This document discusses several types of cyanotic heart disease including tetralogy of Fallot, tricuspid atresia, and transposition of the great arteries. Tetralogy of Fallot is characterized by four defects that result in oxygenated blood mixing with deoxygenated blood. Tricuspid atresia involves failure of the tricuspid valve to develop. Transposition of the great arteries is defined by the aorta arising from the right ventricle and pulmonary artery from the left ventricle, resulting in deoxygenated blood flowing to the systemic circulation. Surgical options are discussed to repair defects and allow for proper blood flow, such as the Fontan procedure for tricuspid atresia

1. Cyanotic Heart Disease

2. Classification of Cyanotic Heart
Disease
Cyanotic
Pulmonary blood flow Mixed blood flow
Tetralogy of fallot Transposition of great
arteries
Tricuspid ateresia Total anomalous pulmonary
Venous return
truncus arteriosis
Hypo plastic left heart
syndrome

3. Tetralogy of Fallot

4. Tetralogy of Fallot
• Introduction:
Tetrology of fallot is the most common type
of cyanotic congenital heart disease.
The classic form include four defect
• Ventricular septal defect
• Pulmonary stenosis
• Overriding aorta
• Right ventricular hypertrophy

6. Pathophysiology
The blood normally returns from the systemic
circulation to the systemic circulation to the
right atrium and right ventricles
The outflow of blood from the right ventricles is
resisted by the pulmonary stenosis so that
the blood flows through the ventricular septal
defect in to the aorta

7. There is right to left shunt. Hypertrophy of the
right ventricles occurs as a result of the
pressure exerted against the pulmonary
stenosis.
Because, the blood from the right ventricles is
unoxygenated, cyanosis results.

10. Clinical Manifestation
• Cyanosis, anoxia blue spells
• Clubbing of the fingers and toes
• Squatting posture
• Slow weight gain
• Dyspnoea no exertion
• Hypoxic spells (low O2 in tissue)
• Transient cerebral ischemia
• Pan systolic murmur
• Poor growth

11. Diagnostic Evaluation
• On Auscultation
A loud harsh systolic murmur may be heard
at the left middle to lower sturnal border.

12. Radiography (X – Ray):
The heart shows the enlarged ventricle
on the right side, the large aorta and the
decrease in size of the pulmonary artery.
The hilar areas of the lungs are usually
clear because of normal or demised
pulmonary blood flow.
The unusual shape of the heart has led
to its being called the wooden shoe or
boot – shaped heart.

13. • ECG:
ECG shows evidence of right ventricular
hypertrophy
• Echocardiography:
Shows evidence of the aortic override,
thick, anterior right ventricular wall and large
aorta.
• Cardiac catheterization:
• Angiocardiography
• Laboratory test – blood test

14. Management
• Oxygen administration for cyanosis
• Maintain nutrition by small and frequent
• Give rest and allow only minimum activities
for the child

15. Surgical Management
• Palliative shunt:
In the infant who cannot undergo primary repair,
a palliative procedure to increase pulmonary blood
flow and increase O2 saturation may be performed.
The preferred procedure is the modified blalock –
taussing shunt, which provides blood flow to the
pulmonary arteries from the left or right subclavion
artery.
In general, however shunts are avoided because
they may result in pulmonary artery distortion.

16. • Pott’s procedure :the upper decending
aorta is anastomosed with left pulmonary
artery
• Waterson shunt:it involves side to side
anastomosis of ascnding aorta with right
pulmonary artery.
• Brock’s procedure:pulmonary valvotomy
done to correct PS

17. • Complete Repair
Elective repair is usually performed in
the first year of life. Indications for repair
include increasing cyanosis and valves
closure of the VSD and resection of the
infundibular stenosis with a pericardial patch
to enlarge the right ventricular outflow tract.
The procedure requires a median sternotomy
and the use of pulmonary bypass.

18. Tricuspid Atresia
• Introduction:
Failure of the tricuspid valve to develop
consequently no communication form right atrium to
right ventricle. Blood flows through an atrial septal
defect (ASD) or patent foramen ovale to the left side
of the heart and through a VSD to the right ventricle
and out to lungs.

19. • It is often associated with PS and
transposition of the great arteries.
• There is complete mixing of unoxygenated
and oxygenated blood in the left side of
the heart.
• Resulting in systemic desaturation and
varying amount of pulmonary obstruction
causing decreased pulmonary blood flow
usually is diminished.

21. Pathoph
ysiology
At birth, the
presence of a
patent foramen
ovale is
required to
permit blood
flow across the
septum into the
left atrium.

22. CLINICAL
MANIFESTATIO
N
Cyanosis
Tachycardia
Dyspnoea
Chronic
hypoxemia with
clubbing

23. Therapeutic Management
For the neonate whose pulmonary blood
flow depend on the patency of the ductus
arteriosus a continuous infusion of
prostaglandin E, is started until surgical can
be arranged.

24. Surgical Management
Palliative treatment is the placement of a
shunt (systemic to pulmonary artery) to
increase blood flow to the lungs. If the ASD is
small, an atrial septostomy is done during
cardiac catheterization some children have
increased pulmonary volume of blood to the
lungs. A bidirectional glannshunt may be
performed at 6 to 9 months as a second
stage.

25. MODIFIED FONTAN PROCEDURE
Systemic venous return is directed to
the lungs without a ventricular pump through surgical
connections between the right atrium and the
pulmonary artery. A fenestration (opening) in the right
arterial baffle is sometimes done to relieve pressure.
Patient must have normal ventricular function and a
low pulmonary vascular resistance for the procedure
to be successful.

26. The modified fontan procedure separates
oxygenated and unoxygenated blood inside the
heart and eliminates the excess volumes load
on the ventricle but does not restore normal
anatomy or hemodynamic.

27. Complication
Dysrhythmias
Systemic venous hypertension
Pericardial effusion

28. TRANSPOSITION OF THE
GREAT ARTERIES
Transposition of great vessels (TGA) is
defined as aorta arising from the right
ventricle and pulmonary artery from the left
ventricle.

30. Sign & Symptoms
Cyanosis
Congestive heart failure
Fatigue
Slow weight gain
Clubbing of the finger
Rapid breathing
Heart size enlarge rapidly

31. Pathophysiology
In this anomaly the aorta has its origin in
the right ventricles and pulmonary artery has
its origins in the left ventricles.
Hence, the aorta carries unoxygenated blood
to the systemic circulation and the pulmonary
circuit carries oxygenated blood back to the
lungs.
The pulmonary venous return is to the left
atrium and the systemic veins returns to the

32. There is two separate circulatory
systemic exist, one pulmonary and
one systemic. An infant can survive
with this malformation initially only if
an associated with defect or PDA is
present
There co-existing lesions provide a
means for mixing venous and arterial
blood.

33. Diagnostic Evaluation
X – Ray
E.C.G
Cardiac catheterization
Angiocardiography
Blood examination
Echocardiogram

34. Treatment
The medical management consist in control of
congestive failure.
PALLIATIVE OPERATION
• Blalock Hanlon procedure -surgical creation or
enlargement of an existing arterial septal defect.
• Rash kind operation – enlargement of an existing
arterial septal defect.
CORRECTIVE SURGERY
Mustard’s operation – the complete repair of
the defect is done.

35. Nursing Management
• Provide comfort and adequate rest to the
child
• Avoid excessive crying.
• Observe the clinical features make the
nursing diagnosis & plan nursing action to be
carried out.
• Report the unusual symptoms like sign of
cardiac failure
• make proper positioning of the child
• Oxygen inhalation is given for cyanosis
• Diuretic to reduce over hydration

36. • Maintained the fluid & electrolyte balance
• Blood test should do to check acidosis.
• Small frequent feeds should be given
• Provide psychological support
• Provide love & affection to the child
• Meet all his hygienic needs with the help
of mother.
• administration of proper antibiotics
• Watch the possible complication