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Adult Congenital Heart

Disease
diagnosis and management

Supomo
 Increased every years
 At the TCCCA: 1500 cases in 1990 , and 7500
cases in 2002
 In USA estimated growth 5% per years
 In Sardjito Hospital about 5 cases per month
I Without previous surgery

II With previous palliative surgery

III Residual defect or sequelae after complete


repair
1. Prolong over load
volume and
pressure
2. Prolong cyanosis
3. Residual or sequelae
post operative
Problem in:
Pre operative
Intra operative
Post operative care
I. Preoperative Evaluation.
 Clinical assessment
 ECG
 Chest X Ray
 Transthoracic echocardiography
 Transesophageal echocardiography
 Heart Catheterization
 CT scan
 MRI/ MSCT Angiography
 USG doppler
 Dental care
 Informed consent
II. Procedures in Intra 0peratif.

 Redo median sternotomy may be difficult becouse


has been adhesiveness.
 Femoral artery and vein cannulation
 May be need deep hypothermia
 Bleeding may a problem during chest opening
III. Myocardial protection

 Right and left ventricular hypertrophy, dilatation, or


dysfunction may be present
 A left ventricular vent is require
 Deeper hypothermia may be require
 Antegrade or/ and retrograde cardioplegia are
given every 10 minutes
IV. Post operative care

Problems :
- RV and LV functions
- PVR may high
- Arrhythmias may present

TEE post operative


RA, PA pressure monitor lines
ECG

INOTROPIC, Vasodilator,
pacemaker wire
 Types : osteum secundum, sinus venousus, and coronary
sinus

 Spesific problems:
1. volume and sometime pressure overload.
2. May causes exercise limitation
3. atrial fibrilation ( usually over age 30 )
4. Right heart failure ( usually over age 40 )
5. Paradoxical embolism TIA/Stroke
6. Pulmonary hypertension
 A persistent opening in the interatrial septum after birth that allows
direct communication between the left and right atrium.

5% 15%

80%

<1%

<1%

Lilly LS. Pathophysiology of heart disease. 5th ed.


Park MK. Park’s pediatric cardiology for practitioners. 6th ed.
 An adequate diagnostic workup , determines of:

1. Type of ASD
2. The size of defect
3. The functional importance of the defect :
- shunt size ( Qp/Qs)
- RV size, RA size
- Pulmonary artery pressure.
4. Identifies other associated that may influence management
( e.g. anomalous pulmonary venous connection, valve
disease, or coronary artery disease)
 The initial workup :
1. Clinical assessment
2. ECG
3. Chest x-ray
4. TTE
5. TEE
6. Resting oxygen saturation
7. Heart catheterization
8. Coronary angiography
ESC. Guidelines for the management of grown-up congenital heart
 Device closure( ASO) : defect size less than 36 mm
Rim : minimal 4 mm

 Surgical closure :
- defect size up 36-38 mm
- sinus venosus or osteum primum types
 Percutaneous closure is contraindicated if the defect is
very large, has inadequate rims
 Associated with other intracardiac anomalies requiring
surgery
 Stretched ASD size greater than 36 mm and native size
greater than 25 mm are contraindications for device
closure
 Relative contraindications include multiple ASDs,
fenestrated septum primum, and redundant or
aneurysmal septum primum

Kouchoukos NT, Hanley FL, Kirklin JK, Blackstone EH. Cardiac surgery. 4th ed.
 Median sternotomy or right thoracotomy
 Cardiopulmonary bypass with hypothermia ( 30 to
32 0C
 Closure of the defect ( primary or patch closure)
 PA pressure monitor line.
 Insert intrapericard drain.
Post operative care

Problems :
- RV and LV functions
- PVR may high
- Arrhythmias may present

TEE post operative


RA, PA pressure monitor lines
ECG

INOTROPIC, Vasodilator,
pacemaker wire
 Device closure :
- early and intermediate follow up is excellent
- long-term outcome unknow
- arrhythmias and thromboembolic complication ?

 Surgical closure :
- For ASD without pulmonal hypertention , surgical mortality
very low ( < 1%).
- Arrhythmias may be may persist
- LV failure may be occur ( associated : CAD, hypertension,
mitral valve incompetence)
- may appear cardiac tamponade for the first several week after
surgery
 May occur in up 1/3 of patient
 Give anticoagulate for first 6 month post-operative
 Anticoagulation may be stoped if they remain
arrhythmia free or no other risk factors
 If atrial fibrilation occors anticoagulants and
antyarrhythmias therapy are indicated
 Periodic follow up about :
- Pulmonary artery pressure
- Atrial arrhythmias
- ventricular dysfunction
- Pericard effusion for several month.
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