Tetralogy of Fallot is the most common cyanotic heart disease characterized by four features: ventricular septal defect, overriding aorta, infundibular pulmonary stenosis, and right ventricular hypertrophy. It presents with cyanosis that is more frequent in the second half of the first year and hypercyanotic spells after exercise or positions like crying and squatting. Treatment involves palliative surgery like Blalock-Taussig shunt initially, followed by complete repair around 6 months of age. Management of hypercyanotic spells includes medications like morphine, oxygen, and positioning changes.
TAPVC defines the anomaly in which the pulmonary veins have no connection with the left atrium. Rather, the pulmonary veins connect directly to one of the systemic veins (TAPVC) or drain in to right atrium.
A PFO or ASD is present essentially in those who survive after birth
When pulmonary veins drain anomalously into the right atrium either because of complete absence of the interatrial septum or malattachment of the septum primum , then it is known as total anomalous pulmonary venous drainage.
When some or all of the pulmonary veins drain anomalously in to RA or its tributaries without being abnormally connected, the terms partially anomalous pulmonary venous drainage (PAPVD) or totally anomalous pulmonary venous drainage (TAPVD) with normal pulmonary venous connections are used.
TAPVC defines the anomaly in which the pulmonary veins have no connection with the left atrium. Rather, the pulmonary veins connect directly to one of the systemic veins (TAPVC) or drain in to right atrium.
A PFO or ASD is present essentially in those who survive after birth
When pulmonary veins drain anomalously into the right atrium either because of complete absence of the interatrial septum or malattachment of the septum primum , then it is known as total anomalous pulmonary venous drainage.
When some or all of the pulmonary veins drain anomalously in to RA or its tributaries without being abnormally connected, the terms partially anomalous pulmonary venous drainage (PAPVD) or totally anomalous pulmonary venous drainage (TAPVD) with normal pulmonary venous connections are used.
TGA is a complex congenital heart disease.Understanding the anatomy,physiology,surgery and anaesthetic management is very important for patient's better outcome.This ppt explains all these points in detail.
Some babies with tricuspid atresia have other conditions, such as pulmonary stenosis or transposition of the great arteries, that also affect blood flow through their heart. These conditions require treatment, too.
A detailed discussion on embryogenesis of heart and ennumeration of all congenital diseases and description of cyanotic congenital heart disease , each disease in detail.
TGA is a complex congenital heart disease.Understanding the anatomy,physiology,surgery and anaesthetic management is very important for patient's better outcome.This ppt explains all these points in detail.
Some babies with tricuspid atresia have other conditions, such as pulmonary stenosis or transposition of the great arteries, that also affect blood flow through their heart. These conditions require treatment, too.
A detailed discussion on embryogenesis of heart and ennumeration of all congenital diseases and description of cyanotic congenital heart disease , each disease in detail.
Acyanotic Congenital Heart Diseases;
1. Left-to-right shunts
a. Ventricular Septal Defect(VSD)
b. Atrial Septal Defect(ASD)
c. Patent Ductus Arteriosus(PDA)
d. Atrioventricular Septal Defect(AVSD)
e. Aortopulmonary window
* Eisenmenger Syndrome – The shunt becomes right-to-left
2. Left-sided obstructive lesions
a. Coarctation of the Aorta(COA)
b. Congenital Aortic Stenosis
c. Mitral Stenosis
d. Interrupted Aortic Arch
Cyanotic Congenital Heart Diseases;
1. Right-to-left shunts
a. Tetralogy of Fallot
b. Pulmonary stenosis
c. Pulmonary atresia
d. Tricuspid atresia
e. Ebstein’s anomaly
2. Complete mixed lesions
a. Transposition of the great vessels
b. Double outlet right ventricle(DORV)
c. Total anomalous pulmonary venous return
d. Truncus arteriosus
e. Hypoplastic left heart syndrome
most common congenital cyanotic heart disease.one of the conotruncal family of heart lesions.. It accounts for 7 to 10% of all congenital heart abnormalities.
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 ageR to L shunt increase)
– RVH
• Severity depend on PS.
• More frequently develops cyanosis in second half
of the first year.
4.
5.
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 hypoxiaPolycythaemiaThrombosis(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
– Morphinesedatereduce hyperventilationreduce
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.
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
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
17.
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
childhoodadulthood 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
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