Cyanotic congenital heart disease 2021

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 Raad13:28

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 4
th
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-Taussigshunt (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-Taussigshunt
•Blalock-Taussigshunt
•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
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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 VSDor 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