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  1. 1. Pediatric Cardiology Department & Adult with Congenital Heart Disease IRCCS Policlinico San Donato Dr Massimo Chessa INTRODUCTION OF THE PROBLEM ADULT WITH CONGENITAL HEART DISEASE
  2. 2. Adults with Congenital Heart Disease P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa More than 75.000 ACHD in Italy 1200-1600 adolescents enter adulthood every year and require life-long care More than 85% of infants are expected to reach adulthood
  3. 3. Workload is Increasing 400% in Canada P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa ACHD Pts numbers Pediatric Pts numbers 1980 2000 2020 G. Webb
  4. 4. An increasing problem P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa Advances Diagnosis Therapy Survival
  5. 5. AN INCREASING PROBLEM P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa The first cause of such increasing number of patients is SURGERY
  6. 6. Extraordinary Management Advances Improved Survival Rate P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa Warnes CA, et al. 32th Bethesda Conference, JACC 2001; 37:1170-5 95% 90% 80% 1980-1989 95% 65% 50% 1960-1979 90% 55% 10% 1940-1959 Simple CHD Moderate CHD Complex CHD Year of birth
  7. 7. First of all improved Surgical Mortality P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa Year 1960 1970 1980 1990 2000 Adults 30% 20% 10% 5% 4% Children 90% 70% 40% 10% 5%
  8. 8. AN INCREASING PROBLEM P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa It is important to outline that most pts with CHD who have had their lives transformed by surgical intervention, had reparative and not corrective surgery
  9. 9. P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa Surgery is corrective, if…. … ..ventricular function is normal … ..life expetancy is normal! … ..there is no need for tharapeutic measures during f-up
  10. 10. Corrective Surgery……. P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa Atrial Septal defect Ventricular Septal Defect Patent Ductus Arteriosus … ..if treated during childhood!!!
  11. 11. P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa Residuae Sequelae Mechanical Electrophysiological Functional
  12. 12. An increasing problem P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  13. 13. P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  14. 14. P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa Why an ACHD is hospitalized Chessa M, et al. J Cardiovasc Med (Hagerstown). 2006 Sep;7(9):701-5. 4.3 52 Other 0.3 3 Extra cardiac surgery 6.3 75 General evaluation 1.7 20 Cardiac Failure 7.0 86 Arrhythmias 25.2 307 Surgery 55.2 670 Interventional procedures % n° of pts
  15. 15. P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa List of diseases treated by Cath or Surgery Chessa M, et al. J Cardiovasc Med (Hagerstown). 2006 Sep;7(9):701-5. 31 (59.6) 21 (40.4) 51 Others 13 (100) 13 PAPVD 13 (100) 13 PAVC 19 (100) 19 MV disease 37 (100) 37 PDA closure 55 (56.7) 42 (43.2) 97 RVOT+PAs stenosis 78 (64) 44 (36) 122 LVOT+Aorta 14 (32.6) 29 (67.4) 43 VSD 188 (100) 188 PFO 84 (21.4) 309 (78.6) 393 ASD n°of pts treated by surgery (%) n°of pts treated by cath (%) n°
  16. 16. P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa Reasons of death 197 pts Mean age at death 37.3 14.6 (18-80) Oechslin E, et al. Am J Cardiol 2000;86:1111-6
  17. 17. P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa Congenital Heart Disease Is a continuum From Fetal Life Until Adulthood
  18. 18. P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa Congenital Heart Disease Is a continuum From Fetal Life Until Adulthood CHILDHOOD TRANSITION ADULTHOOD 0 12 16 Pediatricians Pediatricians GUCH Cardiologist Nursing GUCH Cardiologist Community Cardiologist Primary Caregivers
  19. 19. P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa It is mandatory a correct organization to follow this kind of patients
  20. 20. P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa National Centre for 5 to 10 milion people Regional Centre with some expertise and facilities Primary Caregivers Community Cardiologist
  21. 21. A National Adult CHD Centre must include Physicians with specific training Dedicated surgeons Cath Lab with dedicated interventionists EF lab with dedicated specialists Facilities for Cardio MR and CT scan Specific appropriate links for provision of non cardiac surgery, integrated team of high-risk obstetricians, anesthetists and adult CHD cardiologists
  22. 22. Types of congenital heart defects of great complexity: these patients should be seen regularly at the adult congenital heart disease centres. Conduits, valved or nonvalved Cyanotic congenital heart (all forms) Double outlet ventricles Fontan procedures Mitral atresia Single ventricle Pulmonary atresia Pulmonary vascular obstructive diseases Transposition of the great arteries Tricuspid atresia Truncus arteriosus Eisenmenger syndrome
  23. 23. Types of congenital heart defects of moderate severity: these patients should be seen periodically at general hospitals with some expertise (Regional GUCH centres) Aorto-left ventricular fistulae Anomalous pulmonary venous drainage (partial or total) Atrioventricular canal defects (partial or complete) Coarctation of the aorta Ebstein’s anomaly Infundibular right ventricular outflow obstruction of significance Ostium primum atrial septal defect Patent dusctus arteriosus Pulmonary valve regurgitation (moderate to severe) Sinus of Valsalva fistula/aneurysm   Sinus venosus atrial septal defect Supra valvar or subvalvar aortic stenosis Tetralogy of Fallot Ventricular septal defect with: Absent valve or valves Aortic regurgitation Coarctation of the aorta Mitral disease Right ventricular outflow tract obstruction Straddling tricuspid/mitral valve Subaortic stenosis
  24. 24. Types of simple congenital heart defects: these patients can usually be cared for in the general medical community. Isolated congenital aortic valve disease Isolated congenital mitral valve disease Isolated patent foramen ovale or small atrial septal defect Isolated small ventricular septal defect Mild pulmonary stenosis Previously legated or occluded ductus arteriosus Repaired atrial septal defect without residua Repaired ventricular septal defect (modified from Conelly MS, et al. Canadian Consensus Conference on Adult Congenital Heart Disease, 1996. Can J Cardiol 1998; 14:395-452).
  25. 25. <ul><ul><ul><li>Plan what to do </li></ul></ul></ul>P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  26. 26. … . they born with a CHD but they become Adult with CHD!!! P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  27. 27. Infundibular stenosis VSD Overiding aorta RV hypertrophy P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  28. 28. Palliation BTS BTM Waterston Potts shunt Procedura di Brock P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  29. 29. Palliation <ul><li>PA distorsion </li></ul><ul><li>PH </li></ul><ul><li>Cyanosis </li></ul><ul><li>LV overload </li></ul><ul><li>RVP overload </li></ul>Biventricular disfunction with risk of sudden death P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  30. 30. Complete reparation Different approaches during the years Right ventriculotomy and transanular patch Combined approach Transatrial-transpulmonary; sometime it is necessary a small ventriculotomy with a transanular patch P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  31. 31. Survival rate: 86% at 32 yrs 85% at 36 yrs Murphy JG et al. N Engl J Med 1993;329:593-9 P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  32. 32. Residual problems <ul><li>Residual VSD </li></ul><ul><li>Residual Infundibular Stenosis </li></ul><ul><li>PA branches stenosis </li></ul><ul><li>Severe PR </li></ul><ul><li>Ao dilatation </li></ul><ul><li>Arrhythmias and SD </li></ul>P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  33. 33. What to do? This is the most difficult and also the most important step planning a correct follow-up strategy. Mandatory: a) share follow-up protocols b) have a shared database P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  34. 34. What to do Tetralogy of Fallot (FU annual* ) All patients require endocarditis prophylaxis 22qD: FISH test (bloods to be drawn at Paul Wood Ward by GUCH SHO) if patient or spouse contemplate pregnancy Di George syndrome more common when right aortic arch or pulmonary atresia present P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  35. 35. What to do Tetralogy of Fallot (FU annual* ) Each Visit Sustained palpitations? Presyncope? Exertional dyspnoea? Exam: To and fro murmur in the pulmonary area? Tricuspid regurgitation? Diastolic murmur of aortic regurgitation? Right heart failure? BP and which arm If unrepaired tetralogy, check resting O2 saturations (after 5 mins of rest) ECG: Rhythm, QRS duration (if maximum > 180 ms, or QRS increase from recent ECG’s is more than 3.5 ms/year discuss with consultant) Holter: If clinically indicated, not routinely P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  36. 36. What to do Tetralogy of Fallot (FU annual* ) 2-3 yearly CXR: Cardiothoracic ratio, right of left aortic arch, dilated ascending aorta? dilated main or central pulmonary arteries? Echo: RV size and function Long-axis function Degree of pulmonary and tricuspid regurgitation Gradient across right ventricular outflow tract Estimated systolic RV pressure (from tricuspid regurgitation) Residual VSD LV size and function RA/LA size and function
  37. 37. What to do Tetralogy of Fallot (FU annual* ) “ Baseline” MRI Assessment Exercise test with maximum O2 uptake and anaerobic threshold Signal Average ECG Tests to be arranged following consent (on a Friday day-case basis) Please inform Drs ……………………. (or copy your letter to them in their absence) P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  38. 38. What to do <ul><li>Tetralogy of Fallot (FU annual* ) </li></ul><ul><li>Discuss with consultant if: </li></ul><ul><li>New symptoms (exertional dyspnoea, palpitations, presyncope) develop progressive RV dilation, early RV dysfunction is present </li></ul><ul><li>New onset tricuspid regurgitation is documented </li></ul><ul><li>and if QRS > 180 ms, or QRS increase > than 3.5 ms/year (from recent ECGs) </li></ul>P ediatric C ardiology D epartment & Adult with Congenital Heart Disease Dr Massimo Chessa
  39. 39. Thank you for your attention Massimo Chessa Paracas-Perù

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