1. SC BROWN PAEDIATRIC CARDIOLOGY UFS

2. Introduction  congenital heart disease - lifelong care ○ frequency 1% -USA: 2.9mil pa -Netherlands 20 000 GUCH – only 8000 seen -SA: 47million = 470 000  challenges: ○ best location ○ appropriate antenatal care ○ securing infrastructure  3 most common problems neglected patient weak infrastructure family planning & management of pregnancy

3. neglected patient  lost to follow up  non education ○ patient ○ families  adult physicians: no idea SOLUTIONS ○ educate patient & families ○ orderly transfer

4. infrastructure  more survivors of CHD  few clinics for GUCH ○ no experience ○ shortage of skilled personnel  no orderly way of training  multidisciplinary approach

5. contraception & pregnancy

6. conditions in which pregnancy risk is WHO 4 * Pulmonary arterial hypertension of any cause * Severe systemic ventricular dysfunction NYHA III–IV or LVEF ≤ 30% * Previous peripartum cardiomyopathy with any residual impairment of left ventricular function * Severe left heart obstruction * Marfan syndrome with aorta dilated > 40 mm LVEF, left ventricular ejection fraction; NYHA, New York Heart Association Heart 2006;92:1520-25

7. GUCH: Facts & figures

8. age distribution (Florida)

10. number of re-operations

11. Problems in GUCH  ventricular function ○ poor imaging  arrhythmias ○ SVT ○ early electrical cardioversion  cyanosis policythaemia – phlebotomy (hct > 65) haemostasis renal function gallstones orthopaedic

12.  pulmonary vascular disease ○ irreversible & progressive disease  pregnancy ○ Eisenmenger > 50% mortality risk (after delivery) ○ cyanotic CHD – 12% successful pregnancy  genetic counselling ○ 2 – 50% risk if mother CHD  comorbidity ○ 15 – 20% chromosomal abnormalities  endocarditis  psychosocial ○ anxiety ○ depression  employment

13. surgical issues  planning  specific challenges preservation myocardial function blood salvage techniques ○ autologous transfusion ○ pre-op phlebotomy in cyanotic patients ○ routinely cell saver system intraoperatively redo sternotomy ○ difficult ○ consider cannulation of femoral vessels, bypass before opening sternum aortopulmonary collaterals ○ complicate peri- 7 post operative management ○ excessive return LA: -obscures visualisation, volume overlaod etc anaesthesia

14. Case example  31y female Tricuspid Atresia Fontan @ 6y  29y: atrial flutter – cv disability pension exercise intolerance & ascites  referred – transplant liver & heart by physician  RAPA Fontan extracardiac conduit + Maze great improvement in quality of life physician initial care did not understand Fontan circulation & complexity patient + family did not appreciate the need for expert care or where to find it

15. reasons for inappropriate care  adults CHD lost to follow-up  patients & families not educated ○ what to expect ○ where to go  adult physicians not educated re complexity of GUCH

16. NEED  network of specialist centers  team of experts ○ nurses – physicians  training  recommendations unit located adult environment with multidisciplinary provision associated with strong paediatric cardiology group each center to serve 5-10million population referral relationships with primary caregivers association with transplant centre medical expertise Cardiologist – paed + adult Electro physiologist MRI, CT Minimum 2 congenital heart surgeons Obstetrics for high risk pregnancies Specialist nurses European Heart J 2003. 24:1035-84

17. Why the sharks win

22. And the BULLS ?