4. • Tetralogy of Fallot (TOF) is common
cyanotic congenital heart disorders (CHD)
• Tetralogy of Fallot results in an inadequate
flow of blood to lungs for oxygenation
(right-to-left shunt)
• Patients with Tetralogy of Fallot initially
present with cyanosis
5. ToF
Four anatomic malformations:
A- PulmonaryValve Stenosis
B- Over riding of aorta
C-Ventricular Septal Defect
D- RightVentricular Hypertrophy
6.
7. Clinical Presentation
Birth weight is low
Clinical presentation is directly related to the
degree of pulmonary stenosis
Severe stenosis results in immediate cyanosis
following birth
Mild stenosis will not present until later
Growth is retarded – insufficient oxygen and
nutrients
Development and puberty may be delayed
8. Poor feeding
Cyanosis
Cyanosis during feeding
Anoxic spells
Dyspnea on exertion
Tachypnea
Exercise intolerance
Fussiness and agitation
Clinical Presentation
9. Clinical Presentation
• “Tet spells” at 2-3 yrs
• Paroxysmal attacks of dyspnea
• Anoxic spells
• Predominantly after waking up
• child becomes distressed and inconsolable, without
apparent reason
“Tet Spell”
10. • Child cries→ Dyspnea (hyperpnea not tachypnea)→
progressively deeper Cyanosis → Loss of
consciousness → Convulsion (may experience syncope)
• During the spell diminished/absent murmur
• Frequency
once a few days to many attack everyday
“Tet Spell”
14. Investigations
• CBC
- hematocrit
↑
• ECG
-RVH, RAD
• Echocardiogram
A- PulmonaryValve Stenosis
B- Over riding of aorta
C-V S D
D- RightVentricular Hypertrophy
15.
16. CXR
•boot-shaped heart
secondary to uplifting of the
cardiac apex from RVH
• Decreased pulmonary
vascularity
• Right atrial enlargement
• Right-sided aortic arch (20-25%
of patients)
25. Surgical Intervention
Complete intracardiac repair
• Repair theVSD with a patch
Transcatheter patches
Open repair
• Repair of Pulm stenosis
either PA removed
or
removing the excessive muscle tissue
of PA
28. VENTRICULAR SEPTAL DEFECT
VENTRICULAR SEPTAL DEFECT
• most common ACHD
• 2nd
most common CHD(32%)
• SYNONYMS
* Roger’s disease
* Interventricular septal defect
29. PATHOPHYSIOLOGY
PATHOPHYSIOLOGY
• Primarily depends on ----size ofVSD
----status of pulm. vascular bed
(rather than location)
• Small communication (less than 0.5cm`)VSD is restrictive &
rt.ventricular pressure is normal – does not cause significant
hemodynamic derangement (Qp:Qs =1.75:1.0)
• Moderately restrictive VSD with a moderate shunt(Qp:Qs
=1.5-2.5:1.0) &poses hemodynamic burden on LV
• Large nonrestrictive VSDs(more than 1.0cm`) Rt & Lt
ventricular pressure are equalised (Qp:Qs is >2:1)
30. PATHOPHYSIOLOGY
PATHOPHYSIOLOGY
• LargeVSDs at birth ,PVR may remain higher than
normal and Lt to Rt shunt may intially limited –
involution of media of small pulm.arterioles,PVR
decreases—large Lt to Rt shunt ensues
• In some infants largeVSDs ,pulm. arteriolar
thickness never decreases –pulm. obstructive
disease develops .when Qp:Qs=1:1 shunt becomes
bidirectional,signs of heart failure abate &pt.
becomes cyanotic. (Eisenmenger syndrome)
33. CLINICAL FEATURES
CLINICAL FEATURES
• Race : no particular racial predilection
• Sex :no particular sex preference
• Age :infants
infants– difficult in postnatal
period,although ccf during first 6mths is
frequent,X-ray&ECG are normal.
children
children—after first year variable clinical
picture emerges.
smallVSD – asymptomatic
largeVSD – symptomatic
34. Common Symptoms of largeVSD
• Palpitation
• Breathing dificulty
• Dyspnoea on exertion
• Feeding difficulties
• Poor growth
• Frequent chest infections
35. PHYSICAL FINDINGS
PHYSICAL FINDINGS
• Pulse pressure - wide
• hyperkinetic Precordium with systolic thrill LSB
• S1&S2 are masked by a PSM at Lt. sternal border
• Max. intensity of the murmur is best heard at 3rd
,4th
&
5th
Lt intercostal space
• Lt. 2nd
space –widely split & accentuated P2
• Maladie de Roger
Maladie de Roger – smallVSD presenting in older
children as a loud PSM w/o other significant
hemodynamic changes
37. ECG
Small
restrictive
VSDs
Normal ECG
Medium-sized
VSDs
Lt atrial overload- Broad, notched P wave
Signs of LV volume overload — deep Q
and tall R waves with tall T waves in
leads V5 and V6
Atrial fibrillation
Large VSDs Rt ventr hypertrophy - right-axis
deviation
Biventricular hypertrophy - P waves
notched or peaked
38. COMPLICATIONS
COMPLICATIONS
• Congestive cardiac failure
• Infective endocarditis on Rt. ventricular side
• Aortic insufficiency
• Complete heart block
• Delayed growth & development (FTT) in infancy
• Damage to electrical conduction system during
surgery (causing arrythmias)
• Pulmonary hypertension
39. INTERVENTION
INTERVENTION
3 MAJOR TYPES
• SMALL
SMALL
(
(surface area < 0.5
surface area < 0.5 cm2
or <1/3
or <1/3rd
rd
of Aortic root size)
of Aortic root size)
- hemodynamically insignificant
- b/w 80-85% of allVSDs
- all close spontaneously
* 50% by 2yrs
* 90% by 6yrs
* 10% during school yrs
- muscular close sooner than membranous
40. • MODERATE VSDs
MODERATE VSDs
* surface area
surface area 0.5-1cm2
or <1/2 of
<1/2 of
Aortic root size
Aortic root size
* least common group of children(3-
5%)
* w/o evidence of ccf/ pulm.htn can be
followed until spontaneous closure
occurs.
41. • LARGE VSDs WITH NORMAL PVR
LARGE VSDs WITH NORMAL PVR
* surface area
surface area >1 cm2
or ≥ of Aortic root
≥ of Aortic root
size
size
* usually requires surgery otherwise…
develop CCF & FTT by age of 3-6mths.
Conservative treatment
- Treat CCF & prevent development of pulm.
vascular disease
- prevention & treatment of infective
endocarditis
42. INDICATIONS for SURGERY
INDICATIONS for SURGERY
• VSDs at any age where clinical symptoms and FTT
cannot be controlled medically.
• Infants b/w 6-12mths of age with large defects ass. with
PH ,even if symptoms are controlled by medication.
• Pt.s older than 24mths of age with Qp:Qs is greater
than 2:1.
• Pt.s with supracristalVSD of any size, because of high risk
of development of AI.
CONTRAINDICATION
CONTRAINDICATION –severe pulmonary vascular
disease.
