Management of Adult Congenital Heart Disease

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

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%

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. Trace PR Severe 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 * *: 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. Sudden Cardiac Death Adults with CHD

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

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