children congenital heart disease

1. Congenital
Cyanotic Heart Disease
Prof. Imran Iqbal
Fellowship in Pediatric Neurology (Australia)
Prof of Paediatrics (2003-2018)
Prof of Pediatrics Emeritus, CHICH
Prof of Pediatrics, CIMS
Multan, Pakistan

2. Al Quran surah Raad 13:28

3. Cyanotic Congenital Heart Defects
(Relative Frequency)
• Tetralogy of Fallot (10% of total Congenital Heart
Defects)
• Transposition of the Great Arteries - TGA (7% of total
Congenital Heart Defects)
• Rare Cyanotic Defects –
• Total Anomalous Pulmonary Venous Drainage
• Tricuspid Atresia
• Pulmonary Atresia

4. TOF
Tetralogy of Fallot
Pathophysiology, Clinical Features, Diagnosis
Complications, Management, Prognosis
Prof. Imran Iqbal
Fellowship in Pediatric Neurology (Australia)
Prof of Paediatrics (2003-2018)
Prof of Pediatrics Emeritus, CHICH
Prof of Pediatrics, CIMS
Multan, Pakistan

5. Dr. Etienne Fallot
Tetralogy of Fallot
Is named after
French physician
Etienne-Louis Arthur Fallot,
who accurately described the
4 anatomic abnormalities in
TOF in 1888
Dr. Etienne Fallot, 1850-1911

6. 4 Characteristics in TOF
• VSD
• Pulmonary stenosis
• Overriding aorta
• Right ventricular
hypertrophy

7. TOF – Four Components
• PS (Pulmonary Stenosis)
• Pulmonary valve narrowing results in less deoxygenated
blood transported to the lungs and more deoxygenated
blood passing through VSD to aorta to be circulated
throughout the body
• RVH (Right Ventricular Hypertrophy)
• Secondary to Pulmonary Stenosis resulting in increased
Right Ventricular Pressure
• Transposition of Aorta
• Aorta is displaced and overlies both ventricles
• VSD (Ventricular Septal Defect)
• Produces a shunt from Right to Left Ventricle with mixing of
deoxygenated blood with oxygenated blood

8. Tetralogy of Fallot
http://commons.wikimedia.org/wiki/Image:Tetralogy_of_Fallot.svg

9. Clinical Scenario
• A 4 month old baby
presents with poor feeding.
Baby stops during mother
feeds and starts crying.
• On examination, bluish
tinge is noticed on lips and
face of baby.
• During auscultation, a heart
murmur is heard.
• Congenital Heart Disease is
suspected and further
investigations are
performed.

10. TOF - Clinical Features
• Cyanosis – may appear in neonatal life or later in
infancy
• Clubbing – appears in months to years
• Tachypnea – fast breathing
• Cardiac Examination
• Cardiac Apex beat in 4th intercostal space
• Right ventricular heave may be present
• Single P2 - second heart sound
• Ejection systolic murmur at Pulmonary area - left upper
sternal border

11. TOF – Clubbing and Cyanosis

12. Heart sounds - Normal

13. TOF – Heart murmur
Pulmonary stenosis murmur
Ejection systolic murmur at LUSB (left upper sternal border)

14. TOF - Investigations
• Pulse Oximetry – low oxygen saturation
• Chest X-ray – boot shaped heart
• ECG – Right Axis deviation,
- Right Ventricular Hypertrophy
• Echocardiography –
- Pulmonary stenosis
- Right Ventricular Hypertrophy
- Over-riding of Aorta
- VSD
• Cardiac catheterization – cardiac angiography reveals the exact
anatomy

15. Chest Radiograph in TOF
• Apex of Heart is
raised
• Pulmonary Artery
shadow is not
visible
• Pulmonary
vascular markings
are decreased

16. TOF
Boot-shaped Heart

17. Normal ECG

18. EKG in TOF
(R wave in V1, S wave in V5)

19. TOF - Complications
• Polycythemia – due to hypoxia
• Hyper-cyanotic Spell – paroxysm of tachypnea,
tachycardia, irritability, crying, lethargy & increased
cyanosis produced due to spasm of pulmonary
outflow tract and decreased pulmonary blood flow.
It requires immediate medical treatment.
• Cerebral thrombosis – due to Hypoxia
• Cerebral Abscess – Fever, Headache, Vomiting
• Failure to Thrive – Low weight, Short height

20. TOF – Hyper-cyanotic Spell

21. TOF – Medical Management
• Supportive Management:
• Adequate Hydration and Nutrition
• Oral Iron to prevent anemia
• Specific Management:
• Oral propranolol (dilates pulmonary tract and prevents
hypoxic spells)
• Management of complications:
• Management of Hyper-cyanotic spell
• Antibiotic prophylaxis against SBE

22. Management of Hyper-cyanotic spell
• Knee - chest position
• Oxygen
• Intravenous fluids
• IV Sedation (narcotic analgesics)
• IV Sodium bicarbonate to correct acidosis
• Propranalol (beta blocker)

23. TOF – Surgical Management
• Palliative Surgery – creation of a vascular shunt
between systemic and pulmonary circulation to
increase the pulmonary blood flow (performed in
severe cases)
• Blalock-Taussig shunt (B-T shunt) - is creation of a
vascular channel between Right Subclavian Artery
and Right Pulmonary Artery
• Corrective Surgery – surgical procedure to correct
the anatomical defects in TOF usually after infancy

24. Blalock-Taussig shunt
• Blalock-Taussig shunt
• Anastomotic shunt between
Right subclavian artery &
ipsilateral Pulmonary Artery
• Usually performed at 3 – 12
months of age in severe
cases

25. TOF – Corrective Surgery
• Patch closure of VSD &
widening of RV outflow
tract
• Timing of procedure
depends upon expertise
available
• Usually performed after
one year of age
www.inova.com

26. Prognosis
• Prognosis depends upon severity of disease and the
management available
• Infants with severe disease may die during a hypoxemic
spell
• Many untreated patients may not survive beyond 10
years of age
• Survival is better after first year of life
• Survival is good with adequate surgical management

27. Prognosis
No Surgical Repair With Surgical Repair

28. Transposition of Great Arteries
(TGA)

29. Transposition of the Great Arteries
• Aorta arises from RV
• Pulmonary artery
arises from LV
• VSD in 20% of cases

30. TGA – Pathophysiology
• TGA patients need mixing of right of left heart
circulations in order to provide oxygenated blood
to body
• TGA with VSD or ASD has better survival
• TGA with intact septum (no VSD or ASD) remain
dependent on Patent Ductus Arteriosus, which
tends to close in neonatal life

31. Transposition of Great Arteries
(TGA)

32. TGA – Clinical Features
• TGA presents with increasing cyanosis in first days
of life

33. TGA - Investigations
• Pulse Oximetry – low oxygen saturation
• Chest X-ray – Egg shaped heart, pulmonary vascular
markings increased
• ECG – Right Axis deviation,
• Echocardiography –
- Pulmonary artery from Left Ventricle
- Aorta from Right Ventricle -
- VSD / ASD or no other defects
• Cardiac catheterization – cardiac angiography reveals the
exact anatomy

34. Chest Radiograph in TGA
Egg-shaped Heart

35. TGA – Management
• In TGA with intact septum, Ductus Arteriosus needs
to be kept open to provide partially oxygenated
blood to body
• Newborn baby with TGA is given Prostaglandin
infusion to keep the Ductus Arteriosus open
• An artificial Atrial Septal Defect may need to be
created in the newborn by a catheter and balloon –
Balloon Atrial Septostomy
• Corrective surgery – Arterial Switch operation -
should be performed as soon as possible

36. Medical Profession

37. Thankyou