Cyanotic congenital heart diseases are those with decreased pulmonary blood flow leading to central cyanosis. Tetralogy of Fallot is the commonest cyanotic heart disease, accounting for 10% of congenital heart defects. It is characterized by four anatomical features - pulmonary stenosis, ventricular septal defect, right ventricular hypertrophy, and overriding of the aorta. Patients typically present with cyanosis, clubbing of fingers, and cyanotic spells which can be relieved by squatting. Investigations reveal findings like boot-shaped heart on chest X-ray and right axis deviation on ECG. Without intervention, patients experience complications like infections, stroke, and delayed growth and development.

1. - CSNVittal
Approach to Cyanotic
Congenital Heart Diseases

2. Cyanosis
Bluish discoloration of the mucous membrane and skin
Visible clinically when arterial O2 saturation falls to 60-65%
Primarily depends on the amount of reduced Hb – must be
at least 5g % - provided absolute Hb is normal (Lunds Gaard &
Vanslyke)
Physiological cyanosis may be normal till 20 min. but
beyond that – it is always abnormal
Central cyanosis desaturation of arterial blood.
Peripheral cynosis normal arterial oxygen circulation.

3. Cyanosis - Types
Central – cyanotic CHD
Peripheral – hypothermia, CCF
Mixed Cyanosis – CHD in Shock
Differential cyanosis – PDA with reversal
Reverse differential cyanosis – TGA with PDA
with reversal
Intermittent Cyanosis – Ebsteins anomaly
Circum oral cyanosis
Cyclical cyanosis – Bilateral choanal atersia

4. Complications of Cyanosis /
Cyanotic CHD
Clubbing
Cyanotic Spell
Depressed IQ
Infective endocarditis
Polycythemia
Embolic phenomenon

5. Cyanosis - Etiology
A] Cyanotic Congenital Heart Diseases with  Pulm. Vascularity
1. With RVH
 Severe PS with ASD
 Pulm atresia (with or without VSD)
 TOF
1. With LVH
 Tricuspid atresia
 Pulm. Atresia and hypoplastic Rt. Ventricle
1. With combined ventricular hypertrophy
 TGA with PS
 Truncus arteriosus with hypoplastic pulm. Arteries
1. With neither ventricle predominant
 Ebstein’s anomaly

6. Cyanosis - Etiology
B] Cyanotic Congenital Heart Diseases with  Pulm. Vascularity
1. With RVH
 Hypoplastic Lt. Heart syndrome
 TAPVR
 TGA
 Double outlet Single (Rt.) ventricle
1. With Lt. / Combined ventricular Hypertrophy
 TGA + VSD
 Single ventricle
 Tricuspid atresia with transposition
 Truncus arteriosus

7. Cyanosis - Etiology
C] Non Cardiac Causes
1. Pulmonary
 RDS in newborns
 Pneumonia
 Meconium aspiration
 Pneumothorax
 Chylothorax
 Diagrphmatic hernia
 Hemothorax
 Lobar emphysema
 Pleural effusion
 Pulm. Telangectasia
 Bronchogenic cyst

8. Cyanosis - Etiology
C] Non Cardiac Causes
1. Hematological
 Abnormal hemoglobins
o Hb Kansas
o HB Beth Isreal
o Hb M variants
 Hereditary methemoglobinemia

9. Cyanosis : D D
CardiacCardiac RespiratoryRespiratory
Tachypnoea RR Less ( 40 – 60) More ( > 60 )
Respiratory distress Absent Present
100 % Oxygen No response PO2 > 150 torr

10. Cyanosis with NO CCF
TOF
Pulmonary atresia with VSD
Tricuspid atresia
Transposition of great vessels with pulmonary stenosis

11. Cyanosis with Heart Failure
Complete Transposition of great arteries
Truncus arteriosus
Single ventricle

12. Foetal
Circulation

13. Cyanotic Congenital Heart Disease
Increased
Pulmonary Blood
Flow
Cyanosis, Clubbing, Polycythemia
Decreased
Pulmonary Blood
Flow
Transposition of Great arteries (3-5%)
Truncus Arteriosus (1-2%)
Single Ventricle (1-2%)
TAPVC (1-2%)
HLHS (1-3%)
Tetralogy of Fallot (5-7%)
Tricuspid Atersia
Ebstein’s Anomaly
Pulmonary Atersia
% given for 100 CHDs

14. Approach to a Child with cyanotic CHD
History:
 H/o. cyanotic spells (TOF, Ebstein anomaly)
 Cyanosis increased during defecation, feeding.
 H/o. squatting
 Easy fatigability
 Failure to thrive
 Increased metabolic activity
 Recurrent infections due to  pulm flow
 Decreased intake
 Associated chromosomal anomaly
 Chest pain
 Syncope : TOF, Eisenmenger’s
 Hemoptysis : Eisenmenger’s
 Cough, breathlessness & repeated chest infections due to  pulm flow
 TAPVC, TGA, Truncus arteriosus
 Convulsions : - Cerebral abscess – TOF after 2 yrs.

15. Approach to a Child with cyanotic CHD
Prenatal History:
Consanguinity
Age of parents (esp. mother)
Down’s – more in maternal age > 35 (endocardial cushion
defects)
Maternal medication during pregnancy
Sod. valproate – TOF, VSD
Lithium – ASD, tricuspid atresia, Ebstein’s
Marijuana – Ebstein’s
Clomiphene – TOF
Antitussive - ASDs
Sex hormones – VSD, TGA, TOF

16. Approach to a Child with cyanotic CHD
Age of the Patient:
1. Cyanosis during newborn
period
1. Hypoplastic LV
2. TGA with intact ventricular
septum
3. Tricuspid atresia
4. Hypoplastic Rt. Ventricle
with pulm. Atresia
5. TOF (severe type)
2. Cyanosis develops during 1st
week :
1. Pulmonary atresia
2. Tricuspid atresia
3. TGA
3. Cyanosis after 1 month
1. TOF
2. TGA, TAPVC

17. Approach to a Child with cyanotic CHD
Sex of the Patient:
1. Equal in both sexes
1. Ebstein’s anomaly
2. Pulmonary atresia
3. Tricuspid atresia
4. Truncus arteriosus
5. TOF
6. Cong. venacaval – Lt atrial
communication
2. Females – more common:
1. Endocardial cushion defects
3. Males – more common
1. Univentricular heart
2. TGA
3. Hypoplastic left heart

18. Approach to a Child with cyanotic CHD
General Examination:
Trisomy 21 – Endocardial cushion defects
Cat cry syndrome – Tricuspid atresia
Di George syndrome – Truncus arteriosus
(hypoplastic mandible, defective ears and short filtrum)
Marfan’s and Down syndrome – TOF
Anomalies of 16 – 18 chromosomes – DORV
Clubbing of fingers – takes 6 mo. to develop
Differential clubbing – Eisenmenger’s syndrome – only in toes
Short stature, kyphoscoliosis, arthropathy, congested eyes, bad
teeth, growth retardation

19. Approach to a Child with cyanotic CHD
Jugulars Examination:
Enlarged
Tricuspid atresia
Hypoplastic left ventricle
TAPVR
Endocardial cushion defect
TGA with increased pulm. blood flow
DORV with increased pulm. blood flow
Truncus arteriosus
Normal jugulars
Fallot’s tetrology
TGA with PS
DORV with PS

20. Approach to a Child with cyanotic CHD
Pulse
Collapsing
Truncus arteriosus
AV malformations
Severer TOF with collaterals
Taussig-bing operation fort OF
Decreased Lt. carotid and Lt. brachial pulses
Hypoplastic Lt. heart syndrome
Decreased femoral pulses
DORV

21. Approach to a Child with cyanotic CHD
Precardial Examination
Normal
TOF, DORV, Pulm. atresia with intact ventricular septum,
tricuspid atresia
Precardial bulge
TAPVR, Hypoplastic Lt. ventricle, PS with intact IVS
TGA

22. Approach to a Child with cyanotic CHD
Auscultation
Splitting of S 1 : Ebstein’s anamoly
Loud P 2 : TAPVR, Endocardial cushion defect,
Eisenmenger syndrome, DORV with 
pulm. flow
Single S 2 : TOF, TOF with PS, DORV with PS,
Tricuspid atresia, Truncus arteriosus
Muffled S 2 : Ebstein’s anamoly
Continuous murmur : TOF with collaterals, Truncus arteriosus

23. Approach to a Child with cyanotic CHD
Radiology
Boat shaped heart : TOF
(coeur en sabot)
Enlarged heart : Severe TOF with
collaterals, associated
anemia or hypertension
Concavity in pulm. artery area : PS
Rt. sided aortic arch : 25% cases of TOF
Rib notching : TOF with collaterals
Egg on end appearance : TGA
Figure of Eight appearance : TAPVR

24.  Commonest Cyanotic HD
 10% of CHDs
Nada’s dictum:
If a cyanotic heart disease child over 2 years is admitted
in ward, if it is diagnosed as TOF it is 75% correct
Taussing’s axiom:
If a patient is having cyanosis with normal sized heart
and oligemic lung fields it is definitely TOF

25. 1. Pulmonary stenosis (infundibular)
2. VSD
3. Rt. Ventricular hypertrophy
4. Overriding aorta
Fallot’s Pentology
TOF + ASD
Fallot’s Trilogy
ASD with patent foramen ovale
RVH
PS

27. History
 Nicholas Steno 17th
century – originally
described TOF
 In 1948, Etinne, Louis Arthur Fallot
described the four anatomical features of
TOF
 In 1947 Taussig described various positions
for relief of the dyspnoea

28. Etiology:
 Extracardiac manifestations in 10%
 Syndromes associated with
 Apert’s anomaly (TOF, VSD, CA)
 Silver syndrome (TOF, VSD)
 Goldenhar syndrome (TOF, VSD, ASD)
 De Lange syndrome ( VSD, TOF, PDA, DORV)
 Fetal hydantoin syndrome
 Fetal carbamazepine syndrome
 Fetal alcohol syndrome
 Maternal phenylketonuria (PKU) birth defects
 CATCH 22 – Cardiac defects, abnormal facies, thymic hypoplasia,
cleft palate, hypocalcemia
 Maternal drugs/diseases associated with
 Trimethadone, Sex hormones, Thalidomide

29. Associations:
 RVOT
 infundibular 45%
 valve level 10%
 combined 30%
 atretic 15%
 Pulmonary annulus and MPA hypolastic.
 Right aortic 25%.
 5% cases abnormal coronary arteries.

30. Classification:
 Group I
 Severe PS or pulm. atresia
 Pt. symptomatic at birth
 Mortality high
 Group II
 Classical TOF.
 Pt. becomes symptomatic from 6 mo. of life
 Group III
 Pink Fallot’s tetrology
 Pt. becomes symptomatic between 4 – 5 years

31. Hemodynamcis:
 Pressure in both ventricles equal
 Pulm blood flow depends on severity of PS
 In VSD with mild PS, blood flow from Lt to Rt
ventricle throughout systole
 In TOF with severe PS, most of Rt. ventricular
blood enters into aorta resulting in cyanosis
and hypoxia

32. Tetrology of Fallot

34. Hemodynamics - Squatting &
TOF
During activity → respiration (hyperpnea)↑
Venous return increases but Pulmonary→
flow cannot increase Right to Left shunt→ →
Cyanosis
On squatting, venous return decreases →
Systemic vascular resistance increases,
therefore less or no right to left shunt →
decreases cyanosis

35. Clinical Symptoms:
 Dyspnoea – not associated with cough
 Cyanosis – at birth or from 6 mo. of age
 Cyanosis on exertion
 Squatting
 Cyanotic spells
 Growth retardation

36. Polycythemia

37. Clubbing

38. Squatting Position

39. Presence of CCF ExcludesPresence of CCF Excludes
Tetralogy Physiology exceptTetralogy Physiology except
when complicated bywhen complicated by
Anemia
Infective Endocarditis
Valvar Regurgitation
Surgically created or naturally occurring
large left to right shunts
Systemic hypertension
Unrelated or coincidental myocardial
disease

40. Investigations:- CXR
 coeur en sabot
 Rt. sided aortic arch
 Aneurysmal bulging of Pulm. artery (with
absence of pulm. valves)
 Oligemic lung fields
 Rib corrosions – with collaterals
 Unilateral lesions – after Blalock- Taussig
operation

41. Investigations:- ECG
 RAD
 RVH
 Sudden transition from V1 to V2
(R wave in V1 is suddenly replaced by S wave in V2)

42. Electrocardiography
Right axis deviation, Right ventricular hypertrophy

43. Echocardiogram
Apical Parasternal

44. Echocardiograp
hy

45. Chest roentgenogram
Normal size heart
Pulmonary vascular
markings are decreased
Concave main pulmonary
artery segment with an
upturned apex
– BOOT shaped heart
or coeur en sabot
Right atrial enlargement
(25%)
Right aortic arch (25%)

46. Investigations: Blood
 Polycythemia
 Spontaneous thromboses
 Microcytic hypochromic anemia

47. Other Complications
1. Cerebral venous thrombosis
2. Cerebral abscess
 Entering of contaminated blood in to systemic
circulation without being filtered in lungs
 Infarction of brain due to polycythemia
 Hypoxia – leading to anaerobic infection
 Infective endocarditis can disseminate infection
in cerebrum
1. Infective endocarditis

48. Other Complications
5. Arthropathy
 Gout arthropathy due to production of uric acid from RBC
due to polycythemia
5. Acute renal failure
 Uric acid nephropathy
7. Bleeding diathesis
 Decrease in coagulants due to shrinkage of plasma
 Decreased platelet adhesion and production
7. Delayed puberty

49. Natural history of Fallot’s
Tetrology
Acyanotic TOF – gradually become cyanotic
Patients already cyanotic become deeply
cyanosed as infundibular stenosis increases and
polycythemia develops
Hypoxic spells develop in infants
Severe TOF patients may develop AR
Growth retardation in severe cases
Coagulopathy develops in long standing cases
Iron deficiency anemia develops slowly

50. Tetrology of Fallot
Medical Therapy :
Maintain Hb > 14 g /dL (oral iron or bl.
Transfusion)
Beta blockers to be given in highest
tolerated doses ( 1-4 mg/kg/d in 2-3
divided doses)

51. Tetrology of Fallot
 Timing of Surgery :
 Stable, minimally cyanosed : Total correction at 1-2 yrs of age
 Significant cyanosis (SaO2 < 70%) or H.o spells despite therapy
 < 3 mo. : systemic to pulm. art shunt (class I)
 > 3 mo. : shunt or correction depending on anatomy and surgical center’s
experience (class I)
 VSD with pulm. Atresia. Adequate Pas: Repair at 3-4 yrs, if RV to pulm.
art. conduit required (class I)
Systemic to pulm. artery shunt if symptomatic earlier and repair without
conduit is not possible

52. 1. Blalock – Taussig Operation:
Anastamosis between subclavian artery with ipsilateral pulmonary
artery. In Rt. sided aortic arch, anastamosis is done between Rt.
subclavian artery and pulm. artery.
1. Waterston’s Operation:
Anastamosis between ascending aorta and pulm. artery.
1. Pott’s Operation:
Anastamosis between descending aorta and left branch of pulm.
artery.
1. Gore-Tex Interposition shunt: Placed between the subclavian
and ipsilateral PA, done even in small infants younger than 3 months

53. Blalock Taussig Shunt

55. Corrective Surgery:
 Reconstruction of infundibular area
 Closure of VSD
 Trabeculotomy
Indications
1. In symptomatic patients between 3-4 months
2. In asymptomatic mildly cyanosed patients between 3 – 24
months
3. Mildly cyanosed patients with Blalock-Taussig shunt correction
between 1-2 years
4. Asymptomatic acyanotic patients – between 1-2 years

56. Tetrology of Fallot
Timing of Surgery :
 Stable, minimally cyanosed : Total correction at 1-2 yrs of age
 Significant cyanosis (SaO2 < 70%) or H.o spells despite
therapy
 < 3 mo. : systemic to pulm. art shunt (class I)
 > 3 mo. : shunt or correction depending on anatomy and
surgical center’s experience (class I)
 VSD with pulm. Atresia. Adequate Pas: Repair at 3-4 yrs, if
RV to pulm. art. conduit required (class I)
Systemic to pulm. artery shunt if symptomatic earlier and
repair without conduit is not possible

57. Postoperative complications
Congestive heart failure (right or left, residual
outflow obstruction, VSD, and/or pulmonic
regurgitation
Atrial flutter, ventricular arrhythmias, right
bundle-branch block, or left anterior
hemiblock
Infective bacterial endocarditis

58. If the patient is severely cyanosed with
group I Fallot’s tetrology should be given
prostaglandin infusion to keep ductus
arteriosus patent for augmentation of
blood supply to pulmonary artery.

60. Cyanotic spell (tet spell)
 Medical emergency
 6 mo. To 2 years
 Early hours
 Crying, feeding, defecation and relative anemia
precipitate
Mechanism :
 Local intracardiac production of catecholamines
increased due to stress – which increase infundibular
spasm leading to more R > L shunt
 Vulnerable resp. centre
 Paroxysmal attack of arrhythmias

61. Cyanotic spell (tet spell)
Treatment:
1. Morphine : 0.1 mg/kg SC
(stabilizes resp. centre and
calms)
2. Propronalol : 0.1 mg /kg IV dil. in 50 ml of 5% D/W
3. Methoxamine : 40 mg. dil in 250 ml of 5% D/W - IV
4. Ketamine : 1 to 3 mg/kg IV over 60 min
(increases systemic vascular resistance and calms
patient)
5. Phenylephrine : 0.1 to 0.2 mg / kg IV –
(increases systemic vascular resistance)

62. Cyanotic spell (tet spell)
Supportive Treatment:
1. Knee chest position
2. O2 inhalation
3. Sodium bicarbonate for acidosis
Prevention :
1. Propronalol : 1 - 4 mg/kg up to 2 years
2. Iron supplementation : 1 mg/kg/d

63. Polycythemia – Management:
1. Avoid dehydration
2. Iron therapy
3. Phlebotomy – if Hct. > 65 – 70%

64. Prophylaxis for Infective Endocarditis
1. Maintain oral hygiene
2. Administer prophylactic antibiotic IV
immediately before cardiac surgery and
continue for 1-2 days postoperatively
3. Administer appropriate antibiotic for any
dental procedure or surgical procedures

65. Infective Endocarditis
Prophylaxis
Every child with CHD must be advised to maintain
good oral hygiene and regular dental check up
Unrepaired CCHDs are high risk conditions for IE. So
prophylaxis is mandatory
ASD (secundum type) and valvular PS are low risk
conditions for IE – prophylaxis not recommended
Other acyanotic CHDs including bicuspid aortic valve
are moderate risk & prophylaxis is recommended
Repaired CHDs with prosthetic material need
prophylaxis for first 6 mo. after procedure
Device placement by transcatheter route also require
prophylaxis for the first 6 mo.