Approach to Cyanotic Congenital Heart Diseases

1. - CSN Vittal
Approach to Cyanotic
Congenital Heart Diseases

2. Cyanosis
Bluish discoloration of the mucous membrane and skin
Visible clinically when arterial O
2
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
oHb Kansas
oHB Beth Isreal
oHb M variants
Hereditary methemoglobinemia

9. Cyanosis : D D
CardiacCardiac RespiratoryRespiratory
Tachypnoea RR Less ( 40 – 60)More ( > 60 )
Respiratory distressAbsent 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 1
st

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 17
th
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 Excludes Presence of CCF Excludes
Tetralogy Physiology except Tetralogy 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.O
2
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.