscle, also need to be
re-attached to the new
aorta.

43. NURSING CARE
Assessment
1. Obtain thorough nursing history.
2. Measure and record the height and weight and record.
3. Record vital signs.
4. Assess and record:
a. Skin color: pink, cyanotic, mottled
b. Mucous membrane; dry, cyanotic
c. Extremities; peripheral pulses for quality and symmetry, capillary refill, cool
5. Assess clubbing
6. Assess chest wall for deformities.
7. Assess respiratory pattern. (technique)
8. Assess heart sounds.
a. Rate (brady,tachy or normal) and rhythm
b. Identify murmurs.
9. Assess fluid status
a. Daily weight.
b. Strict I & O
10. Assess and record the child’s level of activity.
a. Observe the child at play, while feeding.

44. NURSING DIAGNOSES
Impaired Gas exchange related to altered
pulmonary blood flow or pulmonary
congestion.
Decreased cardiac output related to decreased
myocardial function.
Activity intolerance related to hypoxia or
decreased myocardial function.
Altered Nutrition; Less than body requirements
related to excessive energy demands required
by increased cardiac workload
Risk for infection related to chronic illness.
Fear and anxiety related to life-threatening
illness.

45. NURSING INTERVENTIONS:
1. Relieving respiratory distress.
a. Positioning.
b. Suction oral and nasal secretions
c. Administer oxygen as prescribed.
d. Administer prescribed meds and document response.
Diuretics
Bronchodilators
e. May need to change oral feeding to NGT feeding.
2. Improving Cardiac Output
a. Organize nsg care and meds schedule to provide periods of
uninterrupted rest.
b. Provide play or educational activities that can be done in bed.
c. Maintain normothermia.
d. Administer prescribed meds
Diuretics (Furosemide, spirinolactone)
Digoxin
Afterload reducing meds (Captopril, enalapril)

46. NURSING INTERVENTIONS:
3. Improving oxygenation and Activity tolerance
a. Continuous monitoring of VS (pulse oximeter)
b. Administer oxygen as needed; assess response
4. Providing adequate nutrition
a. Infants
Small frequent feedings
Fortified formula or breast milk (up to 30 cal/oz)
Limit oral feeding time to 15-20 mins
Supplement oral feeds with NGT feeds as needed.
b. Report feeding intolerance: nausea, vomiting, diarrhea
c. Document daily weight (same time, scale, day)
d. Record accurate I&O
5. Preventing infection
a. Maintain routine childhood immunization.
b. Administer yearly influenza vaccine.
c. Prevent exposure to communicable disease.
d. Good hand washing
e. Report fevers
f. Report signs of URTI
6. Reduce fear and anxiety
a. Educate patient and family.
b. Provide the family with contact phone numbers of a cardiologist.
c. Provide teachings on initial action in case of emergency situations.

47. EVALUATION
Improved oxygenation as evidenced by easy,
comfortable respiration
Improved cardiac output as evidenced by
stable v/s and adequate peripheral perfusion
Increased activity level
Maximal nutritional status demonstrated by
weight gain and increase in growth curve
percentile.
No signs of infection.
Parents discuss diagnosis and treatment
together and with child.

48. End of Discussion