Survival Patterns of Adults with Congenital Heart Disease

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Management of Adult
Congenital Heart Disease
Alpay Çeliker MD.
Hacettepe University
Department of Pediatric Cardiology
Congenital Heart Defects in Newborn
8%
Cardiac Operation
60 %
Possibility to reach adulthood
85%
Major Issues in ACHD
Primary Operation or intervention
 Reoperation or reintervention
 Heart Failure
 Arrhythmia
 Sudden Death

CHD`s that do not Require
Operation
Functionally normal bicuspid aortic
valve
 Mild pulmonary valve stenosis
 Small interatrial connection
 Small VSD!!!
 Uncomplcicated L-transposition

Types of Surgery for
Congenital Heart Disease





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
Conditions with Specific
Interest
Aortic coarctation
 Left-to-right shunts
 Repaired tetralogy of Fallot
 Atrial switch procedures
 Fontan circulation

Coarctation of Aorta

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
• >40 yrs
25 yr survival 75%
15 yr survival 50%
Isolated Aortic Coarctation
Surgery
Balloon Dilation
Dangerous!!!
Stent Implantation
Balloon Expandable Stents
Covered Stents
Neointima Formation
I. Method in Recoarc
Left-to-Right Shunt
Lesions





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
ASD Closure


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
PDA Closure





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
Detechable Coil
Amplatzer Plug
Cardiac Surgery&Frequent
Complications in some CHD’s

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
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
Total Correction and
Arrhythmias

Ventricular arrhythmias
– Late operation\Long follow-up duration
– Residual VSD
– Severe Pulmonary regurgitation
Atrial arrhythmias
 Sinus node and AV conduction
disorders

Risk Assessment
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

ECHO



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
ECG and Holter
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

MRI


Right ventricle
size
Right ventricle
ejection fraction
MRI II
Degree of
Pulmonary
regurgitation
 Determining
fibrotic and
aneursymatic
areas
 Time
consuming

Trace PR
Severe PR
Cardiac EPS in Fallot Patients

Common AV conduction disturbance

Common atrial flutter

Infrequent inducible SMVT

Ablation in tolerated VT’s

ICD in fast VT or cardiac arrest
Hacettepe Experience: EPS in Fallot
Patients *
Result
Patient No
%
NORMAL
12
40
SSS
1
3.3
AVCD
3
10
SSS+AVCD
3
10
NS AFL
2
6.7
SSS+AFL
1
3.3
S AFL
2
6.7
Fibro-flutter
1
3.3
SSS+NSVT
2
6.7
NSVT
3
10
TOTAL
30
100
*: 30 patients after 11 years tetralogy repair
Reoperation in Tetralogy





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
Mustard & Senning Procedures

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
Fontan Circulation
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
Fontan & Arrhythmia

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

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
Heart Failure in ACHD

Chronic Treatment
–
–
–
–
–

ACE inhibitors
Diuretics
-Blockers
Aldosterone antagonism
Digitalis
Acute Treatment
– Dopamine, dobutamine
– Milrinone

Biventricular pacing
Sudden Cardiac Death
Adults with CHD
Sudden Death
Surgically repaired Tetralogy of
Fallot
 Atrial switch operation DTransposition
 Aortic stenosis
 Coarctation of aorta

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