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Sophie Mavrogeni MD FESC

Onassis Cardiac Surgery Center

Athens Greece

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.

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.

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

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

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

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

Congenital Heart Disease (CHD)

Causes of RV dilatation in CHD

• Atrial septal defect

• Pulmonary valve dysfunction

• Tricuspid valve dysfunction

• Diverticula and aneurysm

Atrial septal defect

Beitzke D et al. Br J Radiol. 2011

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

Fallot tetralogy

Beitzke D et al. Br J Radiol. 2011

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

Epstein anomaly

Beitzke D et al. Br J Radiol. 2011

Tricuspid Regurgitation

Beitzke D et al. Br J Radiol. 2011

Diverticula and aneurysm

Beitzke D et al. Br J Radiol. 2011

Congenital absence of the pericardium

Brulotte S et al. Can J Cardiol. 2007

PULMONARY HYPERTENSION

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

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

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

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

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

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

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

AUTOIMMUNE DISEASES WITH RV

INVOLVEMENT

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

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

AMYLOIDOSIS

Prabhakar Rajiah et al. Indian J Radiol Imaging. 2012

CARDIAC SARCOIDOSIS

Shaunagh McDermott, et al. World J Cardiol. 2012

Utility of CMR in assessing right-sided heart

failure in sarcoidosis

Lonborg J et al. BMC Med Imaging. 2013

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

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

ARRYTHMOGENIC RV CARDIOMYOPATHY

(ARVC)

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)

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%

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

ARRHYTHMOGENIC RIGHT VENTRICLE (ARVC)

Mavrogeni S et al. Hell J Cardiol 2007

Naxos Disease

Valsangiacomo ER et al. Eur Heart J 2012

Naxos disease evolution mimicking acute

myocarditis: The role of CMR

Mavrogeni S et al Int J Cardiol 2013

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

OTHER CARDIOMYOPATHIES

RV TAKOTSUBO CARDIOMYOPATHY

Korlakunta H et al. Tex Heart Inst J 2011

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

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

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

ISCHEMIC HEART DISEASE

Rupture of right coronary sinus of Valsalva

aneurysm into RV

Post MC et al. Neth Heart J. 2010

Premature myocardial infarction presenting

with acute pulmonary embolism

Gopaluni S et al. J Med Case Reports. 2009

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

CARDIAC TUMORS INVOLVING RV

Large RV fibroma in a 6-month-old infant.

Horovitz A et al. Pediatr Cardiology 2012

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

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

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

Greek College of Clinical Applications

in CMR

“CARDIOTOMI”

EuroCMR/SCMR level 1

30/9-2/10/2017

in Metropolitan Hotel

Endorsed by EACVI/SCMR

and accredited by EBAC

“Transform CRISIS into INSPIRATION”

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