Algorithm

Congenital Heart Diseases

Cyanotic

Normal Flow

RVHLVH

Non Cyanotic

Plethora

CoAMR

PSMS

CoA Bayi

LVH RVH

VSDPDA

ASDPAVSDPAPVD

Oligemia Plethora

TOFPS + ShuntObstruktif + L→RPAEbstein Anomaly

Common Mixing Atrial• TAPVD• UniatrialCommon mixing AV• CAVSDCommon 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

PDA

Adolescent/Adult

HF (+) PH (-)HF (-) PH (+)

Premature Mature

Medical th/+

IndomethacinControlled Failed

Elective After >12 weeks

L→R L↔R

•Clinical•EKG•CXR•Echo

Medical th/

Closedspontaneously Ligation or Amplatzer Ductal Occluder Conservative

Controlled Failed

reactiveNon

reactiveElective After

>12 weeks

Cath

Neonate/Baby

HF :heart failurePH : Plumonary hipertensionIndomethacin 0,2 mg/kgbb 3x interval 12 hour

<10days

ASD

Big Shunt

Observe

PH (-)

HF (+)

PH (+)Evaluate5-8 yo Elective

> 1 yo

CathControlledFailed

Immediately

PVD (+)

•Clinical•EKG•CXR•Echo

Medical th/

Conservative Ligation or Amplatzer Septal Occluder Conservative

FR < 1.5 reactive Non reactive

Cath

Small Shunt

HF :heart failurePH : Plumonary hipertensionPVD : Pulmonary Vascular DiseasesASO tidak dapat dilakukan pada bayi < 8 Kg

FR > 1.5

Baby Adolescent Adult

HF (-)

> 1 yo

PVD (-)

VSD

HF (-)

ControlledFailed

PVD (+)

•Clinical•EKG•CXR•Echo

Medical th/

VSD Closure

Conservative

FR < 1.5reactive Non reactive

Cath

HF (+)

HF :heart failurePH : Plumonary hipertensionPVD : Pulmonary Vascular DiseasesReactive : PARI < 8 u/m2

FR > 1.5

Natural History

PVD (-)PABIf weight

< 3kg

Evaluate6 mo

ProlapsAo valve

StenosisInfundibulum

PulmonalHypertension Smaller

ClosedSpontaneously

CathCath 5 yo

CathPARI & FRRV : infundibularLV : VSD typeAo : prolaps

VSD + PH

Pulmonary Hypertension

Yes No or

High Flow

Follow upTill Pre School

Catheterization

PARI Flow ratio

Oxygen Test

< 8 u/m2 >8 u/m2

VSD Closure

< 8 u/m2 > 8 u/m2

< 1,5 > 1,5

High Flow

TOF

> 1 yo

Spell (+)

PROPANOLOL

Cath

ControlledFailed

•Clinical•EKG•CXR•Echo

TOTAL CORRECTION OPERATION

Cath

< 1 yo

BTS : Blalock Taussig ShuntPropanolol 0,5-1,5 mg/kg/dose 3-4x

CI : asthma

Spell (-)

BTS

PA/RV graphy

BTS

Cath

evaluate 6 mo

PA/RV graphy

Small PA Good size PA

Criteria for Operation– Good PA size– Good LV function

Cath– PA confluence/size– Anomaly coroner– MAPCA

Spell : – O2 100%– 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 IV 0,02 mg/kg IM 0,1 mg/kg

if not controlledVentilation BT Shunt,sat <30

BCPS CRITERIA

1. PAp < 18 mmHg

2. PARI < 4 Um2

3. PA Confluence

4. PA half size suitable (Kirklin)

CRITERIA FONTAN1. PAp < 15 mmHg2. PARI < 4 Um2

3. PA Confluence4. PA half size suitable (Kirklin)5. AV valve regurg. (-)6. LV dimension & function

adequate for Systemic Pump7. Arrhythmia (-)8. Age over 2-3 yo.

TGA

VSD (+)

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

> 3 mo Dynamic LVOTOor

Can be resected

•Clinical•EKG•CXR•Echo

< 3 mo

ARTERIAL SWITCHARTERIAL SWITCH & PERFORATED VSD RASTELLI

LV > 2/3

PABCath

VSD (-)

LVOTO : left ventricular outflow tract obstruction

Cath

LV < 2/3

Cath

PARI< 8

PARI> 8

Can not be resected

BTS

SEQUENTIAL ANALYSIS

1. Established Atrial Situs

2. Ascertain Atrioventicular connexions

3. Decide Ventriculo-Arterial

4. 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

SITUSEstablished 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 liverMeans / Inference Right Sided• Inferior vena cava & RA• Sinus Node• Tri-lobed, morphologically right Lung• Echo

– short axis Subxiphoid Thoracal XV A

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 middleBilateral right lung type• RA isomerism• AspleniaBilateral 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» Anterior (Ao) posterior (P)

Normal

P

PP

Ao

AoAo

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 ATRIALPANDANGAN SUBCOSTAL ( SAGITAL KORONAL )

Situs solitus : morfologi RA ada di kanan

morfologi LA ada di kiri

IVC ada di kanan kolum vertebrae ke RA

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 ATRIALSITUS ATRIALPANDANGAN SUBCOSTAL ( SAGITAL KORONALPANDANGAN 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 AtroventrikulerPANDANGAN 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 AtroventrikulerPANDANGAN 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 utamaPANDANGAN 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 2 D

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

VSD non Committed

PS (-)PS (+) PS (+)PS (+) PS (-)

TOFalgorithm

PS Nonresectable

•Clinical•EKG•CXR•Echo

PAB

INTRAVENTRICULARTUNNELLING

CONSER

VATIVE

FONTANTCPC

Reactive NonReactive

Cath

VSD Subaortic

DORV : Double Outlet Right VemtriclePAB : Pulmonary Artery BandingBTS : Blalock-Taussig ShuntBCPS : Bi Cavo-Pulmonary ShuntPS : Plumonary StenosisTB : Taussig Bing

VSD SADC VSD SP (TB)

Cath Cath

PAB

< 1 yo

Cath

BTS

EXTRACARDIAC CONDUIT/ FONTAN

PS resectable

ARTERIAL / ATRIALSWITCH

CONSER

VATIVE

> 3 mo< 3 mo

reactive Nonreactive

PS (-)

CathCath

BTS < 6 mo < 6 moPAB

BCPSBCPS

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

Partial

Obstruction (+)

PH (-)

•Clinical•EKG•CXR•Echo

TAPVD CORRECTION

Cath

Total

APVD : Anomaly Pulmonary Vein DrainageSVD : Sinus Venosus DefectBAS : Ballon Atrial Septostomy

Obstruction (-)

BAS

Cath

Supra cardiacIntra cardiacInfra cardiac

PH (+)

PH (+) PH (-)

CONSERVATIVE INTRA ATRIAL BAFFLE

REACTIVE NON REACTIVE REACTIVE

PA + IVS

Tricuspid ValveScore 2 > - 4

< 6 mo

Tricuspid ValveScore 2 < - 4Sinusoid RV

Anomaly Coroner

BTS

> 6 mo

BCPS

Cath

Small PA Big PA

BTS

FONTAN /TCPC

PGE1 BAS

•Clinical•EKG•CXR•Echo

Cath

Valvotomy Pulmonal(closed)+ BTS

+ PDA ligation

ASD CLOSURE + PV REPAIR

PA + VSD

BABY & CHILDNEONATUS

Shunt

Cath

MAPCA (+) MAPCA (-)

Univocalisasi + BTS

RASTELLI OPERATION

PGE1

•Clinical•EKG•CXR•Echo

Cath

Selective Aortography

TRICUSPID ATRESIA

< 6 mo

•Clinical•EKG•CXR•Echo

FONTAN TCPC

Cath

PULMONARY FLOW

PGE1BAS/BH

Cath

< 6 mo > 6 mo

Pap > 15 mmhgPARI < 4 HRU

< 15 mmhg< 4 HRU

PULMONARY FLOW (N) PULMONARY FLOW

> 6 mo

BTS

BTS

Cath

PAB

Cath

> 15 mmhg< 4 HRU

PAB < 2 yo > 2 yo

BCPSBCPS

BCPS CONSERVATIVE

CONGENITAL AS

CHILD / ADULT

SeverePG > 4.75 cm2/m2

INFANT / BABY

BAV

Mild / ModeratePG > 4.75 cm2/m2

Cath

NORWOOD

•Clinical•EKG•CXR•Echo

FONTAN

ValvotomiAorta

Cath

Cath

PG > 60 mmhgPG < 60 mmhg• LV strain• Syncope• Chest Pain

Ao Valvotomy

BAV

COARCTATIO AORTA

CoA + VSD

Ao Arch Hypoplastic

SIMPLE CoA

Hypoplastic LV & MVHLH

•Clinical•EKG•CXR•Echo

CoArc RepairVSD Closure

REPAIR• E-E• Subclavian Flap• Patch

COMPLEX CoA

Ao Arch Normal

Single VSD Multiple/Big VSD

CoArc RepairPAB

Complete RepairIn CPB CoArc Repair

+Intra Cardiac

Repair

NORWOODHIGH RISK

FONTAN