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Massimo Chessa

Department of Pediatric Cardiology
                &
Adult with Congenital Heart Disease


  IRCCS- Policlinico San Donato
  San Donato Milanese – Milano

massimo.chessa@grupposandonato.it




     Managing the RVOT

    Indications andTiming
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




…………….severe pulmonary regurgitation                                                         alone,
requiring valve insertion, is uncommon……..

World Congress of Paediatric Cardiology 1989
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




                 Natural History of PR
One of the reason for the lack of appreciation of the
impact of PR is its very long preclinical natural history

At age 20 years, only 6% of the pt had symptoms, but the
incidence increased to 29% at age 40 years


Shimazaki Y Thorac Cardiovasc Surg1984;32:257-9
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




                   Natural History of PR

At the time of ToF repair the RV is hypertrophied and its
compliance is low; the diameters of the central PA are
either hypoplastic or low-normal, and their capacitance is
low.
The heart rate is relatively high, which leads to a relatively
short duration of diastole

            The combination of these factors
     limits the degree of pulmonary regurgitation.
Department of Pediatric Cardiology & Adult with Congenital Heart Disease



                    Natural History of PR

Over time the increase in RV
stroke volume leads           to
progressive rise in the size and
compliance of the central PA
and     to     increased     RV
compliance



  Combined with a longer duration of diastole as HR
  decreases with age, these changes lead to progressive
  increase in the degree of PR
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




The number of pts free of reinterventions for PVR
decrease during the 3rd-4th decade
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 that


                  PR is a key driver

                         of      RV failure
                                            but


the Timing      for PVR remains Controversial
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




Criteria for PV Replacement


Pt with symptoms
             Exercise intollerance
                Heart failure
                     sVT
                   syncope


     PVR surgically
          or
     transcatheter
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




                Criteria for PV Replacement
Pt 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
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?
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




Natural History of PR

In a pt with a PR
although there is a normal
pattern of ejection during
pressure rise and pressure
fall, there is increase in
volume      during      the
isovolumic       relaxation
period.


                                                   Redington AN Br Heart J 1988;60:57-65
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 volume




Redington AN Br Heart J 1988;60:57-65
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




Once the compensatory mechanisms begin to fail
RV Mass-to-Volume ratio decreases
           End-Systolic Volume increases
                 Ejection Fraction decreases
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




Samyn et al, J Magn Reson Imaging 2007                             Geva et al, J Am Coll Cardiol 2004,
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




RV Structure and Function
More afterload dependent than the LV
Very modest increases in PVR – one component of afterload
- may result in substantial declines in RV stroke volume
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




For determining the optimal timing of
    pulmonary valve replacement
         we must know the

         Natural History
             ant the
    Adverse Clinical Outcomes
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




One of the key point influencing the RV modifications
related 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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




For determining the optimal timing of pulmonary valve
           replacement we must know the
                  Natural History
                       ant the
            Adverse Clinical Outcomes
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




• Mortality rate triples during the 3rd postoperative decade




There 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)
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




           How to Investigate
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




           How to Investigate




                                                                           12
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




           How to Investigate
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




                                     The Timing!

Certainly PVR should be performed when patients develop
first symptoms as dyspnea, but it is not infrequent that they
may have advanced RV dysfunction by the time complain of
symptoms


Serial exercise testing and/or CPE test may help to delineate
subtle changes in exercise capacity before the pt becomes
symptomatic.
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
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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




                                               The Timing!

The most recent RV EDV “cut-off ” proposed has moved
even lower than 150 ml/m2
                          but
Non consistent improvement in RVEF was observed!!
Dave HH 2005;80:1615-20
Frigiola A 2008;34:576-82




         Maybe the Focus should be on the
    preservation of RVEF rather than RV volume
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




                             The Timing!

  In asymptomatic children after repair of ToF,
   pulmonary regurgitation is associated with
impaired regional systolic RV deformation indices
                (Cadiac Doppler Myocardial Imaging)

                                        not

         demonstrate by routine RVEF
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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




Tricuspid Valve Repair
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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




                                                 The Timing!

QRS duration may be a “proxy”for RV function
A bad RV is associated with an increased risk for VT and SD
PVR alone does not usually result in shortening of the QRS
duration
Harrild 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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease




Conclusions

We are probably still operating too late because the
limited 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
I Thank you for your attention……

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Timing for PVR

  • 1. Massimo Chessa Department of Pediatric Cardiology & Adult with Congenital Heart Disease IRCCS- Policlinico San Donato San Donato Milanese – Milano massimo.chessa@grupposandonato.it Managing the RVOT Indications andTiming
  • 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. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Natural History of PR One of the reason for the lack of appreciation of the impact of PR is its very long preclinical natural history At age 20 years, only 6% of the pt had symptoms, but the incidence increased to 29% at age 40 years Shimazaki Y Thorac Cardiovasc Surg1984;32:257-9
  • 4. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Natural History of PR At the time of ToF repair the RV is hypertrophied and its compliance is low; the diameters of the central PA are either hypoplastic or low-normal, and their capacitance is low. The heart rate is relatively high, which leads to a relatively short duration of diastole The combination of these factors limits the degree of pulmonary regurgitation.
  • 5. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Natural History of PR Over time the increase in RV stroke volume leads to progressive rise in the size and compliance of the central PA and to increased RV compliance Combined with a longer duration of diastole as HR decreases with age, these changes lead to progressive increase in the degree of PR
  • 6. Department of Pediatric Cardiology & Adult with Congenital Heart Disease The number of pts free of reinterventions for PVR decrease during the 3rd-4th decade
  • 7. Department of Pediatric Cardiology & Adult with Congenital Heart Disease
  • 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 but the Timing for PVR remains Controversial
  • 9. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Criteria for PV Replacement Pt with symptoms Exercise intollerance Heart failure sVT syncope PVR surgically or transcatheter
  • 10. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Criteria for PV Replacement Pt 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. 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. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Natural History of PR In a pt with a PR although there is a normal pattern of ejection during pressure rise and pressure fall, there is increase in volume during the isovolumic relaxation period. Redington AN Br Heart J 1988;60:57-65
  • 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 volume Redington AN Br Heart J 1988;60:57-65
  • 14. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Once the compensatory mechanisms begin to fail RV Mass-to-Volume ratio decreases End-Systolic Volume increases Ejection Fraction decreases
  • 15. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Samyn et al, J Magn Reson Imaging 2007 Geva et al, J Am Coll Cardiol 2004,
  • 16. Department of Pediatric Cardiology & Adult with Congenital Heart Disease RV Structure and Function More afterload dependent than the LV Very modest increases in PVR – one component of afterload - may result in substantial declines in RV stroke volume
  • 17. Department of Pediatric Cardiology & Adult with Congenital Heart Disease For determining the optimal timing of pulmonary valve replacement we must know the Natural History ant the Adverse Clinical Outcomes
  • 18. Department of Pediatric Cardiology & Adult with Congenital Heart Disease One of the key point influencing the RV modifications related 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. Department of Pediatric Cardiology & Adult with Congenital Heart Disease For determining the optimal timing of pulmonary valve replacement we must know the Natural History ant the Adverse Clinical Outcomes
  • 20. Department of Pediatric Cardiology & Adult with Congenital Heart Disease • Mortality rate triples during the 3rd postoperative decade There 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. Department of Pediatric Cardiology & Adult with Congenital Heart Disease How to Investigate
  • 22. Department of Pediatric Cardiology & Adult with Congenital Heart Disease How to Investigate 12
  • 23. Department of Pediatric Cardiology & Adult with Congenital Heart Disease How to Investigate
  • 24. Department of Pediatric Cardiology & Adult with Congenital Heart Disease The Timing! Certainly PVR should be performed when patients develop first symptoms as dyspnea, but it is not infrequent that they may have advanced RV dysfunction by the time complain of symptoms Serial exercise testing and/or CPE test may help to delineate subtle changes in exercise capacity before the pt becomes symptomatic.
  • 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. 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. Department of Pediatric Cardiology & Adult with Congenital Heart Disease The Timing! The most recent RV EDV “cut-off ” proposed has moved even lower than 150 ml/m2 but Non consistent improvement in RVEF was observed!! Dave HH 2005;80:1615-20 Frigiola A 2008;34:576-82 Maybe the Focus should be on the preservation of RVEF rather than RV volume
  • 28. Department of Pediatric Cardiology & Adult with Congenital Heart Disease The Timing! In asymptomatic children after repair of ToF, pulmonary regurgitation is associated with impaired regional systolic RV deformation indices (Cadiac Doppler Myocardial Imaging) not demonstrate by routine RVEF
  • 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. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Tricuspid Valve Repair
  • 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. Department of Pediatric Cardiology & Adult with Congenital Heart Disease The Timing! QRS duration may be a “proxy”for RV function A bad RV is associated with an increased risk for VT and SD PVR alone does not usually result in shortening of the QRS duration Harrild 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. Department of Pediatric Cardiology & Adult with Congenital Heart Disease Conclusions We are probably still operating too late because the limited 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. I Thank you for your attention……