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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
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)
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