1. MODERN DAY APPROACH TO AORTIC COARCTATION SUSAN VOSLOO CHRISTIAAN BARNARD MEMORIAL HOSPITAL CAPE TOWN

2. HISTORY 1760 Morgagni 1760 Morgagni Congenital narrowing of aorta adjacent to attachment of ductus Congenital narrowing of aorta adjacent to attachment of ductus Uncommon between LCA & LSA, or in lower thoracic or abdominal aorta Uncommon between LCA & LSA, or in lower thoracic or abdominal aorta AORTIC COARCTATION

3. MORPHOLOGY

4. COARCTATION SEGMENT AORTIC COARCTATION

5. FETAL CIRCULATION AORTIC COARCTATION

6. CO-EXISTING LEFT HEART ANOMALIES (up to 50%) Supravalvar mitral ring Mitral stenosis with or without a single papillary muscle (parachute mitral valve) Endomyocardial fibrosis Left ventricular hypoplasia or hypertrophy Aortic atresia and hypoplasia of ascending aorta Supra-valvar, valvar, sub-valvar aortic stenosis or hypoplasia AORTIC COARCTATION

7. MAJOR COLLATERAL CHANNELS AORTIC COARCTATION

8. AGES AT PRESENTATION AORTIC COARCTATION 1ST OPERATION (92)RECOARCTATION (8) 3 2 3 40 (43.5%) 31 (33.7%) 19 (20.6%) 2 (2.2%)

9. AGES AT CLINICAL PRESENTATION NEONATAL PERIOD (40) first month of life (12 pre-op vent, inotropes incl 5 isolated coarct, 7 co-existing lesions) NEONATAL PERIOD (40) first month of life (12 pre-op vent, inotropes incl 5 isolated coarct, 7 co-existing lesions) INFANCY (34) from 1 month - 1 year INFANCY (34) from 1 month - 1 year CHILDHOOD (21) age 1 – 14 years CHILDHOOD (21) age 1 – 14 years ADOLESCENTS AND ADULTS (5) beyond 14 years ADOLESCENTS AND ADULTS (5) beyond 14 years AORTIC COARCTATION

10. SPECIAL INVESTIGATIONS ECHOCARDIOGRAPHY ECHOCARDIOGRAPHY CARDIAC CATHETERIZATION OR AORTOGRAPHY CARDIAC CATHETERIZATION OR AORTOGRAPHY MRI MRI CT CT AORTIC COARCTATION

11. MR AORTIC COARCTATION AORTIC COARCTATION

12. CT AORTIC COARCTATION AORTIC COARCTATION

13. PRIMARY ANGIOPLASTY vs SURGERY OLDER PATIENTS: Primary angioplasty & stenting > surgery with comparable if not superior risk & recurrence rates HIGH RISK INFANTS: Still better served with surgery AORTIC COARCTATION

14. Do High-Risk Infants Have a Poorer Outcome From Primary Repair of Coarctation? Analysis of 192 Infants Over 20 yrs (JG McGuinness,et al, Our Lady’s Childrens Hospital, Dublin, Ireland, AnnThorac Surg 2010; 90:2023-2027) Primary angioplasty reports ( 8 studies last 10 yrs): 6 studies represented only low risk pts, no initial mortality, re-intervention rate of 14-83% 6 studies represented only low risk pts, no initial mortality, re-intervention rate of 14-83% 2 studies included high risk patients: 2 studies included high risk patients: - mortality 17 & 21% - re-intervention 73% in 10 days, 77% by 12 yrs Both studies reported lost femoral pulses 12-18%, long term sequelae unknown Both studies reported lost femoral pulses 12-18%, long term sequelae unknown AORTIC COARCTATION

15. Do High-Risk Infants Have a Poorer Outcome From Primary Repair of Coarctation? Analysis of 192 Infants Over 20 yrs (JG McGuinness,et al, Our Lady’s Childrens Hospital, Dublin, Ireland, AnnThorac Surg 2010; 90:2023-2027) Higher vs lower risk surgical pts (pre-op PG, ventilation, LV dysfunction, inotropic support) were: -Smaller (3.3 vs 4.2 kg), younger (18 vs 57 days), PAB (25 vs 15%), - same technique, similar X-clamp times -mortality(7 vs 3%), recurrence (11%) -treated easily with single balloon angioplasty,mean 3.8 yrs later AORTIC COARCTATION

16. SURGICAL HISTORY 1944 Crafoord & Nylin 1944 Crafoord & Nylin 1945 Gross 1945 Gross Original technique resection with end-to-end anastomosis (REE) Original technique resection with end-to-end anastomosis (REE) Other techniques followed Other techniques followed Choice of technique mostly based on individual preference Choice of technique mostly based on individual preference AORTIC COARCTATION

17. SURGICAL APPROACH AORTIC COARCTATION LEFT THORACOTOMY

18. SURGICAL TECHNIQUES AORTIC COARCTATION ALL OPERATIONS (n=100) 73 14 10 3

19. SURGICAL TECHNIQUES AORTIC COARCTATION FIRST OPERATION (92) RECOARCTATION (8) 2 3 3 14 71 7 M/s (9) M/s (2)

20. SIMPLE RESECTION & END- END ANASTOMOSIS (SEE) AORTIC COARCTATION

21. MONITORING PRE-REPAIR AORTIC COARCTATION

22. MONITORING POST-REPAIR AORTIC COARCTATION

23. EXTENDED RESECTION & END- END ANASTOMOSIS (Amato 1977) AORTIC COARCTATION

24. GROWTH & ARCH RE- INTERVENTION FACTORS AORTIC COARCTATION Mortality (8/36) and arch re-intervention (5/36) common in neonates weighing < 2.5 kgs Mortality (8/36) and arch re-intervention (5/36) common in neonates weighing < 2.5 kgs SEE (2/3); EEE (3/16); SCF (7/15); patch aortoplasty (1/2) SEE (2/3); EEE (3/16); SCF (7/15); patch aortoplasty (1/2) Catch-up growth of transverse arch and isthmus does occur post coarctation repair, especially in smallest arch parameters, where EEE was favoured Catch-up growth of transverse arch and isthmus does occur post coarctation repair, especially in smallest arch parameters, where EEE was favoured This may be increased using extended rather than simple resection and end-to-end anastomosis This may be increased using extended rather than simple resection and end-to-end anastomosis (T Karamlou et al: Hosp for Sick Children,Toronto; J Thorac Cardiovasc Surg 2009; 137: 1163-7)

25. ALTERNATIVE SURGICAL TECHNIQUES Subclavian flap & reversed subclavian flap Subclavian flap & reversed subclavian flap Patch aortoplasty (indirect aortoplasty) & Direct aortoplasty Patch aortoplasty (indirect aortoplasty) & Direct aortoplasty Interposition or Bypass grafts Interposition or Bypass grafts AORTIC COARCTATION

26. SUBCLAVIAN FLAP Waldhausen & Nahrwold 1966 AORTIC COARCTATION

27. REVERSED SUBCLAVIAN FLAP AORTIC COARCTATION

28. DIRECT ISTHMOPLASTY Vosschulte 1957 AORTIC COARCTATION

29. PATCH AORTOPLASTY Indirect Isthmoplasty AORTIC COARCTATION

30. CAUSES OF ANEURYSM AORTIC COARCTATION Accelerated proximal aortic wall growth due to compliance mismatch Cystic medial necrosis in aortic wall adjacent to coarctation Disruption of intima or sub-intima with or without patch aortoplasty Infection

31. ANEURYSMS POST COARCTATION REPAIR AORTIC COARCTATION Predictors of aneurysm formation after surgical correction of aortic coarctation (Y von Kodolitsch, Hamburg, Germany, J Am Coll Cardiol, 2002; 39:617-624) Reported 25 aneurysms (9% of coarctation repairs),8 ascending, 17 local aneurysms, with 36% mortality if left untreated Independent predictors for aneurysm formation: * Higher age at repair (72% had surgery after age 13.5 yrs) * Patch graft technique * Higher pre-op gradient & bicuspid aortic valve favoured ascending aneurysm formation

32. INTERPOSITION GRAFTS Schusler 1962 Brom 1965 AORTIC COARCTATION

33. BYPASS GRAFTS Weldon 1973 Edeie 1975 AORTIC COARCTATION

34. MID-TERM OUTCOMES OF RESECTION & EEE 201 pts coarctation without/with VSD (14%) 201 pts coarctation without/with VSD (14%) Neonates (53%); pre-op shock(20%) Neonates (53%); pre-op shock(20%) Sternotomy 44 pts (22%); thoracotomy 157 pts (78%) Sternotomy 44 pts (22%); thoracotomy 157 pts (78%) Early mortality 2% (PHT&CDH, MAS, MOF, RSV) Early mortality 2% (PHT&CDH, MAS, MOF, RSV) Re-intervention 8 pts (3 balloon angioplasty; 5 re- ops; 75% in 1 st po yr) Re-intervention 8 pts (3 balloon angioplasty; 5 re- ops; 75% in 1 st po yr) (S Kaushal; Children’s Memorial Hosp, Chicago; Ann Thor Surg 2009; 88: 1932-8) AORTIC COARCTATION

35. OUTCOME - MORTALITY No deaths 1 year No deaths 1 year 2 early deaths (both hospitalized since birth) 2 early deaths (both hospitalized since birth) 1. F, ex-prem, 6 weeks, 1.8 kg, pre-op vent, Coarctation & AP Window, po pneumonia, ECMO day 5-19, off ECMO, recurrent pneumonia week later, died respiratory failure 2. F, ex-prem, 3 months, 2.1 kg, large hydrocephalus, massive pericardial effusion, Klebsiella septicaemia, died day 7 po No late deaths, including all subsequent surgery for intracardiac repairs post palliation No late deaths, including all subsequent surgery for intracardiac repairs post palliation AORTIC COARCTATION

36. OUTCOME – EARLY MORBIDITY Transient Hypertension common Transient Hypertension common PO Ventilation > 3 days (3 – 2 died) PO Ventilation > 3 days (3 – 2 died) Phrenic Nerve injury(2); Both required diaphragmatic plication Phrenic Nerve injury(2); Both required diaphragmatic plication Chylothorax (2); 1 thoracic duct ligation Chylothorax (2); 1 thoracic duct ligation No postop bleeding, spinal cord complications No postop bleeding, spinal cord complications AORTIC COARCTATION

37. FACTORS DETERMINING SPINAL CORD INJURY RISK The location and length of narrowing The presence of the collateral circulation The clamping time required for the procedure AORTIC COARCTATION

38. OUTCOME – LATE MORBIDITY PPM (2) – LV dysfunction at 1 & 4 yrs PPM (2) – LV dysfunction at 1 & 4 yrs Late Aneurysms – nil Late Aneurysms – nil Hypertension – continuous anti-HT therapy (2) Hypertension – continuous anti-HT therapy (2) Recoarctation ( 8 single balloon angioplasty < 6m; 2 at 4 & 6 yrs po; 1 redo surgery REE – patch at 6m) Recoarctation ( 8 single balloon angioplasty < 6m; 2 at 4 & 6 yrs po; 1 redo surgery REE – patch at 6m) AORTIC COARCTATION

39. CAUSES AORTIC RECOARCTATION AORTIC COARCTATION

40. PATIENTS (n=100) ISOLATED COARCTATION (66) including 12 pts with stable left heart obstructive lesions, being observed ISOLATED COARCTATION (66) including 12 pts with stable left heart obstructive lesions, being observed CO-EXISTING CARDIAC LESIONS (34) CO-EXISTING CARDIAC LESIONS (34) M 58; F 42 M 58; F 42 PRIMARY OPERATION (92) PRIMARY OPERATION (92) RECOARCTATION (8) RECOARCTATION (8) AORTIC COARCTATION

41. CO-EXISTING CARDIAC DEFECTS (n=46/100) Bicuspid Aortic Valve (8) Bicuspid Aortic Valve (8) Stable Shone complex (4) (12) Stable Shone complex (4) (12) Significant LVOTO (5) (34) Significant LVOTO (5) (34) VSD (16) VSD (16) Other (13) Other (13) DORV (4) TGA&VSD (2) UVH (5) AP- window (1) IHD (1) AORTIC COARCTATION

42. COARCTATION PLUS SIGNIFICANT LVOTO (n =5) AORTIC VALVOTOMY (3) AORTIC VALVOTOMY (3) Aortic valvotomy with aortic coarctation (1), Aortic valvotomy at 3 & 5 months post coarct (2) Aortic valvotomy with aortic coarctation (1), Aortic valvotomy at 3 & 5 months post coarct (2) PROGRESSIVE LVOTO POST-COARCT REPAIR PROGRESSIVE LVOTO POST-COARCT REPAIR Ross procedure at 5 yrs (1) Resection Subaortic stenosis at 4 yrs,then Ross- Konno at 10 yrs (1) AORTIC COARCTATION

43. COARCTATION PLUS VSD (n = 16) RECOARCTATION (4) RECOARCTATION (4) Primary VSD & coarctation (2) PAB & coarctation; later VSD closure (2) PRIMARY VSD & COARCTATION (3) PRIMARY VSD & COARCTATION (3) PAB & COARCTATION (9) PAB & COARCTATION (9) CBMH; later VSD closure @ 4-22m age (5) RXH; all awaiting definitive procedures (4) AORTIC COARCTATION

44. COARCTATION WITH OTHER CARDIAC DEFECTS (n=13) Primary repair with coarctation (5) Primary repair with coarctation (5) - APW (1), - IHD (LIMA – LAD) (1); - TGA & VSD primary ASO & VSD (1), - DORV (2) Palliation PAB (8) Palliation PAB (8) -TGA & VSD at 11m (1), -DORV at 11 & 15 m(2) -UVH: Glenn (3/5), TCPC (1/3) - Awaiting repairs(2) AORTIC COARCTATION

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