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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