131009_catatan kuliah penyakit jantung bawaaningris

8/13/2019 131009_catatan Kuliah Penyakit Jantung BawaanIngris

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Heart Diseases in Infant andChildren

Dr. Mulyadi M. Djer, SpA(K)

Department of Child HealthMedical School University of Indonesia


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Structures of the heart


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Cardiac performance

Preload Afterload

ContractilityRate


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Heart disease in childrenCongenital heart disease

Acyanosis congenital heart diseaseCyanosis congenital heart disease

Acquired heart disease Acute rheumatic feverChronic rheumatic heart diseaseKawasaki disease

Cardiac involvement in systemic diseaseThalasemiaKidney diseaseetc


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Congenital Heart Disease


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Incidence of Congenital

Heart DiseaseThe incidence: 8-10 in 1000 live birth

Indonesia:

Total population : 220,000,000Birth rate: 2.3 %Incidence CHD per year: 40,000 cases

Jakarta:Total population: 10,000,000Incidence CHD per year: 4,000 cases.


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Classification of CHD AcyanosisNormal pulmonary blood flow

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

Increased pulmonary blood flowPatent Ductus Arteriosus (PDA) Atrial Septal Dfect (ASD) Ventricular Sseptal Defect (VSD)

CyanosisNormal pulmonary blood flow

TGA without PSIncreased pulmonary blood flow

TGA with VSDTruncus arteriosusTotal anomaly pulmonary vein drainage

Decreased pulmonary blood flowToFPulmonary atresiaTicuspid atresia


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Classification of CHD AcyanosisNormal pulmonary blood flow

PS ASCoA

Increased pulmonary blood flowPDA ASD VSD

CyanosisNormal pulmonary blood flow

Transposition of Great Artery (TGA) without PSIncreased pulmonary blood flow

TGA with VSDTruncus arteriosusTotal anomaly pulmonary vein drainage (TAPVD)

Decreased pulmonary blood flowTetralogy of Fallot (ToF)Pulmonary atresia (PA)Ticuspid atresia


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Pathophysiology acyanotic and cyanotic

Hemodynamic acyanotic Hemodynamic cyanotic


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EtiologyGenetic 10 %

Chromosome 7 %Monogenic 3 %Environment 3 %

Multifactor 90 %


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Sign and Symptom of CHDCyanosisDyspneuExercise intolerance

Infant Feeding problemIntermittent feedingProlonged feeding

Big children Dyspneu on exertionOrthopneuRecurrent respiratory tract infectionPoor weight gain

Asymptomatic murmur


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DiagnosisClinical findingSupporting examination

Level 1Periphery blood examination

Arterial blood gas analysisChest X rayElectrocardiography

Level 2Echocardiography

Level 3Cardiac catheterization

DiagnosticTherapeutic

OthersCT ScanMRI


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Level 3

Cardiac catheterizationDiagnosticTherapeutic

OthersCT ScanMRI


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Level 3


OthersCT ScanMRI


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Level 3


OthersCT ScanMRI


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Level 3


OthersCT ScanMRI


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MR-guided diagnostic and interventional procedures


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Early diagnosis is important because:Management of disease and education to parentdepend on itCertain CHD has optimal age to undergo definitivetreatment

TGA: 2 weeksComplete AVSD: 3-6 monthsTruncus arteriosus: < 6 months

Most CHD does not need intervention / surgery attime of diagnosis:

Intervention / surgery will be needed at anyage in which the risk of intervention or surgeryis low (usually above 1- 2 year), but dont late.Early surgery / intervention is needed ifconservative treatment fail.


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Early diagnosis is important because:Management of disease and education to parentdepend on itCertain CHD has optimal age to undergo definitivetreatment

Severe CoA / Interrupted Ao arch: as soon aspossible

TGA: 2 weeksComplete AVSD: 3-6 monthsTruncus arteriosus: < 6 months

Most CHD does not need intervention / surgery attime of diagnosis:



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

depend on itCertain CHD has optimal age to undergo definitivetreatment

TGA: 2 weeks

Complete AVSD: 3-6 monthsTruncus arteriosus: < 6 monthsMost CHD does not need intervention / surgery attime of diagnosis:


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Management of Congenital HeartDisease

Do not required treatment or intervention, some of defect closedspontaneouslyTreatment

Medical treatmentInitial treatment (PGE 1, 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 CHDSurgery Complex CHD

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Treatment of Congenital Heart

DiseaseMedical treatment

Initial treatment (PGE 1, indomethacin)

Complication treatment (anti failure, anti spell)Conservative treatment (Eisenmenger)

PalliativeIntervention non-surgery (BAS, PDA stenting)

Surgery (BT shunt, PA banding)Definitive


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Treatment of Congenital HeartDisease

Medical treatmentInitial treatment (PGE 1, indomethacin)





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Treatment of Congenital Heart

DiseaseMedical treatment

Initial treatment (PGE 1, indomethacin)





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State of the Art of Treatmentof Congenital Heart Disease

Invasiveness

E f f e c

t i v e n e s s

Good

Bad

Conventional

Surgery

MinimalInvasiveSurgery

Interven

tion


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ComplicationsHeart failure

preload afterload contractility heart rate

Cyanotic spell

EndocarditisEisenmenger syndromeetc

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