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Απεικόνιση της δεξιάς κοιλίας.
Πότε η υπερηχοκαρδιογραφία δεν είναι αρκετή;
Sophie Mavrogeni MD FESC
Onassis Cardiac Surgery Center
Athens Greece
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RV structure and function (1)
• RV differs from the LV
• (more complex shape being ‘wrapped around’ the LV).
• This complex geometry precludes imaging the inflow and
outflow tract in a single two-dimensional plane.
• Compared with the LV, the RV myocardium is significantly
more trabeculated
• The RV wall is much thinner with a normal compacted wall
thickness of 3–5 mm in the adult population.
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RV structure and function (2)
• LV wall has a 3-layered structure with the epicardial cells oriented obliquely, the midmyocardial cells circumferentially and the endocardial cells again obliquely.
• The midwall circumferential layer is responsible for predominanceof circumferential shortening and radial thickening in LV.
• RV epicardial fibres are oriented obliquely and contiguous withepicardial LV fibres, the midwall circumferential layer is poorlydeveloped and the endocardial fibres are oriented longitudinally.
• This fibre structure explains why RV ejection is determined by longitudinal shortening rather than by circumferential deformation.
• The normal RV contraction results in a PERISTALTIC contraction from the inflow to the outflow part of RV.
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Echocardiography for RV assessment
• RV morphology can be adequately described by TTE in most patients.
• Recent guidelines recommend quantitative RV function by using at least one of the following as surrogate of RV volumetric assessment:
• Percent fractional area change (FAC),
• Tricuspid annular plane systolic excursion (TAPSE),
• RV index of myocardial performance (RIMP)
• FAC: correlates with RV EF by CMR, but visualization of endocardial borders are limited mainly in RV lateral wall and RV apex.
• TAPSE: easy but normal values are limited and TR may influence the values obtained.
• RIMP: load dependent and due to short RV isovolumic time intervals, its use remains controversial.
Valsangiacomo ER et al. Eur Heart J 2012
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Limitations of speckle tracking in RV
assessment
• Strain values are influenced by loading conditions, as it has been
demonstrated in patients with PAH, in whom RV longitudinal strain
was related to pulmonary arterial systolic pressures.
• Strain values are influenced by RV size and stroke volume.
• Feasibility is poor in the thin RV wall; still NO normal values
• Standardization among different software still under investigated.
• TDI and speckle tracking are NOT READY yet for routine use.
Valsangiacomo ER et al. Eur Heart J 2012
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CMR in pulmonary hypertension
• Cardiac morphology, function and mass
• Ventricular mass index (VMI), obtained by dividing the mass of RV by the mass of LV (sensitivity 84%, specificity 71%, strong correlation with MPAP by RHC)
• IVS configuration
• Late gadolinium enhancement (LGE)
• Pulmonary circulation (Quantification of the pulmonary flow profile revealed a reduction in the peak flow velocity in the main pulmonary artery in patients with PAH).
• Distensibility of pulmonary artery
• Stress CMR (reduced MPRI)
• RV remodeling after treatment for PAH
Dimitroulas T, Mavrogeni S, Kitas GD. Nature Review Rheumatology 2012
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Clinical application of non-invasive
imaging in conditions affecting the RV
• Congenital heart disease (CHD)
• Pulmonary arterial hypertension (PAH)
• Autoimmune diseases involving RV
• Arrhythmogenic RV Cardiomyopathy (ARVC) and other cardiomyopathies involving the RV
• Ischaemic RV disease and RV failure
• Cardiac tumors involving RV
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Congenital Heart Disease (CHD)
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Causes of RV dilatation in CHD
• Atrial septal defect
• Pulmonary valve dysfunction
• Tricuspid valve dysfunction
• Diverticula and aneurysm
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Atrial septal defect
Beitzke D et al. Br J Radiol. 2011
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Superior sinus venosus atrial septal defect (ASD)
with associated partial anomalous pulmonary
return of the right upper lobe pulmonary vein
Beitzke D et al. Br J Radiol. 2011
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Fallot tetralogy
Beitzke D et al. Br J Radiol. 2011
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A 29-year-old woman with partial
anomalous pulmonary venous return
• A) MR angiography (a) subtracted
image shows anomalous drainage
of both superior and inferior right
pulmonary veins (white arrows)
into the superior vena cava
• B) subsequent signs of RV
volume overload on cine-SSFP (b,
c RV enlargement + septal
flattening) without hypertrophy
reflecting low pulmonary vascular
resistance
Galea N et al. Insights Imaging. 2013
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Epstein anomaly
Beitzke D et al. Br J Radiol. 2011
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Tricuspid Regurgitation
Beitzke D et al. Br J Radiol. 2011
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Diverticula and aneurysm
Beitzke D et al. Br J Radiol. 2011
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Congenital absence of the pericardium
Brulotte S et al. Can J Cardiol. 2007
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PULMONARY HYPERTENSION
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Diagnostic accuracy of CMR of RV morphology
and function in the assessment of suspected
pulmonary hypertension: ASPIRE registry.
• Ventricular mass index (VMI) was the CMR measurement
with the strongest correlation with mPAP (r = 0.78) and the
highest diagnostic accuracy for the detection of PAH (area
under the ROC curve of 0.91) compared to an ROC of 0.88
for echocardiography calculated mPAP.
• LGE, VMI ≥ 0.4, retrograde flow ≥ 0.3 L/min/m² and PA
relative area change ≤ 15% predicted the presence of PH
with a high degree of diagnostic certainty with a positive
predictive value of 98%, 97%, 95% and 94% respectively.
• No single CMR parameter could confidently exclude PAH.
Swift AJ et al, J Cardiovasc Magn Reson 2012
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Short axis slice through the ventricles showing
epicardial and endocardial border segmentation of
the RV (white) and the LV (red).
• The inter-venticular septum is included in the LV mass, Ventricular mass index (VMI) is defined as RV mass divided by LV mass.
• The endocardial borders of the RV and LV were traced for calculation of EDV and ESV.
• EDV and ESV were calculated by summation of the product (area × slice distance) for all slices. SV is given by SV = EDV-ESV calculated for both RV and LV.
Swift AJ et al, J Cardiovasc Magn Reson 2012
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Pulmonary Arterial Hypertension:
MR Imaging-derived First-Pass Bolus Kinetic Parameters
Are Biomarkers for Pulmonary Hemodynamics, Cardiac
Function, and Ventricular Remodeling
Skrok J et al, Radiology 2012
CE MR-derived PTT, LV FWHM, and LV TTP are noninvasive compound markers
of pulmonary hemodynamics and cardiac function in patients with PAH. Their
predictive value for patient outcome needs further investigation
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Contrast-enhanced MDCT vs. Time-resolved MRA vs.
contrast-enhanced perfusion MRI: assessment of
treatment response by patients with inoperable CTEPH.
• Dynamic perfusion MRI has better capability for
assessment of therapeutic effect on CTEPH
patients than does MDCT.
Ohno Y et al, J Magn Reson Imaging 2012
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Idiopathic pulmonary hypertension
• Severe idiopathic pulmonary hypertension; PAP at right cath 70 mmHg
• Focal LGE are observed at the level of both ventricular junctions.
• Severe concentric RV hypertrophy on short-axis cine-SSFP withflattening and inversion of the IVSduring contraction (c)
Galea N et al. Insights Imaging. 2013
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LGE CMR predicts clinical worsening in PAH
• The presence of
RVIP-LGE in patients
with PH is a marker
for more advanced
disease and poor
prognosis.
• CMR-derived RVEF is
an independent non-
invasive imaging
predictor of adverse
outcomes PH.
Freed BH et al, J Cardiovasc Magn Reson 2012
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Abnormalities of Pulmonary Vasculature in PAH
• (A) White blood anatomy showing right upper pulmonary vein stenosis at the site of a prior ablation for AF (arrow).
• (B) Congestion and infarction in the right upper lobe (asterisk).
• (C) MRA from a patient with PH due to fibrosing mediastinitis; a varix is seen bypassing a stenosed left upper pulmonary vein (not shown) alongside stenoses of both right sided pulmonary veins (arrows).
• (D) MRA in patient with CTPH. The most striking feature is loss of the left descending pulmonary artery (arrow head).
Bradlow WM et al, J Cardiovasc Magn Reson 2012
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AUTOIMMUNE DISEASES WITH RV
INVOLVEMENT
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Is there a place for CMR in the evaluation of
cardiovascular involvement in rheumatic diseases?
• CMR is a noninvasive, nonradiating imaging technique, which provides novel information for the evaluation of cardiovascular diseases.
• Currently, it is considered the gold standard for the evaluation of volumes, mass, ejection fraction of atriums and ventricles, quantification of iron overload in different organs, detection and follow-up of myocardial inflammation, myocardial infarction and its complications, evaluation of the aorta, detection of anomalous coronary arteries, and ectatic or aneurysmatic coronary arteries.
• All the above applications and mainly the CMR ability to detect myocardial inflammation, perfusion defects, fibrosis, coronary and great arteries aneurysms make it a valuable tool for cardiovascular system assessment, commonly affected during the course of rheumatic diseases.
• The technique has been already successfully used in the evaluation of vasculitides, systemic lupus erythematosus, myositis, and scleroderma.
Mavrogeni S et al, Semin Arthritis Rheum 2011
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Cardiovascular magnetic resonance in
rheumatology: Current status and
recommendations for use.
• The present report outlines the recommendations of the participating CMR and rheumatology experts with regards to:
• (a) indications for use of CMR in rheumatoid arthritis, the spondyloarthropathies, systemic lupus erythematosus, vasculitis of small, medium and large vessels, myositis, sarcoidosis and scleroderma;
• (b) CMR protocols, terminology for reporting CMR and diagnostic CMR criteria for assessment and quantification of cardiovascular involvement in CTDs;
• (c) a research agenda for the further development of this evolving field.
Mavrogeni S et al Int J Cardiol 2016
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AMYLOIDOSIS
Prabhakar Rajiah et al. Indian J Radiol Imaging. 2012
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CARDIAC SARCOIDOSIS
Shaunagh McDermott, et al. World J Cardiol. 2012
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Utility of CMR in assessing right-sided heart
failure in sarcoidosis
Lonborg J et al. BMC Med Imaging. 2013
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Comparison of the diagnostic utility of CMR, CT, and Echo
in assessment of suspected PAH in CTDs
• 81 patients with CTD, 55 had PAH, 22 had no PH, and 4 had PH due to LV disease.
• There was good correlation between mPAP and PVR measured by RHC
• VMI derived from MRI (mPAP, r = 0.69, p < 0.001; PVR, r = 0.78, p < 0.001) and
• Systolic area ratio (mPAP, r = 0.69, p < 0.001; PVR, r = 0.68, p < 0.001) and
• TG derived from echo (mPAP, r = 0.84, p < 0.001; PVR, r = 0.76, p < 0.001).
• In contrast, CT measures showed only moderate correlation.
• VMI ≥ 0.45 had a sensitivity of 85% and specificity 82%;
• TG ≥ 40 mm Hg had a sensitivity of 86% and specificity 82%.
• Cox regression analysis showed that CMR was better at predicting mortality.
• Patients with RV end diastolic volume < 135 ml had a better prognosis than those with a value > 135 ml, with a 1-year survival of 95% versus 66%, respectively.
Rajaram S et al, J Rheumatol 2012
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Pulmonary blood volume indexed to lung volume is
reduced in newly diagnosed SSc compared to normals –
a prospective CMR study addressing PV changes
• This study is the first to measure the PBV in humans using
CMR. Compared to healthy controls, newly diagnosed SSc
patients have a reduced amount of blood in the pulmonary
vasculature (PBVI) but unchanged pulmonary vascular
distensibility (PBVV/stroke volume).
• PBVI is unrelated to DLCO, pulmonary artery pressure, vital
capacity, and the presence of pulmonary fibrosis. PBVI may
be a novel parameter reflecting vascular lung involvement
in early-stage SSc, and these findings may be consistent
with pathophysiological changes of the pulmonary
vasculature.
Kanski M et al, J Cardiovasc Magn Reson 2013
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ARRYTHMOGENIC RV CARDIOMYOPATHY
(ARVC)
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Structural and functional criteria for ARVC
Major criteria
2D echo Regional RV akinesia, dyskinesia, or aneurysm
And 1 of the following (end-diastole):
RVOT ≥32 mm (19 mm/m2)/parasternal long-axis view
RVOT ≥36 mm (21 mm/m2)/parasternal short-axis view
or RV fractional area change ≤33%
CMR Regional RV akinesia or dyskinesia, or dyssynchronous RV
contraction
And 1 of the following:
RV end-diastolic volume ≥110 mL/m2 (male) or ≥100 mL/m2 (female)
or RV ejection fraction ≤40%
Imaging task force criteria for diagnosing arrhythmogenic
right ventricular cardiomyopathyopathy (ARVC)
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Imaging task force criteria for diagnosing arrhythmogenic
right ventricular cardiomyopathyopathy (ARVC)
• Minor criteria
• 2D echo Regional RV akinesia or dyskinesia
• And 1 of the following (end-diastole):
• RVOT ≥29 mm ,32 mm (≥16 ,19 mm/m2)
• RVOT ≥32 ,36 mm (≥18 ,21 mm/m2)
• or fractional area change 33 to ≤40%
• CMR Regional RV akinesia or dyskinesia, or dyssynchronous RV contraction
• And 1 of the following:
• RV end-diastolic volume ≥100 ,100 mL/m2 (male) or ≥90 ,100 mL/m2 (female) or RV ejection fraction 40 ≤45%
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ARRYTHMOGENIC RIGHT VENTRICLE (ARVC)
• Dilated hypokinetic RV
• Increased RVESV, RVEDV
• Localized aneurysms
• RV-free wall bulging
• Increased signal intensity from fibrofatty myocardial replacement of RV after Gadolinium
• Mild decrease of LV function in 15% of cases
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ARRHYTHMOGENIC RIGHT VENTRICLE (ARVC)
Mavrogeni S et al. Hell J Cardiol 2007
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Naxos Disease
Valsangiacomo ER et al. Eur Heart J 2012
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Naxos disease evolution mimicking acute
myocarditis: The role of CMR
Mavrogeni S et al Int J Cardiol 2013
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The triangle of dysplasia in ARVC
• (A) Structural anomalies can
be observed in a region
including the subtricuspidal
RV wall, the RV apex, and the
RV outflow tract.
• (B) Steady-statefree
precession images showing
severe aneurysmatic
abnormalities of the RV free
wall subtricuspidal (arrows) in
a patient with ARVC
Valsangiacomo ER et al. Eur Heart J 2012
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OTHER CARDIOMYOPATHIES
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RV TAKOTSUBO CARDIOMYOPATHY
Korlakunta H et al. Tex Heart Inst J 2011
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Biventricular hypertrophic
cardiomyopathy• (a). Severe concentric
biventricular hypertrophy
• Inhomogeneous bi-ventricular spotty areas of signal hyperintensity are depicted on T2WI four-chamber (a) view reflecting diffuse myocardial oedema;
• LGE image (b) shows extensive tissue damage with late enhancement involving both ventricles including the RV free apical wall (arrow).
• (c) EMB confirms a severe hypertrophy and disarray of myocardiocytes,interrupted by fibrosis
Galea N et al. Insights Imaging. 2013
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Hypertrophic Cardiomyopathy in a Young Adult
with RV Aneurysm: Report of a Rare Finding
and Review of the Literature.
Abdel-Razek AM et al. Heart Views 2011
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Glycogen storage disease in a 29-year-old man
with family history of juvenile sudden cardiac
death and unexplained increased LV wall
thickness at echocardiography
• a. T2WI shows an hyper-trabeculated RV with subendocardial hyperintense signal related to “slow-flow” caused by diffused hypokinesis.
• b. LGE-T1WI reveals a diffuse and homogeneous enhancement of the RV myocardium (black arrows) and right side of the IVS (arrowheads).
• The LGE of the LV is subepicardial (white arrows) in lateral wall.
• c. EMB shows massive accumulation of citosolic glycogen, sometimes engulfing autophagosoms (asterisk)
Galea N et al. Insights Imaging. 2013
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ISCHEMIC HEART DISEASE
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Rupture of right coronary sinus of Valsalva
aneurysm into RV
Post MC et al. Neth Heart J. 2010
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Premature myocardial infarction presenting
with acute pulmonary embolism
Gopaluni S et al. J Med Case Reports. 2009
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RV Injury in ST-elevation myocardial
infarction
• Severe inferior infarction:
• A, proximal occlusion of RCA
• B, After PCI to RCA
• C, Area at risk extends from
the LV inferior wall to the IVS
and RV inferior and free wall.
• D, microvascular obstruction
of the LV and scar area
Grothoff M et al Circ Cardiovasc Imaging 2012
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CARDIAC TUMORS INVOLVING RV
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Large RV fibroma in a 6-month-old infant.
Horovitz A et al. Pediatr Cardiology 2012
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RV fibroelastoma
• RV fibroelastoma in an
asymptomatic 32-year-old
man with an intracavitary
nodule incidentally
depicted at trans-thoracic
echocardiography.
• TSE T1-weighted image
shows the presence of a
nodular rounded RV mass
(arrow) attached to a large
trabecula
Galea N et al. Insights Imaging. 2013
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RV metastasis
• RV metastasis of renal
cancer in a 62-year-old man
(histologically proven).
• A large ovoid secondary
lesion is depicted at the
lateral atrio-ventricular
junction compressing the
RV free wall
Galea N et al. Insights Imaging. 2013
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CONCLUSIONS
• Comprehensive, operator independent study by CMR
• Assessment of function, mass, morphology, oedema,
fibrotic changes, infiltrative processes, lung circulation
dynamics
• Ability to assess remodeling, viability, perfusion, oedema
• Useful to monitor therapy
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Greek College of Clinical Applications
in CMR
“CARDIOTOMI”
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EuroCMR/SCMR level 1
30/9-2/10/2017
in Metropolitan Hotel
Endorsed by EACVI/SCMR
and accredited by EBAC
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“Transform CRISIS into INSPIRATION”
THANK YOU!