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Congenital Heart Congenital Heart Lesions-Lesions-
Right to Left ShuntsRight to Left Shunts
Zeev Perles MDZeev Perles MD
Pediatric CardiologyPediatric Cardiology
Hadassah Jerusalem, 11/2013Hadassah Jerusalem, 11/2013
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Congenital Heart Congenital Heart Lesions-Lesions-
Right to Left ShuntsRight to Left Shunts
R-L shunting = R-L shunting = blue bloodblue blood contaminating contaminating systemic cyclesystemic cycle
R-L shunting R-L shunting Cyanotic Cyanotic CHDCHD
??????
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Congenital Heart Congenital Heart Lesions-Lesions-
Right to Left ShuntsRight to Left Shunts
R-L shunting = R-L shunting = blue bloodblue blood contaminating contaminating systemic cyclesystemic cycle
R-L shunting R-L shunting Cyanotic Cyanotic CHDCHD
??????
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Congenital Heart Congenital Heart Lesions-Lesions-
Right to Left ShuntsRight to Left Shunts
R-L shunting = R-L shunting = blue bloodblue blood contaminating contaminating systemic cyclesystemic cycle
R-L shunting R-L shunting Cyanotic Cyanotic CHDCHD
??????
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CyanosiCyanosiss
CyanosiCyanosiss
Kuanosis (Greek) “blueness”Kuanosis (Greek) “blueness” Deoxygenated capillary bloodDeoxygenated capillary blood Lundsgaard & Van Slyke (1923)Lundsgaard & Van Slyke (1923)
≥≥3-5 gram% of reduced Hgb3-5 gram% of reduced Hgb Central or PeripheralCentral or Peripheral Look at skin, mucosa, nailbedsLook at skin, mucosa, nailbeds
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Cyanotic CHDCyanotic CHD
TTetralogy of Fallot (TOF)etralogy of Fallot (TOF)
TTransposition of the Great Arteries (TGA)ransposition of the Great Arteries (TGA)
TTricuspid valve atresia + ricuspid valve atresia +
Pulmonary aPulmonary aTTresia (with VSD or IVS)resia (with VSD or IVS)
TTotal anomalous pulmonary venous return otal anomalous pulmonary venous return
(TAPVR)(TAPVR)
TTruncus arteriosusruncus arteriosus
EbsEbsTTeinein’’s Anomaly of the s Anomaly of the TTricuspid Valvericuspid Valve
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R-L shunting = R-L shunting = Cyanotic CHD !!!Cyanotic CHD !!!
Qp/QS ?Qp/QS ?
100 100 10060 60 60
90 80 70
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♥ Qp/Qs << 1Qp/Qs << 1♥ Severe desaturation/cyanosis (RA Severe desaturation/cyanosis (RA
SiO2 60-75%)SiO2 60-75%)♥ Oligemic CXROligemic CXR♥ Normal respiratory statusNormal respiratory status♥ Normal growthNormal growth♥ Need: Need: BT ShuntBT Shunt RV-PA conduit + RV-PA conduit +
VSDVSD
♥ Qp/Qs >> 1Qp/Qs >> 1♥ Mild/No desaturation (RA SiO2 85-Mild/No desaturation (RA SiO2 85-
99%)99%)♥ CXR CXR –– increased PVM increased PVM’’ss♥ Tachypneic and dyspneicTachypneic and dyspneic♥ FTT !!!FTT !!!♥ Need: Need: RV-PA conduit + VSDRV-PA conduit + VSD
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♥ Qp/Qs << 1Qp/Qs << 1♥ Severe desaturation/cyanosis (RA Severe desaturation/cyanosis (RA
SiO2 60-75%)SiO2 60-75%)♥ Oligemic CXROligemic CXR♥ Normal respiratory statusNormal respiratory status♥ Normal growthNormal growth♥ Need: Need: BT ShuntBT Shunt RV-PA conduit + RV-PA conduit +
VSDVSD
♥ Qp/Qs >> 1Qp/Qs >> 1♥ Mild/No desaturation (RA SiO2 85-Mild/No desaturation (RA SiO2 85-
99%)99%)♥ CXR CXR –– increased PVM increased PVM’’ss♥ Tachypneic and dyspneicTachypneic and dyspneic♥ FTT !!!FTT !!!♥ Need: Need: RV-PA conduit + VSDRV-PA conduit + VSD
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♥ Qp/Qs << 1Qp/Qs << 1♥ Severe desaturation/cyanosis (RA Severe desaturation/cyanosis (RA
SiO2 60-75%)SiO2 60-75%)♥ Oligemic CXROligemic CXR♥ Normal respiratory statusNormal respiratory status♥ Normal growthNormal growth♥ Need: Need: BT ShuntBT Shunt RV-PA conduit + RV-PA conduit +
VSDVSD
♥ Qp/Qs >> 1Qp/Qs >> 1♥ Mild/No desaturation (RA SiO2 85-Mild/No desaturation (RA SiO2 85-
99%)99%)♥ CXR CXR –– increased PVM increased PVM’’ss♥ Tachypneic and dyspneicTachypneic and dyspneic♥ FTT !!!FTT !!!♥ Need: Need: RV-PA conduit + VSDRV-PA conduit + VSD
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♥ Qp/Qs << 1Qp/Qs << 1♥ Severe desaturation/cyanosis (RA Severe desaturation/cyanosis (RA
SiO2 60-75%)SiO2 60-75%)♥ Oligemic CXROligemic CXR♥ Normal respiratory statusNormal respiratory status♥ Normal growthNormal growth♥ Need: Need: BT ShuntBT Shunt RV-PA conduit + RV-PA conduit +
VSDVSD
♥ Qp/Qs >> 1Qp/Qs >> 1♥ Mild/No desaturation (RA SiO2 85-Mild/No desaturation (RA SiO2 85-
99%)99%)♥ CXR CXR –– increased PVM increased PVM’’ss♥ Tachypneic and dyspneicTachypneic and dyspneic♥ FTT !!!FTT !!!♥ Need: Need: RV-PA conduit + VSDRV-PA conduit + VSD
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♥ Qp/Qs << 1Qp/Qs << 1♥ Severe desaturation/cyanosis (RA Severe desaturation/cyanosis (RA
SiO2 60-75%)SiO2 60-75%)♥ Oligemic CXROligemic CXR♥ Normal respiratory statusNormal respiratory status♥ Normal growthNormal growth♥ Need: Need: BT ShuntBT Shunt RV-PA conduit + RV-PA conduit +
VSDVSD
♥ Qp/Qs >> 1Qp/Qs >> 1♥ Mild/No desaturation (RA SiO2 85-Mild/No desaturation (RA SiO2 85-
99%)99%)♥ CXR CXR –– increased PVM increased PVM’’ss♥ Tachypneic and dyspneicTachypneic and dyspneic♥ FTT !!!FTT !!!♥ Need: Need: RV-PA conduit + VSDRV-PA conduit + VSD
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♥ Qp/Qs << 1Qp/Qs << 1♥ Severe desaturation/cyanosis (RA Severe desaturation/cyanosis (RA
SiO2 60-75%)SiO2 60-75%)♥ Oligemic CXROligemic CXR♥ Normal respiratory statusNormal respiratory status♥ Normal growthNormal growth♥ Need: Need: BT ShuntBT Shunt RV-PA conduit + RV-PA conduit +
VSDVSD
♥ Qp/Qs >> 1Qp/Qs >> 1♥ Mild/No desaturation (RA SiO2 85-Mild/No desaturation (RA SiO2 85-
99%)99%)♥ CXR CXR –– increased PVM increased PVM’’ss♥ Tachypneic and dyspneicTachypneic and dyspneic♥ FTT !!!FTT !!!♥ Need: Need: RV-PA conduit + VSDRV-PA conduit + VSD
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♥ Qp/Qs << 1Qp/Qs << 1♥ Severe desaturation/cyanosis (RA Severe desaturation/cyanosis (RA
SiO2 60-75%)SiO2 60-75%)♥ Oligemic CXROligemic CXR♥ Normal respiratory statusNormal respiratory status♥ Normal growthNormal growth♥ Need: Need: BT ShuntBT Shunt RV-PA conduit + RV-PA conduit +
VSDVSD
♥ Qp/Qs >> 1Qp/Qs >> 1♥ Mild/No desaturation (RA SiO2 85-Mild/No desaturation (RA SiO2 85-
99%)99%)♥ CXR CXR –– increased PVM increased PVM’’ss♥ Tachypneic and dyspneicTachypneic and dyspneic♥ FTT !!!FTT !!!♥ Need: Need: RV-PA conduit + VSDRV-PA conduit + VSD
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Cyanotic CHDCyanotic CHD
Take-home messageTake-home message::Some Some CYANOTICCYANOTIC CHDs are not CHDs are not necessarily bluenecessarily blue
The degree of “blueness” The degree of “blueness” depends on Qp/Qs ratiodepends on Qp/Qs ratio
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ונעבור לנציגנו במיון ילדים...ונעבור לנציגנו במיון ילדים...
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Can You Guess Can You Guess –– O O22SS? ?
OO22SS= = OO22SS= =
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Can You Guess Can You Guess –– O O22SS? ?
OO22SS= = OO22SS= =
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Can You Guess Can You Guess –– O O22SS? ?
OO22S = 80%S = 80%OO22S = S = 80%80%!!! !!!
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Can You Guess Can You Guess –– O O22SS? ?
OO22S = 80%S = 80%OO22S = 80%S = 80%!!! !!!
Total Hb- 10g% Total Hb- 10g% Deox Hb=10*20%=Deox Hb=10*20%=2 g2 g%%
Total Hb- 20g% Total Hb- 20g% Deox Hb=20*20%=Deox Hb=20*20%=4 g4 g%%
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Can You Guess Can You Guess –– Hgb g Hgb g? %? %
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Can You Guess Can You Guess –– Hgb g Hgb g? %? %
All- All- 75%75%!!! !!!
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Can You Guess Can You Guess –– Hgb g Hgb g? %? %
All- All- 75%75%!!! !!!
HgbHgb??? ???
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Can You Guess Can You Guess –– Hgb g Hgb g? %? %
All- All- 75%75%!!! !!!
HgbHgb??? ???
9g9g!!! %!!! %
21g21g!!! %!!! %
18g18g!!! %!!! %
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Can You Guess Can You Guess –– O O22SS? ?
Take-home messageTake-home message::A child with A child with CYANOTICCYANOTIC CHD is CHD is not necessarily bluenot necessarily blue
The degree of “blueness” The degree of “blueness” depends on hemoglobin leveldepends on hemoglobin level
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Breathing PatternsBreathing Patterns
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Breathing PatternsBreathing Patterns
Large VSD+PDA
Tricuspid Atresia (Qp/QS
1.3)
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Can You Guess Can You Guess –– O O22SS? ?
Truncus Arteriosus
Tricuspid Atresia
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Medical HistoryMedical History
Large VSD
Tetralogy of
Fallot
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Can You Guess Can You Guess –– O O22SS? ?
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Can You Guess Can You Guess –– O O22SS? ?
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Can You Guess Can You Guess –– O O22SS? ?
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Can You Guess Can You Guess –– O O22SS? ?
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Cyanotic CHD- Cyanotic CHD- Pulmonary flow obstruction Pulmonary flow obstruction
with R-L Shuntingwith R-L Shunting
TTetralogy of Fallot (TOF)etralogy of Fallot (TOF)
TTricuspid valve atresia (with PS)ricuspid valve atresia (with PS)
Pulmonary aPulmonary aTTresia (with VSD or resia (with VSD or
IVS)IVS)
Classic Cyanotic Heart Classic Cyanotic Heart lesionslesions
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Cyanotic CHD- Cyanotic CHD- Pulmonary flow obstruction Pulmonary flow obstruction
with R-L Shuntingwith R-L Shunting
After birth- closure of arterial After birth- closure of arterial
duct:duct:
Baby dies!!!!Baby dies!!!!
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Cyanotic CHD- Cyanotic CHD- Pulmonary flow obstruction Pulmonary flow obstruction
with R-L Shuntingwith R-L Shunting
After birth- closure of arterial After birth- closure of arterial
duct:duct:
Baby dies!!!!Baby dies!!!!
iv PGEiv PGE
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Blue Baby SyndromeBlue Baby Syndrome
Helen B. Taussig 1898-Helen B. Taussig 1898-19861986
♥Orphan- age 11Orphan- age 11
♥XXXX
♥Severe DyslexiaSevere Dyslexia
♥DeafnessDeafness
Founder of Pediatric Founder of Pediatric CardiologyCardiology
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TOF TOF Natural Natural historyhistory
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ACQUIRED ACQUIRED CONDITIONSCONDITIONS
Brain abscessesBrain abscesses– most commonly with TOFmost commonly with TOF– rarely before 2 years of agerarely before 2 years of age– headaches, fever, seizures, or headaches, fever, seizures, or
neurologic defecitsneurologic defecitsCerebrovascular accidentsCerebrovascular accidentsInfectious endocarditisInfectious endocarditisMay be pre-disposed to otitis mediaMay be pre-disposed to otitis media
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Blue Baby SyndromeBlue Baby Syndrome
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Physical Exam in Tetralogy of Physical Exam in Tetralogy of FallotFallot
S2 single S2 single
S1S1
ECEC
Systolic Systolic murmur of murmur of PSPS
1)1) Cyanosis - degree depends on Cyanosis - degree depends on PSPS
2)2) Clubbing (>6m)Clubbing (>6m)
3)3) Normal pulsesNormal pulses
4)4) Increased RV impulse at RSBIncreased RV impulse at RSB
5)5) SS2SS2
6)6) Murmur along LSBMurmur along LSB
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CXRCXR Heart size enlargedHeart size enlarged Small RVOT Small RVOT
segment+ large RV-segment+ large RV- Coeur en sabot Coeur en sabot or or boot shaped heartboot shaped heart
May see right sided May see right sided aortic arch aortic arch
Pulmonary Pulmonary vasculature vasculature decreased (oligemic)decreased (oligemic)
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Anatomy/Anatomy/PhysiologyPhysiology
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Edwards, JE: Congenital Heart Disease. WB Saunders and Co. Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 19651965
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Anatomy/Anatomy/PhysiologyPhysiology
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Anatomy/Anatomy/PhysiologyPhysiology
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Anatomy/Anatomy/PhysiologyPhysiology
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Anatomy/Anatomy/PhysiologyPhysiology
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TOF managementTOF management
SquattingSquatting
SoothingSoothing
SedationSedation
SelineSeline
Slowing the rateSlowing the rate
SurgerySurgery
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TOF managementTOF management
SquattingSquatting
SoothingSoothing
SedationSedation
SelineSeline
Slowing the rateSlowing the rate
SurgerySurgery
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BLALOCK-TAUSSIG BLALOCK-TAUSSIG SHUNTSHUNT
11/07/200311/07/2003המלאך השחור של הילדים הכחולים, הארץ המלאך השחור של הילדים הכחולים, הארץ
((קרונזון יצחקקרונזון יצחק) )
נעשה בבית חולים בבולטימור ניתוח ניסיוני בתינוקת שסבלה ממומים מולדים בלבה. 1944בנובמבר הד"ר בליילוק, מומחה בעל שם עולמי, נעצר פתאום. "צריך לקרוא לויויאן תומאס", אמר לאחות.
.תומאס, עובד מעבדה שחור, שלא הורשה עד אז לעטות חלוק לבן, שינה את עולם הרפואה
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CLASSIC BLALOCK-TAUSSIG CLASSIC BLALOCK-TAUSSIG SHUNTSHUNT
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BLALOCK-TAUSSIG SHUNTBLALOCK-TAUSSIG SHUNT
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BLALOCK-TAUSSIG SHUNTBLALOCK-TAUSSIG SHUNT
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BLALOCK-TAUSSIG SHUNTBLALOCK-TAUSSIG SHUNT
Denton Cooley
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BLALOCK-TAUSSIG SHUNTBLALOCK-TAUSSIG SHUNT
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BLALOCK-TAUSSIG SHUNTBLALOCK-TAUSSIG SHUNT
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BLALOCK-TAUSSIG SHUNTBLALOCK-TAUSSIG SHUNT
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BLALOCK-TAUSSIG SHUNTBLALOCK-TAUSSIG SHUNT
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MODIFIED BLALOCK-MODIFIED BLALOCK-TAUSSIG SHUNTTAUSSIG SHUNT
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TOF - HISTORYTOF - HISTORY
Stenson 1671Stenson 1671 First descriptionFirst description
Fallot 1888Fallot 1888 Description of featuresDescription of features
Blalock and Taussig 1945Blalock and Taussig 1945 First systemic-to-pulmonary First systemic-to-pulmonary
anastamosisanastamosisLillehei 1955Lillehei 1955
First definitive surgical repairFirst definitive surgical repair
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INCIDENCEINCIDENCE
Most common cyanotic cardiac Most common cyanotic cardiac
lesionlesion
Third most common cardiac lesionThird most common cardiac lesion
Occurs 3-5 times per 10,000 birthsOccurs 3-5 times per 10,000 births
5-10% of all cardiac defects5-10% of all cardiac defects
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Anatomy/Anatomy/PathologyPathology
Ventricular septal defectVentricular septal defect
Pulmonary stenosisPulmonary stenosis
Aortic overrideAortic override
Right ventricular hypertrophyRight ventricular hypertrophy
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Anatomy/Anatomy/PathologyPathology
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Gross example of Tetrology of Fallot. The interior of the right ventricle is exposed showing a large VSD (D) and an aorta (A)straddling (ie over-riding) the VSD. A probe (arrow) is positioned inside of the stenotic subpulmonary infundibular channel.
Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 1965
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This is an example of tetralogy of Fallot opened sagittally. The aorta and its valve (AV) clearly straddle the VSD (D) such that the aorta appears to arise equally from each ventricle. The right ventricular (RV) is clearly hypertrophic as it is nearly as thick as left ventricle (LV).
Edwards, JE: Congenital Heart Disease. WB Saunders and Co. 1965
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ASSOCIATED ANOMALIESASSOCIATED ANOMALIES
Pulmonary arteries:Pulmonary arteries:– Valvar, main, branch PS Valvar, main, branch PS – Discontinuous PA’sDiscontinuous PA’s
Atrial septal defect (up to 80%)Atrial septal defect (up to 80%)Additional VSD’sAdditional VSD’sConotruncal defects-Conotruncal defects-
– Right sided aortic arch (25%)Right sided aortic arch (25%)– Aortic branch anomaliesAortic branch anomalies– LSVCLSVC
PDAPDA
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CORONARY ANOMALIESCORONARY ANOMALIES
Present in 2-9% of patientsPresent in 2-9% of patientsSingle right or left coronary arterySingle right or left coronary arteryLAD from right coronary arteryLAD from right coronary arteryAccessory LAD from right coronary Accessory LAD from right coronary arteryartery
Pre-operative identification Pre-operative identification importantimportant
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CYANOSISCYANOSIS
Two-thirds acyanotic at birthTwo-thirds acyanotic at birth75-90% have cyanosis within 6 75-90% have cyanosis within 6 monthsmonths
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CYANOSISCYANOSIS
Two-thirds acyanotic at birthTwo-thirds acyanotic at birth75-90% have cyanosis within 6 months75-90% have cyanosis within 6 months
Progressive Progressive disease!!!disease!!!
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ACQUIRED ACQUIRED CONDITIONSCONDITIONS
Brain abscessesBrain abscesses– most commonly with TOFmost commonly with TOF– rarely before 2 years of agerarely before 2 years of age– headaches, fever, seizures, or headaches, fever, seizures, or
neurologic defecitsneurologic defecitsCerebrovascular accidentsCerebrovascular accidentsInfectious endocarditisInfectious endocarditisMay be pre-disposed to otitis mediaMay be pre-disposed to otitis media
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PHYSICAL EXAMPHYSICAL EXAM
Vital signs normalVital signs normal Normal ventricular impulsesNormal ventricular impulses First heart sound normalFirst heart sound normal Second heart sound usually Second heart sound usually
singlesingle Ejection click may be heardEjection click may be heard Normal pulsesNormal pulses Clubbing after 6 months of ageClubbing after 6 months of age
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MURMURSMURMURS
Systolic murmur at upper-left sternal borderSystolic murmur at upper-left sternal border– varies in intensityvaries in intensity– turbulent flow across right-ventricular outflow turbulent flow across right-ventricular outflow
tracttract– typically typically crescendo-decrescendocrescendo-decrescendo– may be difficult to distinguish from VSD murmurmay be difficult to distinguish from VSD murmur
Continuous murmurs uncommonContinuous murmurs uncommon– ductus arteriosusductus arteriosus– pulmonary collateralspulmonary collaterals
Diastolic murmurs rareDiastolic murmurs rare
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Physical Exam in Tetralogy of Physical Exam in Tetralogy of FallotFallot
S2 single
S1
EC
Systolic murmur of PS
1) Cyanosis - degree depends on PS
2) Normal pulses
3) Increased RV impulse at RSB
4) Murmur along LSB
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CXRCXR Heart size enlargedHeart size enlarged Small RVOT Small RVOT
segment+ large RV-segment+ large RV- Coeur en sabot Coeur en sabot or or boot shaped heartboot shaped heart
May see right sided May see right sided aortic arch aortic arch
Pulmonary Pulmonary vasculature vasculature decreased (oligemic)decreased (oligemic)
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ELECTROCARDIOGRAMELECTROCARDIOGRAM
RVHRVH Right axis deviationRight axis deviation Difficult to discern Difficult to discern
in neonatein neonate
Evident by 3 monthsEvident by 3 months RAE rare in childrenRAE rare in children LAD with AV canalLAD with AV canal
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ECHOCARDIOGRAPHYECHOCARDIOGRAPHY
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CARDIAC CARDIAC CATHETERIZATIONCATHETERIZATION
Usually not Usually not neededneeded
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TOF managementTOF managementMedicalMedical::
–Cyanotic spellsCyanotic spells
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TET SPELLSTET SPELLS
Increased cyanosis, abnormal Increased cyanosis, abnormal respirationsrespirations
Lethargy or unconsciousnessLethargy or unconsciousnessRapidly developing metabolic acidosisRapidly developing metabolic acidosisMore common in the morningMore common in the morningMay be precipitated by feeding, May be precipitated by feeding, crying, having a bowel movementcrying, having a bowel movement
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TET SPELLSTET SPELLS
Incidence peaks between 3 months and 5 yearsIncidence peaks between 3 months and 5 years– uncommon in neonatal perioduncommon in neonatal period
– rare after 5 years of agerare after 5 years of age
Increased right-to-left shuntingIncreased right-to-left shunting Decrease in intensity of systolic pulmonary Decrease in intensity of systolic pulmonary
outflow tract murmuroutflow tract murmur Usually self-limited, but may be life-threateningUsually self-limited, but may be life-threatening
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TET SPELLS-TET SPELLS-MECHANISMMECHANISM
Exact cause unknownExact cause unknown May be infundibular spasmMay be infundibular spasm
– may be primary or induced by catecholaminesmay be primary or induced by catecholamines– doesndoesn’’t explain occurrence with pulmonary t explain occurrence with pulmonary
atresiaatresia Tachypnea may be primary causeTachypnea may be primary cause
– increased systemic venous returnincreased systemic venous return– work of breathing increases oxygen work of breathing increases oxygen
consumptionconsumption– doesndoesn’’t explain decrease in murmurt explain decrease in murmur
Rarely, precipitated by SVTRarely, precipitated by SVT
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TET SPELLS-TREATMENTTET SPELLS-TREATMENT
Calm and comfort childCalm and comfort child OxygenOxygen Knee-chest positionKnee-chest position
– compresses femoral arteriescompresses femoral arteries– increases blood return from the increases blood return from the
legslegs MorphineMorphine
– may directly relax infundibulummay directly relax infundibulum– may act at the level of the CNSmay act at the level of the CNS
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TET SPELLS-TREATMENTTET SPELLS-TREATMENT
Knee-chest position- SquattingKnee-chest position- Squatting
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TET SPELLS-TET SPELLS-TREATMENTTREATMENT
Intravenous fluidsIntravenous fluidsBicarbonateBicarbonateBeta-blocker (propranalol)Beta-blocker (propranalol)Vasoconstrictor (phenylephrine)Vasoconstrictor (phenylephrine)External compression of External compression of abdominal aortaabdominal aorta
General anesthesiaGeneral anesthesia
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SURGICAL OPTIONSSURGICAL OPTIONS
Palliation followed by complete repairPalliation followed by complete repair– pulmonary-to-systemic anastamosis (shunt)pulmonary-to-systemic anastamosis (shunt)– modified Blalock-Taussig most commonly modified Blalock-Taussig most commonly
usedusedPrimary complete repairPrimary complete repair
– may not be possible because may not be possible because size of patientsize of patientsmall caliber of pulmonary arteriessmall caliber of pulmonary arteriescoronary artery anomaliescoronary artery anomalies
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TOTAL REPAIRTOTAL REPAIR
Closure of ventricular septal defectClosure of ventricular septal defect– transatrial or transverse ventricular incisiontransatrial or transverse ventricular incision– patch of synthetic material (Dacron or Teflon)patch of synthetic material (Dacron or Teflon)– avoid damaging conduction system avoid damaging conduction system
(perimembranous)(perimembranous)
Relief of right ventricular outflow tract Relief of right ventricular outflow tract obstructionobstruction
– resection of infundibular tissueresection of infundibular tissue– may need a transannular patch or valved conduitmay need a transannular patch or valved conduit
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TOF managementTOF management
VSD closureVSD closure
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TOF managementTOF managementRVOT reliefRVOT relief
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TRANSANNULAR TRANSANNULAR PATCHPATCH
Infundibulum short Infundibulum short and hypoplasticand hypoplastic
Simple resection of Simple resection of tissue does not tissue does not relieve obstructionrelieve obstruction
Can be extended to Can be extended to pulmonary arteriespulmonary arteries
Causes post-Causes post-operative pulmonary operative pulmonary regurgitationregurgitation
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TOF TOF –– Postop MRI Postop MRI
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VALVED CONDUITVALVED CONDUIT
Used when Used when pulmonary pulmonary regurgitation will be regurgitation will be poorly toleratedpoorly tolerated
– Pulmonary Pulmonary hypertensionhypertension
– Distal PA stenosisDistal PA stenosis Dacron conduitDacron conduit Porcine or human Porcine or human
valvevalve
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TOF- Late TOF- Late ComplicationsComplications Lancet. 2000 Sep 16;356(9234):975-81Lancet. 2000 Sep 16;356(9234):975-81 Risk Risk
factors for arrhythmia and sudden cardiac death late factors for arrhythmia and sudden cardiac death late after repair of TOF: a multicentre study.after repair of TOF: a multicentre study.
793 repaired TOF patients793 repaired TOF patients mean age at repair 8.2 years mean time from repair mean age at repair 8.2 years mean time from repair
21.1 years [8.7])21.1 years [8.7]) 33 - sustained monomorphic VT33 - sustained monomorphic VT
16 - died suddenly 16 - died suddenly 29 - had new-onset sustained 29 - had new-onset sustained
atrial atrial flutter or fibrillationflutter or fibrillation
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The Beginning………….The Beginning………….
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Anatomy of Tetralogy of FallotAnatomy of Tetralogy of Fallot