Department of Cardiology and Vascular Medicine Faculty of...

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Poppy S. RoebionoDepartment of Cardiology and Vascular MedicineFaculty of Medicine University of IndonesiaNational Cardiovascular Center Harapan Kita

CARDIOVASCULAR MODULE 2008 - 2009

anomalies of the heart‟s structure or its major blood vessels and circulatory function that are present at birth

due to disturbances or failure in the development of the fetus„ heart during the early weeks of pregnancy

approximately 8 – 10 out of every 1,000 live newborns have congenital heart disease

Indonesia: approximately 9 per 1,000 live births

Harimurti GM, Asian Congress of Cardiology, 1997

with birth rate 20 per 1,000 per year there will 40,500 baby born per year with congenital heart disease

Rahajoe AU, Cardiol Young 2007; 17: 584 – 8

DIAGNOSIS

CONGENITAL

HEART DISEASE

• History• Physical Examination• ECG• Chest X-Ray• Echocardiography• Cardiac catheterization

UNDERSTANDCARDIOVASCULAR• Embryology• Anatomy• Physiology• Pathophysiology

Knowledge of fetal and perinatalcirculation is helpful in understanding the clinical manifestations and natural

history of CHD

CHANGES IN CIRCULATION AFTER BIRTH

Shift of blood flow for gas exchange from placenta to the lungs

1. Interruption of the umbilical cord• Increase of SVR• Closure of ductus venosus

2. Lung expansion• Reduction of PVR• Functional closure of PFO• Closure of PDA

near or at term: PVR is as high as the SVR

at birth: rapid fall of PVR

6 – 8 weeks after birthslower fall of PVR

first 2 yearsfurther decline of PVR

Divided into 2 types

Non-cyanotic

Cyanotic

Clinical manifestations

acyanosis or cyanosis

pulmonary blood flow: normal, increased (plethora) or decreased (oligemia)

ventricular hypertrophy: LVH, RVH or BVH

Left-to-right lesions

Ventricular septal defect

Patent ductus arteriousus

Atrial septal defect

Obstructive lesions

Pulonary stenosis

Aortic stenosis

Coarctation of the aorta

Increased pulmonary blood flow

Magnitude of theleft-to-right shunt

Size of the defectLevel of pulmonary vascular resistance

Asymptomatic• small left-to-right shunt

Symptomatic• large left-to-right shunt• short of breath• feeding difficulties• recurrent respiratory tract infections• failure to thrive

• Congestive Heart Failure• Pulmonary Hypertension large left-to-right shunt high pulmonary vascular resistance pulmonary vascular obstructive disease bi-directional shunt right-to-left shunt cyanosis

EISENMENGER SYNDROME

Increased pulmonary blood flow

P = Q X R

Small VSD

Asymptomatic

Large VSD

Symptomatic at 2 – 3 months old

rapid fall of PVRCARDIOVASCULAR MODULE 2008 - 2009

left-to-right shunt

LV volume overload

LA, LV and PA

enlargement

congestive heart failure

pulmonary hypertension

HEMODYNAMIC

Small VSD

• normal P2

• holosystolic murmur

Large VSD

• accentuated P2 pulmonary hypertension

• holosystolic murmur left to right shunt

• mid diastolic murmur increased blood flow through the mitral valve relative MS

Large VSD with Pulmonary Vascular Obstructive Disease

• loud and single S2

• decreased loudness of the holosystolic murmur (or disappear)

Small VSD

Large VSD

AUSCULTATION

CHEST X-RAY

LA, LV and PA dilatation

Prominent pulmonary artery segment

Increased pulmonary vascular marking (plethora)

ELECTROCARDIOGRAM

Left ventricular hypertrophy

Bi-ventricular hypertrophy

ECHOCARDIOGRAM

MANAGEMENT

Asymptomatic• Small VSD spontaneous closure

• FR (Qp/Qs) > 1.5 intervention: VSD closure

Symptomatic• Failure to thrive and congestive heart failure

• Anti heart failure treatment: digoxin, diuretics, vasodilator

• Intervention: VSD closure

INTERVENTION Non-surgical: trans-catheter closure with AMVSO

Surgical closure

Small PDA Asymptomatic

Small left-to-right shunt

Large PDA Symptomatic

Large left-to-right shunt Increased pulmonary blood flow at 2 – 3 months old rapid fall of PVR

PREMATURE NEWBORNS Pulmonary vascular smooth muscle is not well developed Fall in PVR occurs more rapidly Early onset of a large left-to-right shunt and congestive

heart failure

left-to-right shunt

LA and LV volume

overload

LA, LV, Ao and PA

enlargement

congestive heart failure

pulmonary hypertension

HEMODYNAMIC

Normal P2 intensity• small PDA

Continuous “machinery” murmur• left to right shunt occurs throughout the cardiac cycle

• significant pressure gradient between Ao and PA during systole and diastole

Apical mid diastolic murmur• increased blood flow through the mitral valve relative MS

Large PDA with pulmonary hypertension• single and loud S2

• no longer continuous murmur ejection systolic murmur

AUSCULTATION

CHEST X-RAY

• LA, LV, ascending Ao and PA

dilatation

• prominent pulmonary artery

segment

• increased pulmonary vascular

marking (plethora)

ELECTROCARDIOGRAM

Left ventricular hypertrophy

Bi-ventricular hypertrophy

MANAGEMENT

Asymptomatic• Small PDA spontaneous closure (neonates)• > 3 – 4 months old intervention : PDA closure

Symptomatic• Failure to thrive and congestive heart failure• Anti heart failure treatment: digoxin, diuretics, vasodilator• Neonates: indomethazine treatment• Intervention: PDA closure

INTERVENTION Non-surgical: trans-catheter closure with ADO or coils Surgical closure: PDA ligation

TRANS CATHETER CLOSURE

Magnitude of the shunt

Size of the defect

Relative compliance of the RV and LV

Asymptomatic Children Congestive heart failure if complicated

with with severe mitral regurgitation

Symptomatic 30 – 40 years of age Pulmonary hypertension

HEMODYNAMIC

Left-to right-shunt

RA, RV and PA dilatation

RV volume overload

Pulmonary hypertension 30 – 40 years of age

large left-to-right shunt

high pulmonary vascular resistance

pulmonary vascular obstructive disease

bi-directional shunt right-to-left shunt cyanosis

Widely split and fixed S2 RV volume overload prolonged RV ejection time delays

the closure of the pulmonary valve large pulmonary venous return to RA fixed split

Systolic ejection murmur not caused by the shunt originates from the increased blood flow passing through the

normal-sized pulmonary valve relative PS

Mid diastolic murmur increased blood flow through the tricuspid valve relative TS large left to right shunt

Accentuated P2 pulmonary hypertension

AUSCULTATION

CHEST X-RAY

• RA, RV and PA dilatation

• prominent pulmonary artery segment

• increased pulmonary vascular marking (plethora)

ELECTROCARDIOGRAM

Right ventricular hypertrophy

ECHOCARDIOGRAM

ASD --

MANAGEMENT

Asymptomatic intervention : ASD closure

• Pre school age 3 – 4 years old • FR (Qp/Qs) > 1.5 intervention: VSD closure

Symptomatic Large ASD with or without MI Failure to thrive – congestive heart failure Anti heart failure treatment: digoxin, diuretics and

vasodilator Intervention: ASD closure

INTERVENTION Non-surgical: trans-catheter closure with ASO Surgical closure: ASD closure

Increased pulmonary blood fow Transposition of the Great Arteries

Truncus arteriosis

Decreased pulmonary blood flow Pulmonary obstruction

Right-to-left shunt through defect

Tetrallogy of Fallot

Decreased PBF

cyanosis clubbing finger hypoxic spell squatting

PS / PA

+PFO / ASD / VSD( R – L SHUNT )

• Tetralogi Fallot• PS + PFO / ASD• PA + VSD

• anterior deviation of the infundibularseptum

• Bulbus malrotasion

• perimembranus VSD• overriding Ao• valvular-infundibular PS• RV hypertrophy

MANAGEMENT

• PALIATIVE BT shunt• spell sianotik berulang• menambah aliran darah ke paru

• DEFINITIVE total correction• VSD closure• rv outflow tract reparation• around 1 year of age

perlu mixing antara sirkulasi pulmonal dan sistemik

SIRKULASI PARALEL

atrium : PFO, ASDventrikel : VSDpembuluh darah besar: PDA

Complete TGA• AV concordance : RA – RV dan LA – LV• VA discordance : RV – Ao dan LV – PA

• TGA – IVS (Intact Ventricular Septum) without VSD

• TGA – VSD

Corrected TGA• AV discordance : RA – LV dan LA – RV• VA discordance : LV – PA dan RV – Ao

Neonatus

duct dependent systemic circulation

diperlukan percampuran darah vena

dan arteri melalui PDA atau PFO

• sianosis

• hipoksia

• asidosis

duktus

menutup

Balloon Atrial Septostomy

membuat lubang

Infus PGE1

mempertahankan

PDA terbuka

Department of Cardiology and Vascular Medicine Faculty of...

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