Poppy S.

Roebiono Department of Cardiology and Vascular Medicine Faculty of Medicine University of Indonesia National Cardiovascular Center Harapan Kita

CARDIOVASCULAR MODULE 2008 - 2009

anomalies of the hearts 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

CARDIOVASCULAR MODULE 2008 - 2009

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

CARDIOVASCULAR MODULE 2008 - 2009

UNDERSTAND CARDIOVASCULAR Embryology Anatomy Physiology Pathophysiology

History Physical Examination ECG Chest X-Ray Echocardiography Cardiac catheterization

DIAGNOSIS CONGENITAL HEART DISEASE

CARDIOVASCULAR MODULE 2008 - 2009

Knowledge of fetal and perinatal circulation 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
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 near or at term: PVR is as high as the SVR at birth: rapid fall of PVR 6 8 weeks after birth slower fall of PVR first 2 years further decline of PVR

CARDIOVASCULAR MODULE 2008 - 2009

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
CARDIOVASCULAR MODULE 2008 - 2009

Left-to-right lesions

Ventricular septal defect Patent ductus arteriousus Atrial septal defect

Obstructive lesions

Pulonary stenosis Aortic stenosis Coarctation of the aorta

CARDIOVASCULAR MODULE 2008 - 2009

Size of the defect

Level of pulmonary vascular resistance

Magnitude of the left-to-right shunt

Increased pulmonary blood flow

CARDIOVASCULAR MODULE 2008 - 2009

Asymptomatic

Symptomatic
large

small left-to-right shunt

Increased pulmonary blood flow

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

P=QXR 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
Congestive

EISENMENGER SYNDROME

CARDIOVASCULAR MODULE 2008 - 2009

CARDIOVASCULAR MODULE 2008 - 2009

Small VSD Asymptomatic

Large VSD
Symptomatic
at 2 3 months old rapid fall of PVR

CARDIOVASCULAR MODULE 2008 - 2009

HEMODYNAMIC

left-to-right shunt LV volume overload LA, LV and PA enlargement congestive heart failure pulmonary hypertension EISENMENGER SYNDROME

CARDIOVASCULAR MODULE 2008 - 2009

AUSCULTATION

Small VSD

Small VSD normal P2 holosystolic murmur Large VSD 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)
CARDIOVASCULAR MODULE 2008 - 2009

CHEST X-RAY

LA, LV and PA dilatation Prominent pulmonary artery segment Increased pulmonary vascular marking (plethora)

CARDIOVASCULAR MODULE 2008 - 2009

ELECTROCARDIOGRAM

Left ventricular hypertrophy

Bi-ventricular hypertrophy
CARDIOVASCULAR MODULE 2008 - 2009

ECHOCARDIOGRAM

VSD

CARDIOVASCULAR MODULE 2008 - 2009

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

CARDIOVASCULAR MODULE 2008 - 2009

CARDIOVASCULAR MODULE 2008 - 2009

CARDIOVASCULAR MODULE 2008 - 2009

Small PDA Asymptomatic

Small left-to-right shunt

Large PDA Symptomatic

at 2 3 months old rapid fall of PVR

Large left-to-right shunt Increased pulmonary blood flow

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
CARDIOVASCULAR MODULE 2008 - 2009

HEMODYNAMIC

left-to-right shunt LA and LV volume overload LA, LV, Ao and PA enlargement congestive heart failure pulmonary hypertension EISENMENGER SYNDROME

CARDIOVASCULAR MODULE 2008 - 2009

AUSCULTATION

Normal P2 intensity

small PDA

Continuous machinery murmur

Apical mid diastolic murmur

left to right shunt occurs throughout the cardiac cycle significant pressure gradient between Ao and PA during systole and diastole
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

CARDIOVASCULAR MODULE 2008 - 2009

CHEST X-RAY

LA, LV, ascending Ao and PA dilatation prominent pulmonary artery segment increased pulmonary vascular marking (plethora)

CARDIOVASCULAR MODULE 2008 - 2009

ELECTROCARDIOGRAM

Left ventricular hypertrophy

Bi-ventricular hypertrophy
CARDIOVASCULAR MODULE 2008 - 2009

MANAGEMENT
Asymptomatic

Small PDA spontaneous closure (neonates) > 3 4 months old intervention : PDA closure Failure to thrive and congestive heart failure Anti heart failure treatment: digoxin, diuretics, vasodilator Neonates: indomethazine treatment Intervention: PDA closure Non-surgical: trans-catheter closure with ADO or coils Surgical closure: PDA ligation

Symptomatic

INTERVENTION

CARDIOVASCULAR MODULE 2008 - 2009

TRANS CATHETER CLOSURE

CARDIOVASCULAR MODULE 2008 - 2009

TRANS CATHETER CLOSURE

CARDIOVASCULAR MODULE 2008 - 2009

TRANS CATHETER CLOSURE

CARDIOVASCULAR MODULE 2008 - 2009

CARDIOVASCULAR MODULE 2008 - 2009

Magnitude of the shunt Size of the defect Relative compliance of the RV and LV Asymptomatic

Symptomatic

Children Congestive heart failure if complicated with with severe mitral regurgitation 30 40 years of age Pulmonary hypertension
CARDIOVASCULAR MODULE 2008 - 2009

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 EISENMENGER SYNDROME

CARDIOVASCULAR MODULE 2008 - 2009

AUSCULTATION

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

Mid diastolic murmur

Accentuated P2 pulmonary hypertension

CARDIOVASCULAR MODULE 2008 - 2009

CHEST X-RAY
RA, RV and PA dilatation prominent pulmonary artery segment increased pulmonary vascular marking (plethora)

CARDIOVASCULAR MODULE 2008 - 2009

ELECTROCARDIOGRAM

Right ventricular hypertrophy

CARDIOVASCULAR MODULE 2008 - 2009

ECHOCARDIOGRAM

ASD --

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MANAGEMENT
Asymptomatic intervention : ASD 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

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

INTERVENTION

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

CARDIOVASCULAR MODULE 2008 - 2009

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

CARDIOVASCULAR MODULE 2008 - 2009

CARDIOVASCULAR MODULE 2008 - 2009

PS / PA PFO / ASD / VSD ( R L SHUNT )

Tetralogi Fallot PS + PFO / ASD PA + VSD

Decreased PBF
cyanosis clubbing finger hypoxic spell squatting

CARDIOVASCULAR MODULE 2008 - 2009

 anterior deviation of the infundibular septum 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

PDA

perlu mixing antara sirkulasi pulmonal dan sistemik

VSD PDA ASD

atrium : PFO, ASD ventrikel : VSD pembuluh darah besar: PDA

SIRKULASI PARALEL

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

PDA

Neonatus duct dependent systemic circulation

diperlukan percampuran darah vena dan arteri melalui PDA atau PFO

duktus menutup
Infus PGE1

sianosis hipoksia asidosis

Balloon Atrial Septostomy (BAS)

mempertahankan PDA terbuka

membuat lubang ASD