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Congenital Heart Diseases
Non Cyanotic
Normal Flow
Plethora
LVH
RVH LVH
CoA
MR
VSD
PDA
PS
MS
CoA Bayi
Cyanotic
Oligemia
TOF
PS + Shunt
Obstruktif + L→R
RVH PA
Ebstein Anomaly
ASD
PAVSD
PAPVD
Plethora
Common Mixing Atrial
• TAPVD
• Uniatrial
Common mixing AV
• CAVSD
Common Mixing Ventricle
• Single ventricle
• HLHS, TA, MA
• DORV, DILV
Truncus (A-P Window)
TGA + VSD
Congenital Heart Diseases
Acyanotic
Normal Flow
LVH
CoA
MR
RVH
Cyanotic
Plethora
LVH
RVH
LVH
RVH
TA
PA-IVS
VSD
PDA
ECD
PS
MS
CoA(infant)
Oligemia
ASD
PAPVR
PVOD
TOF
PA-VSD
Ebstein anomaly
Plethora
BVH
TGA+PS
PTA +
hypoplastic
PA
Single
ventricle with
PS
LVH
RVH
PTA
Single
ventr
TGA+VSD
TGA
TAPVR
HLHS
Common Mixing
• Pressure & saturation of O2 in Aorta &
pulmonal is the same
HF :heart failure
PH : Plumonary hipertension
Indomethacin
0,2 mg/kgbb 3x interval 12 hour
PDA
Neonate/Baby
Adolescent/Adult
HF (-)
HF (+)
Premature
<10days
Medical th/
+
Indomethacin
Mature
•Clinical
•EKG
•CXR
•Echo
Elective After
>12 weeks
PH (-)
PH (+)
L→R
Medical th/
Controlled
L↔R
Cath
Failed
reactive
Controlled
Non
reactive
Failed Elective After
Closed
spontaneously
>12 weeks
Ligation or Amplatzer Ductal Occluder
Conservative
HF :heart failure
PH : Plumonary hipertension
PVD : Pulmonary Vascular Diseases
ASO tidak dapat dilakukan pada bayi < 8 Kg
•Clinical
•EKG
•CXR
•Echo
ASD
Small Shunt
Big Shunt
Baby
Observe
HF (-)
Evaluate
5-8 yo
Elective
> 1 yo
Adolescent
Adult
HF (+)
Medical th/
Failed
Cath
FR < 1.5
Immediately
Controlled
PH (-)
PH (+)
PVD (-)
> 1 yo
FR > 1.5
Conservative
PVD (+)
Cath
reactive
Ligation or Amplatzer Septal Occluder
Non reactive
Conservative
HF :heart failure
PH : Plumonary hipertension
PVD : Pulmonary Vascular Diseases
Reactive : PARI < 8 u/m2
VSD
HF (+)
•Clinical
•EKG
•CXR
•Echo
Cath
PARI & FR
RV : infundibular
LV : VSD type
Ao : prolaps
HF (-)
Medical th/
Failed
Natural History
Controlled
Prolaps
Stenosis
Ao valve Infundibulum
PAB
Pulmonal
Hypertension
PVD (-)
If weight
< 3kg
Closed
Spontaneously
Smaller
PVD (+)
Cath
Cath
5 yo
Cath
Evaluate
6 mo
reactive Non reactive
FR < 1.5
Conservative
VSD Closure
FR > 1.5
VSD + PH
Pulmonary Hypertension
No or
High Flow
Yes
High Flow
Catheterization
PARI
Follow up
Till Pre School
< 8 u/m2
Flow ratio
>8 u/m2
< 1,5
Oxygen Test
< 8 u/m2
VSD Closure
> 8 u/m2
> 1,5
BTS : Blalock Taussig Shunt
Propanolol 0,5-1,5 mg/kg/dose 3-4x
CI : asthma
TOF
Criteria for Operation
– Good PA size
– Good LV function
< 1 yo
Spell (+)
Spell (-)
PROPANOLOL
Cath
Failed
Controlled
Small PA
PA/RV graphy
•Clinical
•EKG
•CXR
•Echo
Spell :
– O2 100%
> 1 yo – Knee Chest Position
– MO 0,1 mg/kgbb
– Diazepam 0,1 mg/kgbb
– BicNat 3-5 meq/kgbb
– Propanolol 0,02-0,1 mg/kg
– Fenilefrine
CI 2-5 mg/kgbb/mt
Cath
IV 0,02 mg/kg
IM 0,1 mg/kg
if not controlled
Ventilation
Good size PA BT Shunt,sat <30
BTS
Cath
evaluate 6 mo
Cath
– PA confluence/size
– Anomaly coroner
– MAPCA
BTS
PA/RV graphy
TOTAL CORRECTION OPERATION
LVOTO :
left ventricular outflow tract obstruction
TGA
•Clinical
•EKG
•CXR
•Echo
VSD (+)
VSD (-)
LVOTO (-)
< 1 mo
LVOTO (+)
> 1 mo
< 3 mo
> 3 mo
Dynamic LVOTO
or
Can be resected
Cath
Cath
LV > 2/3
LV < 2/3
Can not be
resected
BTS
PARI
<8
PARI
>8
Cath
ARTERIAL SWITCH &
PERFORATED VSD
RASTELLI
PAB
ARTERIAL SWITCH
DORV : Double Outlet Right Vemtricle
PAB : Pulmonary Artery Banding
BTS : Blalock-Taussig Shunt
BCPS : Bi Cavo-Pulmonary Shunt
PS : Plumonary Stenosis
TB : Taussig Bing
VSD Subaortic
DORV
VSD SADC
VSD SP (TB)
PS (+)
PS (+)
VSD non Committed
PS (+)
PS (-)
BTS
Cath
< 3 mo
Cath
> 3 mo
Cath
reactive
Reactive
PAB
< 1 yo
INTRA
VENTRICULAR
TUNNELLING
PS (-)
PS (-)
PAB
TOF
algorithm
•Clinical
•EKG
•CXR
•Echo
Non
PS
Reactive
resectable
PS Non
resectable
CON
SER
VATIVE
EXTRACARDIAC
CONDUIT/
FONTAN
BTS
< 6 mo
< 6 mo
PAB
Cath
Cath
Cath
Non
reactive
BCPS
ARTERIAL /
ATRIAL
SWITCH
CON
SER
VATIVE
BCPS
FONTAN
TCPC
Taussig Bing
• Echo
– Great arteries side by side
– Conus between
• MV & PV
• PV & Ao poss. Stenosis post arterial switch.
– Often associated with Ao Arch Hypoplastic
IN TGA there uss. Without Conus.
APVD : Anomaly Pulmonary Vein Drainage
SVD : Sinus Venosus Defect
BAS : Ballon Atrial Septostomy
Total
Obstruction (+)
APVD
•Clinical
•EKG
•CXR
•Echo
Supra cardiac
Intra cardiac
Infra cardiac
Partial
Obstruction (-)
PH (-)
PH (+)
PH (-)
PH (+)
BAS
Cath
REACTIVE
TAPVD CORRECTION
NON REACTIVE
CONSERVATIVE
Cath
REACTIVE
INTRA ATRIAL BAFFLE
PA + IVS
•Clinical
•EKG
•CXR
•Echo
BAS
PGE1
Tricuspid Valve
Tricuspid Valve
Score 2 < - 4
Sinusoid RV
Anomaly Coroner
Score 2 > - 4
< 6 mo
> 6 mo
Valvotomy Pulmonal
(closed)
+ BTS
+ PDA ligation
BTS
Cath
Cath
Small PA
Big PA
BTS
BCPS
FONTAN /TCPC
ASD CLOSURE + PV REPAIR
PA + VSD
NEONATUS
•Clinical
•EKG
•CXR
•Echo
BABY & CHILD
PGE1
Cath
Shunt
Cath
Selective Aortography
MAPCA (+)
MAPCA (-)
Univocalisasi + BTS
RASTELLI OPERATION
•Clinical
•EKG
•CXR
•Echo
TRICUSPID ATRESIA
PULMONARY FLOW
< 6 mo
PULMONARY FLOW (N)
> 6 mo
< 6 mo
> 6 mo
PGE1
BAS/BH
PULMONARY FLOW
BTS
PAB
Cath
BTS
Cath
Cath
Pap > 15 mmhg
PARI < 4 HRU
BCPS
BCPS
PAB
Cath
FONTAN TCPC
< 15 mmhg
< 4 HRU
< 2 yo
BCPS
> 15 mmhg
< 4 HRU
> 2 yo
CON
SER
VA
TIVE
CONGENITAL AS
INFANT / BABY
CHILD / ADULT
Severe
Mild / Moderate
PG > 4.75 cm2/m2
PG > 4.75 cm2/m2
PG > 60
mmhg
PG < 60
mmhg
• LV strain
• Syncope
• Chest Pain
BAV
Cath
Valvotomi
Aorta
•Clinical
•EKG
•CXR
•Echo
NORWOOD
BAV
Cath
Cath
FONTAN
Ao
Valvotomy
•Clinical
•EKG
•CXR
•Echo
COARCTATIO AORTA
SIMPLE CoA
CoA + VSD
Ao Arch
Normal
REPAIR
• E-E
• Subclavian Flap
• Patch
Ao Arch
Hypoplastic
COMPLEX CoA
Hypoplastic LV & MV
HLH
NORWOOD
Complete Repair
In CPB
Single VSD
Multiple/Big VSD
CoArc Repair
VSD Closure
CoArc Repair
PAB
HIGH RISK
CoArc Repair
+
Intra Cardiac
Repair
FONTAN
BCPS CRITERIA
1.
2.
3.
4.
PAp < 18 mmHg
PARI < 4 Um2
PA Confluence
PA half size suitable (Kirklin)
CRITERIA FONTAN
1.
2.
3.
4.
5.
6.
PAp < 15 mmHg
PARi < 4 Um2
PA Confluence
PA half size suitable (Kirklin)
AV valve regurg. (-)
LV dimension & function
adequate for Systemic Pump
7. Arrhythmia (-).
8. Age over 2-3 yo.
SEQUENTIAL ANALYSIS
1.
2.
3.
4.
Established Atrial Situs
Ascertain Atrioventicular connexions
Decide Ventriculo-Arterial
Ascertain relationships
– Right – Left & Anterior – Posterior relationship
Situs
Morphology Right Atrium
• Atrial appendages “blunt ending”
• Receives Systemic Venous Return
• Coronary sinus enter to the smooth wall
sinus venorum separated by from
trabeculated right auricle by crista
terminalis
Morphology Left Atrium
• Atrial Appendages “Finger Shaped”
• Receive blood from Pulmonary Vein
• Smooth walled is not separated from
trabeculated wall by crista
Morphology Right Ventricle
•
•
•
•
Coarse trabeculation of the wall
Shape “Rounded”
Contain infundibulum & tricuspid valve
Tricuspid valve separated from Pulmonary valve by
crista supraventricularis
trabecula septomarginalis
• Insertion of papillary muscle of Tricuspid
– Single Anterior
– Multiple Posterior
– Medial
MORPHOLOGY LEFT VENTRICLE
•
•
•
•
Fine Trabeculation
Shape “ellipse”
Mitral valve & Ao Valve in fibrous continuity
Bileaflet mitral valve
• No medial papillary insertion, all to free wall
SITUS
Established Atrial Situs
• Situs Solitus
• Morphology right Atrium right side
• Morphology left Atrium on the left side
• Situs Inversus
• Morphology right Atrium left side
• Morphology left Atrium on the right side
• Situs Ambigus
• Not possible to separate right & left atria by
morphological
Situs Solitus
By Plain Ro
• Right sided liver
Means / Inference Right Sided
• Inferior vena cava & RA
• Sinus Node
• Tri-lobed, morphologically right Lung
• Echo
– short axis Subxiphoid Thoracal X
A
V
Spine
Bronchial Branches
• Strong Xray
• Right side three lobed distance from bifurcatio
shorter
• Left side two lobed distance from the
bifurcatio shorter
• IVC always to RA
• In LA isomerism, there must be an interrupted
IVC.
Azygos to SVC (Left)
Hemiazygos to SVC (right)
• SVC doesn’t always into RA, can be bilateral
SITUS AMBIGUS
By Plain Ro
• Liver both side, stomach in the middle
Bilateral right lung type
• RA isomerism
• Asplenia
Bilateral left lung type
• LA isomerism
• Polysplenia
AV connection
•
•
•
•
•
Discordant
Ambigus
Double inlet
Single inlet (univentricular)
Straddling,
– insertion of papillary muscle MV in RV or
– insertion of papillary muscle TV in LV
• Overriding
– Insertion papillary of overriding mitral in the LV
• Ventricle inversion can be determined by EKG
– Normal V1 RSR, V6 qRS
– Ventricle inversion V1 qRS, V6 RSR
VA c Ao onnection
• Physical examination
– 2nd Heart sound single, not accentuated : PA
– 2nd Heart sound single, loud : TGA
Ao
» Side by side
P
» Anterior (Ao) posterior (P)
Normal
P
Ao
Ao
P
Hyperoxidation Test
• O2 100% 10-20 minutes
• Lung problem
– Saturation O2 increased to 100%
• Cardiac problem
– saturation O2 increased less than 30%