1. To summarize T.O.F.
To summarize T.G.A.
To summarize tricuspid atrsia.
To summarize Ebstein’s anomaly
To outline the non cardiac complications of cynotic C.H.D.

2. Cyanotic Congenital Heart Disease

3. Lesions Associated with Decreased Pulmonary Blood Flow
Tetralogy of Fallot

4. Epidemiology and pathology
Tetralogy of Fallot is the most common cyanotic congenital heart defect, representing about 10% of all congenital heart defects . Anatomically, there are four structural defects: VSD, pulmonary stenosis, overriding aorta and right ventricular
hypertrophy. The VSD
is large and the
pulmonary stenosis is
most commonly
subvalvularor (infundibular).

5. Clinical features
A- Symptoms
1.  Most patients are symptomatic with cyanosis
at birth or shortly thereafter. Dyspnea on
exertion, squatting, or hypoxic spells develop
later, even in mildly cyanotic infants .
2. Occasional infants with acyanotic TOF may be asymptomatic or may show signs of CHF from a large left-to-right ventricular shunt.
3.Immediately after birth, severe cyanosis is seen in patients with TOF and pulmonary atresia.

6. 4-Hypoxic ("Tet") spells occur during the 1st 2 yr of life, they are usually progressive. During a spell, the child typically becomes restless and agitated, crying inconsolably, hyperpneic with gradually increasing cyanosis and of the murmur. In severe spells, they develope unconsciousness , convulsions, hemiparesis, or death may occur.
Independent of hypoxic
spells, patients with tetralogy
are at increased risk for
cerebral thromboembolism and
cerebral abscesses resulting in
part from their right-to-left
intracardiac shunt.

7. Placement of the infant on the abdomen in the knee-chest position while making certain that the infant's clothing is not constrictive. Premature attempts to obtain blood samples may cause further agitation and –ve effect.
O2 adminstration
S.c morphine not > 0.2mg/kg.
B-adrenergic blockers e.g propranolol ( ) mg /kg i.v and slowly.
Treatment of spells

8. Physical examination
1-Growth and developmental delay in patients with severe untreated tetralogy of Fallot, particularly when oxygen saturation is chronically <70%. Puberty may also be delayed in patients who do not undergo
Surgery.
2-Varying degrees of cyanosis, tachypnea, and clubbing (in older infants and children) are present.
3-The pulse is usually normal, as is venous and arterial pressure.

9. 4-The left anterior hemithorax
may bulge anteriorly because
of RVH.
5-In about half the cases,
a systolic thrill is felt along the
left sternal border .
6-The murmur is usually loud,
systolic, most intense at the left
sternal border. It is due to PS and
generally ejection in quality , but it
may sound more holosystolic
toward the lower sternal border.

10. 1-The classic chest x-ray finding is a boot-shaped heart created by the small main pulmonary artery and upturned apex secondary to right ventricular hypertrophy with olegaemic lungs.

11. 2-The ECG usually has right axis deviation and right ventricular hypertrophy.

12. VSD

13. 4-Cardiac catheterisation and right ventriculography may be required to show the detailed anatomy of the pulmonary arteries, which may be small or stenosed.
Lateral view of a selective right ventriculogram in a patient with the tetralogy of Fallot. I- is the infundibular stenosis that is below the infundibular chamber (C). V-is the narrowed pulmonary valve orifice is seen at the distal end of the infundibular chamber. V I

14. Natrural history and complications
1. Worsening of cyanosis with time.
2. Physicians need to watch for the development of relative iron-deficiency state (i.e., hypochromia).
3. Polycythemia develops secondary to cyanosis.
4. Growth retardation may be present if cyanosis is severe.
5. Hypoxic spells may develop in infants.
6. Brain abscess and cerebrovascular accident rarely occur
7. SBE is occasionally a complication.
8.Coagulopathy is a late complication of a long-standing cyanosis.

15. Management
Initial management is medical, with corrective surgery at around 6 months of age. It involves closing the VSD and relieving right ventricular outflow tract obstruction with an artificial patch, which sometimes extends across the pulmonary valve.

16. Infants who are very cyanosed in the neonatal period require a shunt to increase pulmonary blood flow. This is usually done by surgical placement of an artificial tube between the subclavian artery and the pulmonary artery (a modified Blalock-Taussig shunt) or sometimes by balloon dilatation of the right ventricular outflow tract

17. *Frequently there is no significant murmur.
TRICUSPID ATRESIA
Etiology, Epidemiology and pathophysiology
Tricuspid atresia accounts for approximately 2% of all congenital
heart defects . The absence of the tricuspidvalve results in a hypoplastic
right ventricle. All systemic venous return must cross the atrial septum
Into the left atrium. A PDA or VSD is
necessary for pulmonary blood flow
andsurvival.
Clinical Manifestations
Severe cyanosis
If a VSD is present, there may be a murmur. A diastolic murmur across the mitral valve may be audible.
*Frequently there is no significant murmur.

18. TRICUSPID ATRESIA Diagnosis ECG shows LVH and Lt axis deviation.
CXR shows cardiomegaly with decreased pulmonary blood flow
Echo is diagnostic and shows the abnormalities.
Treatment
If there is no VSD PGE1 is infused to maintain ductal patency and pulmonary flow. Surgical correction involve (Blalock-Taussig procedure) then Fontan opertation.
SVC
RPA
IVC

19. Ebstein Anomaly Pathophysiology
Ebstein anomaly consists of downward displacement of an abnormal tricuspid valve into the right ventricle.
Pathophysiology
The right ventricle is divided into 2 parts by the abnormal tricuspid valve: the 1st, a thin-walled “atrialized” portion, is continuous with the cavity of the right atrium; the 2nd, often smaller portion consists of normal ventricular myocardium.
RV output is decreased due to a combination of the poorly
functioning small right ventricle
and tricuspid valve
regurgitation,
“atrialized” portion

20. *Neonates with severe form present with severe cyanosis,
Ebstein Anomaly
Clinical Manifestations
Symptoms
Severity is variable depending on the severity of pathology.
Mild cases present at teenage or adulthood with fatigue and
palpitation as a result of cardiac dysrhythmias.
There may be atrial right-to-left shunt through an ASD
causing cyanosis and polycythemia.
Physical signs
1- High JVP.
2- A holosystolic murmur caused by tricuspid regurgitation is
audible over most of the anterior left side of the chest.
*Neonates with severe form present with severe cyanosis,
heart failure and pulmonary hypoplasia and may die.

21. Ebstein Anomaly Diagnosis
ECG shows RBB, large tall p wave, prolonged PR interval and SVT due to Wolf-Parkinson white syndrome.
CXR shows cardiomegaly
Echo is diagnostic and shows the abnormalities.
Treatment
In non severe cases by surgical repair of TV
In severe cases by single ventricle repair ( Fontan opertation)
SVC
RPA
IVC

22. D-Transposition of the Great Arteries
Lesions Associated with Increased Pulmonary Blood Flow
D-Transposition of the Great Arteries

23. Etiology and Epidemiology
T.G.A represents about 5% of congenital
heart defects, and it is the most common
cyanotic lesion to present in the Newborn
period . T.G.A is ventriculoarterial
discordance secondary to abnormalities of
septation of the truncus arteriosus, the
aorta arises from the right ventricle,
anterior and to the right of the pulmonary
artery, which arises from the left
ventricle. This transposition results in desaturated blood returning to the right heart and being pumped back out to the body, while well-oxygenated blood returning from the lungs enters the left heart and is pumped back to the lungs. Without mixing of the two circulations, death occurs quickly. Mixing can occur at the atrial (patent foramen ovale/ASD), ventricular (VSD), or great vessel (PDA) level

24. CLINICAL MANIFESTATIONS
1.    History of cyanosis from birth is always present.
2.    Symptoms of congestive heart failure (CHF) with dyspnea and feeding difficulties may develop during the newborn period
On examination:-
1.Moderate to severe cyanosis and tachypnea.
2. No VSD no murmur, VSD holosystolic murmur, and there may be murmur of RVOT or LVOT obstruction.
3. There may be features of heart failure.

25. 1. Cardiomegaly with increased pulmonary vascularity .
   Investigations
X-ray Studies
1. Cardiomegaly with increased pulmonary vascularity .
2. An egg-shaped cardiac silhouette .
The electrocardiogram shows the normal neonatal right-sided dominant pattern.

26. Echocardiography.
Two-dimensional echo and color flow Doppler studies usually provide all the anatomic and functional information needed for the management of infants with D-TGA.

27. Treatment Initial medical management includes prostaglandin E1 to
maintain ductal patency. If
the infant remains significantly
hypoxic on prostaglandin
therapy, a balloon atrial
septostomy is performed to
improve mixing between the
two circulations.

28. Complete surgical repair is most often an arterial switch; the atrial switch is rarely done. The arterial switch usually is performed within the first 2 weeks of life, when the left ventricle still can maintain a systemic pressure

29. Extracardiac Complications of Cyanotic Congenital Heart Disease and Eisenmenger Physiology

30. CNS, central nervous system; DIC, disseminated intravascular coagulation; RSV, respiratory syncytial virus.