Chd nanoti sir

1. CONGENITAL HEART
DISEASE
PART-1
BY
Dr Girish Nanoti

2. Learning objectives
1. Differences between foetal and neonatal
circulation.
2. Anatomical and Physiological changes after
birth in circulation.
3. Patho physiological changes occuring in LR
shunt .
4. Etiology ,clinical features ,investigation and
management of VSD .

4. Fetal Circulation
• In Fetus the placenta is organ for the exchange
of gases and nutrients ,while in adults /
paediatric population the lung is organ for
gaseous exchange
• In fetus lung is a solid organ hence PVR is very
high ,while in pediatric population lung is
pnematic organ and PVR is almost 4 times less
than SVR.

5. Changes at birth
1. Removal of placenta results in
1. Increase in SVR
2. Cesation of blood flow in umblical vein  closure of Ductus
venosus
2. First breath results in decrease in PVR (Oxygen is a
potent vasodilator)  results in fall in Pulmonary
Arterial Pressure
3. Due to increase pressure in left atrium  closure of
Foramen ovale .
4. Due to increase oxygen in blood  functional closure of
PDA. Anatomical closure within 48-72 hrs.

6. CONGENITAL HEART DISEASE
0.5 – 0.8 % Live Birth.
2-3 neonates in 1000 newborn with CHD symptomatic in 1st week.
ETIOLOGY :
1. Multifactorial ( genetic predisposition + environmental stimulus )
2. 2-3 % single gene defect ( Marfan syndrome )
3. Chromosomal anomalies
1. Down syndrome trisomy 21 : endocardial cushion defect, ASD,VSD
2. TRISOMY 18 (90%) : VSD , ASD, PDA
3. TRISOMY 13 : VSD , ASD, PDA
4. TURNER SYNDROME : biscuspid aortic stenosis , coarctation of aorta
4. Syndrome Complexes :
1. CHARGE
2. CATCH 22 (cardiac defect, abnormal facies)
3. VATER

7. 5. Teratogenic Agents:
1. congenital rubella
2. fetal hydantoin, infant of diabetic mother
3. Maternal PKU

8. CONGENITAL (ACYANOTIC/CYANOTIC HEART DISEASE)
Five Basic Questions
1. Congenital Cyanotic Vs Acyanotic
2. Acyanotic Heart Disease:
Shunt Absent Shunt Present
3. Dominant Ventricle
4. Lesion Present On Left Side Vs Right Side
5. Whether Pulmonary Hypertension Is Present
• With The Help Of :
1. Physical Examination
2. X Ray Chest
3. ECG

9. ACYANOTIC DEFECTS
• PVOD : Pulmonary vascular obstructive disease
• Increased PBF – S/O of plethora on X rays
Increased PBF Normal PBF
LVH
VSD
PDA
RVH
ASD
PVOD
LVH
AS/AR
COA
MR
RVH
PS
MS

10. PATHOPHYSIOLOGY IN VOLUME
OVERLOAD
• Communication Between Systemic And Pulmonary Side Of
Circulation
• Shunt ( Qp : Qs)
• Shunt Will Depend On
– Size Of Defect
– Relative Pulmonary & Systemic Pressure & Vascular Resistance
• Dynamic : Change Dramatically With Age
1. Size (VSD) Muscular Type Decrease With Age
2. PVR In Neonate Gradually Decreases
[ Chronic Exposure Of Pulmonary Circulation To High Pressure &
Blood Flow Results Gradual Increase of PVR  Eisenmenger
Physiology ]

11. Effects :
1. Increase Pulmonary Circulation Leads To Decreased
Pulmonary Compliance And Increased Work Of
Breathing
2. Fluid Leaks In Interstitial Space : Pulmonary Edema
3. Tachypnea , Chest Retractions, Nasal Flaring
4. Increase Left Ventricular Output :
– HR And SV Increases (Overactivity of Sympathetic
Nervous System) & sweating
5. Increase in Total Oxygen Consumption as metabolic
rate increases  Irritability And Failure To Thrive

12. Changes in Pulmonary blood flow , PVR ,
Pulmonary arterial pressure

13. VENTRICULAR SEPTAL DEFECT
• Most common form of CHD (15 - 20 % )
• TYPES
a) Site :
Membranous 70% : Muscular
(perimembranous) Perimembranous(inlet)
(Endocardial cushion defect) 1)outlet
perimembranous (outlet) 2)Inlet
b) Size (VSD in TOF)
3)Trabecular
a)Anterior.
b)Apical
c)Middle
b) Size :
mild moderate Severe
<0.5cm2 0.5 – 1 cm2 >1cm2
1.5 : 1 1.5-2 : 1 More than 2

14. Patho-physiology
SMALL VSD
1. Restrictive
2. Shunt depends on the size
• Pulmonary : systemic flow  1.5 : 1
• So absence of pulmonary congestion and minimal chamber enlargement
X ray
ECG
• Pansystolic murmur (+) at parasternal area
• Intensity of P2 normal
WNL

15. Moderate size VSD
• Shunt 1.5 :1
• Pressure Of Right Ventricle Elevated Slightly Hence No Volume Overload
And Pressure Overload Of Right Ventricle.
• Volume Overload By Left Ventricle Is Significant.  Causes Left
Ventricular Hypertrophy
• Pulmonary Arterial Pressure Mildly Elevated  Intensity Of P2 May
Increase Slightly.
• Pansystolic Murmur (+) In Addition Due To Increased Flow Across Mitral
Valve
• Mid Diastolic Murmur across mitral area May Be Heard

16. large VSD
1. Increase in PVR ,
2. Greater shunt  volume overload in both ventricle causing
cardiomegaly.(Pressures in both ventricles are equal)
3. Shunt depend on pulmonary vascular resistance.
4. As PVR decrease after birth by 4 to 6 weeks hence CCF ( left
ventricular volume overload)
5. But as time progresses due to increase pulmonary blood flow 
causes hyperkinetic pulmonary hypertension.
6. If not operated, it causes irreversible pulmonary hypertension due
to hypertrophy of Media & proliferation of Intima
7. Finally it leads to  Eisenmenger’s syndrome
8. Paradoxically patient feels better as Cardiomegaly decreases
9. Only RVH (+) with p2 loud with reversal cyanosis (+)
10. Shunt murmur decrease in the intensity

17. Symptoms
• Small vsd
1. Asymptomatic
• Moderate to large VSD
1. Delayed growth and development.
2. Decreased exercise intolerance.
3. Repeated pulmonary infections.
4. CHF during infancy +
• Large VSD (Long standing more than 10yrs)
1. cyaniosis (+)

18. Physical Signs & Symptoms
• Small VSD
1. Pan systolic murmur (+) . systolic thrill (+)
• Moderate VSD
1. Growth may be affected [ Left Ventricular Apex (+) ]
2. systolic thrill
3. pansystolic murmur (+)
4. P2 slightly loud and MDM over mitral area
• Large vsd
1. growth failure (+)
2. Precordial bulge & pulsation (+),
3. biventricular apex , parasternal heave (+) ,
4. pansystolic murmur + , MDM + , P2 loud
5. With eisenmengerisation p2 loud, RVH, shunt murmur dec.

19. MILD MODERATE LARGE LARGE(PVR )
EISENMINGER
ECG NO CHANGE LVH BVH RVH
X RAY NO CHANGE LV (+)
LA (+)
PULMONARY
PLETRHORA (+)
LV (+)
LA (+)
RV (+)
PULMONARY
PLETHORA (+)
RV (+)

20. DIAGNOSTIC : ECHOCARDIOGRAPHY &
COLOUR DOPPLER
• To See
1. Number ,Size and exact location of defect .
2. Pulmonary arterial pressure
3. Magnitude of shunt
4. Other associated defects

21. Natural History Of VSD
1. Spontaneous closure of perimembranous and muscular VSD can occur .
2. Patient with membranous VSD and muscular VSD close mostly during
1ST two years of life.
3. About 60 % small to moderate VSD close spontaneously during 1st 8 yrs
of life while 35 % of Small perimembranous VSD close within 1st 5 yrs of
life .
4. but INLET AND OUTLET defect do not close spontaneously or do not
decrease in size.
5. CHF develops in infant with large VSD but usually not until 6 to 8 wks
6. Pulmonary vascular obstructive disease may begin to develop as early 6
to 12 months of age in patients with large VSD but resulting right to left
shunt usually not develop untill teenage years

22. Management
• Medical
1. Treatment of CHF :
1. DIURESIS ,
2. DIGOXINE,
3. OTHER DRUGS  ENALAPRIL, SPIRONOLACTONE
2. Treatment of anemia
3. Treatment of infection
4. Good dental hygiene
5. Antibiotic prophylaxis
6. Non Surgical device closure of muscular VSDs.
7. Nutritional – High energy Diet

23. • SURGICAL : Indication and timing
1. Large VSD in infants if growth failure ++( operated
within 6 months )
2. If PA pressure 50 % greater than systemic pressure
3. After 1 yr of age significant left to right shunt (2:1)
4. Small VSD if shunt less than 1.5 : 1  intrevention
done to close the defect
5. Outlet VSD – Risk of AI .
6. Surgery is CONTRAINDICATED if
1. pulmonary vascular obstructive disease
2. with pulmonary to systemic resistance ratio greater than 0.5

24. COMPLICATION
1. RBBB
2. Residual VSD
3. Tricuspid incompetence

25. Post Operative Follow Up
1. Review examination done every early.
2. On ECG RBBB  50-90 % post-op cases of
VSD who are operated by right
ventriculostomy.
• Genetic Counselling
– Recurrence risk is 4% in a multifactorial
inheritence

26. • Long Question
– Describe patho-physiolgy & management of
ventricular septal defect.
• Short question
– foetal circulation.

27. THANK YOU