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Ruxandra Jurcut
RV function in
congenital heart diseases and
pulmonary hypertension
Department of Cardiology
University of Medicine and Pharmacy “Carol Davila”
Bucharest, Romania
Declaration of conflict of interest: none to declare
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Agenda
Background
RV – complex anatomy & function
Specific question
RV myocardial function in PH and
congenital heart disease = RV in different
loading conditions
deformation imaging and loading conditions
Take home messages
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RV remodeling
The RV and LV differ markedly in their anatomy, mechanics, and
loading conditions:
Structure: crescent-shaped, thin-walled RV
Hemodynamics: volume pump, ejecting in a low-resistance pulmonary
arterial circulation.
Special contraction pattern – peristaltic movement
87% from the RV stroke volume – RV sinus (apical 4CV)
80% from the RV stroke volume - longitudinal contraction
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RV remodeling/failure and prognosis
PHT and RV failure - strong predictors of mortality in
LV failure, AMI and COPD
RV dysfunction is a strong prognostic factor in:
PulmReg postrepair for Tetralogy of Fallot
systemic RV in TGA
PH (TAPSE only)…
RV pressure overload associated with pulmonary valve
stenosis - better prognosis than PAH
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Load and contractility
Bijnens et al EJE 2008
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Cardiac mechanics –
how is it balanced?
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Frank Starling law
LVEDP (mmHg) LVEDP (mmHg)
SV
(m
l)
SV
(m
l)
↑ afterload
↓ inotropy
↓ afterload
↑ inotropy
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Load, geometry and contractility
Laplace’s law
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RV function assessment
Gold standard: pressure-volume loops
Load dependence of dp/dt, stroke work
Load independence of end systolic elastance, PRSW
Bleeker, Heart 2006
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Myocardial deformation imaging –
several techniques, several parameters
t
t
IVV
IVA=IVV/t
Jurcut et al, Eur J Echo 2010
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Load and contractility – in practice
Rosner et al, EJE 2008
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RV deformation
Afterload alterations
Pulmonary hypertension
Pulmonary stenosis
Transposition of the great arteries
(“systemic RV”)
Preload alterations
Atrial septal defect
Tricuspid regurgitation
Pulmonary regurgitation
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Pulmonary Hypertension
Puwanant et al, Circulation 2010
PASP LVEI
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Pulmonary Hypertension
Puwanant et al, Circulation 2010
PASP LVEI
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Pulmonary Hypertension
Simon et al, Congest Heart Fail 2009
-26±1%
-19±1%
-14±1%
Study group RV FW LS
ROC curve cutoff
Sn Sp
Normal PAPs ≤ -27.2% 70% 93.5%
PH compensated vs decompensated ≥ -14.9% 61.5% 85%
RAP<10 mmHg RAP>10 mmHg
* * #
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Increased afterload: PHT vs PStenosis
Jurcut et al, JASE 2011
RVP
73±34 mmHg
RVP
88±31 mmHg
RVP
25±4 mmHg
p = 0.37
p<0.001
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Arterial PHT
F, 25 years
RVP = 100 mmHg
Pulmonary stenosis
F, 27 years
RVP = 106 mmHg
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Increased afterload: PHT vs PStenosis
Jurcut et al, JASE 2011
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Pulmonary artery banding ~
PulmSten
Angioproliferative pulmonary
vascular remodeling ~ aPHT
Bogaard et al, Circulation 2009
PS PHT
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RV and PH etiology – does it matter?
48 patients PH
14 pts iPAH
42±14 yo
7 pts PH assoc CTD
53±15 yo
16 pts CTEPH
53±12 yo
11 pts Eisenmenger
41±16 yo
pVarsta = NS Giusca, Jurcut et al, data on file
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Similar sPAP…
iPAH PH_CTD CTEPH EisenSy
sPAP (mmHg) 96±36 72±12.5 89±12 96±14
PANOVA=0.1
HTPAI HTP_BTC HTPTC Sindr Eisen.0
50
100
150
200
PA
Ps (
mm
Hg
)
Giusca, Jurcut et al, data on file
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…but different RV morphology…
Grosimea perete VD
HTPAI HTP_BTC HTPTC Sind Eisen0.0
2.5
5.0
7.5
10.0
GP
VD
(m
m)
Pposthoc <0.05 Pposthoc <0.01 PANOVA=0.002
RVfw 7,6±1,8 6,2±1,2 7,2±1,4 9,2±1,5
Diametru telediastolic VD
HTPAI HTP_BTC HTPTC Sindr Eisen.0
10
20
30
40
50
DT
VD
(m
m)
Pposthoc <0.05 Pposthoc <0.05 PANOVA=0.04
RVEDD 45,9±10 36,6±6 47,9±6 43±10,8
Giusca, Jurcut et al, data on file
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RVEDA RVESA
Rata de modificare a ariei VD
HTPAI HTP_BTC HTPTC Sindr Eisen.0
5
10
15
20
25
30
35
40
45
RV
FA
C (
%)
PANOVA=0.005
Pposthoc <0.05
Pposthoc <0.05
Pposthoc <0.01
areaicenddiastolRV
areacendsystoliRVareaicenddiastolRVRVFAC
RVFAC 30,9±10 27,5±11 27,5±9,5 41±8,9
…and different RV function
Giusca, Jurcut et al, data on file
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Pposthoc <0.05
PANOVA=0.007
Long S (%) -15,6±6,8 -17,4±6,8 -12,6±4,8 -20,6±3,5
Strain la nivelul perete liber VD
HTPAI HTP_BTC HTPTC Sindr Eisen.
-30
-20
-10
0
Str
ain
(%
)
Pposthoc <0.05
…and different RV function
Free RV wall longitudinal strain
Giusca, Jurcut et al, data on file
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Is RV adaptation/remodeling identical for all PH forms?
And what does it mean?
Eisenmenger Syndrome
iPAH
CTEPH
Hopkins et al, Coronary Artery Disease 2005
So…
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However, also in Eisenmenger syndrome…
Van de Bruaene et al, Am J Cardiol 2012
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Eisenmenger syndrome
– linking echo to prognosis
Van de Bruaene et al, Am J Cardiol 2012
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TGA – the “systemic right ventricle”
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TGA – the “systemic right ventricle”
Pettersen et al, JACC 2007
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TGA – the “systemic right ventricle”
Di Salvo et al, Int J Cardiol 2010
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TGA – the “systemic right ventricle”
Di Salvo et al, Int J Cardiol 2010
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Atrial septal defect
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Open atrial septal defect
*
Jategaonkar et al, EJE 2009
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Atrial septal defect – open vs closed
Van de Bruaene et al, EJE 2011
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Van de Bruaene et al, Eur J Prev Cardiol 2012
Atrial septal defect – exercise physiology
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RV function after ToF repair
•Different echocardiographic techniques have
become available that allow to reliably and
accurately assess RV volumes and function.
• Both 3D an 3DR can be used in
postoperative pediatric patients after TOF
correction.
• Cardiac MRI currently still is the reference
technique.
Dragulescu et al, Eur J Echo 2011
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Eyskens et al, Eur J Echocard 2010
RV function after ToF repair
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Eyskens et al, Eur J Echocard 2010
RV function after ToF repair
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RV function after ToF repair
Dragulescu et al, Euroecho 2011
In patients after ToF repair, RV dilatation due to chronic
Pulm Reg is associated with reduced strain.
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Take home messages
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Interpretation of MDI parameters must always
incorporate loading conditions:
Influence of volume vs pressure overload
Influence of geometrical changes (e.g.RV dilation)
RV function may not be adequately described by
longitudinal function alone.
RV myocardial adaptation to load in different conditions:
RV wall transverse fibers hypertrophy with increased afterload
Different cellular and molecular adaptation mechanisms to
pressure overload (e.g. PS vs PHT)
Take home messages