Massimo ChessaDepartment of Pediatric Cardiology                &Adult with Congenital Heart Disease  IRCCS- Policlinico S...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease…………….severe pulmonary regurgitation              ...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                 Natural History of PROne of the r...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                   Natural History of PRAt the tim...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                    Natural History of PROver time...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseThe number of pts free of reinterventions for PVRd...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease During the past 2 decades it has become apparent ...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseCriteria for PV ReplacementPt with symptoms       ...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                Criteria for PV ReplacementPt asym...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseWhy timing is so important?Why timing is so diff...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseNatural History of PRIn a pt with a PRalthough the...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                                                  ...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseOnce the compensatory mechanisms begin to failRV M...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseSamyn et al, J Magn Reson Imaging 2007            ...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseRV Structure and FunctionMore afterload dependent ...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseFor determining the optimal timing of    pulmonary...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseOne of the key point influencing the RV modificati...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseFor determining the optimal timing of pulmonary va...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease• Mortality rate triples during the 3rd postoperat...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease           How to Investigate
Department of Pediatric Cardiology & Adult with Congenital Heart Disease           How to Investigate                     ...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease           How to Investigate
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                                     The Timing!Ce...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                                    The Timing!RV ...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                                   The Timing!RV S...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                                               The...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                             The Timing!  In asymp...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                                    The Timing!RV ...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseTricuspid Valve Repair
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                                  The Timing!RV Si...
Department of Pediatric Cardiology & Adult with Congenital Heart Disease                                                 T...
Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseConclusionsWe are probably still operating too lat...
I Thank you for your attention……
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Timing for PVR

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Timing for PVR

  1. 1. Massimo ChessaDepartment of Pediatric Cardiology &Adult with Congenital Heart Disease IRCCS- Policlinico San Donato San Donato Milanese – Milanomassimo.chessa@grupposandonato.it Managing the RVOT Indications andTiming
  2. 2. Department of Pediatric Cardiology & Adult with Congenital Heart Disease…………….severe pulmonary regurgitation alone,requiring valve insertion, is uncommon……..World Congress of Paediatric Cardiology 1989
  3. 3. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Natural History of PROne of the reason for the lack of appreciation of theimpact of PR is its very long preclinical natural historyAt age 20 years, only 6% of the pt had symptoms, but theincidence increased to 29% at age 40 yearsShimazaki Y Thorac Cardiovasc Surg1984;32:257-9
  4. 4. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Natural History of PRAt the time of ToF repair the RV is hypertrophied and itscompliance is low; the diameters of the central PA areeither hypoplastic or low-normal, and their capacitance islow.The heart rate is relatively high, which leads to a relativelyshort duration of diastole The combination of these factors limits the degree of pulmonary regurgitation.
  5. 5. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Natural History of PROver time the increase in RVstroke volume leads toprogressive rise in the size andcompliance of the central PAand to increased RVcompliance Combined with a longer duration of diastole as HR decreases with age, these changes lead to progressive increase in the degree of PR
  6. 6. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseThe number of pts free of reinterventions for PVRdecrease during the 3rd-4th decade
  7. 7. Department of Pediatric Cardiology & Adult with Congenital Heart Disease
  8. 8. Department of Pediatric Cardiology & Adult with Congenital Heart Disease During the past 2 decades it has become apparent that PR is a key driver of RV failure butthe Timing for PVR remains Controversial
  9. 9. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseCriteria for PV ReplacementPt with symptoms Exercise intollerance Heart failure sVT syncope PVR surgically or transcatheter
  10. 10. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Criteria for PV ReplacementPt asymptomatic with PR ≥ 25-35% + at least 2 criteria RV EDVi ≥ 150 mL/m2 or RV/LV >1.5 RV ESVi ≥ 80 mL/m2 RV volumes and function RV EF ≤ 45% CPET ≤ 65% of the predicted VO2 max QRS ≥ 180 msec (better before 180 because no improvments after PVR) TR ++ Residual VSD RVOTO (RVP 2/3 LVP or ΔP ≥ 50 mmHg AR ++ LV Dysfunction
  11. 11. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseWhy timing is so important?Why timing is so difficult?What do we know OR DON’T know?Which are possible future directions?
  12. 12. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseNatural History of PRIn a pt with a PRalthough there is a normalpattern of ejection duringpressure rise and pressurefall, there is increase involume during theisovolumic relaxationperiod. Redington AN Br Heart J 1988;60:57-65
  13. 13. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Natural History of PR There is a linear relationship between the amount of pulmonary incompetence measured during the isovolumic relaxation period and the end diastolic volumeRedington AN Br Heart J 1988;60:57-65
  14. 14. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseOnce the compensatory mechanisms begin to failRV Mass-to-Volume ratio decreases End-Systolic Volume increases Ejection Fraction decreases
  15. 15. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseSamyn et al, J Magn Reson Imaging 2007 Geva et al, J Am Coll Cardiol 2004,
  16. 16. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseRV Structure and FunctionMore afterload dependent than the LVVery modest increases in PVR – one component of afterload- may result in substantial declines in RV stroke volume
  17. 17. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseFor determining the optimal timing of pulmonary valve replacement we must know the Natural History ant the Adverse Clinical Outcomes
  18. 18. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseOne of the key point influencing the RV modificationsrelated to the PR is the RV Diastolic Performance While this appears to be disadvantageous in the early postoperative period, restrictive physiology has many potential advantages during late postoperative follow-up
  19. 19. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseFor determining the optimal timing of pulmonary valve replacement we must know the Natural History ant the Adverse Clinical Outcomes
  20. 20. Department of Pediatric Cardiology & Adult with Congenital Heart Disease• Mortality rate triples during the 3rd postoperative decadeThere are three major categories of outcome predictors on the risk of death in survivors of ToF repair 1. History (syncope, older age at repair) 2. Electrophysiologic markers (prolonged QRS duration, sVT, positive ventricular stimulation study) 3. Hemodynamic sequelae (RV dilatation, Ventricular dysfunction)
  21. 21. Department of Pediatric Cardiology & Adult with Congenital Heart Disease How to Investigate
  22. 22. Department of Pediatric Cardiology & Adult with Congenital Heart Disease How to Investigate 12
  23. 23. Department of Pediatric Cardiology & Adult with Congenital Heart Disease How to Investigate
  24. 24. Department of Pediatric Cardiology & Adult with Congenital Heart Disease The Timing!Certainly PVR should be performed when patients developfirst symptoms as dyspnea, but it is not infrequent that theymay have advanced RV dysfunction by the time complain ofsymptomsSerial exercise testing and/or CPE test may help to delineatesubtle changes in exercise capacity before the pt becomessymptomatic.
  25. 25. Department of Pediatric Cardiology & Adult with Congenital Heart Disease The Timing!RV Size and function TR functional or mechanic Symptomatic atrial and ventricular arrhythmias Coexistent PS
  26. 26. Department of Pediatric Cardiology & Adult with Congenital Heart Disease The Timing!RV Size and function TR functional or mechanic Symptomatic atrial and ventricular arrhythmias Coexistent PS
  27. 27. Department of Pediatric Cardiology & Adult with Congenital Heart Disease The Timing!The most recent RV EDV “cut-off ” proposed has movedeven lower than 150 ml/m2 butNon consistent improvement in RVEF was observed!!Dave HH 2005;80:1615-20Frigiola A 2008;34:576-82 Maybe the Focus should be on the preservation of RVEF rather than RV volume
  28. 28. Department of Pediatric Cardiology & Adult with Congenital Heart Disease The Timing! In asymptomatic children after repair of ToF, pulmonary regurgitation is associated withimpaired regional systolic RV deformation indices (Cadiac Doppler Myocardial Imaging) not demonstrate by routine RVEF
  29. 29. Department of Pediatric Cardiology & Adult with Congenital Heart Disease The Timing!RV Size and function TR functional or mechanic Symptomatic atrial and ventricular arrhythmias Coexistent PS
  30. 30. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseTricuspid Valve Repair
  31. 31. Department of Pediatric Cardiology & Adult with Congenital Heart Disease The Timing!RV Size and function TR functional or mechanic Symptomatic atrial and ventricular arrhythmias Coexistent PS
  32. 32. Department of Pediatric Cardiology & Adult with Congenital Heart Disease The Timing!QRS duration may be a “proxy”for RV functionA bad RV is associated with an increased risk for VT and SDPVR alone does not usually result in shortening of the QRSdurationHarrild DM 2009;119:445-451 It is possible that in both groups, the RV size and dysfunction were already advanced and surgery was too late to confer a survival advantage Warnes CA JACC 2009;54:1903-10
  33. 33. Department of Pediatric Cardiology & Adult with Congenital Heart DiseaseConclusionsWe are probably still operating too late because thelimited life expectancy of all valves inserted in the pulmonary position…. …. but further development of transcatheter techniques for implantation and re-implantation may lower the threshold for PVR
  34. 34. I Thank you for your attention……

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