2. Objective
ďRevise the fetal circulation in short
ďClassification of congenital heart disease
ďDiscuses the common acyanotic and cyanotic congenital heart diseases and their
clinical presentation, Investigation and principles of management.
3. Fetal circulation
The right and left ventricles exist in a parallel circuit.
In the fetus gas and metabolite exchange provided by the placenta
Parallel circuit maintained
ďź Foramen ovale
ďź Ductus arteriosus
ďź Ductus venosus
4. Fetal circulation
placenta
ďź largest amount of combined ventricular output
ďź Has lowest vascular resistance in the fetus
ďź Placenta empties into IVC therefore IVC oxygen saturation
greater than SVC saturation
ďź Highest PO2 (32 mmHg )is found in the umbilical vein
Ductus Venosus
ďź Oxygenated blood running from the placenta flow to the fetus
through umbilical vein and 50 % bypass the liver enters IVC
via DV
5. Fetal circulation
Foramen Ovale
ďźAbout one third of IVC blood directed in to LA through
the foramen ovale and to LV ejected to the ascending
aorta
ďźThe result is higher saturated blood being delivered to
the brain and coronary arteries
ďźMost SVC blood drains into RV through tricuspid valve
ďźFrom the RV to the pulmonary artery and because of
pulmonary arterial circulation constricted only 10% of the
RV out put enters the lungs .
6. Fetal circulation
Ductus arteriosus
ďźLess oxygenated blood shunted through the ductus
arteriosus away from the lungs and into the descending
aorta to perfuse the lower part of the body.
ďźThe blood return to the placenta via the to umbilical
arteries
ďźThis low oxygen content increases the efficiency of
oxygen exchange in the placenta
7. Transitional circulation
1. Interruption of Umbilical cord
ďź Removal of the low resistance placental circulation result in
an Increased systemic vascular resistance
ďź lack of blood flow through the placenta leads to closure of
Ductus venosus
2. Expansion of lungs
ď Mechanical expansion of the lungs and increased arterial P02
result in a rapid decrease in pulmonary vascular resistance
ď The output from RV flows entirely to the pulmonary
circulation
ď The increased blood volume from the pulmonary circulation
increased the LA volume and pressure sufficiently to close
the foramen ovale
8. Transitional circulation
3. PDA closure
The ductus flow become left to right and later the ductus will
obliterated and become ligamentum arteriosus
ďźFunctional closure at 10 to 15 hours after birth
ďźIncreased systemic O2 saturation is strongest stimulus or
constriction of ductal smooth muscle
ďźGestational age: decreased responsiveness of ductal smooth
muscle to oxygen in premature infant
ďźDecreasing prostaglandin levels leads to closure
9. Transitional circulation
ďźThe right ventricle is coupled with low
resistance pulmonary circulation and its wall
thickness and mass decreased
ďźThe left ventricle coupled to high resistance
systemic circulation and its wall thickness and
mass increased and deliver the entire systemic
cardiac out put.
10. Congenital Heart disease
Introduction:
Def: Structural or functional heart disease that present at birth.
It is not static ,there is always a continuous anatomical or physiological
change
⢠The incidence is higher in abortus and still births
⢠Estimate in live birth range from 4-10.2 per 1000live birth.
11. ContâŚ.
⢠The incidence of specific type of CHD varies from one country to
another
⢠Specific aetiology only known 10%
⢠8% genetic
⢠2% environmental (rubella, foetal-alcohol syndrome
⢠90% Multifactorial inheritance
12. Incidence of Congenital heart Disease
Developed Ethio-Swedish
1986
2003-5
TAH
VSD 28.3% 41 27%
PDA 12% 13% 15.8%
ASD 10.7% 13.6% 10.7%
COA 8.8% <2% 0.6%%
TOF 7% 9% 5.1%
PS 6% 9.9% 8.5%
AS 2.3% 3.5% 3.4%
13. cont.âŚ.
⢠The risk of recurrence in siblings varies from 1-4%
⢠Third Pregnancy 20-30%
⢠Parents with CHD 4-6%
⢠Varies with type of inheritance
⢠Except PDA and ASD males are more affected than females.
14. Classification of Congenital Heart
Diseases:
Common Acyanotic Heart Diseases:
⢠Ventricular septal defect
⢠Atrial septal defect
⢠Patent ductus arteriosus
⢠Coarctation of the aorta
⢠Pulmonary stenosis
⢠Aortic stenosis
15. Classification of CHD
Common cyanotic Heart Diseases:
⢠Tetralogy of Fallot
⢠Transposition of the Great Arteries
⢠Truncus Arteriosus
⢠Severe Pulmonary stenosis
⢠Pulmonary Atresia
⢠Tricuspid Atresia
⢠Hypoplastic heart diseases
16. Clinical manifestation of CHD
CHD suspected in any child with:
⢠Feeding difficulty
â˘Recurrent attack of respiratory tract infection
â˘Growth failure
â˘Cyanosis unresponsive to 100% oxygen-failed
hyperoxia test(pO2<100)
â˘Tachycardia
â˘Respiratory distress
â˘Rhythm disturbance
â˘Murmur
17. Managementt of CHD
General principle
⢠1) Correction of underlying defect (timing depend on the type and severity).
⢠2) Treatment of Congestive Heart Failure
ďźDiuretics
ďźInotropic support
ďźAfter load reduction
⢠3)Prevention and treatment of complication
ďźPulmonary hpt : early surgical correction
ďźInfective endocarditis: Administration of antibiotic chemoprophylaxis as indicated.
ďźNon infective thromboembolism : prevent polycythemia
ďź polycythemia :partial exchange transfusion
⢠Counselling of parents on the risk of recurrence
18. Ventricular Septal Defect
â˘Most common CHD
â˘Both sexes are equally
affected
â˘Incidence of 1/3000
â˘Can be single or multiple
â˘Can be associated with
other congenital heart
diseases
19. VSD cont.âŚ
Types of VSD
⢠70% membranous close to pulmonary valve and Pulmonary artery
⢠20% muscular
⢠5% Aortic valve (sub aortic)
⢠5% near junction of mitral and tricuspid valve (A-V canal defect)
20. Clinical manifestations
I) Asymptomatic:
⢠Small VSD , trivial shunt ,the pulmonary pressure is normal
⢠Loud harsh Holosystolic murmur at LLSB, with thrill
⢠X-ray is normal
⢠EKG normal
21. VSD CONTâŚ.
II ) Large defects:
⢠Excessive pulmonary blood flow lead to pulmonary hypertension
⢠Dyspnea, feeding difficulty
⢠Poor growth
⢠Profuse perspiration
⢠Recurrent pulmonary infection
22. Physical Examination:
⢠Prominence of the precordium
⢠Palpable parasternal lift
⢠Apical trust with systolic thrill
⢠Holosystolic murmur at LLSB less harsh and more blowing
⢠Diastolic murmur at the apex
⢠Increased P2 indicate pulmonary hpt.
23. VSD contâŚ.
Severity depend on :
â˘The size of the defect
â˘Level of pulmonary resistance to systemic
resistance.
â˘Defects < 0.5 restrictive
> 1cm sq non restrictive
â˘Majority closes spontaneously
â˘Large defects lead to CHF at early age
28. CLINICAL COURSE:
Small sized defects
⢠are closed spontaneously in the first year of life
⢠The risk of endocarditis is independent of the size
Moderate to large defects:
⢠decreased in size but not closed
⢠Heart failure and growth failure is common at the early age
⢠Risk of pulmonary hypertension lead to pulmonary vascular
diseases.
⢠Eisenmengers syndrome due to reversal of shunt which
presented with absence of thrill and cyanosis, decreased heart
size.
29. Treatment
Small size
⢠Reassurance
⢠No surgical treatment
⢠Maintain integrity of primary and permanent teeth
⢠Give anti- infective endocarditis prophylaxis
ďź Antibiotic prophylaxis before
ďś dental visit
ďś Tonsillectomy
ďś instrumentation of GUT,GIT
30. Treatment contâŚ.
Large VSD:
⢠Control CHF
â˘Prevent development of Pulmonary vascular
disease.
ďź Surgical closure in the first year of life (6M-12M).
ďźPalliative : pulmonary banding if surgery is not
possible for the time being.
ďźDevice closure of the VSD with Amplatzer device
Umbrella)
31. Patent Ductus Arteriosus(PDA)
⢠During foetal life blood from PA
shunted through the DA in to the
Aorta.
⢠After birth closed functionally
⢠Prematurity and hypoxia
predispose for patencey
⢠Females are more affected than
males Commonly associated with
rubella of the mother
⢠Isolated PDAs are common in high
altitude
32. Pathophysiology
Blood flow from the aorta to the pulmonary.
Extent of the shunt depend on
⢠size of the ductus
⢠ratio of pulmonary and systemic vascular resistance
33. Clinical manifestation
Depend on the :
â˘Size of the defect and direction of flow
â˘Small defects no symptom
â˘Large defect result in Large left to right shunt
ďźCHF
ďźGrowth Failure
ďźRepeated ARI
ďźReversal of shunt ,(Eisenmengers ) result in dyspnea and
cyanosis
34. Physical EXAMINATION
â˘Bounding pulse
â˘Wide pulse pressure
â˘Heave , thrill in the 2nd ics
â˘Continuous machinery murmur 2nd ics
â˘EKG: bi-ventricular hyperthrophy
â˘X-ray: prominent PA, increases PA marking enlarged
chambers(LA,LV),
â˘ECHO: size of the PDA, direction of flow, chamber size
â˘Catheterization: a step up oxygen saturation, PDA
anatomy in angiography
35. Clinical course:
â˘Small defects : few or no cardiac symptoms
â˘Large defects:
â˘CHF
â˘Infective endocarditis
â˘Systemic emboli
â˘Calcification of the ductus
â˘Non infective thrombosis with embolization
â˘Paradoxical emboli
â˘Eisenmengers syndrome if left untreated.
36. Treatment
Medical therapy:
⢠Congestive heart failure treatment
⢠infective endocarditis prophylaxis
⢠Surgical closure of the PDA (banding)
⢠Closure of the PDA coil embolization
37. Coarctation of the Aorta
⢠Occurs at any point from transverse arch
to iliac bifurcation
⢠98% below the origin of left sub clavian at
the origin of Ductus
⢠Male to female ratio: 2:1
Associated with:
⢠Turner syndrome
⢠Bi cuspid aortic valve (70%)
⢠Left sided obstructive lesions ( Shone
complex-AS,MS,PS,HOCM)
⢠Mitral valve abnormality
⢠Supravalvar mitral ring
⢠Parachute mitral valve
⢠Sub aortic stenosis
38. Pathophysiology
⢠Collaterals develop to by pass the obstruction.
⢠Hypertension of the aortic branch proximal to coarctation
⢠In Pre ductal type the RV blood ejected through the ductus to supply the
descending aorta lead to differential cyanosis.
43. Treatment
â˘Neonatal: closure of the ductus lead to hypo perfussion
and acidosis, thus give infusion of prostaglandin(PGE1-
Alprostadil) to reopen the ductus, after stabilization
surgical treatment.
â˘Older children with CHF and no hypertension medical
treatment followed by surgery or angioplasty.
â˘Re -stenosis balloon angioplasty is safe.
44.
45. Incidence of CHD
Developed Ethio-Swedish
1986
2003-5
TAH
VSD 28.3% 41 27%
PDA 12% 13% 15.8%
ASD 10.7% 13.6% 10.7%
COA 8.8% <2% 0.6%%
TOF 7% 9% 5.1%
PS 6% 9.9% 8.5%
AS 2.3% 3.5% 3.4%
47. Pathophysiology:
â˘Severity directly proportional to the degree of RVOT obstruction.
â˘Change in pulmonary and systemic vascular resistance and the degree of
RVOT obstruction affect degree of R-L shunt.
â˘Infundibular stenosis is progressive.
48. Clinical Manifestations:
Variable depend on RVOT obstruction
ď pink to cyanosis
ďCHF is not a usual manifestation of TOF
ďSquatting
ďDyspnoea on exertion
ďHypoxic spells
ďGrowth failure
52. Chest x ray (TOF)
⢠Normal sized, boot
shaped heart.
â˘Reduced pulmonary
vascular marking
(oligemic depend on the
degree of RVOT
obstruction.
53. Echocardiography (TOF)
⢠Location and size of the VSD
⢠The aortic override
⢠The degree of RVOT obstruction
⢠The size of pulmonary valve
annulus
⢠Look for additional pulmonary
artery branch stenosis.
⢠Look for other associated
anomalies
ďźRight aortic arch
ďźCoronary arteries anatomic
variations
55. Complications:
ďCardiovascular accidents : occurs in 4-5% of
cases is due to cerebral embolism.
ďBrain abscess: rare in the first two years of life
may be due to small cerebral infract which is
super infected due to bacteraemia.
ďInfective endocarditis
ďPolycythemia
56. Management
Medical management:
A) Neonatal period if pulmonary flow is dependent of DA, give
prostaglandin to prevent ductal closure and followed by palliative
shunt (B-T Shunt)
B)Recognition and treatment of hyper cyanotic spell.
ďKnee chest positioning of the patient
ďAdministration of oxygen
ďVolume expansion
ďCorrection of acidosis
ďSedation with morphine
ďPropranolol
C) Treat infective endocarditis
D) Anaemia should be corrected
E) Severe polycythemia correction with phlebotomy should be
done.
57. Management
Surgical Management:
A) Palliative: Modified Blalock-Taussig shunt between pulmonary
artery branch and subclavian artery
B) Corrective surgery : closing of the VSD and reliving all possible
source of RVOT obstruction.
58.
59. CompleteTranspostion of the Great Arteries
⢠The great arteries arise from
morphologically wrong
ventricles. (The aorta arise
from the RV and Pulmonary
arises from LV in the setting
of a concordant
atrioventricular connection.
60. Transposition of the Great Arteries
â˘TGA occurs in 8-9% of
CHD
â˘Male are affected than
Females
â˘Different variety exist
61. Hemodynamics
ďThe RV pressure is systemic
ďThe blood returning from the lung pass to the lung via PA
ďSystemic venous return passes back to the systemic
circulation via the aorta.
ďBoth ventricles are volume overloaded.
ďThe right ventricle also pressure overloads and result in CHF
ďSurvival depend on associated Large ASD, VSD,PDA.
62. Clinical features:
ďCyanosis detected 87% of the neonates
immediately ; rest later at the age of 1 to 3
month and is progressive.
ďClubbing
ďSquatting only 20% of patients
ďDyspnoea
ďCardiac failure
ďCough
63. Physical findings
1)TGA with no VSD
ďCyanosis intense
ďPrecordial lift
ďLoud first sound
ďSplitted S2
ďMurmur may not be heard
2)TGA with large VSD
ďCyanosis less intense
ďCardio -Respiratory distress, retraction
ďOedema
ďHepatomegally
ďLeft and right ventricular pulsations are prominent
ďSystolic murmur at the LLSB
ďDiastolic rumble at the apex.
65. Electrocardiography (TGA)
TGA with intact ventricular septum:
â˘P wave tall
â˘P upright T wave in lead V1 and V3R
â˘Right ventricular hypertrophy
â˘Right axis deviation
TGA with VSD:
â˘Bi atrial enlargement
â˘Bi Ventricular hypertrophy
â˘Right axis deviation
66. Echocardiography(TGA)
Shows the origion of :
ďźpulmonary artery from
the LV
ďźAorta from RV
â˘Associated lesions like
VSD and ASD be
identified.
â˘Coronary artery anatomy
67.
68. Prognosis
⢠Patient die of anoxia and or CHF in the first 6 month of life if there is
little communication.
⢠TGA with VSD and PS favourable
69. Management
Medical management:
â˘Prostaglandin to keep the DA open until palliation
done
â˘Give oxygen
â˘Treat metabolic acidosis with bicarbonate.
Surgical:
â˘Palliative balloon septostomy creating /increasing
ASD Rashkind procedure.
â˘Large VSD do pulmonary banding
â˘Intra atrial switch (Mustard procedure)
â˘Arterial Switch (Jaten Procedure)