6. Cyanotic CHD with Decreased PBF
RV Dominance
PAH
Acyanotic CHD
with ES
VSD
PDA, AP window
ASD
Cyanotic CHD
with PAH
DORV
TGA
TAPVC
Truncus
No PAH
RVOTO
Quiet Precordium
Single S2 ± PS murmur
TOF
DORV WITH PS
TGA WITH PS
L-TGA WITH PS
ASD with PS
7. Cyanotic CHD with Decreased PBF
LV dominance
LV type Apex (Exam/CXR)
No Parasternal Heave
LAD ± LV dominance on ECG
Tricuspid Atresia with PS
Pulmonary Atresia with intact IVS
Single Ventricle with PS
Ebstein’s
8. Cyanotic CHD with Near Normal PBF
• Pulmonary AV Fistula
• Unroofed coronary sinus into LA
• Anomalous drainage of vena cava to LA
13. TOF PHYSIOLOGY
• Cyanotic CHD with decreased PBF
having 2 key components anatomically
– Severe RVOTO – Decreasing PBF
– Large VSD – causing equalization of RV and LV
pressure with right to left shunt due to outflow
obstruction
(Acyanotic TOF not included)
14. TOF PHYSIOLOGY
• Cyanotic CHD with decreased PBF
having 2 key symptoms Physiologically
– History of Spells
– History of Squatting
– No CHF symptoms
15. Cyanotic CHD with Decreased PBF
RV Dominance
PAH
Acyanotic CHD
with ES
VSD
PDA, AP window
ASD
Cyanotic CHD
with PAH
DORV
TGA
TAPVC
Truncus
No PAH
RVOTO
Quiet Precordium
Single S2 ± PS murmur
TOF
DORV WITH PS
TGA WITH PS
L-TGA WITH PS
ASD with PS
16. Cyanotic CHD with Decreased PBF
LV dominance
LV type Apex (Exam/CXR)
No Parasternal Heave
LAD ± LV dominance on ECG
Tricuspid Atresia with PS
Pulmonary Atresia with intact IVS
Single Ventricle with PS
Ebstein’s
17. Cyanotic CHD with Decreased PBF
LV dominance
LV type Apex (Exam/CXR)
No Parasternal Heave
LAD ± LV dominance on ECG
Tricuspid Atresia with PS
Pulmonary Atresia with intact IVS
Single Ventricle with PS
Ebstein’s
19. “Tetralogy of Fallot” History
• 1671: First reported by Niels Stenson a.k.a
Nicholas Steno
• 1777; 1784; 1839; 1866; 1872
Similar Case reports
20. • 1888:
Etienne Louis Arthur Fallot
– Anatomic diagnosis
at bedside
– Confirmed at
postmortem
– Coined term Tetralogie
(Fr.)
21. • 1894: Pierre Marie (French), first used term
“Tetralogie de Fallot”
• 1924: Maude Abbott, first used term
“Tetralogy of Fallot” &
“Fallot’s Tetralogy”
22. Pathology
• Van Praagh called TOF “Monology of Stenson”
• Central pathology –
Underdevelopment/hypoplasia of
Subpulmonary infundibulum
• Gives rise to 4 components of ‘tetralogy’
– Obstructive RVOT
– Large Malaligned VSD
– Aortic override
– Dominant RV hypertrophy
23. Pathology
• Van Praagh called TOF “Monology of Stenson”
• Central pathology –
Underdevelopment/hypoplasia of
Subpulmonary infundibulum
• Gives rise to 4 components of ‘tetralogy’
– Obstructive RVOT
– Large Malaligned VSD
– Aortic override
– Dominant RV hypertrophy
25. Natural History
• Survival
– 66% 1st yr
– 50% 3rd yr
– 25% 1st decade
• Poor survival with PA
– 50% 1st yr
– 10% 1st decade
26. Symptoms/Presentation
• Cyanosis
– 1-2 weeks after birth
– More severe the PS, earlier the presentation
• Hypercyanotic Spells
– 2 months to 2 years of age
• Exertional dyspnoea
– Older child
• Squatting
– To alleviate a spell or dyspnoea
27. Physical Exam
• Physically underdeveloped
• Cyanosis (Depending on PBF)
• Pulse
– NORMAL (irrespective of PS severity)
– Wide PP – only in Large MAPCA/Severe AR
28. Physical Exam
• JVP
– NORMAL (Height and waveform)
(RAP stays normal unless significant TR present)
29. Physical Exam
Palpation
• RV impulse
– Gentle; like normal neonatal RV; but stays like that
even as child grows
– 4th LICS
– 5th LICS & Subxyphoid (if Sub-Infundibular stenosis)
32. Physical Exam
Palpation
• Right sternoclavicular joint pulsation
– Right sided Aortic arch
• No thrill due to RVOTO
– BF goes uninterrupted to dilated Aorta
33. Physical Exam
Auscultation
• Aortic area (2nd RICS)
– Loud aortic EC from aortic root
– Maximum in expiration
• Pulmonary area
– Very delayed and soft P2
– EC and P2 almost inaudible (Bicuspid PV – decreased mobility)
34. Physical Exam
Auscultation
• 3rd LICS
– Superficial murmur starting with S1
Duration and intensity decreases with severity of PS
• No S4 (RA contraction not forceful, RAP normal)
• No S3 (No RVF)
51. Natural History
• Mortality rate in TGA without PS
– 30% 1st week
– 50% 1st month
– 90% 1st year
• Better survival with PS – may survive
adolescence
52. Physical Exam
• Birth weight > normal (Contrast to other CHDs)
• Deep Cyanosis
• Scalp & Arm varicose veins (Systemic volume
overload with desaturated blood)
53. • Pulse
– Full volume bounding pulse
– Warm extremities
• JVP
– Elevated RAP with dominant A wave
(Systemic volume overload with desaturated blood)
54. • Palpation
– RV impulse gentle at birth
– Soon after 1 week – Prominent RV impulse
(Systemic volume overload with desaturated blood)
– Rt sternoclavicular impulse – Rt arch
(11-16% in TGA-VSD-PS)
Just like TOF
56. ECG
• P wave
– Tall, Peaked (Hypervolemic RA)
• RAD, RVH
• T
– Usually positive in ALL
precordial leads
– Taller in right precordial leads
• Counterclockwise depn
57. CXR
• Thymic shadow absent
(after 12 hrs of birth)
• Characteristic narrow
pedicle (egg on side)
ABSENT in severe PS –
due to dilated right
anterior aorta
• CXR like TOF
67. • Q /q
– Present in right precordial leads / absent in left
(Septal activation right to left directed)
– Present in III, avf (III>avf) / absent in I, aVL
(Septal activation superiorly directed)
• T
– Usually positive in ALL precordial leads
• Clockwise depn
68. CXR
• Thymic shadow absent
• Ao & PA side by side
• Ao left and Anterior
• Straight left border
• Hump shaped heart
• RPA and LPA at same
level
70. Tricuspid Atresia (TA)
• 1 in 17000 live births
• 90% TA
– No TGA
• 90% have Restrictive VSD – Physiologically PS
• 10% TA
– TGA
• 90% have no PS – Increased PBF
72. Natural History
• TA-NRGA-PS
– 80% mortality in 1st yr
– Already restrictive VSD – decreases in size and
closes! (Like a PM-VSD!)
– Acquired Pulmonary atresia without
embryological collaterals – fatal!
87. ECG
• P wave
– Tall, Peaked (RAE)
• Inverted OC – RAD,
Clockwise depn
• Noninverted OC – LAD,
Counterclockwise depn
• Stereotyped rS or RS
complexes V2-V5
• Tall R in V1 despite LVH
88. CXR
• Non-inverted OC
• Doesn’t form left
border – just like D-TGA
• LV apex
• Characteristic waterfall
appearance of RPA –
NOT present in PS –
Low PBF
89. CXR
• Inverted OC
• Form left border – just
like L-TGA
• Maybe visible as
convexity on left
• Left straight border
• LV apex