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SURGERY IN PAEDIATRIC RHEUMATIC CARDITIS. DR VILJEE JONKER DEPT CARDIOTHORACIC SURGERY YUNIVESITHI YA FREISTATA. NATURAL HISTORY. ARF : 5% < 5y Rare >35y 85% ARF major manifestations- Carditis Valvar disease rather than myocarditis M&M
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SURGERY IN PAEDIATRIC RHEUMATIC CARDITIS DR VILJEE JONKER DEPT CARDIOTHORACIC SURGERY YUNIVESITHI YA FREISTATA
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
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
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
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
3 Stages Braunwald, E :Heart Disease; 1706-17
PATHOLOGY:AUTE CARDITIS • Pericarditis • “bread and butter”, fibrous exudate • NO Constriction
Myocarditis • Remarkably normal • Aschoff lesions • Interstitial cellular infiltrate, oedema Antunes MJ: Mitral valve repair; 31-43 Walter, JB: Pathology of human disease; 471-74
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
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
Thickening and fusion of Triangular base of chordaetendineae Antunes MJ: Mitral valve repair; 31-43
CHRONIC PHASE • Depends on original involvement & predominant healing process • Fibrous tissue- commisural fusion/ contraction leaflets • Calcification
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
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
SURGERY REPAIR VS REPLACEMENT
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
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
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
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
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
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
REPALCEMENT • Mitral valve • Bioprosthesis - early degeneration • Homograft - midterm failure • Metallic valve • Aortic valve • Bioprosthesis • Ross • Metallic
AV + MV • REPLACE BOTH Kuwaki, K 2007: Ann Thorac Surg 2007;83:558-63
Conclusion • Patient selection determines success of repair • Surgery for decompensated ARC • Active carditis: ? replacement