1. Management of Adult Congenital Heart Disease Alpay Çeliker MD. Hacettepe University Department of Pediatric Cardiology

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

4. Major Issues in ACHD Primary Operation or intervention Reoperation or reintervention Heart Failure Arrhythmia Sudden Death

5. CHD`s that do not Require Operation Functionally normal bicuspid aortic valve Mild pulmonary valve stenosis Small interatrial connection Small VSD!!! Uncomplcicated L-transposition

6. 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

7. Conditions with Specific Interest Aortic coarctation Left-to-right shunts Repaired tetralogy of Fallot Atrial switch procedures Fontan circulation

8. 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%

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

10. 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

11. 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

12. 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

13. Detechable Coil Amplatzer Plug

14. 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

15. 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

16. 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

17. 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

18. 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

19. 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

20. MRI Right ventricle size Right ventricle ejection fraction

21. MRI II Degree of Pulmonary regurgitation Determining fibrotic and aneursymatic areas Time consuming

22. Severe PR Trace PR

23. 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

24. Hacettepe Experience: EPS in Fallot Patients ResultPatient No% NORMAL1240 SSS13.3 AVCD310 SSS+AVCD310 NS AFL26.7 SSS+AFL13.3 S AFL26.7 Fibro-flutter13.3 SSS+NSVT26.7 NSVT310 TOTAL30100 * *: 30 patients after 11 years tetralogy repair

25. 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

27. 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

28. 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

29. Stent implantation in LPA stenosis in Fontan

30. 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

31. Coronary sinus angio Coronary sinus lead in place

33. 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

34. Heart Failure in ACHD Chronic Treatment –ACE inhibitors –Diuretics –  -Blockers –Aldosterone antagonism –Digitalis Acute Treatment –Dopamine, dobutamine –Milrinone Biventricular pacing

35. Adults with CHD Sudden Cardiac Death

36. Sudden Death Surgically repaired Tetralogy of Fallot Atrial switch operation D- Transposition Aortic stenosis Coarctation of aorta