Hacettepe Üniversitesi Tıp Fakültesi · HACETTEPE ONivER5irESi . Created Date: 10/8/2019 3:19:28 PM
Management of Adult Congenital Heart Disease Alpay Çeliker MD. Hacettepe University Department of...
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Transcript of Management of Adult Congenital Heart Disease Alpay Çeliker MD. Hacettepe University Department of...
Management of Adult Congenital Heart
Disease
Alpay Çeliker MD.Hacettepe University
Department of Pediatric Cardiology
Congenital Heart Defects in Newborn8%
Cardiac Operation60 %
Possibility to reach adulthood85%
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 25 yr survival 75%•>40 yrs 15 yr survival 50%
Isolated Aortic Coarctation
Surgery Balloon Dilation
Stent Implantation
Dangerous!!!
Balloon Expandable StentsCovered Stents
Neointima FormationI. 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
Severe PR
Trace 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
Adults with CHD
Sudden Cardiac Death
Sudden Death
Surgically repaired Tetralogy of Fallot
Atrial switch operation D-Transposition
Aortic stenosis Coarctation of aorta