pem. cardiovaskular dr. mulyadi.ppt

CURICULUM VITAE

Name : Mulyadi M. Djer, MD, SpA(K), PhDPlace / Date of Birth : Padang, 29 October 1964Adress : Jl. Taman Sari VIII/23, Jatinegara Baru, Buaran, Jakarta Timur 13940. Phone 021 48636322Current Position : Lecturer and Medical Staff, Department of Child Health FKUI-RSCM JakartaOrganization : Secretary of Indonesia Society of Pediatric Cardiology (Perkani)

Educational Qualifications:Year: 1989 Degree: Medical Doctor (MD)

Institution: FKUI 1997 Pediatric Specialist (SpA) FKUI

2003 Pediatric Cardiologist FKUI

2005 Consultant Pediatric Cardiologist [(SpA(K)] IDAI

2008 Doctor of Phylosophy (PhD) FKUI

Awards, Fellowship, Grants:2001-2002 Fellowship training in Pediatric Cardiology at Institut Jantung

Negara (National Heart Institute), Kuala Lumpur, Malaysia

2004 Live course in Pediatric Cardiac Intervention, Beijing, China2004 & 2006 Live course in Pediatric Catheter Intervention , Kuala Lumpur, Malaysia2004 Short course in Pediatric Cardiac Intensive Care, Miami, USA2005 & 2007 International Workshop on Interventional Pediatric Cardiology, Millan, Italy2005 Live course in Pediatric Interventional Cardiology and Emerging

New Technique in Cardiac Surgery, Buenos Aires, Argentina2006 Live course in Pediatric Interventional Cardiology and Adult

Congenital Heart Disease, Las Vegas, USA 2009 Live course in Pediatric and Adult Interventional Cardiac

Symposium, Cairns, Australia

Heart Disease in Infant and Children

Heart Disease in Infant and Children

Mulyadi M. Djer, MD, SpA(K), PhD

Mulyadi M. Djer, MD, SpA(K), PhD

Department of Child HealthMedical School University of Indonesia

Department of Child HealthMedical School University of Indonesia

Structures of the heart

Cardiac performanceCardiac performance

PreloadAfterloadContractilityRate

PreloadAfterloadContractilityRate

Normal Heart

Heart disease in childrenHeart disease in children Congenital heart disease

Acyanosis congenital heart disease Cyanosis congenital heart disease

Acquired heart disease Acute rheumatic fever Chronic rheumatic heart disease Kawasaki disease Cardiac involvement in systemic disease

Thalasemia Kidney disease etc

Congenital heart disease Acyanosis congenital heart disease Cyanosis congenital heart disease

Acquired heart disease Acute rheumatic fever Chronic rheumatic heart disease Kawasaki disease Cardiac involvement in systemic disease

Thalasemia Kidney disease etc

Congenital Heart DiseaseCongenital Heart Disease

Incidence of Congenital Heart DiseaseIncidence of Congenital Heart Disease The incidence: 8-10 in 1000 live birth

Indonesia: Total population : ± 235,000,000 Birth rate: 2.3 % Incidence CHD per year: 50,000

cases

The incidence: 8-10 in 1000 live birth Indonesia:

Total population : ± 235,000,000 Birth rate: 2.3 % Incidence CHD per year: 50,000

cases

Classification of CHDClassification of CHD Acyanosis

Normal pulmonary blood flow Pulmonary Stenosis (PS) Aortic Stenosis (AS) Coarctatio Aorta (CoA)

Increased pulmonary blood flow Patent Ductus Arteriosus (PDA) Atrial Septal Defect (ASD) Ventricular Septal Defect (VSD)

Cyanosis Normal pulmonary blood flow

TGA without PS Increased pulmonary blood flow

TGA with VSD Truncus arteriosus Total anomaly pulmonary vein drainage

Decreased pulmonary blood flow ToF Pulmonary atresia Ticuspid atresia

Acyanosis Normal pulmonary blood flow

Pulmonary Stenosis (PS) Aortic Stenosis (AS) Coarctatio Aorta (CoA)

Increased pulmonary blood flow Patent Ductus Arteriosus (PDA) Atrial Septal Defect (ASD) Ventricular Septal Defect (VSD)

Cyanosis Normal pulmonary blood flow

TGA without PS Increased pulmonary blood flow

TGA with VSD Truncus arteriosus Total anomaly pulmonary vein drainage

Decreased pulmonary blood flow ToF Pulmonary atresia Ticuspid atresia

PDA

Located between aorta and pulmonary arteryLocated between aorta and pulmonary artery

ASD

Defect between LA and RADefect between LA and RA

VSD VSD

Tetralogy Fallot

Syndrome consist of 4 items: VSD Pulmonary stenosis Aortic over-riding RVH


Transposition of Great arteryTransposition of Great artery

Fetal vs. Neonatal Circulation

Fetal vs. Neonatal Circulation

Changes in Pulmonary Vascular Resistance 7 weeks preceding birth, at birth and 7 weeks after birth

Changes in Pulmonary Vascular Resistance 7 weeks preceding birth, at birth and 7 weeks after birth

Park MK. Pediatric cardiology for practitioner. 5th Ed. Philadelphia: Elsevier, 2008

Pathophysiology Acyanotic and Cyanotic Pathophysiology Acyanotic and Cyanotic

Hemodynamic acyanoticHemodynamic acyanotic Hemodynamic cyanoticHemodynamic cyanotic

Critically Congenital Heart DiseaseCritically Congenital Heart Disease Complex CHD in which circulation to

lungs /systemic depend on PDA Duct dependent pulmonary circulation

Pulmonary Atresia Duct dependent systemic circulation

Hypoplastic left heart syndrom Duct dependent mixing circulation

Transposition of great artery

Complex CHD in which circulation to lungs /systemic depend on PDA Duct dependent pulmonary circulation

Pulmonary Atresia Duct dependent systemic circulation

Hypoplastic left heart syndrom Duct dependent mixing circulation

Transposition of great artery

Critically CHDCritically CHD

Duct Dependent PulmonaryCirculation

Duct Dependent Systemic Circulation

Duct Dependent Mixing Circulation

EtiologyEtiology

Genetic 10 % Chromosome 7 % Monogenic 3 %

Environment 3 % Multifactor 90 %

Genetic 10 % Chromosome 7 % Monogenic 3 %

Environment 3 % Multifactor 90 %

Sign and Symptom of CHDSign and Symptom of CHD Cyanosis Dyspneu Exercise intolerance

Infant Feeding problem Intermittent feeding Prolonged feeding

Big children Dyspneu on exertion Orthopneu Recurrent respiratory tract infection Poor weight gain Asymptomatic murmur

Cyanosis Dyspneu Exercise intolerance

Infant Feeding problem Intermittent feeding Prolonged feeding

Big children Dyspneu on exertion Orthopneu Recurrent respiratory tract infection Poor weight gain Asymptomatic murmur

No murmur does not exclude CHD

The presence of murmur does not mean that there is CHD

No murmur does not exclude CHD

The presence of murmur does not mean that there is CHD

DiagnosisDiagnosis Clinical finding Supporting examination

Level 1 Periphery blood examination Arterial blood gas analysis Chest X ray Electrocardiography

Level 2 Echocardiography

Level 3 Cardiac catheterization

Diagnostic Therapeutic

Others CT Scan MRI

Clinical finding Supporting examination





Others CT Scan MRI

History

A complete birth history: maternal history; prenatal, perinatal, and postnatal complications; history of labor and delivery;

Neonatal course should be obtained, especially the exact time when the cyanosis developed, because certain CHD present at birth, while others may take as long as one month to present themselves.

History

A complete birth history: maternal history; prenatal, perinatal, and postnatal complications; history of labor and delivery;

Neonatal course should be obtained, especially the exact time when the cyanosis developed, because certain CHD present at birth, while others may take as long as one month to present themselves.

Clinical Manifestations

General Physical examination

Initial physical examination should focus on vital signs and cardiac and respiratory examinations

Evaluate for rales, stridor, grunting, flaring, retractions, and evidence of consolidation or effusion on pulmonary examination.

General Physical examination

Initial physical examination should focus on vital signs and cardiac and respiratory examinations

Evaluate for rales, stridor, grunting, flaring, retractions, and evidence of consolidation or effusion on pulmonary examination.


Physical Extremities: strength and symmetry of

the pulses in the upper and lower extremities, edema, and cyanosis of the nail beds.

Hepatosplenomegaly may be consistent with right ventricular or biventricular heart failure.

Physical Extremities: strength and symmetry of

the pulses in the upper and lower extremities, edema, and cyanosis of the nail beds.

Hepatosplenomegaly may be consistent with right ventricular or biventricular heart failure.


Cardiac Physical examination…… Inspection: precordial impulse, Palpation: thrill, left precordial lift, right

ventricular heave Percussion Auscultation: S1, S2 systolic or diastolic

murmurs, splitting abnormalities, S3 or S4 gallop, ejection click, opening snap, or rub.

Cardiac Physical examination…… Inspection: precordial impulse, Palpation: thrill, left precordial lift, right

ventricular heave Percussion Auscultation: S1, S2 systolic or diastolic

murmurs, splitting abnormalities, S3 or S4 gallop, ejection click, opening snap, or rub.


CyanosisCyanosis Bluish discoloration of skin & mucous

membrane ↑ reduced Hb to 5 g/100 mL in cutaneous veins

Central cyanosis Associated with desaturation of arterial

blood Peripheral cyanosis

Normal arterial oxygen saturation Increased extraction of oxygen by

peripheral tissue Circulatory shock Hypovolemia Vasoconstriction from cold

Bluish discoloration of skin & mucous membrane ↑ reduced Hb to 5 g/100 mL in cutaneous veins

Central cyanosis Associated with desaturation of arterial

blood Peripheral cyanosis

Normal arterial oxygen saturation Increased extraction of oxygen by

peripheral tissue Circulatory shock Hypovolemia Vasoconstriction from cold

Cyanosis Acro vs. CentralCyanosis Acro vs. Central

Acrocyanosis part of normal

transition may last 72hr beware APGAR

of 10 hypoperfused severe anemia

Acrocyanosis part of normal

transition may last 72hr beware APGAR

of 10 hypoperfused severe anemia

Lefkowitz B, 2000

Types of CyanosisTypes of Cyanosis

Peripheral vs CentralPink Mucous membranes,

tongue, lips, trunk (?)Blue

Cool Extremities Warm cool

Decreased Perfusion Normal to decreased

Normal PaO2 Low

Usually benign Outcome Urgent management

(pulmonary, CHD*, sepsis, shock)

Differential cyanosis:•Pink right hand/head with blue feet-pulmonary hypertension; preductal coarctation with PDA or interrupted arch•Pink feet, blue hands-transposition with coarctation*CHD = congenital heart disease

Thompson TR, The Cyanotic Newborn Infant

http://www.med.umn.edu/img/assets/9223

CyanosisCyanosis Normally there are 2 g/100 mL

required another 3 g/100 ml reduced Hgb to produce cyanosis

Hgb X Desaturation = 3, so Desat = 3/Hgb

For example Hgb 15 g/dl cyanosis appear at

desaturation 3/15 = 20%, or cyanosis appears at SaO2 80%

Hgb 6 g/dL cyanosis appear at desaturation 3/6 = 50% or cyanosis appears at SaO2 50%

Normally there are 2 g/100 mL required another 3 g/100 ml reduced Hgb to produce cyanosis

Hgb X Desaturation = 3, so Desat = 3/Hgb

For example Hgb 15 g/dl cyanosis appear at

desaturation 3/15 = 20%, or cyanosis appears at SaO2 80%

Hgb 6 g/dL cyanosis appear at desaturation 3/6 = 50% or cyanosis appears at SaO2 50%






Others CT Scan MRI






Others CT Scan MRI

Sa O2 and pO2Sa O2 and pO2

100% pO2 ↑

100% pO2 ↑

70%

70%

80%pO2 ↓

80%pO2 ↓70%

70% 70%

70%

100% pO2 ↑

80%pO2 ↓

Central CyanosisLung disease Heart disease

100% pO2 ↑

100% pO2 ↑

70%

70%

80%pO2 ↓

Peripheral Cyanosis

80%pO2 ↓

80%pO2 ↓

70%

70%70%

70%

100% pO2 ↑↑

100% pO2 ↑↑

21% O2 -Room Air 100% O2-HyperoxiaLung disease

100% pO2 ↑

80%pO2 ↓

70%

70% 70%

70%

100% pO2 ↑↑

80%pO2 ↓

21% O2- Room air 100% O2-Hyperoxia

Heart Disease

Hyperoxia testHyperoxia testOxygen

Concentration (%)

Ventilation Status

PaCO2 Goal

PaO2 Values

PPHN Lung Disease

RL Cardiac

21 %-room air Spontaneous 40 40 40 40

100 %-hyperoxia Spontaneous or MV

40 40 >100 40

100 %-pre and postductal shunt

Spontaneous or MV

40 >10-15 <5 <5

100 %-hyperoxia, hyperventilation

MV

(Mechanical ventilation)

20-25 >100 >150 40

Thompson TR, The Cyanotic Newborn Infant

http://www.med.umn.edu/img/assets/9223

How to read chest X rayHow to read chest X ray

Ebstein anomaly

TAPVD

“Figure of eight”“Snowman Appearance”

ToF

“Boot shape”

“Egg on Side”

TGA

ElectrocardiographyElectrocardiography

Cardiac Potential Cardiac Potential action recording on action recording on ECC electrode placing ECC electrode placing on the surface of the on the surface of the bodybody

Reference value Reference value ageage

Cardiac Potential Cardiac Potential action recording on action recording on ECC electrode placing ECC electrode placing on the surface of the on the surface of the bodybody

Reference value Reference value ageage






Others CT Scan MRI






Others CT Scan MRI

EchocardiographyEchocardiography






Others CT Scan MRI






Others CT Scan MRI

Atrial septal defectAtrial septal defect

ASD ASD






Others CT Scan MRI






Others CT Scan MRI

MR-guided diagnostic and interventional proceduresMR-guided diagnostic and interventional procedures

Early diagnosis is important because: Management of disease and education to

parent depend on it Certain CHD has optimal age to undergo

definitive treatment TGA: 2 weeks Complete AVSD: 3-6 months Truncus arteriosus: < 6 months

Most CHD does not need intervention / surgery at time of diagnosis:

Intervention / surgery will be needed at any age in which the risk of intervention or surgery is low (usually above 1-2 year), but don’t late.

Early surgery / intervention is needed if conservative treatment fail.









definitive treatment Severe CoA / Interrupted Ao arch: as soon

as possible TGA: 2 weeks Complete AVSD: 3-6 months Truncus arteriosus: < 6 months






definitive treatment Severe CoA / Interrupted Ao arch: as soon

as possible TGA: 2 weeks Complete AVSD: 3-6 months Truncus arteriosus: < 6 months




Management of CHD Management of CHD

Transcatheter Intervention

HybridIntervention

Surgery

Palliative Definitive

Medical Treatment

Invasiveness

Effe

ctiv

enes

s

Good

Bad

State of ArtState of Art

Intervention

Minimal InvasiveSurgery

ConventionalSurgery

MedicalTreatment

Management of Congenital Heart DiseaseManagement of Congenital Heart Disease

Do not required treatment or intervention, some of defect closed spontaneously

Treatment Medical treatment

Initial treatment (PGE1, indomethacin) Complication treatment (anti failure, anti spell) Conservative treatment (Eisenmenger)

Palliative Intervention non-surgery (BAS, PDA stenting) Surgery (BT shunt, PA banding)

Definitive Intervention non-surgery Non-complex CHD Surgery Complex CHD

Do not required treatment or intervention, some of defect closed spontaneously

Treatment Medical treatment

Initial treatment (PGE1, indomethacin) Complication treatment (anti failure, anti spell) Conservative treatment (Eisenmenger)



Treatment of Congenital Heart DiseaseTreatment of Congenital Heart Disease

Medical treatment Initial treatment (PGE1, indomethacin) Complication treatment (anti failure, anti spell) Conservative treatment (Eisenmenger)



Medical treatment Initial treatment (PGE1, indomethacin) Complication treatment (anti failure, anti spell) Conservative treatment (Eisenmenger)



Medical TreatmentMedical Treatment1. Initial treatment: Prostaglandin E1

Critical CHD To open PDA Fast response Doses 10 nanogram/kg/minute Side effect:

Apneu Hypotension

1. Initial treatment: Prostaglandin E1

Critical CHD To open PDA Fast response Doses 10 nanogram/kg/minute Side effect:

Apneu Hypotension

...Medical treatment...Medical treatment

2. Complication treatmentCyanotic spella. Kneechest positionb. Acid-base correctionc. Sedation: Morphin sulphat 0,2 mg/kg

IM/SCd. Propranolol: 0,01-0,25 mg/kg

(average 0,05 mg/kg) IV slowly

2. Complication treatmentCyanotic spella. Kneechest positionb. Acid-base correctionc. Sedation: Morphin sulphat 0,2 mg/kg

IM/SCd. Propranolol: 0,01-0,25 mg/kg

(average 0,05 mg/kg) IV slowly

...Medical treatment...Medical treatmentHeart failure ↓ preload

Diuretic; Frusemide : 1-2mg/kg/day 2 X

Sprironolakton:

0-10 kg: 6,25mg/kg 12H; 11-20 kg: 12,5 12H mg/kg 2X; 21-40 kg: 25 mg/kg 12H; >40 kg: 25 mg/kg 12H

↓ afterload Vasodilator

Captopril: 0,3-6 mg/kg/day divided 2-3 dose

Heart failure ↓ preload

Diuretic; Frusemide : 1-2mg/kg/day 2 X

Sprironolakton:

0-10 kg: 6,25mg/kg 12H; 11-20 kg: 12,5 12H mg/kg 2X; 21-40 kg: 25 mg/kg 12H; >40 kg: 25 mg/kg 12H

↓ afterload Vasodilator

Captopril: 0,3-6 mg/kg/day divided 2-3 dose

...Medical treatment...Medical treatment ↑ Contractility

Dopamine : 5-10µg/kg/minute Dobutamine: 5-10 µg/kg/minute Digoxin (µg/kg/day)

Digitalization Maintenance

Premature 20 5 < 30 day 30 8 < 2 year 40-50 10-12 > 2 year 30-50 8-10

↑ Contractility Dopamine : 5-10µg/kg/minute Dobutamine: 5-10 µg/kg/minute Digoxin (µg/kg/day)

Digitalization Maintenance

Premature 20 5 < 30 day 30 8 < 2 year 40-50 10-12 > 2 year 30-50 8-10

↓ heart rate Adenosine: 0,1 mg/kg fastly Beta blocker: Propranolol: 0,01-0,25 mg/kg (average 0,05 mg/kg) IV slowly.

↓ heart rate Adenosine: 0,1 mg/kg fastly Beta blocker: Propranolol: 0,01-0,25 mg/kg (average 0,05 mg/kg) IV slowly.

PalliativePalliative

Aim: to release sign or symptom Non-surgery:

BAS PDA stenting

Surgery: BT Shunt PA banding

Aim: to release sign or symptom Non-surgery:

BAS PDA stenting

Surgery: BT Shunt PA banding

Balloon Atrial SeptostomiBalloon Atrial Septostomi

Transposition Great Artery

Balloon Atrial SeptostomyBalloon Atrial Septostomy

PDA stentingPDA stenting

Hypoplastic Left Heart Syndrome

PDA stenting

Pulmonary Artery Banding (PA banding)Pulmonary Artery Banding (PA banding)

VSD pada bayi

Blallock Tausig Shunt (BT shunt)Blallock Tausig Shunt (BT shunt)

Blallock Tausig Shunt (BT shunt)

Definitive TreatmentDefinitive Treatment

Non-surgery: Non-Complex CHD Surgery:

Bi-ventricular circulation Single-ventricular /univentricular

circulation One and half ventricle Heart transplantation

Non-surgery: Non-Complex CHD Surgery:

Bi-ventricular circulation Single-ventricular /univentricular

circulation One and half ventricle Heart transplantation

Biventricular Circulation

Transposition of Great arteryTransposition of Great artery

Uni / Single Ventricular Circulation

One and Half Ventricular Circulation

ComplicationsComplications Heart failure

preload afterload contractility heart rate

Cyanotic spell Endocarditis Eisenmenger syndrome etc

Heart failure preload afterload contractility heart rate

Cyanotic spell Endocarditis Eisenmenger syndrome etc

Patent Ductus ArteriosusPatent Ductus Arteriosus

PDA

Located between aorta and pulmonary arteryLocated between aorta and pulmonary artery

Patent Ductus Arteriosus Patent Ductus Arteriosus

Incidence + 10% Female : Male = 1.2 to 1.5 : 1 Premature and LBW higher

Embryology Fetus: ductus arteriosus connects PA and

aorta. If ductus does not closs Patent Ductus

arteriosus

Incidence + 10% Female : Male = 1.2 to 1.5 : 1 Premature and LBW higher

Embryology Fetus: ductus arteriosus connects PA and

aorta. If ductus does not closs Patent Ductus

arteriosus

RA

RV

LA

LV

RA LA

RV LV

Patent Ductus Arteriosus


Clinical findings Small defect:

Symptom (-) Growth and development normal

Significant defect: Decreased exercise tolerant Weigh gained not good Frequent URTI

Specific case: pulsus seler at 4th extremities

Clinical findings Small defect:

Symptom (-) Growth and development normal

Significant defect: Decreased exercise tolerant Weigh gained not good Frequent URTI

Specific case: pulsus seler at 4th extremities


Auscultation : continuous murmur at upper LSB 2Auscultation : continuous murmur at upper LSB 2

ECG LVH

ECG LVH

Patent Ductus ArteriosusPatent Ductus Arteriosus Chest X- Ray

Prominent PA segment LVH

Chest X- Ray Prominent PA segment LVH

Diagnosis Differential AP-window Arterio-venous fistulae

Management Medical treatment

Premature: indometacin Anti-failure

Definitive treatment PDA closure :

Transcatheter closure Surgery

Diagnosis Differential AP-window Arterio-venous fistulae


Premature: indometacin Anti-failure

Definitive treatment PDA closure :

Transcatheter closure Surgery


PDAPDA

Neonates/InfantsNeonates/Infants Children/AdultsChildren/Adults

Heart failure (+)Heart failure (+) Heart failure (-)Heart failure (-)

PrematurePremature Full termFull term

Anti failureIndomethacinAnti failure

Indomethacin

SuccessSuccess FailFail

Spontaneous closure

Spontaneous closure

Anti failureAnti failure

SuccessSuccessFailFail

Surgical ligation

Surgical ligation

Transcatheter closureTranscatheter closure

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

LRLR RLRL

HyperoxiaHyperoxia

ReactiveReactive Nonreactive

Nonreactive

ConservativeConservative

Age >12wksW >4kg

Age >12wksW >4kg

FailFail

Transcatheter Closure of PDATranscatheter Closure of PDA

Plastic viseCombinationTouhy borst/hemostatis valve

Amplatzer occluder

Delivery cable

Delivery sheath

1 way stop cock

Amplatzer Ductal OccluderAmplatzer Ductal Occluder

ProcedureProcedure

Under general anesthesia

Access from femoral artery and vein

Under general anesthesia

Access from femoral artery and vein


PDA before occluded by device

PDA before occluded by device


Device collapsed into catheter and pushed out to open distal disk

Device collapsed into catheter and pushed out to open distal disk


Device and catheter withdrawn into PDA followed by opening proximal disk

Device and catheter withdrawn into PDA followed by opening proximal disk


Immediateresults after occluded by ADO

Immediateresults after occluded by ADO


10 minutes after occluded10 minutes after occluded


During unscrewedthe device During unscrewedthe device


PDA after occluded byADO

PDA after occluded byADO


PDA before occludedusing coil

PDA before occludedusing coil


PDA after occludedusing coilPDA after occludedusing coil

PDA ligationPDA ligation

Atrial Septal DefectAtrial Septal Defect

ASD

Defect between LA and RADefect between LA and RA

Atrial Septal defect( ASD )Atrial Septal defect( ASD )

Incidence + 10 %♂: ♀ ratio = 1,5 to 2 : 1

Anatomy :Defect on foramen ovale : Secundum ASDDefect at SVC and RA junction: sinus venosus

ASDDefect at ostium primum : primum ASD

Incidence + 10 %♂: ♀ ratio = 1,5 to 2 : 1

Anatomy :Defect on foramen ovale : Secundum ASDDefect at SVC and RA junction: sinus venosus

ASDDefect at ostium primum : primum ASD

Atrial Septal Defect


Diagram of ASDDiagram of ASD

RA

RV

LA

LV

RA

RV

LA

LV

Atrial septal Defect

Clinical findings Asymptomatic Auscultation :

Normal 1st HS or loudWide and fixed split 2nd HSEjection systolic murmur

Clinical findings Asymptomatic Auscultation :

Normal 1st HS or loudWide and fixed split 2nd HSEjection systolic murmur

Atrial septal DefectAtrial septal Defect

Normal Split 2nd Heart SoundNormal Split 2nd Heart Sound

ExpExp

InspInsp

A2 = P2A2 = P2

A2A2

P2P2

RARA

RARA LALA

RVRV

RVRV

LVLV

LALA

LVLV

Wide and Fixed Split 2nd Heart Sound in ASDWide and Fixed Split 2nd Heart Sound in ASD

ExpExp

InspInsp

A2A2

A2A2

P2P2

P2P2

RARA

RVRV

LALA

LVLV

RARA

RVRV

LALA

LVLV

Paradoxical Split 2nd Heart Sound in Aortic StenosisParadoxical Split 2nd Heart Sound in Aortic Stenosis

ExpExp

InspInsp

A2 = P2A2 = P2

A2A2P2P2

RARA

RARA LALA

RVRV

RVRV

LVLV

LALA

LVLV


Auscultation :•1st HS N or loud•Wide and fixed split 2nd HS •Ejection systolic murmur

Auscultation :•1st HS N or loud•Wide and fixed split 2nd HS •Ejection systolic murmur

ECG : IRBB , right ventricular hypertrophyECG : IRBB , right ventricular hypertrophy


•Right atrial enlargement•RVH•Prominence the MPA segment•Increased pulmonary vascular marking

•Right atrial enlargement•RVH•Prominence the MPA segment•Increased pulmonary vascular marking

Chest X-RayChest X-Ray

CatheterizationCatheterization


Diagnosis Differential

Partial Anomalous Pulmonary Vein Drainage

Pulmonary Stenosis

Innocent Murmur

Diagnosis Differential

Partial Anomalous Pulmonary Vein Drainage

Pulmonary Stenosis

Innocent Murmur

Atrial Septal defectAtrial Septal defect


Anti-failure Definitive treatment

Transcatheter closure using ASO (Amplatzer septal occluder)

Recent treatment Surgery : Preschool age


Anti-failure Definitive treatment

Transcatheter closure using ASO (Amplatzer septal occluder)

Recent treatment Surgery : Preschool age

ASDASD

Small ShuntSmall Shunt Large ShuntLarge Shunt

ObservationObservation

EvaluationAt age 5-8 yrs

EvaluationAt age 5-8 yrs

CathCath

FR<1.5FR<1.5 FR>1.5FR>1.5


InfantsInfants Children/AdultsChildren/Adults

Heart Failure (-)

Heart Failure (-)

Heart Failure (+)

Heart Failure (+)

Age >1yrsW >10kg

Age >1yrsW >10kg

Transcatheter closure (Secundum ASD) /Surgical Closure(other type of ASD)Transcatheter closure (Secundum ASD) /Surgical Closure(other type of ASD)



FailFailSuccessSuccess

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

PVD (-)

PVD (-)

PVD (+)

PVD (+)

HyperoxiaHyperoxia

Reac-tive

Reac-tive

Nonreactive

Nonreactive

SurgicalClosureSurgicalClosure

Amplatzer Septal OccluderAmplatzer Septal Occluder

AMPLATZER Septal OccluderAMPLATZER Septal Occluder


ASD before occlusionASD before occlusion

During balloon sizingDuring balloon sizing



ASD after occluded using ASOASD after occluded using ASO

….ASD Surgery….ASD Surgery

Ventricular Septal DefectVentricular Septal Defect

VSD VSD

Ventricular septal defectVentricular septal defect Incidence

20 % of all CHD No sex influenced

Anatomy Subarterial defect : below pulmonary and

aortic valve Perimembranous defect: below aortic valve at

pars membranous septum Muscular defect

Incidence 20 % of all CHD No sex influenced

Anatomy Subarterial defect : below pulmonary and

aortic valve Perimembranous defect: below aortic valve at

pars membranous septum Muscular defect

Ventricular Septal Defect

RA

RV

RA LALA

RV LVLV

Ventricular septal defect

Ventricular Septal Defect


Clinical findings Day 1st after birth: murmur (-) After 2-6 weeks : murmur (+) Murmur : pansystolic grade 3/6 or higher at

LLSB Small muscular defect: early systolic murmur Significant defect: Mid diastolic murmur at

apex

Clinical findings Day 1st after birth: murmur (-) After 2-6 weeks : murmur (+) Murmur : pansystolic grade 3/6 or higher at

LLSB Small muscular defect: early systolic murmur Significant defect: Mid diastolic murmur at

apex

Small VSD Small VSD

Large VSD Large VSD


Murmur: pansystolic grade 3/6 or higher at LSB 3

Murmur: pansystolic grade 3/6 or higher at LSB 3

ECG LVH

ECG LVH


•Cardiomegaly•Apex down ward•Prominence pulmonary artery segment•Increased pulmonary vascular marking

•Cardiomegaly•Apex down ward•Prominence pulmonary artery segment•Increased pulmonary vascular marking

Ventricular septal DefectVentricular septal Defect

Diagnosis Differential PDA with PH Tetralogy Fallot non cyanotic Innocent murmur

Diagnosis Differential PDA with PH Tetralogy Fallot non cyanotic Innocent murmur

Ventricular septal defectVentricular septal defect Management:

Medical treatment Anti-failure

Digoxin Diuretic

Palliative PA banding

Definitive : VSD closure

Surgery Transcatheter closure

Management: Medical treatment

Anti-failure Digoxin Diuretic

Palliative PA banding

Definitive : VSD closure

Surgery Transcatheter closure

DSVDSV

Heart failure (+)Heart failure (+) Heart failure (-)Heart failure (-)


FailFail SuccessSuccess

PABPAB

Evaluate in 6 mothsEvaluate in 6 moths

Surgical closure/Transcatheter closureSurgical closure/Transcatheter closure

Aortic valve prolaps

Aortic valve prolaps

Infundibular stenosis

Infundibular stenosis

PHPH SmallerSmallerSpontaneousclosure

Spontaneousclosure

CathCath

PVD(-)PVD(-) PVD(+)PVD(+) CathCath

CathCath

ReactiveReactive Non-reactive

Non-reactive


FR>1.5FR>1.5FR<1.5FR<1.5

….VSD Occlusion Amplatzer Perimembranous VSD Occluder

….VSD Occlusion Amplatzer Perimembranous VSD Occluder

Amplatzer Perimembranous VSD OccluderAmplatzer Perimembranous VSD Occluder

Ventricular septal defectVentricular septal defect

VSD before occlusionVSD before occlusion


Snaring wire at PA and pull it out to FV

Snaring wire at PA and pull it out to FV


VSD during deploying the deviceVSD during deploying the device

VSD after occludedusing ASOVSD after occludedusing ASO

…VSD Surgery…VSD Surgery

Tetralogy of FallotTetralogy of Fallot

Tetralogy Fallot



Tetralogy FallotTetralogy Fallot

Incidence5-8% from all CHD

AnatomyCaused: Left-anterior deviation of infundibular septum

Incidence5-8% from all CHD

AnatomyCaused: Left-anterior deviation of infundibular septum

What is the cause of ToFWhat is the cause of ToF Left deviation

Malalignment VSD Overriding aorta

Left deviation Malalignment VSD Overriding aorta

What is the cause of ToFWhat is the cause of ToF Anterior deviation

PS RVH VSD

Anterior deviation PS RVH VSD

Anterior Anterior


DiagnosisClinically :

CyanosisSingle 2nd HS, ejection systolic murmur

DiagnosisClinically :

CyanosisSingle 2nd HS, ejection systolic murmur


Single 2nd HS, ejection systolic murmurSingle 2nd HS, ejection systolic murmur

Tetralogi FallotTetralogi Fallot

CXR : Boot-shaped Concave

pulmonary segment

Apex upturned Decreased

pulmonary blood flow

CXR : Boot-shaped Concave

pulmonary segment

Apex upturned Decreased

pulmonary blood flow



ECG :

RAD

RVH

ECG :

RAD

RVH

Echocardiography: to confirm diagnosis

Echocardiography: to confirm diagnosis

Cardiac CatheterizationCardiac Catheterization


Diagnosis Differential Pulmonary Atresia Double outlet right ventricle and pulmonary stenosis Transposition of great artery and pulmonary

stenosis Management

Medical treatment Anti-spell

Palliative treatment: Blalock-Taussig shunt PDA stenting

Definitive: total correction

Diagnosis Differential Pulmonary Atresia Double outlet right ventricle and pulmonary stenosis Transposition of great artery and pulmonary

stenosis Management

Medical treatment Anti-spell

Palliative treatment: Blalock-Taussig shunt PDA stenting

Definitive: total correction

Tetralogy of FallotTetralogy of Fallot

< 1 yr< 1 yr > 1 yr> 1 yr

spell (+)spell (+) spell (-)spell (-)propranololpropranolol

failedfailed succeedsucceed

BTS or

PDA Stent

BTS or

PDA Stent

total correctiontotal correction

cath cath

small PAsmall PA good sized PAgood sized PA

• clinically• ECG

• clinically• ECG

• CXR• echo

• CXR• echo

age 1 yrage 1 yr

cathcath BTS/

PDA Stent

BTS/

PDA Stent

evaluationevaluation

PDA stenting New alternative need for

palliative surgery in neonate

required

PDA stenting New alternative need for

palliative surgery in neonate

required

PDA stentingPDA stenting

Interventional Treatment of Congenital Heart disease

Interventional Treatment of Congenital Heart disease

Invasiveness

Effe

ctiv

enes

s

Good

Bad

State of ArtState of Art

Intervention

Minimal InvasiveSurgery

ConventionalSurgery

Transcatheter treatment of CHD offers a number of advantages over surgery

Transcatheter treatment of CHD offers a number of advantages over surgery

Less invasive Fast recovery Eliminates thoracotomy No surgical complication No post surgical pain No chest scar Shortened hospitalization Minimal hospital service

requirements

Less invasive Fast recovery Eliminates thoracotomy No surgical complication No post surgical pain No chest scar Shortened hospitalization Minimal hospital service

requirements

SurgerySurgery InterventionIntervention

http://www.kkh.com.sg/NR/rdonlyres/DF14FB94-F731-447C-AAAB-F423E9C79901/1389/Fig2702_Acc_ANI_large.gif

Interventional treatment in CHDInterventional treatment in CHDPalliative

Balloon atrial septectomy (BAS)PDA stenting

DefinitivePercutaneous occlusion of cardiac defect

PDA, ASD,VSDCollateralArtery-venous malformation

Balloon angioplasty / valvuloplasty Balloon valvuloplasty: PS, AS or MSBalloon dilatation / stent branch of pulmonary artery stenosisBalloon angioplasty Coarctation of aortaRadio frequency assisted valvotomi in PA-IVS

PalliativeBalloon atrial septectomy (BAS)PDA stenting

DefinitivePercutaneous occlusion of cardiac defect

PDA, ASD,VSDCollateralArtery-venous malformation

Balloon angioplasty / valvuloplasty Balloon valvuloplasty: PS, AS or MSBalloon dilatation / stent branch of pulmonary artery stenosisBalloon angioplasty Coarctation of aortaRadio frequency assisted valvotomi in PA-IVS

020406080

100120140160180

PDA ADO ASD VSD

RSCM RSJHK RS SOETOMO RS M HOESIN RS SARDJITO

020406080

100120140160180

PDA ADO ASD VSD

RSCM RSJHK RS SOETOMO RS M HOESIN RS SARDJITO

175

66

145 1

75

94

7 0 0

204 0 0 0

Interventional Pediatric Cardiology in IndonesiaInterventional Pediatric Cardiology in Indonesia

• Dr. Mazeni Alwi, MRCP (Kuala Lumpur)• Dr. Hasri Samion, MMed Paed (Kuala Lumpur)• Dr. Mulyadi M. Djer, SpAK (Jakarta)• Dr. Sukman T. Putra, SpAK, FACC, FESC (Jakarta)• Prof. Bambang Madiyono, SpJP, SpAK (Jakarta)• Prof. DR. Sudigdo Sastroasmoro, SpAK (Jakarta)• Dr. Ismet N Oesman, SpAK (Jakarta)• Dr. Najib Advani, SpAK, MMed Paed (Jakarta)• Dr. Syarif Rohimi, SpA (Jakarta)• Dr. Sasmito Nugroho, SpA (Yogyakarta)• Dr. Noormanto, SpAK (Yogyakarta)• Dr. Mahrus A. Rahman, SpAK (Surabaya)• Dr. Ria Nova, SpAK (Palembang)• All Fellow of School of Pediatric Cardiology

• Dr. Mazeni Alwi, MRCP (Kuala Lumpur)• Dr. Hasri Samion, MMed Paed (Kuala Lumpur)• Dr. Mulyadi M. Djer, SpAK (Jakarta)• Dr. Sukman T. Putra, SpAK, FACC, FESC (Jakarta)• Prof. Bambang Madiyono, SpJP, SpAK (Jakarta)• Prof. DR. Sudigdo Sastroasmoro, SpAK (Jakarta)• Dr. Ismet N Oesman, SpAK (Jakarta)• Dr. Najib Advani, SpAK, MMed Paed (Jakarta)• Dr. Syarif Rohimi, SpA (Jakarta)• Dr. Sasmito Nugroho, SpA (Yogyakarta)• Dr. Noormanto, SpAK (Yogyakarta)• Dr. Mahrus A. Rahman, SpAK (Surabaya)• Dr. Ria Nova, SpAK (Palembang)• All Fellow of School of Pediatric Cardiology

AcknowledgementAcknowledgement

pem. cardiovaskular dr. mulyadi.ppt

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