Transcript PDA - Jantung Sehat

Grown –Up
Congenital Heart Disease
(GUCH)
Mohammad Saifur Rohman, MD. PhD
Interventional Cardioloy Consultant
Lab. Cardiology and Vascular Medicine
Faculty of Medicine
University of Brawijaya
1
Definition
• Adults with congenital heart defects are a
group of patients which is fast growing in
numbers, due to advances in cardiac surgery
and intensive care in the 1970s and 80s.
• Congenital heart disease (CHD) : Cardiac
lesions present from birth
• CHD in adult: Small defect, no correction,
partial correction, post correction
2
Causes of congenital heart
disease
• No one cause can explain all cases. Many
factors :
• Genetic
• Environmental
Affect cardiac development in the uterus;
3
Causes of congenital heart
disease
• Maternal rubella- in addition to cataracts,
deafness, and microcephaly, cause patent
ductus arteriosus (PDA) and pulmonary
stenosis
• Fetal alcohol syndrome- associated with
cardiac defects (as well as microcephaly,
micrognathia, microphthalmia, and growth
retardation)
4
Causes of congenital heart
disease
• Maternal systemic lupus erythematosus
– associated with fetal complete heart
block (due to transplacental passage of
anti-Ro antibodies)
5
Genetic associations with
congenital heart disease
• Trisomy 21- endocardial cushion defects,
atrial septal defect (ASD), ventricular septal
defect (VSD), tetralogy of Fallot.
• Turner’s syndrome (X0)- coarctation of the
aorta
• Marfan syndrome- aortic dilatation and aortic
and mitral regurgitation
• Kartagener’s syndromeBrochiectasis,chronic sinusitis, situs inversus
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Cardiac malformations
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Ventricular septal defect (VSD)
Atrial septal defect (ASD)
Patent ductus arteriosus (PDA)
Pulmonary stenosis – causes cyanosis if severe
Coarctation of the aorta
Aortic stenosis
Tetralogy of Fallot – causes cyanosis
Transposition of the great arteries – causes cyanosis
Other causes of cyanotic congenital heart diseasepulmonary atresia, hypoplastic left heart, severe
Ebstein’s anomaly with ASD
7
Classification of cyanotic congenital heart disease
Classification of cyanoctic congenital heart disease by the amount
of pulmonary blood flow seen on chest x ray.
Increased Pulmonary Blood Flow
Normal or Decreased Pulmonary Blood Flow
Tricuspid atresia with large VSD
Tricuspid atresia with restrictive VSD
Total anomalous pulmonary venous return
Pulmonary atresia with intact ventricular septum
Truncus arteriosus
Ebstein’s anomaly
D-transposition of the great arteries
D-transposition of the great arteries with pulmonary
stenosis
Taussig-Bing anomaly
Double outlet right ventricle with pulmonary stenosis
Tetralogy of Fallot with minimal right ventricular
outflow tract obstruction
Tetralogy of Fallot
Tetralogy of Fallot with pulmonary atresia and
increased collateral flow
Tetralogy of Fallot wit pulmonary atresia
Single ventricle without pulmonary stenosis
Single ventricle with pulmonary stenosis
Interrupted aortic arch with PDA
Vena cava to left atrium communication
Hypoplastic left heart syndrome
ASD with Eisenmenger’s syndrome
VSD with Eisenmenger’s syndrome
PDA with Eisenmenger’s syndrome
Non Cyanotic Heart Disease
2 groups:
Left to right shunting (Septa l defects)
- PDA (Patent ductus Arteriosus)
– VSD (Ventricular septal defect)
– ASD (Atrial Septat defect)
Obstruction lesion in left or right heart
without shunt
AS (Aortic stenosis)
CoA (Coarctation of Aorta)
PS (Pulmonary stenosis)
Acyanotic heart disease
Chest X
ray
Blood flow to lung
Normal
RVH
-PS
-MS
Increased
LVH
-MI
-AS
-KoA
RVH
-ASD
-PAPVR
-PVOD
LVH/BVH
-VSD
-PDA
-AVSD
Echocardiography, cardiac catheterization
EKG
Non cyanotic heart disease with
left to right shunting
size - location
of defect
Pulmonary
vascular resistance
Shunting via defect
Overflow to pulmonary
Non Cyanotic Heart Disease
Symptoms
Pulmonary blood
flow overload
• asymptomatic
• symptomatic
Depend on Pulmonary
vascular resistance
•Dyspnea
•Recurent respiratory infection
• Fail to thrive
• Heart failure
asymptomatic
Symptomatic
Clinical features
• Neonates:
– Small VSD: asymptomatic
– Large VSD  left ventricular failure :
• Failure to thrive, feeding difficulty, sweating on
feeding
• Tachypnea and intercostal recession
• Hepatomegaly
• Adult:
– Asymptomatic
– Dyspnea due to PH or Eisenmenger syndrome
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• Volume overload of LV
• Big left to right shunt
• Dilatation of LA, LV dan PA
• Congestive heart failure
A. Small VSD
• Holosistolic murmur
 Left to right shunt
B. Moderate VSD
• Holosistolic murmur
• Early diastolic murmur
 Flow through mitral
valve
C. Big VSD : PVR < SVR
• Loud S2 (P2)
• Ejection systolic murmur
 Flow through RV
outflow tract
D. VSD besar : PVR > SVR
• Load S2 (P2)
• No  Shunting (-)




Cardiomegaly
LA and LV dilatation
RV dilatation (PH)
Increased pulmonary
vascularization (pletora)
Pulmonary hypertension in adult
Echocardiography
19
Echocardiography
20
Management
• 30% of cases close spontaneously,
mostly by the time the child is 3 years of
age.
• Some do not close until the child is 10
years old.
• Defects near the valve ring or near the
outlet of the ventricle do not usually
close
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Management
• Operative closure is the treatment of
choice and is recommended for all
lesions that have not undergone
spontaneous closure
• VSD  risk factor for infective
endocarditis  appropriate prophyalctic
measures should be taken
22
PDA
• In the fetus most of the output of the right
ventricle bypasses the lungs via the ductus
arteriosus
• This vessel joins the pulmonary trunk (artery)
to the descending aorta distal to the left
subclavian artery.
• The ductus arteriosus normally closes about
1 month after birth in full-term infants and
takes longer to close in premature infants
23
• Overload of LA and LV
• Big left to right shunting
• Dilatation LA, LV, AO and PA
• Congestive heart failure
Pulmonary hypertension in adult
Clinical features
• The factors that determine the nature of
clinical features are the same as in VSD and
ASD i.e the size of the defect, the presence of
PH, the development of Eisenmenger’s
syndrome
• A patent PDA is more likely in babies born at
high altitude, probably due to low
atmospheric oxygen concentration; it may
also occur In babies who have fetal rubella
syndrome
25
History
• Small PDA: asymptomatic
• Large PDA: large left-to-right shunt 
left ventricular failure with pulmonary
edema causing failure to thrive and
tachypnea
• Adults with undiagnosed PDA may
develop PH and present with dyspnea
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Small PDA
 Continuous murmur – machinery murmur
 Left to right shunting during systolic and diastolic
Big PDA – PH
 pulsus celler S2 (P2) Loud
 Continuous murmur
 Left to right shunt
fase sistolik dan diastolik
 Systolic murmur
 Left to right shunt
fase sistolik
 Murmur (-)
 Early Diastolic murmur
 Mitral flow
Management
• Pharmacological closure in neonates –
indomethacin may induce closure if
given early
• Operative closure – this can be
performed as an open procedure in
which the PDA is ligated or divided
• Or using ADO (Amplatzer ductal
occluder) by cardiac catheter
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SYMPTOMATIC
Heart failure
• digitalis
• diuretika
• vasodilator
PH – PVR ?
• LIGTION PDA
• ADO
(BW> 8 kg)
Management
• PDA is a risk fractor for infective
endocarditis  antibiotic prophylaxis is
required for all patients before operative
procedures.
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Shunting depend upon
• Defect size
• Compliant of RV < LV
Left to right shunt
3 types of Atrial septal defect
• (1) Septum primum (ostium primum
ASD)- this defect lies adjacent to
atrioventricular valves, which are often
also abnormal and incompetent
• (2) Septum secundum (ostium
secundum ASD)- the most common
form of ASD, it is midseptal in location
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3 types of Atrial septal defect
• (3) Sinus venosus ASD – this lies high
in the septum and may be associated
with anomalous pulmonary venous
drainage (in which one of the pulmonary
veins drains into the right atrium instead
of the left.
33
History
• Early life: asymptomatic
• Adult life: dyspnea, fatigue, recurrent
chest infections
• As time goes by, the increased
pulmonary blood flow results in
pulmonary hypertension and eventually
reversal of the shunt and Eisenmenger
syndrome
34
• Volume overload in RV
• Dilatation of RA, RV and PA
• Pulmonary Hipertension
 sindroma Eisenmenger
S2 wide fixed splitting
• pengosongan RV lama – katup
pulmonal terlambat menutup
• tidak berubah dengan respirasi
Sistolic ejection murmur
• Flow through RVOT and PA
Diastolic murmur
• Flow through trikuspid
valve
Examination
• The second heart sound is widely split
because closure of the pulmonary valve is
delayed due to increased pulmonary blood
flow.
• The splitting is fixed in relation to respiration
because the communication between the
atria prevents the normal pressure differential
between right and left sides that occurs
during respiration.
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Examination
• The increased pulmonary blood flow causes
a mid systolic pulmonary flow murmur.
• If PH has developed  reduction of the leftto-right shunt, the pulmonary flow murmur
disappears; there is a loud pulmonary
component to the second heart sound
• If Eisenmenger’s syndrome occurs 
centrally cyanosed, finger clubbing
38
Investigations
• EKG
– Ostium secundum ASD: right axis deviation
– Ostium primum defect: left axis deviation
• Chest radiography
– Pulmonary arteries: dilated, its branches are
prominent
– Enlarged right atrium, enlarged right ventricle
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 RA dilatation
 RV dilatation 
prominent pulmonary
segment
 Increased pulmonary
vaskularisation (pletora)
PH
 Wide of pulmonal and
hilus
 Ischemic of peripheral
pulmonary vascular
(pruning)
Echocardiography
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Echocardiography
42
Cardiac catheterization
• To reveal ASD, because the catheter can be
passed across it.
• Serial oxygen saturation measurements are
made at different levels from the superior
vena through the atrium and the right
ventricle into the pulmonary artery.
• At the level of the left-to-right shunt there will
be a step up increase of the oxygen
saturation as blod flow from the left side
enters the right.
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ASYMPTOMATIC
usia pra sekolah
(3 – 4 tahun)
SYMPTOMATIC
HEART FAILURE
• digitalis
• diuretika
• vasodilator
PH : PVR ?
ASD CLOSURE
FR (Qp/Qs) > 1,5
• SURGERY
•ASO
(BW > 8 kg)
Complications of congenital
heart disease
• Cyanosis – the presence of more than
5g/dL of reduced hemoglobin in arterial
blood
• Congestive heart failure – this occurs
due to the inability of the heart to
maintain sufficient tissue perfusion as a
result of the cardiac lesion
45
Complications of congenital
heart disease
• Pulmonary hypertension-this occurs as
a result of an abnormal increase in
pulmonary blood flow due to left-to-right
shunt (e.g ASD, VSD, PDA)
• Infective endocarditis – congenital heart
disease may result in lesions prone to
bacterial colonization
46
Pulmonary Hypertension
47
Infective Endocarditis
48
Complications of congenital
heart disease
• Heart failure
• Sudden death – this may be due to
arrhythmias (more common in these
disorders) or outflow obstruction as
seen in aortic stenosis
49
Eisenmenger’s syndrome
50
Eisenmenger’s syndrome
• Refers to the situation in which a
congenital cardiac abnormality initially
causes acyanotic heart disease, but
cyanotic heart disease develops as a
consequence of raised pulmonary
pressure and shunt reversal
51
Eisenmenger’s syndrome
• These clinical features are also seen in
patients who have cyanotic congenital
heart disease
• Cyanosis develops when the level of
reduced hemoglobin is over 5 g/dL.
52
Complications of
Eisenmenger’s syndrome
• Clubbing fingers and toes
• Polycythemia and hyperviscosity- with
resulting complications of stroke and venous
thrombosis. Regular phlebotomy is the
treatment of choice
• Cerebral abscesses-especially in children
• Paradoxical emboli- emboli from venous
thrombosis may pass across the shunt and
give rise to systemic infarcts
53
Clubbing fingers and toes
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