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Eur Heart J Cardiovasc Imaging Abstracts Supplement, 2017

doi:10.1093/ehjci/jex121

CASES FROM EASTERN EUROPE AND AROUND THE WORLD

153. CIRCUMFLEX CORONARY ARTERY ORIGINATING FROM THE
RIGHT PULMONARY ARTERY IN A PATIENT WITH COARCTATION OF
THE AORTA
 1, L KORNYEI
A TOTH 2
, A SZATMARI  2, L MOLNAR 1, P MAUROVICH-HORVAT1,
 O
H VAG  1, T SIMOR3, B MERKELY1
1 1
Semmelweis University, Heart and Vascular Center, Budapest, HUNGARY;
2
Gottsegen Gyorgy Hungarian Institute of Cardiology, Budapest, HUNGARY;
3
University of Pecs, Heart Institute, Pecs, HUNGARY
Objectives: Patient history: 24 year old female patient came for a follow-up cardiac MR
exam due to coarctation of the aorta treated with isthmical patch plastic surgery. Stent
placement procedure is planned in the future, the patient also had atypical complains.
Coronary fistula has been described previously.
MR imaging : Cardiac MR exam was carried out using a 1.5T Philips Achieva imaging in-
strument. It included transversal stack of slices and views tilted according to the thoracic
aorta and the aortic arch. The aortic valve was also evaluated. Flow measurements were
taken at the isthmical region, above the diaphragm and at the aortic valve. Non-contrast
enhanced, T2-prep, fat suppressed 3D balanced SSFP MRA have been acquired covering
the thoracic aorta in the parasagittal view using respiratory navigation.
Findings: According to the MR data, 35 Hgmm gradient could be determined at the site of
the prior coarctation, the smallest diameter was 12mm according to a circular stenosis. The
bicuspid aortic valve (LR-type) showed no metionable stenosis (Vmax: 209 cm/s) or regur-
gitation (2%). High resolution 3D MR images demonstrated well defined left main (LM) and
right coronary arteries (RCA). The left anterior descending (LAD) artery followed a normal
course, while it could be also demonstrated, that the circumflex artery (CX) originates from
the right pulmonary artery (RPA).
Further imaging and intervention: Conventional catheter angiography confirmed the find-
ings of the cardiac MR exam: the origin of the CX from the RPA and its unusual course.
Marked collateralisation between the CX and the other two coronaries could be also visual-
ized. The patient underwent successful stenting of the coarctation. Future plans with the
ALCAPA variant is still under consideration.
Discussion: Complex cardiac MR protocol can demonstrate unsuspected additional find-
ings havent been registered before despite multiple follow-ups. This kind of ALCAPA vari-
ant is well known, theres also a prior publication of a case combined with coarctation of the
aorta in the literature. This is the first time to our knowledge the abnormality was discovered
using cardiac MR. This is the second patient with this type of coronary anomaly.
154. RIGHT VENTRICULAR FAILURE SECONDARY TO REVERSIBLE
PERICARDIAL INFLAMMATION AND CONSTRICTIVE-EFFUSIVE
PERICARDITIS : THE VALUABLE ROLE OF CMR IN DIAGNOSIS AND
MANAGEMENT
M PAGE  1, M EL-RAYES1, C ZAIANI1, M ARCHAMBAULT1, C SAMSON1
Hopital du Sacre-Coeur de Montreal, Montreal, CANADA
Objectives: An 80 year-old male patient with a history of hypertension and mild aortic re-
gurgitation was admitted for decompensated right ventricular failure with peripheral edema
and bilateral pleural effusions. Transthoracic echocardiogram (TTE) revealed a moderate-
to-severe circumferential pericardial effusion with a paradoxical septum on M-mode. The
left ventricular (LV) cavity was not dilated and the measured LV ejection fraction (EF) was
55%, with mild-to-moderate aortic regurgitation and trivial mitral regurgitation. Visualisation
of the right ventricle was suboptimal, but the right-sided chambers appeared slightly dilated,
with moderate tricuspid regurgitation. The inferior vena cava was dilated and fixed. Due to
enlarged liver, the usual windows for pericardial tap were judged unsafe. Blood chemistry
revealed a mild acute renal failure and mild leukocytosis. C-reactive protein was moder-
ately elevated, and the patient had no fever or systemic manifestations. The patient was
put on intra-venous furosemide boluses, and then on furosemide infusion.
After 7 days of aggressive diuresis and pleural drainage, persisting signs of right heart fail-
ure and progressive renal failure, it was decided to perform a CMR scan to rule-out infiltra-
tive process with restrictive cardiomyopathy. The pericardial effusion was measured
around 1.5cm. Cine sequences confirmed a small-cavity LV with an LVEF of 51%, normal-
sized RV with mildly reduced RVEF (43%) and severely dilated atria. On real-time imaging,
a frank inspiratory septal flattening was seen (fig1AB). TI-scout showed a normal nulling
pattern. No myocardial late-gadolinium enhancement was observed. The pericardial layers
Abstract 154 Figure.

Abstract 153 Figure.

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were slightly thickened (5-6mm) with frank late-gadolinium enhancement, suggesting acute
pericardial inflammation (fig2). Because the patient had relative contre-indication to non-
steroidal anti-inflammatory agents, a trial of colchicine was started and the response was
drastic with rapid reduction of the peripheral edema, pleural and pericardial effusion, as
confirmed by TTE 5 days later, and a radical clinical improvement. Colchicine was contin-
ued for 3 months after discharge. No recurrence had occurred as of one-year follow-up.
This case is an exemple of reversible constrictive-effusive pericarditis in which CMR
allowed the accurate diagnosis, ruled-out restrictive cardiomyopathy and guided the man-
agement. Anti-inflammatory treatment with colchicine alone was successful.
155. SUBEPICARDIC HEMATOMA - A RARE POST-ABLATION
COMPLICATION
A NICULA1, A MARINESCU1, A MARINESCU1, G IANA1, C SILISTE2, D VINEREANU2
1 157. PECTINEAL MUSCLE HYPERTROPHY IN TWO SIBLINGS WITH
Emergency University Hospital, Imaging Department, Bucharest, ROMANIA;
2
Emergency University Hospital, Cardiology Department, Bucharest, ROMANIA
Objectives: A 48-year-old female was admitted for radiofrequency ablation in the setting of
frequent symptomatic monomorphic ventricular premature beats (VPB), resistant to amio-
darone and beta-blockers. Cardiac clinical examination was normal, while resting 12-lead
ECG showed monomorphic VPB of presumed left ventricular (LV) origin, without other
abnormalities. Precordial echocardiography showed mildly dilated LV with increased sferic-
ity index, but preserved LV ejection fraction. Cardiac magnetic resonance confirmed mildly
dilated LV with borderline LVEF, and no myocardial late enhancement.
Cardiac activation and pace-mapping revealed the arrhythmogenic site, at the level of the
posterior mitral annulus. Radiofrequency ablation was performed, with good efficacy
criteria.
Two hours after the procedure, the patient developed pericardial pain without signs of
hemodynamic compromise. Precordial echocardiography showed circumferential pericar-
dial effusion and a hyperechoic subepicardial mass, adjacent to the ablation zone.
Emergency contrast-enhanced computed tomography described only a 21 mm hemoperi-
cardium, adjacent to the infero-basal LV wall. However, cardiac magnetic resonance imag-
ing, performed 32 hours post-ablation, identified a round subepicardial area with
hyperintense signal in T1 Black Blood and T2 Black Blood sequences, at the level of the
infero-lateral LV wall (basal section), which bulged the adjacent visceral pericardium and
the sero-hematic fluid above. The multi-imaging modalities concluded the final diagnosis of
subepicardial hematoma with hemopericardium.
The patient was managed conservatively and had a favorable evolution, with rapid reso-
lution of pain and with near-complete regression of pericardial effusion at discharge.
Discussion: Subepicardial hematoma is a known complication of myocardial infarction,
thrombolysis, chest trauma, and percutaneous coronary interventions (particularly in previ-
ously by-passed patients), but very rare described following radiofrequency ablation.
Keywords: ablation, subepicardial hematoma, MRI.
156. LIBMAN-SACKS ENDOCARDITIS - AN UNUSUAL PRESENTATION
M MIGLIORANZA1, P SALGADO-FILHO1, P SCHWARTSMAN2, LH SAITO3,
JR LOPES3, AC GUEDES3, LAK SANTOS3, ALG SCARSI3, JC HAERTEL1
1
Cardiology Institute of Rio Grande do Sul and PREVENCOR - Ma ~e de Deus
Hospital, Porto Alegre, BRAZIL; 2Ma ~e de Deus Hospital, Porto Alegre, BRAZIL;
3
Cardiology Institute of Rio Grande do Sul, Porto Alegre, BRAZIL
Objectives: INTRODUCTION: Cardiac masses are generally a challenging diagnosis, es-
pecially in asymptomatic and young patients.
CASE REPORT: A 29yrs-old woman was referred for a cardiac evaluation due to a new
onset of arterial hypertension. She had a history of three spontaneous abortions in the last
4 years with a recent diagnosis of antiphospholipid syndrome. During the echocardio-
graphic evaluation, a huge mass (3 x 4 x 3,5 cm) with homogeneous echogenicity and regu-
lar spheric shape was detected attached to septal tricuspid leaflet. Due to the possibility of
Abstract 155 Figure.
Eur Heart J Cardiovasc Imaging Abstracts Supplement, 2017
a thrombus, an anticoagulation therapy as chosen as initial therapy. After 3 weeks of anti-
coagulation, a new echocardiographic study was performed and no chance in mass size or
aspect were detected. A cardiac magnetic resonance (CMR) study was performed to better
characterize the mass etiology and demonstrated no penetration of contrast and absence
of necrosis, reinforcing the thrombus hypothesis. The patient underwent to cardiac surgery
for mass resection and the anatomo-pathological study confirmed the diagnosis of libman
sacks endocarditis.
CONCLUSION: Libman-Sacks endocarditis is the most characteristic cardiac manifest-
ation of the autoimmune disease systemic lupus erythematosus, commonly involving the
mitral and aortic valves as small masses. It this case, we describe for the first time a diagno-
sis of Libman-Sacks endocarditis presenting with a huge asymptomatic mass on the tricus-
pid valve.
LEFT VENTRICULAR HYPERTROPHY PHENOTYPE WITH
PRESENTATION OF SUPRAVENTRICULAR TACHYCARDIA
LM MOLINA CANCINO1, G MELENDEZ RAMIREZ1, A MEAVE GONZALEZ1,
MV ALVAREZ BRAN1, R CANO ZARATE1
1
Instituto Nacional de Cardiologia - Department of Cardiac magnetic resonance,
Mexico, MEXICO
Objectives: We present the case of 2 siblings with family background of asymmetrical sep-
tal hypertrophy myocardiopathy on the fathers side.
A. Woman 17 years old, suffering since 8 years of age. Symptoms of palpitations and dys-
pnoea and isolated event of wide-complex tachycardia.
Electrograms with pre-excitation. Electrophysiological study at 11 years by Wolf-
Parkinson-White syndrome, with 2 accessory pathways: a left lateral pathway with suc-
cessful ablation, and the other parahisian, mid-septal with anterograde conduction, without
ablation.
Serial electrocardiograms with pre-excitation and auricular growth represented by an in-
crease in voltage of the P wave (fig 1.a).
Cardiac magnetic resonance with hypertrophy predominant in the basal (19 mm) and mid
(17 mm) septum. With hypertrabeculae in the inferior, lateral and anterior wall in mean third
and globally in the apical third. Ventricular mass was estimated at 127 gr. Right ventricle
with increase in trabeculae, stressing a papillary muscle of 15 mm. Right auricle of 49 x
35 mm in its anteroposterior and midlateral dimensions, with an area of 21 cm2. Thickening
in the pectineal muscles of 15 mm. Systolic function: LVEF 65%. RVEF 74%. Late enhance-
ment with gadolinium with increase in the intensity of the signal in hypertrabeculae areas
(fig 2.a).
Genetic study showed a homozygote mutation in the gene PRKAG2/R531G (rs21908990).
B. Man 20 years old, symptoms of dyspnoea, palpitations and dizziness. Debut with auricu-
lar Flutter 3:1. Later with syncope and complete AV blockage. Electrocardiograms with
biventricular hypertrophy and auricular growth (fig 1.b). Cardiac magnetic resonance with
hypertrophy generalized in the left ventricle with predominance of basal and mid sections,
maximum thickening of the septal wall (18 mm). Mass of 209 gr.
Right auricle of 65 x 45 mm (anteroposterior and midlateral). With hypertrophy of pectoral
muscles of 12 mm, of the interatrial septum of 10 mm. and free wall of right ventricle
(10 mm).
Systolic function: LVEF 55%. RVEF 50%. Late enhancement with hyperintensity in the an-
terolateral papillary muscle (Fig 2.b).
Cardiofibrillator (ICD) was implanted with primary prevention.
Awaiting genetic study.
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Abstract 157 Figure.
158. TWO ADULTS WHO UNDERWENT CLASSICAL BLALOCK
TAUSSIG SHUNTS IN CHILDHOOD - TWO STORIES AND TWO
DIFFERENT OUTCOMES, ELUCIDATED BY CMR - DEVELOPING
COUNTRY SCENARIOS
M KAPPANAYIL1, R KANNAN2
1
Amrita Institute of Medical Sciences and Research Centre, Department of Pediatric
Cardiology, Kochi, INDIA; 2Amrita Institute of Medical Sciences and Research Centre,
Department of Radiology, Kochi, INDIA
Objectives: Classical Blalock Taussig shunt involves anastomosis of subclavian-artery to
ipsilateral branch pulmonary artery.In low-resource countries, many patients with complex
CHD main unoperated/partially-palliated resulting in unique, often dramatic pathophysio-
logical outcomes.
We present two illustrative cases of cyanotic CHD palliated at similar ages in childhood, but
having very different outcomes related to the anatomy and physiology of the classical BT
shunt. Both patients were adults (24 years old) when they underwent CMR evaluation for
considering definitive surgery.
Case 1: Complex Cyanotic CHD (situs inversus, dextrocardia, atrioventricular discordance,
large VSD, pulmonary atresia) - palliated with classical BT shunt at 2 years age. Presented
with severe symptoms - effort intolerance, heart failure, SO2 90%.Symptoms appeared
about a year ago, worsening progressively.
Case 2 : Double-outlet-right-ventricle (DORV), large VSD, d-malposed great arteries, se-
vere pulmonic stenosis. He underwent Classical BT shunt at 2.5 years.Remained relatively
symptom-free until recent worsening of effort tolerance.
Both underwent CMR at our centre - GE 1.5TGE SIGNA HDxt scanners with our CHD-
CMR protocol.
Intracardiac anatomy was well elucidated in both patients on 2D SSFP scans - both cases
were amenable to anatomical correction.However physiology was contrasting.
BT shunt was stenotic at the pulmonary artery (PA) end in case 2, while it was wide open in
case 1.
Case 1 showed evidence of previously high blood flow through BT shunt - dilated proximal
branch PAs, However, Phase Contrast showed striking difference in differential pulmonary
blood flow- right lung flows 3.7L/min, left lung flows 12L/min. Gadolinium-enhanced angio-
gram showed poor arborization and rat-tailing typical of pulmonary vascular occlusive dis-
ease (PVOD). Left lung vasculature was normal with high flows.Overall Qp:Qs 0.8:1.
Abstracts ii97
Case 2: Stenotic BT shunt regulated PA blood flows optimally - Right lung 4.8L/min, left
lung 4L/min, Qp:Qs 2.2:1. Bilateral lung arborisations were equal with no signs of PVOD.
Case 1 was not suitable for surgery due to differential PVOD (of right lung) and expired a
few months later. Case 2 was successfully operated and is doing well.
CMR allowed excellent elucidation of not just the anatomy, but the complex physiology that
allowed decision-making.
159. A WEIRD FOCAL WALL MOTION ABNORMALITY FOR
INVESTIGATION
C CHESHIRE1, N NERLEKAR1,2, A NASIS1,2, J. D. CAMERON1,2, S MOIR1,2
1
Monash Heart, Monash Health, Melbourne, AUSTRALIA; 2Monash Cardiovascular
Research Centre, Monash University, Melbourne, AUSTRALIA
Objectives: CASE: A previously well 62-year-old female presented to the emergency de-
partment with central chest pain. Initial electrocardiogram demonstrated sinus rhythm with
ST-elevation in V1-V2 and she underwent emergent coronary angiography which demon-
strated no obstructive coronary artery stenosis, however left ventriculography demon-
strated focal dyskinesis / aneurysm of the mid anterior left ventricular (LV) wall. Contrast
enhanced transthoracic echocardiography (TTE) confirmed the appearance and demon-
strated a pericardial effusion, raising the suspicion of a contained LV rupture.
Cardiac magnetic resonance (CMR) imaging confirmed the presence of an anterior wall an-
eurysm with associated oedema on T2 weighted images (Fig 1) however there was no evi-
dence of associated late gadolinium enhancement (LGE) in the region (Fig 1). The patient
had been involved in an argument before presenting to hospital and the findings were
thought to be most consistent with an atypical Takotsubo cardiomyopathy (TC), and repeat
transthoracic echocardiography 1 month later demonstrated complete normalisation of LV
contraction.
DISCUSSION: TC is a rare syndrome characterized by transient regional systolic dysfunc-
tion of the LV, mimicking myocardial infarction, but in the absence of angiographic evidence
of obstructive coronary artery disease or acute plaque rupture. Focal involvement of the
myocardium is its rarest form. A large multi-centre study in this population found CMR can
accurately identify TC through a typical pattern of LV dysfunction and demonstrates myo-
cardial edema in 81% patients, and no significant LGE in 91%. These findings were consist-
ent with our patient. Interestingly in this study 40% of this patient population also had a
pericardial effusion.
Eur Heart J Cardiovasc Imaging Abstracts Supplement, 2017
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Abstract 158 Figure.

Abstract 159 Figure.

160. CARDIAC MRI CAN REVEAL A CONCEALED PATHOLOGY; LOOK
CAREFULLY
A ABD ELNABY1, M MAHROUS1, A ELSAYED AHMED1, O IBRAHEEM1,
M EL NADY1, S ROMEIH1, W EL MOZY1
1 anterior mitral leaflet. Interventricular septum and posterior wall thickness of 6 mm .There
Aswan heart centre, Aswan, EGYPT
Objectives:
Clinical history
A 53-year-old male, ex-smoker, not diabetic, not hypertensive with no previous cardiovas-
cular history presented with recurrent exertional chest tightness associated with dysnea.
ECG showed left ventricular hypertrophy with T wave inversion in the precordial leads.
Echocardiogram reported normal left ventricular dimensions with good systolic function, No
regional wall motion abnormality, normal cardiac valves in structure and function (Figure 1).
Due to his symptoms, abnormal ECG and risk factors for ischemia, a stress cardiac MRI
(CMR) with adenosine was obtained.
CMR examination was performed on 1.5 T MRI scanner (Siemens Magnetom Aera,
Siemens Medical Systems, Germany ), the imaging protocol includes steady state free
precession
(SSFP) cine images, dynamic scans with adenosine and late gadolinium enhancement
imaging (LGE).
CMR revealed an isolated apical left ventricular hypertrophy (17 mm) with a spade sign
(moustache sign), no left ventricular outflow obstruction and no systolic anterior motion of
was no myocardial fibrosis.
There was a reversible perfusion defect at apical segments with adenosine infusion that
completely disappeared during rest (Figure 2)
Conclusion
By CMR examination a diagnosis of apical hypertrophic cardiomyopathy (HCM) was estab-
lished which might explain the patients symptoms. Apical hypertrophic cardiomyopathy is
a hereditary disease and rare variant of hypertrophic cardiomyopathy. CMR findings initi-
ated a revision of the patients ECG showing that giant T wave inversion in precordial leads
with absent septal Q wave were overlooked signs of apical (HCM). CMR with the use of
various imaging sequences (myocardial tissue characterization and dynamic myocardial
stress images) without any acoustic window limitation revealed a concealed pathology
which dramatically changed the management plan.
Figure 1: (A) Transthoracic Echocardiographic 4-chamber view, (B): 12 lead ECG.

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Abstract 160 Figure.

Figure 2: CMR examination: (A-B) SSFP (4-chamber and apical short axis) cine images
showing apical hypertrophy with spade sign (arrows) .(C-D) LGE images showing no ap-
ical myocardial fibrosis . (E-G) Stress dynamic images with adenosine showing perfusion
defect (dashed arrows). (F-H) Dynamic images during rest showing completely disappear-
ance of the perfusion defect.

Eur Heart J Cardiovasc Imaging Abstracts Supplement, 2017