This document provides information on various cyanotic heart lesions including Tetralogy of Fallot, Transposition of the Great Arteries, Tricuspid Atresia, Ebstein's Anomaly and Total Anomalous Pulmonary Venous Return. It describes the anatomy, clinical features, diagnosis and management of these conditions. Key cyanotic heart lesions are characterized by mixing of oxygenated and deoxygenated blood resulting in central cyanosis.

1. Cyanotic Heart Lesions
Dr. Kalpana Malla
MBBS MD (Pediatrics)
Manipal Teaching Hospital
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2. Cyanotic Heart Lesions
• The 5 Ts
– Tetralogy of Fallot
– Transposition of the Great Arteries
– Truncus Arteriosus (Persistent)
– Tricuspid Atresia
– Total Anomalous Pulmonary Venous Return
(TAPVR)

3. Cyanotic Heart Lesions
• Hypoplastic left heart syndrome (HLH)
• Pulmonary atresia (PA) / critical PS
• Double outlet right ventricle (DORV)
• Ebstein anomaly
• Single ventricle

4. R L
R L with pulmonary stenosis
• TOF
• Tricuspid atresia
• Ebstein’s anomaly

5. Evaluation possible congenital heart
Exam: rate, rhythm, impulse, murmur, pulses
(brachial and femoral)
• Oxygen saturation - Hyperoxia test
• ABG
• Chest X- ray
• Echocardiogram
• Cardiac catheterization

6. Tetralogy of Fallot (TOF):
• Most common (75% )cyanotic CHD in >2yrs
• ~ 10% of all CHD

7. Tetralogy of Fallot
• TOF = consists
– Ventricular septal defect
– Rt ventricular outflow obstruction – infundibular or
infundibular + pulmonary valve stenosis
– Aorta position is shifted to the right and over-rides the VSD
– Hypertrophy of the right ventricle

8. Essential components:
VSD
Pulmonary stenosis
Other components :
overriding of Aorta
RVH
Pentalogy of Fallot: all above + ASD

9. Hemodynamics:
• Large, non-restrictive VSD, perimembranous type,
extending upto right ventricular outlet allows
equalisation of pressures in two ventricles VSD is
silent
• Pulmonary stenosis Shunting of blood from R L
ventricle mixing of oxygenated & deoxygenated
blood in left ventricle circulated to whole body

10. • Severity depends upon degree of pulmonary stenosis
• Pulmonary stenosis causes concentric rt ventricular
hypertrophy without cardiac enlargement & ↑rt vent
pressure
• Flow from Rt vent to pul artery  across pul stenosis
 produce ejection systolic murmur
• If obstruction small, RL shunt minimal or absent
(pink or acyanotic TOF)

11. • P2  Delayed & reduced in intensity due to rt
vent outflow obstruction reduced PA pressure
• S2 single and A2 audible
• Severity of cyanosis  directly proportional
to severity of pul stenosis but intensity of
systolic murmur inversely related to severity
of pulmonic stenosis

12. Clinical features:
• May become symptomatic any time after birth
– usually 2nd half of 1st yr
• Anoxic spells (synonyms- hypoxic,
hypercyanotic, blue, tet ) – paroxysmal attack
of dyspnea
• Common symptoms – dyspnea on
exertion,exercise intolerance
• Cyanosis
• H/O squatting during dyspneaic episodes

13. Anoxic spells
• Occur predominantly after waking up or
following exertion
• Most commonly start around 4 to 6 months of
age and are charcterized by
1.Sudden crying
2.Sudden onset or deepening of cyanosis
3.Sudden onset of dyspnea

14. Anoxic spells
4. Alterations of consciousness
5. Convulsions
6.Decrease in intensity of systolic murmur
Frequency varies from once in a few days
to numerous attacks every day

15. • Mild outflow obstruction: cyanosis in later part of 1st year
• Severe outflow obstruction: cyanosis immediately after
birth (as ductus starts to close)
CCF unusual in children with TOF except in:
Severe anemia
Valvular regurgitation
Infective endocarditis
Systemic hypertension
Coincidental myocardial diseases

16. Physical examination:
• Cyanosis
• Clubbing
• Polycythemia
• Normal sized heart
• Mild parastrnal impulse

17. Auscultation
• S1 –normal
• S2 – Single, (A2 heard,P2 soft &delayed
• Murmur –
• Shunt – absent
• Flow - Loud short pulmonary ejection systolic
murmur grade 3-5/6 at 3rd ICS in left side

18. Diagnosis:
• Blood: polycythemia
• CXR:
1. Upturned apex (RVH)- Small boot shaped
heart
2. Oligemic lung fields
3. Absence or concavity of pulmonary artery
segment gives the shape described as cor-
en sabot
4. Right aortic arch ~25 -30%

19. • Diagnosis:
• ECG:
RAD, RVH with tall peaked p waves
• Echo: overriding aorta,RVH,outflow
obstruction
• Cardiac catheterisation

20. Complications:
• Cerebral thrombosis
• Brain abscess
• Bleeding diathesis
• Infective endocarditis
• CCF-in acyanotic or pink TOF

21. Complications:
• CNS - Embolism to CNS - sluggish circulation
from polycythemia
• Hemiplegia - infarction in CNS during anoxic
spell

22. Management:
• Management of Tet spells:
- knee-chest position
- humidified O2 inhalation
- morphine 0.1 mg/kg s/c/ iv

23. Management of Tet spells:
- IV fluids
- Correct metabolic acidosis- Na-bicarbonate
- Propanolol – 0.1mg/kg/iv during spell (0.5-1
mg/kg PO 6 hrly

24. Management:
• Vasopressors- methoxamine IM or IV drip
penylephrine
• Correct anemia
• Consider surgery

25. • General measures:
- Correction of iron deficiency anemia
- Adequate hydration
- Antibiotics for infection
- Prophylaxis with propranolol

26. Surgery
• Palliative surgery:
- Blalock-Taussig shunt – subclavian artery to
pulm artery
- Pott’s shunt-descending aorta to PA
- Waterson operation – ascending aorta to
Rt pulm artery
-Modified Blalock-Taussig shunt

27. • Corrective surgery: open heart surgery for –
closer of VSD
- resecting the infundibular obstruction PS
Surgery can be performed at any age
Success – 85-90%

28. Complications of surgery
• Complete heart block
• RBBB
• Residual VSD & Pulm stenosis
• Pulm regurgitation

29. Tricuspid atresia
• Cong absence of
Tricuspid valve
• Rt ven hypoplastic
• Absent inflow portion
• 2% of CHD

30. Hemodynamics
• No communication between Rt atrium rt
ventricle (hypoplastic)
• Blood from Rt atrium lt atrium through
patent foramen ovale or ASD.mixing of
oxygenated + deoxygenated blood to lt
ventricle aorta
• Lt vent rt vent there is VSD pul artery ( lt
.ventricle maintains both systemic &
pulmonary circulation saturation of blood is
identicle in pulm artery and aorta

31. Clinical features
• Depends on state of pulmonary flow
• 90 % are with diminished blood flow
• Features :As TOF
• Differentiating points :
1.Cyanosis from birth
2.More sicker than TOF
3.Lt ventricular type of apical impulse
4.Enlarged liver with presystolic pulsations
5.ECG- LAD,LVH

32. Diagnosis
• Blood: polycythemia
• CXR:
1.Oligemic lung fields
2.Left ventricular configuration
3.Prominent SVC shadow
• ECG:
Rt & Lt atrial hypertrophy, LAD,LVH
• Echo: large single ventricular cavity

33. Tricuspid Atresia
Repair not usually performed in neonatal
period- over a series of procedures
– Systemic to PA shunt
– SVC to PA shunt (followed by ligation of first
shunt)– Glenn Shunt
– IVC to PA shunt– completion Fontan

34. Ebsteins Anomaly
• Rare CCHD
• Post and septal leaflet of TV – displaced downwards
–
• The upper part of the right ventricle is part of the
right atrium - atrialized rt ventricle
• Rt ventricle is too small and Rt. atrium is too large.
• Leaflets – malformed and fused – obstruction of
flow to rt ventricle

35. Ebsteins Anomaly
• Often Associated with other heart lesions
– ASD
– Pulmonary Stenosis
– Pulmonary Atresia

36. Hemodynamics :
Abnormal leaflets obstruction to forward
flow & regurgitation from Rt ven to Rt
atrium atrium dilates Patent FO / ASD
allows R L shunt( cyanosis) Lt atrium
(enlarged) Lt ventricle (enlarged &
hypertrophied)

37. Clinical picture
• Cyanosis
• Effort intolerance
• Fatigue
• Paroxysmal attack of tachycardia
• Clubbing
• Lt ventricular apical impulse
• Systolic thrill may be palpable LSB

38. Auscultation
• S1- normal
• S2 – widely split but variable
• Rt ventricular 3rd soundor rt atrial 4th sound
audible – triple/quadruple sound usually
heard
• Murmur-midsystolic ejection or pansystolic
• Short tricuspid delayed diastolic M

39. Investigations
• CXR cardiomegaly –square shaped
Lung – oligemic
ECG- ‘p’ pulmonale ‘p’mitrale,RBBB
Wolff Parkinson white type conducton defect
maybe seen
ECHO- displaced tricuspid valve

40. Treatment
• Surgical – obliteration of atrialised portion of
rt.ventricle and repairof tricuspid valve

41. Fallot’s physiology
• Presence of large VSD with PS
• Useful for bedside identification of group of
condition with similar clinical findings
• Defects with Fallot’s physiology:
1. Complete TGA with VSD & PS
2. DORV with PS & large VSD (subaortic)
3. Tricuspid atresia with diminished pul flow
4.Single ventricle with PS
5. corrected TGA with VSD & PS

42. Transposition of the Great Arteries
• Most common cyanotic condition that
requires hospitalization in first 2 weeks of
life
• Aorta arises from RV
• Pulmonary artery originates in the left
ventricle

43. • Oxygenated pulmonary venous blood
recirculates in lungs and systemic venous
blood recirculates in systemic circulation

44. Transposition of the Great Vessels
• A PDA,ASD,VSD, is necessary for these infants to
survive until they can have corrective surgery
• More common in infants of diabetic mothers

45. Classification
1. Complete variety
2. Physiologically corrected type

46. Complete variety
• Rt atrium →Rt ventricle →aorta
• Lt atrium →Lt ventricle →pulmonary artery
• Systemic & pulmonary circulation separate
→survival possible only if there is
ASD,VSD,PDA
Classification
A) With intact ventricular septum – mixing site is
atrial communication PFO
B) with VSD with/without pul stenosis

47. Physiologically corrected type
• Rt atrium → morphologically inverted left
ventricle →pulmonary artery
• Lt atrium → morphologically inverted Rt
ventricle →aorta
• Route of blood flow is normal

48. C /F with intact VS
• Cyanotic at birth
• Interatrial mixing poor (PFO) – rapid breathing
,congestive failure due to hypoxia within 1st wk of life
• CCF
• S1 –normal
• S2- single
• Ejection systolic murmur grade 1-2/6
• CXR – egg on side appearance,plethoric lung field

49. With VSD
• Good mixing at ventricular level, large
pulmonary blood flow – cyanosis milder
• CCF at 4-10 wks
• Exam –cyanosis,CCF
• S1- Normal
• S2 – single
• Murmur – ejection systolic grade 2-4/6

50. Diagnosis
• CXR- egg on side appearance ,cardiomegaly
with narrow base, plethoric lung field
• ECG without VSD – RAD,RVH
• ECG with VSD – RAD, biventricular
hypertrophy
• Cardiac catheterization
• Angiocardiography

51. Medical management
• Control CCF
• Balloon atrial septotomy by cardiac catheterization -
Inter-atrial septum opened
• Definitive repair – Jatene’s switch operation -
removal of aorta and pulmonary artery from their
origins and re-attached to the correct ventricles
• Less preferred – atrial switch operation –mustard or
senning

52. Corrected TGA
• Normal route of blood flow
• Commonly associated with other anomalies
98% - symtoms are due associated anomalies:
1.VSD with/without PS
2.Lt sided Ebstein’s anomaly of tricuspid valve
3.Atrioventricular conduction abnormalities

53. Truncus Arteriosus
• Truncus fails to divide completely during fetal life,
leaving a connection between the aorta and
pulmonary arteries
• Mixed oxygenated and de-oxygenated blood exits the
heart and enters the systemic circulation

54. Truncus Arteriosus
• Single artery arises from the heart, supplying both aorta
and pulmonary artery.
• VSD below the truncal valve allows mixing of right and left
ventricular blood
• Degree of cyanosis is variable
• Presents with progressive heart failure

55. Truncus Arteriosus
• Medical Management
– Digoxin and Diuretics
• Surgical Repair
– Usually required by 2-3 months of age
– VSD is closed
– PA trunk is separated from truncus
– Conduit created between RV and PA using a
valved graft

56. TAPVR
• The pulmonary veins, instead of being connected to
the left atrium , are connected to the right atrium
or superior vena cava, and return oxygenated blood
to the right side of the heart.

57. Total Anomalous Venous Return
• Uncommon CCHD
• Cyanosis
• CCF at age 4-10 wks
• Murmur : pul ejection systolic + tricuspid flow
murmur
• Continuous venous hum audible at upper left
or rt sternal border or in suprasdternal notch

58. Diagnosis
• CXR- snowman or figure of 8 configuration
• ECG – RAD,RVH,
• ECHO- demonstrate abnormal course of pul
veins

59. Total Anomalous Venous Return
• Control of CCF, pul infections
• The only accepted treatment is surgery
• Surgical connection is made between pulmonary
venous confluence and the LA
• Connection to systemic venous circulation is
ligated.

60. Hypoplastic Left Heart
• Fatal without early surgical
intervention

61. Treatment- continued
• General procedure for cyanotic heart lesions
involves a systemic to PA shunt.
• Procedure known as the Blalock-Taussig shunt
– Uses a small Gore-Tex® shunt to connect either
left or right subclavian to left or right branch PA.
– Allows partially desaturated blood to enter PA,
increasing pulmonary blood flow and oxygenation

62. CCHD with PA HTN
• This group is named – Eisenmenger syndrome
– severe PA HTN resulting in R→L shunt at
atrial, ventricular or pulmonary arterial level
• Eisenmenger complex – severe PA HTN with
VSD resulting in R→L shunt

63. • Eisenmenger's syndrome named by Dr. Paul
Wood after Dr. Victor Eisenmenger, who first
described the condition in 1897.

64. Hemodynamics
• L→R shunt in the heart causes:-
- increased flow through PA
- High O2 saturation in PA
- Hyperreactive pul vasculature → Pul
vascular obstructive Ds →PA HTN
• PA HTN → causes increased pressures in the right
side of the heart and reversal of the shunt into R → L
shunt
• R → L shunt with VSD & PDA → decompresses rt
ventricle →RV has only concentric hypertrophy with
no increase in size ( no heave)

65. • R → L shunt with ASD → RVH +dilatation →rt
ven failure
• R → L with ASD or VSD →mixing of blood
reaches ascending aorta → distributed to
whole systemic circulation → equal cyanosis
• R → L with PDA → mixed blood directed
downwards to descending aorta (junction is
distal to lt Subclavian artery → cyanosis +
clubbing of toes only (differential cyanosis)

66. Examination
• Cyanosis
• Clubbing
• Fatigue
• Effort intolerance
• Dyspnea
• h/o recurrent chest infection

67. Sounds
• S1- normal
• S2- ASD- wide fixed split
VSD- single
• PDA- normally split
• Murmurs
• Pulmonary regurgitation ( graham steel)
• Ejection systolic

68. Investigations
• CXR- prominance of pul artery,heart size –
normal to large
• ECG – RVH
• ECHO-
• Cardiac catherization-bi-directional shunt

69. Treatment
• Heart-lung transplant is required to fully treat
the syndrome
• If this option is not available - treatment is
palliative-
• Anticoagulants
• Pulmonary vasodilators
• Antibiotic prophylaxis to prevent endocarditis
• Phlebotomy to treat polycythemia
• Maintaining proper fluid balance

70. Thank you
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