Congenital Heart Diseases

Non Cyanotic Normal Flow Plethora Cyanotic Oligemia Plethora

LVH CoA MR

RVH LVH VSD PDA

TOF PS + Shunt Obstruktif + LR RVH PA Ebstein Anomaly

PS MS CoA Bayi

ASD PAVSD PAPVD

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

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

Clinical EKG CXR Echo

Neonate/Baby HF (+) Premature

Medical th/ + Indomethacin

Adolescent/Adult HF (-) PH (-) LR PH (+) LR

Cath Non reactive

<10days

Mature
Medical th/

Elective After >12 weeks

Controlled Controlled Failed Elective After

>12 weeks

Failed reactive

Closed spontaneously

Ligation or Amplatzer Ductal Occluder

Conservative

HF :heart failure PH : Plumonary hipertension PVD : Pulmonary Vascular Diseases ASO tidak dapat dilakukan pada bayi < 8 Kg

ASD

Clinical EKG CXR Echo

Small Shunt Baby

HF (-) Evaluate 5-8 yo
Elective > 1 yo

Big Shunt
Adolescent Adult HF (+)
Medical th/ Failed Controlled > 1 yo

Observe

PH (-)

PH (+) PVD (-) PVD (+) Cath

reactive Non reactive

Cath

Immediately

FR < 1.5

FR > 1.5

Conservative

Ligation or Amplatzer Septal Occluder

Conservative

HF :heart failure PH : Plumonary hipertension PVD : Pulmonary Vascular Diseases Reactive : PARI < 8 u/m2

VSD

Clinical EKG CXR Echo

Cath PARI & FR RV : infundibular LV : VSD type Ao : prolaps

HF (+)
Medical th/ Failed Controlled

HF (-)

Natural History

Prolaps Stenosis Ao valve Infundibulum

Pulmonal Hypertension PVD (-) PVD (+)

Closed Spontaneously

Smaller

PAB
If weight < 3kg

Cath

Cath
5 yo FR < 1.5 FR > 1.5

Cath
Evaluate 6 mo reactive Non reactive Conservative

VSD Closure

VSD + PH
Pulmonary Hypertension No or High Flow Yes High Flow Catheterization

PARI
Follow up Till Pre School < 8 u/m2 >8 u/m2

Flow ratio
< 1,5 > 1,5

Oxygen Test < 8 u/m2 > 8 u/m2

VSD Closure

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

Clinical EKG CXR Echo

Cath PA confluence/size Anomaly coroner MAPCA

< 1 yo
Spell (+)
PROPANOLOL Failed Controlled

Spell (-) Cath

PA/RV graphy

Small PA

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
evaluate 6 mo

Cath

BTS

PA/RV graphy

TOTAL CORRECTION OPERATION

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.

LVOTO : left ventricular outflow tract obstruction

TGA

Clinical EKG CXR Echo

VSD (-) LVOTO (-) < 1 mo > 1 mo

< 3 mo > 3 mo

VSD (+) LVOTO (+)

Dynamic LVOTO or Can be resected

Cath
Cath
LV > 2/3 LV < 2/3

Can not be resected

BTS
PARI <8 PARI >8

Cath

PAB

ARTERIAL SWITCH

ARTERIAL SWITCH & PERFORATED VSD

RASTELLI

SEQUENTIAL ANALYSIS
1. 2. 3. 4. Established Atrial Situs Ascertain Atrioventicular connexions Decide Ventriculo-Arterial Ascertain relationships
Right Left & Anterior Posterior relationship

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
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 doesnt 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
Side by side Ao P Anterior (Ao) posterior (P)
Ao P

Normal
Ao

Hyperoxidation Test
O2 100% 10-20 minutes Lung problem
Saturation O2 increased to 100%

Cardiac problem
saturation O2 increased less than 30%

Posisi jantung dalam rongga toraks

5 Langkah Utama
Situs Atrial Loop bulbo ventrikuler Koneksi atrioventrikuler Relasi kedua pembuluh darah utama Koneksitas ventrikulo arterial

Anomali pada setiap segmen

SITUS ATRIAL
PANDANGAN SUBCOSTAL ( SAGITAL KORONAL )

Situs solitus
RA

: morfologi RA ada di kanan

morfologi LA ada di kiri IVC ada di kanan kolum vertebrae ke AoD ada di kiri kolum vertebrae

Situs Inversus : morfologi RA ada di kiri

moprfologi LA ada di Kanan IVC ada di kiri kolum vertebrae ke
RA AoD ada di kanan kolum vertebrae

SITUS ATRIAL
PANDANGAN SUBCOSTAL ( SAGITAL KORONAL )

Situs ambigus :
RA isomerisme ( asplenia )
Keduanya morfologi RA IVS dan AOD satu sisi di kanan atau di kiri kolum vertebrae.

LA isomerisme ( polisplenia )
Keduanya morfologi LA IVS terputus melalui v. azygos / v.hemoazygos masuk ke VCS dan RA

LOOP BULBO VENTRIKULER

D loop :
Morfologi RV di kanan Morfologi LV di kiri

L loop :
Morfologi RV di kiri Morfologi LV di kanan

Morfologi Ventrikel
PANDANGAN PARASTERNAL DAN PANDANGAN APIKAL 4 RUANG

Ventrikel kanan
Katup trikuspid : lebih dekat ke apex insersi khorda ke septum (+) Moderator band Trabekular kasar

Ventrikel kiri
Katup mitral ( bikuspid) : lebih jauh dari apex Insersi khorda ke septum (-) 2 muskulus papalaris besar ada di dinding ventrikel Trabekel halus

Koneksi Atroventrikuler
PANDANGAN APIKAL / SUBKOSTAL 4 RUANG

Konkordan :
Morfologi RA berhubungan dengan morfologi RV Morfologi LA berhubungan dengan morfologi LV

Diskordan :
Morfologi RA berhubungan dengan morfologi LV Morfologi LA berhubungan dengan morfologi RV

Koneksi Atroventrikuler
PANDANGAN APIKAL / SUBKOSTAL 4 RUANG

Ambigus :
Apa bila morfologi ke 2 atrium : RA atau LA (ambiogus)

Double inlet :
Kedua atrium berhubingan dengan satu ventrikel

Satu katup AV absen

Atresia katip trikuspid atau katup mitral

Relasi kedua pembuluh darah utama

PANDANGAN PARASTERNAL SUMBU PENDEK

A. Pulmonalis : bifucartio
bercabang dua

Relasi normal :
Aorta di posterior kanan PA

Malposisi
Aorta di : Anterior PA Anterior kiri PA Kiri dan kanan PA ( side by side )

Anomali tiap Segmen Jantung

Alir balik vena :
sistemik Bilateral SVC pulmonal APVD

Rongga atrium :
Septal atrium ASD Cor triatriatum

Atrioventrikular junction :
Katup AV : stenosis, atresia, cleft, regurgitasi, stradlling Septum : AVSD

Anomali tiap Segmen Jantung

Rongga ventrikel :
Anomalous muscle band
VSD Obstruksi alur keluar

Pembuluh darah Utama :

Katup : stenosis, atresia, regirgitasi, overriding PDA , AP window Arkus aorta : koartasio aorta, interuptus

KESIMPULAN
Ekokardiografi 2 dimensi paling penting untuk diagnosis PJB Diagnosis lengkap dan akurat bila dilakukan secara sistimatis ( analisa squensial ) Pemeriksa harus :
1.Mengerti anatomi dan morfologi jantung 2.Mengetahui gambaran karakteristik dari echo 2D 3.Trampil dan teliti

Hyperoxidation Test
O2 100% 10-20 minutes Lung problem
Saturation O2 increased to 100%

Cardiac problem
saturation O2 increased less than 30%

DORV : Double Outlet Right Vemtricle PAB : Pulmonary Artery Banding BTS : Blalock-Taussig Shunt BCPS : Bi Cavo-Pulmonary Shunt PS : Plumonary Stenosis TB : Taussig Bing

DORV
VSD SP (TB) PS (-) < 3 mo

Clinical EKG CXR Echo

VSD Subaortic

VSD SADC PS (+)

VSD non Committed PS (+)

BTS

PS (-) < 6 mo < 6 mo

Cath

PS (+)
PAB TOF algorithm

PS (-)
BTS

> 3 mo
Cath

PAB

Cath

Cath

Cath

Cath

reactive Reactive
PAB

< 1 yo
INTRA VENTRICULAR TUNNELLING

Non PS Reactive resectable PS Non resectable CON SER VATIVE EXTRACARDIAC CONDUIT/ FONTAN

Non reactive
BCPS

BCPS

ARTERIAL / ATRIAL SWITCH

CON SER VATIVE

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

APVD

Clinical EKG CXR Echo

Total
Obstruction (+)

Supra cardiac Intra cardiac Infra cardiac

Partial
PH (+) PH (-)

Obstruction (-)

PH (-)

PH (+)

BAS
Cath Cath

REACTIVE

NON REACTIVE

REACTIVE

TAPVD CORRECTION

CONSERVATIVE

INTRA ATRIAL BAFFLE

PA + IVS
PGE1 Tricuspid Valve
Score 2 < - 4 Sinusoid RV Anomaly Coroner

Clinical EKG CXR Echo

BAS
Tricuspid Valve
Score 2 > - 4

< 6 mo BTS

> 6 mo

Cath

Valvotomy Pulmonal (closed) + BTS + PDA ligation

Cath

Small PA
BTS BCPS

Big PA

FONTAN /TCPC

ASD CLOSURE + PV REPAIR

PA + VSD
NEONATUS
PGE1

Clinical EKG CXR Echo

BABY & CHILD

Cath Shunt

Cath
Selective Aortography MAPCA (+)
Univocalisasi + BTS

MAPCA (-)

RASTELLI OPERATION

TRICUSPID ATRESIA
PULMONARY FLOW
< 6 mo
PGE1 BAS/BH BTS

Clinical EKG CXR Echo

PULMONARY FLOW (N)

< 6 mo

PULMONARY FLOW
> 6 mo

> 6 mo
BTS

PAB

Cath Cath
BCPS

Cath
Pap > 15 mmhg PARI < 4 HRU PAB < 15 mmhg < 4 HRU > 15 mmhg < 4 HRU

BCPS

< 2 yo
BCPS

> 2 yo

Cath

FONTAN TCPC

CON SER VA TIVE

CONGENITAL AS
INFANT / BABY Severe
PG > 4.75 cm2/m2

Clinical EKG CXR Echo

CHILD / ADULT
PG > 60 mmhg PG < 60 mmhg LV strain Syncope Chest Pain BAV

Mild / Moderate
PG > 4.75 cm2/m2

BAV

Cath Valvotomi Aorta

NORWOOD
Cath

Cath

FONTAN

Ao Valvotomy

COARCTATIO AORTA
SIMPLE CoA
Ao Arch Normal

Clinical EKG CXR Echo

CoA + VSD
Ao Arch Hypoplastic

COMPLEX CoA
Hypoplastic LV & MV HLH

REPAIR E-E Subclavian Flap Patch

NORWOOD
Complete Repair In CPB
Multiple/Big VSD
HIGH RISK

Single VSD

CoArc Repair + Intra Cardiac Repair

CoArc Repair VSD Closure

CoArc Repair PAB

FONTAN