Congenital Heart Defects in Newborn
8%
Cardiac Operation
60 %
Possibility to reach adulthood
85%

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Primary Operation or intervention
Reoperation or reintervention
Heart Failure
Arrhythmia
Sudden Death

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Functionally normal bicuspid aortic valve
Mild pulmonary valve stenosis
Small interatrial connection
Small VSD!!!
Uncomplcicated L-transposition

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Curative : No postoperative residua, sequelae, or complications
Reparative : Anatomic repair or reconstruction with obligatory postoperative residua or sequelae
Palliative : Basic morphologic anomaly is neither repaired or reconstructed
Reoperative : Late reoperation after reparative or palliative surgery
Organ transplantation

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Aortic coarctation
Left-to-right shunts
Repaired tetralogy of Fallot
Atrial switch procedures
Fontan circulation

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Major Concerns :
– Residual hypertension, aneursym formation, recoarctation
Survival&Hypertension
– Hypertension
• Operation between 20-40 yrs may result
80% residual hypertension.
– Operation age
• 20-40 yrs 25 yr survival 75%
• >40 yrs 15 yr survival 50%

Surgery
Stent Implantation
Balloon Expandable Stents
Covered Stents
Neointima Formation
I. Method in Recoarc
Isolated Aortic Coarctation
Balloon Dilation
Dangerous!!!

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Major problem is pulmonary vascular disease
Unrestricted VSD`s rarely reach adult age without PAH
PDA and ASD can be successfully managed by transcatheter methods
Small VSD should be followed clinically, unless AVP and Aortic regurgitation
May result with Eisenmenger syndrome

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ASD II can be closed by interventional methods.
Two major problem may contribute
– Pulmonary vascular disease
– Decreased left ventricle compliance
– Balloon occlusion test should be performed

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Small PDA Endarteritis
Moderate size PDA Left ventricle and atrial dilation
Large PDA Pulmonary vascular disease
Transcatheter closure avoids from general anasthesia, thoracotomy
Large PDA’s can be closed surgically

Amplatzer Plug
Detechable Coil

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Total correction for tetralogy of Fallot
– Atrial and ventricular arrhythmias
– Pulmonary regurgitation
Atrial switch procedures for D-TGA
– Atrial arrhythmias, Sick sinus syndrome
– Right ventricle failure
– Baffle obstruction
Fontan circulation
– Atrial arrhythmias, sick sinus syndrome
– Protein losing enteroptahy
– Conduit obstruction

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Late Complications after Tetralogy
Repair
Endocarditis
Aortic Regurgitation
LV Dysfunction
Residual RVOT Obstruction
Residual Pulmonary regurgitation
RV Dysfunction
Exercise Intolerance
Heart Block
Atrial Fl and Fib
Sustained Ventricular
Tachycardia
Sudden Cardiac Death

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Ventricular arrhythmias
– Late operation\Long follow-up duration
– Residual VSD
– Severe Pulmonary regurgitation
Atrial arrhythmias
Sinus node and AV conduction disorders

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ECHO
– Residual VSD, PS
– Degree of Pulmonary& Tricuspid Regurgitation
– Right ventricle status
ECG
– Prolonged QRS duration
– Abnormal late potentials
Holter
– Ventricular ectopy, NSMVT or SMVT
Exercise
– Increased ectopy, VT
Invasive EPS
MRI

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It is helpful in determining left ventricle function, residual VSD and residual PS
There is no concensus determining Pulmonary regurgitation with
ECHO
Right ventricle ejection fraction can not be measured

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Positive late potentials and wide QRS
(>180 msec) is well-known risc factors associated with ventricular tacyhcardia
Ventricular ectopic beats and nonsustained monomorphic VT are other factors related with SMVT

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Right ventricle size
Right ventricle ejection fraction

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Degree of
Pulmonary regurgitation
Determining fibrotic and aneursymatic areas
Time consuming

Severe PR
Trace PR

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Common AV conduction disturbance
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Common atrial flutter
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Infrequent inducible SMVT
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Ablation in tolerated VT’s
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ICD in fast VT or cardiac arrest

Hacettepe Experience: EPS in Fallot
Patients
*
Result Patient No %
NORMAL
SSS
AVCD
SSS+AVCD
NS AFL
SSS+AFL
S AFL
Fibro-flutter
SSS+NSVT
NSVT
TOTAL
2
1
2
1
2
3
30
3
3
12
1
6.7
3.3
6.7
3.3
40
3.3
10
10
6.7
10
100
*: 30 patients after 11 years tetralogy repair

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Residual VSD with a QP/QS>1.5
Residual PS with RV/LV>2/3
RVOT aneursyms
Branch PS & Pulmonary regurgitation
Severe pulmonary regurgitation with;
– Right ventricle enlargement
– New onset tricuspid regurgitation
– Ventricular tachycardia
– Deteriorating exercise intolerance
Significant aortic regurgitation

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Right ventricle dysfunction
– ACE inhibitors, digitalis, diuretics
Atrial flutter
– AA treatment, catheter ablation, antitachycardia pacemaker
Sick sinus syndrome
– Brady pacing
Baffle obstruction
– Surgery or intervention

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Arrhythmia: 41 % sustained IART and many of them SSS findings
Protein Losing Enteropathy (PLE)
Ventricular Dysfunction
Thromboembolism
Conduit obstruction
Pulmonary artery stenosis
Pulmonary arterivenous fistulae
Plastic bronchitis

Stent implantation in LPA stenosis in Fontan

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SSS or AV Block
– Epicardial pacing
– Pacing from coronary sinus
IART or atrial flutter
– DC cardioversion
– AA drug therapy
– Catheter ablation with
3D mapping
– Arrhythmia surgery

Coronary sinus angio
Coronary sinus lead in place

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PLE
– Diuretics
– Supplemental albumin infusion
– High protein and medium-chain triglyceride intake
– Oral steroids, heparin
– Atrial fenestration
Thromboembolism:
– Anticoagulation and antiplatelet therapy
Heart Failure
– Conversion to Cavopulmonary anastomosis

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Chronic Treatment
– ACE inhibitors
– Diuretics
–  -Blockers
– Aldosterone antagonism
– Digitalis
Acute Treatment
– Dopamine, dobutamine
– Milrinone
Biventricular pacing

Adults with CHD

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Surgically repaired Tetralogy of
Fallot
Atrial switch operation D-
Transposition
Aortic stenosis
Coarctation of aorta