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MODERN DAY APPROACH TO AORTIC COARCTATION

SUSAN VOSLOO CHRISTIAAN BARNARD MEMORIAL HOSPITAL CAPE TOWN. MODERN DAY APPROACH TO AORTIC COARCTATION. HISTORY. 1760 Morgagni Congenital narrowing of aorta adjacent to attachment of ductus Uncommon between LCA & LSA, or in lower thoracic or abdominal aorta. MORPHOLOGY.

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MODERN DAY APPROACH TO AORTIC COARCTATION

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

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

  3. MORPHOLOGY AORTIC COARCTATION

  4. COARCTATION SEGMENT AORTIC COARCTATION

  5. FETAL CIRCULATION AORTIC COARCTATION

  6. CO-EXISTING LEFT HEART ANOMALIES (up to 50%) • Supravalvarmitral 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-valvaraortic stenosis or hypoplasia AORTIC COARCTATION

  7. MAJOR COLLATERAL CHANNELS AORTIC COARCTATION

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

  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) • INFANCY (34) from 1 month - 1 year • CHILDHOOD (21) age 1 – 14 years • ADOLESCENTS AND ADULTS (5) beyond 14 years AORTIC COARCTATION

  10. SPECIAL INVESTIGATIONS • ECHOCARDIOGRAPHY • CARDIAC CATHETERIZATION OR AORTOGRAPHY • MRI • 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% • 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 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 • 1945 Gross • Original technique resection with end-to-end anastomosis (REE) • Other techniques followed • Choice of technique mostly based on individual preference AORTIC COARCTATION

  17. SURGICAL APPROACH LEFT THORACOTOMY AORTIC COARCTATION

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

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

  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 • 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) • 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 (T Karamlouet al: Hosp for Sick Children,Toronto; J ThoracCardiovascSurg 2009; 137: 1163-7) AORTIC COARCTATION

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

  26. SUBCLAVIAN FLAPWaldhausen & Nahrwold 1966 AORTIC COARCTATION

  27. REVERSED SUBCLAVIAN FLAP AORTIC COARCTATION

  28. DIRECT ISTHMOPLASTYVosschulte 1957 AORTIC COARCTATION

  29. PATCH AORTOPLASTYIndirect Isthmoplasty AORTIC COARCTATION

  30. CAUSES OF ANEURYSM • 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 AORTIC COARCTATION

  31. ANEURYSMS POST COARCTATION REPAIR 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 AORTIC COARCTATION

  32. INTERPOSITION GRAFTS Schusler 1962 Brom 1965 AORTIC COARCTATION

  33. BYPASS GRAFTSWeldon 1973 Edeie 1975 AORTIC COARCTATION

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

  35. OUTCOME - MORTALITY • No deaths < 1 month or > 1 year • 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, Klebsiellasepticaemia, died day 7 po • No late deaths, including all subsequent surgery for intracardiac repairs post palliation AORTIC COARCTATION

  36. OUTCOME – EARLY MORBIDITY • Transient Hypertension common • PO Ventilation > 3 days (3 – 2 died) • Phrenic Nerve injury(2); Both required diaphragmatic plication • Chylothorax (2); 1 thoracic duct ligation • 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 • Late Aneurysms – nil • 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) 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 • CO-EXISTING CARDIAC LESIONS (34) • M 58; F 42 • PRIMARY OPERATION (92) • RECOARCTATION (8) AORTIC COARCTATION

  41. CO-EXISTING CARDIAC DEFECTS (n=46/100) • Bicuspid Aortic Valve (8) • Stable Shone complex (4) (12) • Significant LVOTO (5) (34) • VSD (16) • 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 with aortic coarctation (1), Aortic valvotomy at 3 & 5 months post coarct (2) • 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) Primary VSD & coarctation (2) PAB & coarctation; later VSD closure (2) • PRIMARY VSD & COARCTATION (3) • 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) - APW (1), - IHD (LIMA – LAD) (1); - TGA & VSD primary ASO & VSD (1), - DORV (2) • 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

  45. THANK YOU!

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