TOF, VSD in children. MBBS lecture

1. Congenital
Heart Disease
Dr. Muhammad Sajjad Sabir
MBBS, DCH, MCPS, FCPS
Assistant Professor of Paediatrics

2. VSD
ASD
PDA
Tetralogy of Fallot(TOF)
Transposition of great
arties(TGA)
Ebstein anomaly
Hypoplastic left heart
syndrome
Total anomalous
pulmonary venous return
(TAPVR)
Aortic Stenosis
Pulm. Stenosis
Coarctation of
aorta

3. Tetralogy of Fallot

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

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”

11. • Sitting posture – squatting
• Compensatory mechanism
• Squatting ↑ses peripheral (systemic) vascular resistance
• Which diminishes right-to-left shunt
• Increases pulmonary blood flow
• Increases oxygenation of blood
“Tet Spell”

12. Physical Examination
• Clubbing + Cyanosis (Variable)
• Squatting position
• bulging left hemithorax
• Prominent “a” waves JVP
• Normal heart size
• Mild parasternal impulse
• Systolic trill (30%)

14. Investigations
• CBC
- hematocrit↑
• ECG
-RVH, RAD
• Echocardiogram
A- PulmonaryValve Stenosis
B- Over riding of aorta
C-V S D
D- RightVentricular Hypertrophy

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)

17. Course and Complication
• Each anoxic spell is potentially fatal
• Polycytemia
• Cerebral thrombosis
• Cerebral abcess
• Seizures
• Hypoxic damage
• Infective Endocarditis & vegetations
• Postoperative strokes

18. MANAGEMENT

19. Management of anoxic spell
1)Calm the baby
2) increase SVR
3)Knee chest position
4)Humidified O2
5)Morphine 0.1 -0.2 mg/Kg Subcutaneous
6)Correct acidosis – Sodium Bicarb IV
6)Inj Phenyelphrine
7)Propranolol 0.1mg/kg/IV during spells
0.5 to 1.0 mg/kg/ 4-6hourly orally
7)Vasopressors
8)Correct anemia
9) PHELABOTOMY if polycythemia

20. Blalock Taussig Shunt
• Classic BT shunt 1945…
• Lt Subclavian artery – Lt Pulmonary artery anastomosis

21. Modified Blalock Taussig Shunt
Rt Subclavian artery – Rt Pulmonary artery anastomosis

22. Potts Shunt
Side - side anastomosis of
Lt Pulmonary Artery to
Descending Aorta

23. Waterston shunt
Side-side anastomosis of Rt
Pulmonary Artery to
Ascending Aorta

24. Palliative Procedures

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

26. Complications:
•Infective bacterial endocarditis
•pulmonic regurgitation
•Arrhythmias
•RBBB
•Left anterior hemiblock

27. VENTRICULARVENTRICULAR
SEPTAL DEFECTSEPTAL DEFECT
(VSD)(VSD)

28. VENTRICULAR SEPTAL DEFECTVENTRICULAR SEPTAL DEFECT
• most common ACHD
• 2nd
most common CHD(32%)
• SYNONYMS
* Roger’s disease
* Interventricular septal defect

29. PATHOPHYSIOLOGYPATHOPHYSIOLOGY
• 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. PATHOPHYSIOLOGYPATHOPHYSIOLOGY
• 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)

31. ANATOMICAL CLASSIFICATION
MEMBRANOUS SEPTUM
paramembranous/perimembranous defect
(or infracristal, subaortic, conoventricular)
MUSCULAR SEPTUM
inlet, trabecular, central, apical, marginal or
swiss-cheese
OUTLET SEPTUM deficient
supracristal,subpulmonary,infundibular or conoseptal
SEPTAL DEFICIFNCY
AVseptal defect (AVcanal)

33. CLINICAL FEATURESCLINICAL FEATURES
• Race : no particular racial predilection
• Sex :no particular sex preference
• Age :infantsinfants– difficult in postnatal
period,although ccf during first 6mths is
frequent,X-ray&ECG are normal.
childrenchildren—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 FINDINGSPHYSICAL 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 RogerMaladie de Roger – smallVSD presenting in older
children as a loud PSM w/o other significant
hemodynamic changes

36. INVESTIGATIONSINVESTIGATIONS
• ECHOCARDIOGRAPHYECHOCARDIOGRAPHY
two-dimensional & doppler colour flow
• CHEST RADIOGRAPHYCHEST RADIOGRAPHY
- normal
- biventricular hypertrophy
- pulmonary plethora
• ANGIOGRAPHY
(cardiac catheterization and angiography)

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. COMPLICATIONSCOMPLICATIONS
• 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. INTERVENTIONINTERVENTION
3 MAJOR TYPES
• SMALLSMALL
((surface area < 0.5surface area < 0.5 cm2
or <1/3or <1/3rdrd
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 VSDsMODERATE VSDs
* surface areasurface area 0.5-1cm2
or <1/2 of<1/2 of
Aortic root sizeAortic 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 PVRLARGE VSDs WITH NORMAL PVR
* surface areasurface area >1 cm2
or ≥ of Aortic root≥ of Aortic root
sizesize
* 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 SURGERYINDICATIONS 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.
CONTRAINDICATIONCONTRAINDICATION –severe pulmonary vascular
disease.

43. Surgical
correction has
to be done
before
irreversible
damage to
pulmonary
vasculature
occurs

44. Operative procedureOperative procedure

45. Patch closure by RV approach

47. Percutaneous Device Closure
• MuscularVSDs can typically be closed
percutaneously

48. Much more to comeMuch more to come
Are we
all still
awake?