PVR Following Repair of TOF Now? When? Candice Silversides, MD Toronto Congenital Cardiac Centre for Adults University of Toronto Toronto, Canada
Late Complications after TOF repair Repair will be necessary in 15% of patients following repairative surgery over a 20 year follow-up Murphy JC et al NEJM 1994
Case Should this patient be offered a PVR? Symptomatic Severe PR RVEDV 205 cc/m2 RVEF 15 % What is the risk of the procedure? What is the anticipated benefit?
Case Pre PVR Symptoms Severe PR RVEDV 205 cc/m2 RVEF 15% Post PVR No change No PR RVEDV 220 cc/m2 RVEF 18%
Timing of PVR Outline Review of the late complications after TOF repair Benefits of PVR Risks of PVR Indications for PVR
Geva T. Surg Pediatr Card Surg Ann 2006
Late Complications after Endpoints - Death, VT or Decline to NYHA III or IV TOF repair Knauth et al Heart 2008
Late Complications after TOF repair Multivariate Analysis LVEF < 55% RVEDV Z > 7 (172 ml/m 2 / 185 ml/m 2 ) Knauth et al Heart 2008
Late Complications after TOF Repair Ventricular Tachycardia Sudden death Atrial flutter/fibrillation Arrhythmia free RVSP TR PR Gatzoulis et al Lancet 2000
Late Complications after TOF Repair Other PV lesions pulmonary stenosis RVOT aneurysms Residual shunts VSD, ASD Aortic root dilation Aortic regurgitation
Risks and Benefits of PVR NYHA Improvement No improvement in Exercise Duration PVR in Tetralogy of Fallot Functional Capacity 25 24% 20 15 P < 0.001 10 NYHA > II 5 0 Pre PVR 0% Post PVR Therrien et al JACC 2000
Risks and Benefits of PVR Frigiola et al Circulation 2008
Risks and Benefits of RVEDV PVR RVEF 227 ml 215 ml 35% 34% 25 symptomatic adults, 34 yo, RNA Therrien et al JACC 2000
GLOBAL RVEDV (cc/m 2 ) Risks and Benefits of PVR 250 230 210 190 170 150 130 110 90 70 50 Pre PVR 17 adults, 34 yo, MRI Post PVR Therrien et al Am J Cardiol 2005
Risks and Benefits of PVR 71 pts, 29 yo, MRI Oosterhof et al Circ 2007
Risks and Benefits of PVR Frigiola et al Circulation 2008
Risks and Benefits of PVR PVR Controls Harrild et al. Circulation 2009
Risks and Benefits of PVR 1995-2006 118 adults with TOF and PVR Early postoperative mortality - 2% Dos et al JTCVS 2009
PVR - Additional Surgical Procedures Dos et al JTCVS 2009
Risks and Benefits of PVR Preoperative Variable Age at operation > 45 yrs Number of prior sternotomies Prolonged PLOS 50% 14% No Prolonged PLOS 1.8 +/- 0.9 1.2 +/- 0.4 Urgent operation 36% 7% Bypass time (min) 159 +/- 49 128 +/- 52 Dos et al JTCVS 2009
Indications for PVR Homografts and porcine bioprosthesis are the most commonly used valves Metallic prosthesis in the pulmonary position have been complicated by high rates of thrombosis The risk of homograft failure (average life span 10-15 years) needs to be weighed against the risk of adverse cardiac outcomes
Indications for PVR Class IIa Free pulmonary regurgitation associated with: Symptoms Progressive or moderate to severe RV enlargement (right ventricular end diastolic volume > 170 cc/m2) Moderate to severe right ventricular dysfunction Important tricuspid regurgitation Canadian Cardiovascular Society 2009 Consensus Conference on the Management of Adults with Congenital Heart Disease ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease
Indications for PVR Class IIa Free PR and sustained clinical arrhythmias (atrial flutter or fibrillation or sustained monomorphic ventricular tachycardia) Canadian Cardiovascular Society 2009 Consensus Conference on the Management of Adults with Congenital Heart Disease ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease
Indications for PVR Class IIa Residual pulmonary stenosis with RV pressure 2/3 of systemic pressure, peak echo gradient 50 mmhg or progressive RV dilation/dysfunction Residual VSD with a shunt > 1.5:1 Combinations of VSD, PS and/or PR of moderate degrees, but resulting in progressive RV enlargement Canadian Cardiovascular Society 2009 Consensus Conference on the Management of Adults with Congenital Heart Disease ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease
Indications for PVR Class IIa Significant aortic regurgitation associated with symptoms and/or progressive left ventricular systolic dysfunction Aortic root enlargement 55 mm in diameter A large right ventricular outflow tract aneurysm Canadian Cardiovascular Society 2009 Consensus Conference on the Management of Adults with Congenital Heart Disease ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease
Indications for PVR Class I Patients who require reoperation for tetralogy of Fallot should be operated on by congenital heart surgeons Canadian Cardiovascular Society 2009 Consensus Conference on the Management of Adults with Congenital Heart Disease ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease
Indications for PVR Volume threshold for PVR if RVEF Normal Volume threshold for PVR if RVEF < 30% Wald et al Congenit Heart Dis 2009
PVR Following Repair of TOF Now? When? Candice Silversides, MD Toronto Congenital Cardiac Centre for Adults University of Toronto Toronto, Canada
Risks and Benefits of PVR Dos et al JTCVS 2009
Case PRE Symptoms Severe PR RVEDV 308 cc/m2 RVEF 38% POST PVR / No PR RVEDV 143 cc/m2 RVEF 30%