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
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
CARDIOVASCULAR MODULE 2008 - 2009
DIAGNOSIS
CONGENITAL
HEART DISEASE
• History• Physical Examination• ECG• Chest X-Ray• Echocardiography• Cardiac catheterization
UNDERSTANDCARDIOVASCULAR• Embryology• Anatomy• Physiology• Pathophysiology
CARDIOVASCULAR MODULE 2008 - 2009
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
CARDIOVASCULAR MODULE 2008 - 2009
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
CARDIOVASCULAR MODULE 2008 - 2009
Left-to-right lesions
Ventricular septal defect
Patent ductus arteriousus
Atrial septal defect
CARDIOVASCULAR MODULE 2008 - 2009
Obstructive lesions
Pulonary stenosis
Aortic stenosis
Coarctation of the aorta
CARDIOVASCULAR MODULE 2008 - 2009
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
CARDIOVASCULAR MODULE 2008 - 2009
Increased pulmonary blood flow
P = Q X R
CARDIOVASCULAR MODULE 2008 - 2009
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
EISENMENGER SYNDROME
CARDIOVASCULAR MODULE 2008 - 2009
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)
CARDIOVASCULAR MODULE 2008 - 2009
Small VSD
Large VSD
AUSCULTATION
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
CARDIOVASCULAR MODULE 2008 - 2009
ECHOCARDIOGRAM
VSD
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
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
CARDIOVASCULAR MODULE 2008 - 2009
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
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
CARDIOVASCULAR MODULE 2008 - 2009
AUSCULTATION
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
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
CARDIOVASCULAR MODULE 2008 - 2009
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
Magnitude of the shunt
Size of the defect
Relative compliance of the RV and LV
CARDIOVASCULAR MODULE 2008 - 2009
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
EISENMENGER SYNDROME
CARDIOVASCULAR MODULE 2008 - 2009
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
CARDIOVASCULAR MODULE 2008 - 2009
AUSCULTATION
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
CARDIOVASCULAR MODULE 2008 - 2009
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
CARDIOVASCULAR MODULE 2008 - 2009
Increased pulmonary blood fow Transposition of the Great Arteries
Truncus arteriosis
CARDIOVASCULAR MODULE 2008 - 2009
Decreased pulmonary blood flow Pulmonary obstruction
Right-to-left shunt through defect
Tetrallogy of Fallot
CARDIOVASCULAR MODULE 2008 - 2009
Decreased PBF
CARDIOVASCULAR MODULE 2008 - 2009
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
PDA
atrium : PFO, ASDventrikel : VSDpembuluh darah besar: PDA
VSD
ASD
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
PDA
• sianosis
• hipoksia
• asidosis
duktus
menutup
Balloon Atrial Septostomy
(BAS)
membuat lubang
ASD
Infus PGE1
mempertahankan
PDA terbuka