1. CYANOTIC HEART DISEASE
Dr.B.BALAGOBI

2. CYANOTIC DISEASE
• TOF(Tetralogy of fallot)
• TGV(Transposition of great vessels)
• Tricuspid atresia
• Truncus arteriosus
• Total anomalous of pulmonary venous drainage
• Hypoplastic left heart syndrome
• Pulmonary atresia
• Ebstein anomaly

3. Tetralogy of Fallot
• TOF is the most common cyanotic heart disease.
• 4 features
– VSD
– Overriding Aorta
– Infundibular Pulmonic Stenosis(more severe with
advancing ageR to L shunt increase)
– RVH
• Severity depend on PS.
• More frequently develops cyanosis in second half
of the first year.

6. Clinical features
• Hyper cyanotic spells after exercise/cry and
squatting position.
• clubbing.
• Long loud ejection systolic murmur(Due to PS)
– No PSM!
– During cyanotic episodes murmur is inaudible
• Chronic hypoxiaPolycythaemiaThrombosis(CVA)
• Infective endocarditis Cerebral abcess Hemiplegia

7. R/S aortic arch
Pulmonary artery Bay
Oligaemic lung field
Uplifted apex
Boot shaped small heart

8. Management of hypercyanotic spell
• Usually selflimiting
• If prolonged(>15 min) need Rx
– Morphinesedatereduce hyperventilationreduce
venous return reduce R-L shunt  reduce cyanosis
– Knee-chest position(y? use ur brain!)
– IV propranolol  relieve infundibular PS
– ABG PH,O2

9. Tetralogy: Surgical Treatment
• Systemic – Pulmonary Shunt[palliative surgery] to
increase pulmonary flow.
– Blalock-Taussig(BT shunt)
• Complete Repair at age of 6 months
– takedown of prior shunt
– patch VSD
– resection of subpulmonic obstruction

10. Transposition of the Great Arteries
• Aorta from right ventricle, pulmonary artery from
left ventricle.
• Cyanosis from birth, hypoxic spells sometimes
present.
• Heart failure often present.
• corrected initially with prostaglandin to keep
ductus open and balloon atrial septostomy to
improve systemic saturation
• repair via “atrial switch” Mustard procedure
• Cardiac enlargement and diminished pulmonary
artery segment on x-ray.

11. D-transposition of the Great Arteries

12. TGA
• The most common cyanotic defect presents
with cyanosis at neonatal period.
• The degree of saturation will depend on the
degree of mixing of the 2 “parallel” circuits.
• The mixing sites are: ASD, PDA, and VSD.
• The more mixing, the higher the “effective
pulmonary blood flow”

13. CLINICAL Findings TGA
• Cyanosis,Clubbing
• Possibly CHF
• Closely split/single S2
• If pulmonary stenosis present, may cause systolic
murmur.
• CXR: egg on side appearance
– Narrow mediastinum
– RVH increased pulmonary vascular markings

14. Total Anomalous Pulmonary Venous
Drainage
• Pulmonary veins do not make a direct connection with the
left atrium.
• Blood reaches the left atrium only through an atrial septal
defect or patent foramen ovale.
• Pulmonary congestion, tachypnea, cardiac failure, and
variable cyanosis.
• Operative repair in all cases
• Cyanosis variable and largely dependant on degree of
pulmonary venous obstruction.
• Snowman on CXR

15. Total Anomalous Pulmonary Venous Connection

16. Truncus Arteriosus
• A SINGLE GREAT VESSEL ARISES FROM THE
HEART AND GIVES OFF THE CA’S,PA’S AND
AORTA
• embryological structure known as the truncus
arteriosus not properly divides into the
pulmonary artery and aorta.
• Large VSD is present.
• Bounding pulses(it’s like a big PDA), There may
be a continous murmur if the PA’s are tight

18. TRUNCUS:Tx
• Decongestive tx pending surgery
• Surgery consists of VSD closure and a graft to
the PA’s.
• Early surgery essential.The average age of
death untreated is 5 wks.
• Sequelae:depends on degree of truncal valve
insufficiency and pulm artery obstruction

19. Ebsteins Anomaly
• Atrialization of RV, sail-like TV, TR
• 50% ASD/PFO
• 50% ECG evidence of WPW
• Age at presentation varies from
childhoodadulthood and depends
on factors such as severity of
TR, Pulm Vascular resistance in
newborn, and associated
abnormalities such as ASD
• Cyanosis and arrhythmias in infancy
are common.
• Right heart failure in half of patients.
• Operative repair with tricuspid valve
replacement.

20. Tricuspid Atresia
• Tricuspid valve is completely absent in about 2% of
newborns with congenital heart disease.
• Blood flows from right atrium to left atrium through
foramen ovale.
• Early cyanosis.DDs
– TGV,TA,PA,Severe TOF,Ebstein’s anomaly.
• Repair consists of shunt from right atrium to
pulmonary artery or rudimentary right ventricle
(Fontan procedure).

21. Adult Congenital Heart Disease
• Atrial Septal Defect
• Coarctation of Aorta
• Tetralogy of Fallot
• Common Ventricle
• Ebstiens Anomaly
• Eisenmenger Syndrome

22. T/F Causes for cyanosis in first 48
hours after birth?
A. Transposition of great vessels
B. VSD
C. Tricuspid atresia
D. Pulmonary atresia
E. PDA
F. Eisenmenger syndrome

23. Which of the following are associated
with recurrent LRTI?
A. PDA
B. TOF
C. AS
D. Small VSD
E. Eisenmenger syndrome

24. T/F TOF?
A.Majority will present at Day 1
B.Overriding of aorta is a feature
C.Cause cardiomegaly
D.Cause plethoric lung fields on CXR
E. Murmur is inaudible during
cyanotic episodes

25. T/F regarding TOF?
A. Is the commonest cyanotic congenital heart
disease
B. Β blockers are used in cyanotic spells
C. BT shunt improves the saturation
D. Cause cerebral abscess
E. Cause anaemia

26. T/F which of the following cyanotic heart diseases are
cause reduced pulmonary blood flow?
A. TGA
B. TOF
C. Tricuspid atresia
D. TAPVD
E. Eisenmenger syndrome

27. T/F Regarding following combinations?
A. Boot shaped heart - TOF
B. Egg on side appearance – TGA
C. Coil embolization – PDA
D. Down syndrome – Atrio ventricular canal
defect
E. Murmur radiates to the neck - AS .

28. T/F causes for ejection systolic murmur in
upper left sternal edge in 2 year old child.?
A. ASD
B. Large VSD
C. Small PDA
D. Anaemia
E. MS

29. T/F which of the following are normal
in child?
A. Parasternal heave
B. S3
C. Sinus arrhythmia
D. Heart rate of 150/min in a neonate
E. Central cyanosis

30. T/F features of cardiac failure?
A. Tender hepatomegaly
B. Ankle oedema
C. Sweating of the scalp
D. Gallop rhythm
E. Cyanosis

31. T/F which of the following are useful in
a cyanotic spell?
A. IV Morphine
B. IV propranalol
C. IV NaHCO3
D. IV frusemide
E. Knee chest position

32. T/F which of the following cause weak
thread pulse,
A. Cardiac failure
B. PDA
C. Septic shock
D. Hypovolaemic shock
E. AR
F. AV malformation

33. T/F, in tetralogy of fallots?
A. Usually cyanosis presents at birth
B. Cardiomegaly is commonly seen
C. ECG at birth shows right axis
deviation
D. Murmur is due to VSD
E. Recurrent infection is common

34. T/F Cyanotic heart disease with
decreased pulmonary blood flow?
A. Tricuspid atresia
B. Transposition of great
Arteries(TGA)
C. Tetralogy of fallot(TOF)
D. Total abnormally in pulmonary
venous drainage(TAPVD)
E. Eisenmenger’s syndrome

35. T/F Biventricular hypertrophy is a
feature of?
A.Septum secondum ASD
B. Small size VSD
C. PDA
D.TOF
E. PS

36. T/F Causes for loud second heart
sound?
A.Large PDA
B. Eisenmenger syndrome
C. Pulmonary stenosis
D.Systemic hypertension
E. Mitral stenosis

37. • The treatment of hypercyanotic spells includes
which of the following?
a) Isoproterenol
b) Adenosine
c) Digoxin
d) Oxygen
e) Furosemide
f)Morphine

38. • Hypercyanotic spells can be provoked by any
of the following:
• a) Pain
• b) Induction of anesthesia
• c) Dehydration
• e) Squatting
• f)Exercise