Download Is identification of early right ventricular dysfunction possible?

740001 / from 14:00 to 14:22
Controversies in adult congenital heart disease
Is identification of early
right ventricular dysfunction
possible?
Philipp Beerbaum MD PhD
Director , Paediatric and Adult Congenital Cardiac MRI Service
King’s College London, Guy’s & St Thomas’ NHS Foundation Trust
ESC, Annual Scientific Meeting 2001, Paris, France
Dept. Imaging Sciences &
Biomedical Engineering
No disclosures
Dept. Imaging Sciences &
Biomedical Engineering
“RV dysfunction” – what’s that?
 Definition?


Systolic, diastolic, combined?
“normal” –” adaptive” – “dysfunction” – “failure”
 Clinical context?






Dept. Imaging Sciences &
Biomedical Engineering
Acute or chronic?
Myocardial disorder? Or secondary to pre-load / after-load
pathophysiology?
Left ventricular contribution to RV function?
RV supporting the aorta or the pulmonary artery?
If subpulmonary, pulmonary function?
RV as single ventricle - Parallel or serial circulation
(e.g. aorto-pulmonary shunt or Fontan circulation)?
RV may suffer in...

Arrythmogenic “RV” cardiomyopathy (ARVC)
(& involvement in any cardiomyopathy)


Pulmonary arterial hypertension (PAH)
Congenital heart disease (CHD)

Pressure load:




Volume load:


Dept. Imaging Sciences &
Biomedical Engineering
ASD, AVSD partial, Ebstein
Combined:



Systemic RV biventricular (Mustard / d-TGA, CC-TGA)
Systemic RV as single ventricle (HLHS)
Eisenmenger (secondary PHT)
Tetralogy of Fallot post repair
Inferior myocardial infarction
LV failure!
The ideal test for “early right
ventricular dysfunction

Valid and reproducible for all of the various clinical
situations where RV may suffer
Non-invasive, no ionising radiation
Fast & simple & cheap, usable for screening
Biomarker quality
Outcome-related





Of course there is no such test and will
never be
 However, a few conditions seem to
indicate the way forward
Dept. Imaging Sciences &
Biomedical Engineering
Early detection = fighting late mortality
Finnland Registry
37 years, 1953-1989, n=6461
CoA
Fallot
Univentric.
Dept. Imaging Sciences &
Biomedical Engineering
Nieminen, H. P. et al. Circulation 2001;104:570-575
Early detection = fighting late mortality
‘CONCOR` Registry, Netherlands
2/3 cardiovascular deaths
Dept. Imaging Sciences &
Biomedical Engineering
Verheugt C L et al. Eur Heart J 2010;31:1220-1229
LV: Sequence of ischemic events
ECG changes
Anginal pain
wall motion
abnormalities
contraction
abnormalities
(dP/dt )
relaxation
disturbances
(Tau )
Ischemia
time
onset = perfusion deficit
Dept. Imaging Sciences &
Biomedical Engineering
RV: Sequence of remodeling?
 Myocyte
remodelling
 Fiber orientation
 Size & wall
thickness
 Global function &
regional wall motion
 Shape
 RV-LV interaction
Dept. Imaging Sciences &
Biomedical Engineering
Normal RV
Adaptive
remodelling
RV dysfunction
RV failure
Normal? Adaptive remodeling?
Dysfunction? Failure?
 Single ventricle
EF 55%
 Repaired Fallot
EF 45%
 ARVC
EF 25%
Dept. Imaging Sciences &
Biomedical Engineering
RV FEATURES
COMPARED TO LV
Dept. Imaging Sciences &
Biomedical Engineering
Anatomy – general features
 Retrosternal, anterior to LV,




wrapped around LV, pumping
bellow-like “against” the
septum
RV thin-walled (3-5 mm), RV
mass ~25% of LV mass
Trabeculated & moderator
band
3-partite: Inlet, sinus, outlet
portion
Subarterial infundibulum
Dept. Imaging Sciences &
Biomedical Engineering
Sheehan F , Redington A Heart 2008;94:1510-1515
Benza R, et al. JACC 2008; Nov 18;52(21):1683-92
Anatomy – Fiber architecture
 RV: Predominantly helical fibers
 mainly longitudinal shortening, little radial
shortening & torsion
 LV: 3 layers, inner & outer layer more helical,
middle layer circumferential
 longitudinal & radial shortening & marked torsion
Dept. Imaging Sciences &
Biomedical Engineering
Sheehan F , Redington A Heart 2008;94:1510-1515
Normal RV physiology
 Lack of isovolumetric relaxation & contraction
 Forward flow in spite of pressure loss:
 High capacitance of the pulmonary circulation
 allows preservation of momentum and maintained forward
flow (“hang-out” period)
Dept. Imaging Sciences &
Biomedical Engineering
Sheehan F , Redington A Heart 2008;94:1510-1515
Inter-ventricular interaction
 Septum + pericardium + common fibers
 LV produces ~ 30%-50% of RV output
RV and LV contribution
to LV pressure
RV and LV contribution
to RV pressure
LV
RV
Dept. Imaging Sciences &
Biomedical Engineering
Damiano RJ et al. Am J Physiol 1991
Heart–lung interaction
 RV is very sensitive to even small increase of
afterload such as PEEP
 Volume loading is tolerated much better
Dept. Imaging Sciences &
Biomedical Engineering
Henning RJ et al. J Appl Physiol 1986;61:819–26.
FIRST EXAMPLE
PRIMARY MYOCARDIAL DISEASE
ARRYTHMOGENIC RV CARDIOMYOPATHY
Dept. Imaging Sciences &
Biomedical Engineering
ARVC





Myocardial desmosomal disorder, autosomaldominant trait
Prevalence 1:1000-1:5000, males more affected
Predominantly RV disease, but LV is involved in 50%
(postero-lateral)
Sudden cardiac death in adolsecents & yound adults
Diagnostic criteria (Task force) based on





Dept. Imaging Sciences &
Biomedical Engineering
Global & regional dysfunction
Tissue characterisation (biopsy)
ECG (negative T in V1 + V2)
Arrhythmias (PVC, VT of LBBB-type)
Family history
ARVC
Intracellular and intercellular components of the
desmosomal plaque
Dept. Imaging Sciences &
Biomedical Engineering
Corrado D et al. Heart 2011;97:530-539
Arrhythmic risk stratification pyramid based on
observational studies on ICD therapy
Dept. Imaging Sciences &
Biomedical Engineering
Corrado D et al. Heart 2011;97:530-539
Early detection of ARVC by MRI



Dept. Imaging Sciences &
Biomedical Engineering
Fibrosis and subtle regional wall motion can be reliably
identified
Much higher sensitivity & specificity when integrating late
gadolinium enhancement MRI, compared to recent TASK
FORCE criteria
Reason:
 Early “RV dysfunction” identified as myocardial fibrosis
may even precede regional wall motion
MRI of ARVC & cardiomyopathies



Dept. Imaging Sciences &
Biomedical Engineering
EARLY DETECTION OF RV DYSFUNCTION IS POSSIBLE
 Clearly differentiated from adaption & failure
This is also the case for iron-deposition in Thalassaemia by T2*
MR imaging
Also likely for storage myopathies involving RV
SECOND EXAMPLE
CHRONIC PROGRESSIVE PRESSURE LOAD
PULMONARY ARTERIAL HYPERTENSION
(PAH)
Dept. Imaging Sciences &
Biomedical Engineering
Sequence of remodeling in PAH?
Adaptive
remodelling
RV dysfunction
RV failure
Dept. Imaging Sciences &
Biomedical Engineering
Champion H C et al. Circulation 2009;120:992-1007
PV loops: Best measurement of load-independent
contractility and ventriculoarterial coupling in PAH
Dept. Imaging Sciences &
Biomedical Engineering
Champion H C et al. Circulation 2009;120:992-1007
Right heart catheterization is the gold standard for
assessing the RV afterload in PAH
•
•
Dept. Imaging Sciences &
Biomedical Engineering
Response to vasodilators
Stress assessment by exercise &
fluid bolus during catheterization
Champion H C et al. Circulation 2009;120:992-1007
Echocardiography for PAH
Utility of CW of tricuspid regurgitation velocity
for “measurement” of RV pressure
Champion H C et al. Circulation 2009
Dept. Imaging Sciences &
Biomedical Engineering
Janda S et al. Heart 2011
Echocardiography for PAH
Septal shift over time: Eccentricity index (EccIx)
(A)Normal heart with diastolic EccIx = 1
(B)Patient with pulmonary hypertension with diastolic EccIx = 1.8
Dept. Imaging Sciences &
Biomedical Engineering
Jurcut R et al. Eur J Echocardiogr 2010;11:81-96
Echocardiography for PAH
- RVOT- fractional shortening (%)
- RV fractional area change (%)
Dept. Imaging Sciences &
Biomedical Engineering
Jurcut R et al. Eur J Echocardiogr 2010;11:81-96
Echocardiography for PAH
Tricuspid annular plane systolic excursion (TAPSE)
Normal
Dept. Imaging Sciences &
Biomedical Engineering
Jurcut R et al. Eur J Echocardiogr 2010;11:81-96
PAH
Echocardiography for PAH
Myocardial performance index by TDI of RV free wall
at tricuspid level (TEI-index)
Normal
Dept. Imaging Sciences &
Biomedical Engineering
Jurcut R et al. Eur J Echocardiogr 2010;11:81-96
PAH
Echocardiography for PAH
Isovolumetric acceleration (IVA) during isovolumic contraction
at the basal segment of the right ventricular free wall
Advantage: IVA seems relatively load-independent
Concerns: Reproducibility & angle dependence
Dept. Imaging Sciences &
Biomedical Engineering
Jurcut R et al. Eur J Echocardiogr 2010;11:81-96
Echocardiography for PAH
Investigational: 3D imaging of the right ventricle for
calculation of EDV, ESV, SV and EF
Advantage: EF noninvasive and better availability than MRI
Concerns: Reproducibility & coverage of apical free wall and RVOT
Dept. Imaging Sciences &
Biomedical Engineering
Jurcut R et al. Eur J Echocardiogr 2010;11:81-96
Echocardiography for PAH
Investigational: TDI & speckle tracking for strain / strain
rate measurements in the right ventricular free wall
Normal
PAH
Dept. Imaging Sciences &
Biomedical Engineering
TDI
Speckle tracking
Jurcut R et al. Eur J Echocardiogr 2010;11:81-96
MRI for RV function
axial
Dept. Imaging Sciences &
Biomedical Engineering
short axis
MRI for PAH
Dept. Imaging Sciences &
Biomedical Engineering
Champion H C et al. Circulation 2009;120:992-1007
MR feature tracking
Dept. Imaging Sciences &
Biomedical Engineering
MRI for tagging & rotation
Enddiastole
Courtesy: Eike Nagel, KCL
Dept. Imaging Sciences &
Biomedical Engineering
Endsystole
Endsystole
MRI cardiac catheterization for
pressure volume loops and VA coupling
Dept. Imaging Sciences &
Biomedical Engineering
Kuehne T et al. Circulation 2004
MRI cardiac catheterization for
pulmonary vascular resistance
Cather = pressure
MRI = blood flow
=> Heart / Circulation 2005 London/Berlin: MRI vs. Oxymetrie/Thermodilution
=> MRI method became part of German guidlines for pulmonary hypertension, 2009/ 2010
Dept. Imaging Sciences &
Biomedical Engineering
PET and the RV-PA unit
Standard FDG-PET imaging can be used to measure RV and
pulmonary microcirculation metabolism
Dept. Imaging Sciences &
Biomedical Engineering
Champion H C et al. Circulation 2009;120:992-1007
Hyperpolarisation for metabolic MRI
13C
B0 = 3.35T
labelled
compound +
free radical
(free electrons)
Dept. Imaging Sciences &
Biomedical Engineering
Courtesy: Tom Eykyn & Sebastian Kozerke, KCL, London
Hyperpolarised 13C Spectroscopy in
preclinical animal model
13C-Pyruvat
13C-Laktat
13C-Bicarbonat
Echo-planar
Spektroskopie (EPSI)
Sequenz
•
2mm x 2mm
•
Zeit-Intervall 5 sec
Dept. Imaging Sciences &
Biomedical Engineering
Courtesy: Tom Eykyn & Sebastian Kozerke, KCL, London
THIRD EXAMPLE
COMDINED PRESSURE-VOLUME LOADING
IN CONGENITAL HEART DISEASE
REPAIRED TETRALOGY OF FALLOT
Dept. Imaging Sciences &
Biomedical Engineering
Echocardiography for RV dysfunction in CHD
Dept. Imaging Sciences &
Biomedical Engineering
Mertens, L. L. & Friedberg, M. K. (2010)
Nat. Rev. Cardiol. doi:10.1038/nrcardio.2010.118
Echocardiography for RV dysfunction in CHD
Dept. Imaging Sciences &
Biomedical Engineering
Mertens, L. L. & Friedberg, M. K. (2010)
Nat. Rev. Cardiol. doi:10.1038/nrcardio.2010.118
Gold standard MRI for Adult CHD
Dept. Imaging Sciences &
Biomedical Engineering
MRI for RV function
axial
Dept. Imaging Sciences &
Biomedical Engineering
short axis
Normal percentiles for RV
function in kids and adults
Gender- differences...
Dept. Imaging Sciences &
Biomedical Engineering
...gender-specific percentiles
Sarikouch S, Peters B, …, Gutberlet M, Kuehne T, Beerbaum P.
Circulation – Cardiovasc Imaging. 2010
RV-EF is worse in females as
related to sex-specific percentiles
RV-EDV in
ml/m²I
220
female
RV EF
p=0,011
200
180
160
140
120
P97
P90
P75
P50
P25
P10
P3
100
80
60
40
4
5
6
7
RV-EDV in
8
9 10 11 12 13 14 15 16 17 18 19
age (years)
male
220 ml/m²
200
180
160
140
P97
P90
P75
P50
P25
P10
120
100
80
60
P3
40
6
7
8
9
10 11 12 13 14 15 16 17 18 19 20
age (years)
Dept. Imaging Sciences &
Biomedical Engineering
Sarikouch S, Boethig D, Peters B, Kuehne T, Beerbaum P. Circulation (under review)
MRI for viability & scar imaging
in r-TOF
RV
Dept. Imaging Sciences &
Biomedical Engineering
LV
Babu-Narayan S et al. Circulation 2006
... And is related to outcome!
Dept. Imaging Sciences &
Biomedical Engineering
Babu-Narayan S et al. Circulation 2006
... and to RV free wall dyskinesis
Segments of
the RV
Three-dimensional
surface models of the
RV free wall
Dept. Imaging Sciences &
Biomedical Engineering
Wald R, Geva T et al. Circulation 2009
Segmental distribution of
regional RV abnormalities
... and thus again to outcome, because RV
dyskinesis correlates with RV-EF, which in
turn predicts adverse events such as SCD,
VT, increase in NYHA class
Correlations between global RV EF
and area-weighted spatial extent of
•dyskinetic area (A)
•magnitude of RV dyskinesis (B)
Dept. Imaging Sciences &
Biomedical Engineering
Wald R, Geva T et al. Circulation 2009
Is identification of early right
ventricular dysfunction possible?
 Yes – in primary myocardial disease (e.g., ARVC),
particularly if tissue information or regional wall motion
is obtained
 Otherwise – very difficult to discriminate dysfunction from
adaption and failure
 Longitudinal studies related to outcome to better understand if a
parameter qualifies as detector of early RV dysfunction
 Tissue-level imaging most likely to qualify
 Fibrotic & metabolic changes may precede regional / global wall
motion abnormality
 EXAMPLEs: FDG-PET in PAH, MRI fibrosis imaging,
hyperpolarised MRI
Dept. Imaging Sciences &
Biomedical Engineering
Acknowledgements
Samir Sarikouch, Andrea Kelter-Kloepping, Siegfried Kropf, Birgit Peters,
Titus Kuehne, Hashim Abdul-Khalid, Ulrike Bauer, Peter Lange
Kompetenznetz AHF, Berlin
Hermann Körperich, Peter Barth, Hermann Esdorn, Andreas
Peterschroeder, Thorsten Laser, Hans Meyer
Herz- und Diabeteszentrum NRW, Bad Oeynhausen
Sebastian Kozerke, Kawal Rhode, Graeme Penney, Gerald Greil, Eike
Nagel, Sven Plein, Israel Valverde, Vicky Parish, Tarique Hussein,
Hannah Bellsham-Revell, Aaron Bell, Kerstin Lenk, John Simpson,
Aphrodite Tzifa, Shak Qureshi, Rene Botnar, Tobias Schaeffter, Reza
Razavi
King’s College London / Evelina Children’s Hospital, London, UK
Dept. Imaging Sciences &
Biomedical Engineering
Many thanks!
Dept. Imaging Sciences &
Biomedical Engineering
NEW HORIZONS
INTRAVENTRICULAR FLOW?
Dept. Imaging Sciences &
Biomedical Engineering
Particle pathlines
Visualisierung des Blutflusses herznaher
grosser Gefässe
Uribe S, Beerbaum P, Schaeffter T, et al. Magn Reson Med 2009
Dept. Imaging Sciences &
Biomedical Engineering
Particle pathlines
Visualisierung & Quantifizierung des
intrakardialen Blutflusses
Inflow
Dept. Imaging Sciences &
Biomedical Engineering
Outflow
Eriksson J, et al. JCMR 2011
NEW HORIZONS
REALTIME-MRI FOR STRESS IMAGING?
Dept. Imaging Sciences &
Biomedical Engineering
Golden Angle - Retro Motion Correction
Data for each cardiac phase is collected over
multiple RR intervals and corrected for respiratory
motion deformation.
Motion Correction
Dept. Imaging Sciences &
Biomedical Engineering
Courtesy: Michael Schacht Hansen, NIH, USA
NEW HORIZONS
IN-VIVO MYOCARDIAL FIBER TRACKING??
Dept. Imaging Sciences &
Biomedical Engineering
Kardiale Diffusionsbildgebung
Niedriges SNR, lange Messzeiten
Dept. Imaging Sciences &
Biomedical Engineering
Courtesy: Toussaint N, Batchelor PG, KCL
Kardiale Diffusionsbildgebung (DT-MRI)
Diffusions-Tensor in 2 Richtungen, mehrere axiale 2DSchichten: 3D-Interpolation
Dept. Imaging Sciences &
Biomedical Engineering
Courtesy: Toussaint N, Batchelor PG, KCL
NEW HORIZONS
METABOLIC IMAGING??
Dept. Imaging Sciences &
Biomedical Engineering
Hyperpolarisierte 13C Spektroskopie
im präklinischen Modell
13C-Pyruvat
13C-Laktat
13C-Bicarbonat
Echo-planar
Spektroskopie (EPSI)
Sequenz
•
2mm x 2mm
•
Zeit-Intervall 5 sec
Dept. Imaging Sciences &
Biomedical Engineering
Courtesy: Tom Eykyn & Sebastian Kozerke, KCL, London
RV-“shape“ & regionale Wandbewegung aus
2D+time Datensätzen des KN-AHF
1.
3D Reformatierung der 2D+time Daten
2.
Statistische (=quantitative) Beschreibung des einzelnen Patienten
durch Registrierung der individuellen RV-Form bezogen auf eine
Fallot-spezifische Referenzform des RV
3.
Mathematische Deskription des individuellen Remodellings
Atlas
S[1]
1
5
Patient 1
Patient 5
2
3
S[k]
4
Patient
S[10]
Patient 2
Dept. Imaging Sciences &
Biomedical Engineering
Patient 3
Patient 4
Sermesant M, Tommaso M, Beerbaum P, Ayache N. WIP
Shape vector s
Reversible RV dysfunction after conversion from
parallel to serial circulation in HLH syndrome
Bellsham-Revell HR, …, Simpson J, …, Beerbaum P,Anderson D, Greil GF, Razavi R.
Serial MRI in hypoplastic left heart syndrome avoids routine catheterization and
gives insights into cardiac and vascular remodeling. Circulation (under review)
Dept. Imaging Sciences &
Biomedical Engineering