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SURGERY IN PAEDIATRIC RHEUMATIC CARDITIS

Learn about the natural history, pathogenesis, and pathology of pediatric rheumatic carditis. Discover the surgical repair options, including mitral valve repair, and the chronic phase management. Understand the pathophysiology of the mitral and aortic valves, surgical repair techniques, and factors affecting post-repair outcomes.

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SURGERY IN PAEDIATRIC RHEUMATIC CARDITIS

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  1. SURGERY IN PAEDIATRIC RHEUMATIC CARDITIS DR VILJEE JONKER DEPT CARDIOTHORACIC SURGERY YUNIVESITHI YA FREISTATA

  2. NATURAL HISTORY • ARF : 5% < 5y Rare >35y • 85% ARF major manifestations- Carditis • Valvar disease rather than myocarditis M&M • Thomas: if no heart disease in hosp/ no recurrence – no cardiac involvement @ 15y • If no recurrence up to 68-76% MR may disappear; less likely if cardiomegaly • MS only after 3/ more attacks Carapetis, JR: Lancet 2005; 366: 155-68 Antunes MJ: Mitral valve repair; 31-43Thomas, GT: Br Med J I:1961; 1635

  3. PATHOGENESIS • Post Lancefield Group A B -hemolytic Streptococcus pharyngitis • Auto-immune response • Factors dictate attack rate of RF • Quantitative factors • Variations in Group A Strep infections • Geography • Host factors Braunwald, E :Heart Disease; 1706-17 Walter, JB: Pathology of human disease; 471-74

  4. Antibodies • Streptolysin O • Streptokinase • DNA’se • Hyaluronidase • AB’s initial endothelium damage activates lymphocyte adhesion molecules • AB’s cross react • Cardiac myocin in myocardium • Laminin on valve surface (laminin=Strep M Protein) • Laminin & other cross reactive protein trap AB on valve surface

  5. Cunningham, IntCongrss Series 2006;1289:14-19

  6. VCAM upregulated on valve surface promotes lymphocyte adhesion-respond to Strep M Protein • Repetitive Strep infections through neovascularised scar • Repeat infection necessary to prime immune response Cunningham, Int Congrss Series 2006; 1289: 14-19

  7. Cunningham, IntCongrss Series 2006;1289:14-19

  8. 3 Stages Braunwald, E :Heart Disease; 1706-17

  9. Carapetis, Lancet 2005; 366:155-68

  10. PATHOLOGY:AUTE CARDITIS • Pericarditis • “bread and butter”, fibrous exudate • NO Constriction

  11. Myocarditis • Remarkably normal • Aschoff lesions • Interstitial cellular infiltrate, oedema Antunes MJ: Mitral valve repair; 31-43 Walter, JB: Pathology of human disease; 471-74

  12. Endocarditis • Verrucous lesions -MV: Atrial aspect -AV: Ventricular aspect • DO NOT EMBOLISE Antunes MJ: Mitral valve repair; 31-43 Walter, JB: Pathology of human disease; 471-74

  13. Annular dilation- Ant valve prolapse- chordalenlongation • MacCullum’s Patch: MR jet leads to posterior leaflet LA thrombus deposition Antunes MJ: Mitral valve repair; 31-43

  14. Thickening and fusion of Triangular base of chordaetendineae Antunes MJ: Mitral valve repair; 31-43

  15. CHRONIC PHASE • Depends on original involvement & predominant healing process • Fibrous tissue- commisural fusion/ contraction leaflets • Calcification

  16. PATHOPHYSIOLOGYMITRAL VALVE • Carpentier: • Normal leaflet Motion – 88% • Excessive leaflet motion- 73% ( co-exist in 78%) • Restricted leaflet motion • MR • Annular Dilation • Enlongation/ rupture of chordae • Restricted movement post leaflet • Secondary Ventricular dilation (MR begets MR) • MS • Rare 2-10 y – recurrent attacks • Commisural fusion • Both leaflets, chordae thickened

  17. AORTIC VALVE • 25-30 % Children with severe RHD • AR • Annular dilation • Leaflet retraction • AS • Commisural fusion Hillman, ND: Ann Thorac Surg 2004; 78:1403-8

  18. SURGERY REPAIR VS REPLACEMENT

  19. Repair • Safe • Allows annulus growth (annuloplasty dependent) • Preserves chordal/ ventricular function • No anticoagulation (Pt compliance) • Replacement • Worse haemodinamics • Thrombo-embolism • Anticoagulation • Growth of annulus impaired • Rapid degeneration bioprosthesis • Unsuitability of Pulmonary autograft Kumar, S: Ann Thorac Surg 2005;79:1921-5 Essop, MR: Circulation 2005;112:3584-91

  20. SURGERY: MV Repair • ANNULAR DILATION • Annuloplasty Ring > 28-30mm • Partial/ posterior annuloplasty • Teflon felt annuloplasty • Kolangos • COMMISSURAL FUSION • Commissurotomy • VALVE • Cusp thinning & leaflet enlargement • CHORDAE • Cusp-level shortening & transfer Kumar, S: Ann Thorac Surg 2005;79:1921-5 Hillman, ND: Ann Thorac Surg 2004; 78:1403-8

  21. Cuspal thinning

  22. Annuloplasty Ring

  23. Chordal shortening

  24. Chordal Transfer

  25. Posterior Leaflet extension

  26. Commisuroplasty

  27. Posterior Commisuroplasty

  28. Video

  29. MITRAL VALVE REPAIR Kumar-2005 • 278 Pt Age 2 – 15y • Reoperation 6% @ 56m FU • Mortality 4.8% Carpentier-2001 • 951 Pt Subgroup < 19y • Reoperation 19% @ 10y FU • Total Mortality 6.2% (early 2%) Kumar, S: J Cariovasc Surg 2005;129:875-9 Carpentier, A: Circulation 2001; 104(1): 1-15 Kumar, S: Ann Thorac Surg 1995;60:1044-7

  30. Grinda-2002 • 21 Pt Mean age 11+-4y • Reoperation 10% 5y FU • Mortality 4.7% Hillman- 2004 • 26 Pt Age: <21y • Reoperation 23% @ 5.3 +-3.3y • Mortality (late 7.7%) Hillman, ND: Ann Thorac Surg 2004;78:1403-8 Grinda, J: Eur J Cardiovasc Surg 2002;21:447-52

  31. Cause of failure • Judgment error • Inherent complexity of disease • Recurrence/ progression of disease • Treatment post repair • Regular FU • 3 weekly IMI Bensatine Pen till 40y Hillman, ND: Ann Thorac Surg 2004;78:1403-8 Carpentier, A: Circulation 2001; 104(1): 1-15 Kumar, S: Ann Thorac Surg 1995;60:1044-7

  32. AORTIC VALVE REPAIR • Criteria for possible repair • Minimal/ no calcifications • Mobility • >2-3mm of central coaptation • TECHNIQUES • Subcommissural annuloplasty • Cusp thinning • Commissural plication- Trusler • Leaflet extension Hillman, ND: Ann Thorac Surg 2004; 78:1403-8 Kumar, S: Ann Thorac Surg 2005;79:1921-5

  33. Commissuroplasty

  34. Cuspal thinning

  35. Leaflet extension

  36. REPALCEMENT • Mitral valve • Bioprosthesis - early degeneration • Homograft - midterm failure • Metallic valve • Aortic valve • Bioprosthesis • Ross • Metallic

  37. AV + MV • REPLACE BOTH Kuwaki, K 2007: Ann Thorac Surg 2007;83:558-63

  38. Conclusion • Patient selection determines success of repair • Surgery for decompensated ARC • Active carditis: ? replacement

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