Right ventricular function / Advances in cardiomyopathies 209

Right ventricular function / Advances in cardiomyopathies
echocardiography including measurement of RV dimension and systolic function;
tricuspid annular plain systolic excursion (TAPSE), fractional area change (FAC)
and pulsed Doppler peak velocity at the annulus (s’). Our exclusion criteria were
as follows, atrial fibrillation, device implantation, significant aortic or mitral valve
disease, history of cardiac surgery, cardiac shunt and pulmonary hypertension.
We classified the patients into 3 groups according to septal e’ and symptom of
heart failure; normal group (e’≥8), preclinical DD group (e’<8, asymtomatic) and
clinical DD group (e’<8, symptomatic).
Results: Normal group, preclinical DD group and clinical DD group consisted
of 38 (19%), 116 (59%) and 44 (22%), respectively. Prevalence of DM was 16%,
34% and 30%, prevalence of hypertension was 21%, 63%, and 84%, respectively.
In preclinical DD and clinical DD group, Left atrial volume index (p=0.003) and E/e’
ratio (p<0.00001) were significantly larger than those of normal group. In clinical
DD group, TRPG (p=0.00001) were significantly larger than other groups, TAPSE
was significantly smaller (p=0.0055), FAC and s’ were smaller than other groups,
but they showed no significant difference.
Conclusion: Patient with HFpEF has reduced RV myocardial function compared
to normal subjects and ppatient with preclinical DD. And also RV function may be
reduced even in patient with preclinical DD.
Impaired right ventricular myocardial perfusion and right
ventricular dysfunction in patients with pulmonary arterial
hypertension: a study of contrast echocardiography and strain
H. Oe 1 , N. Watanabe 1 , N. Toh 2 , Y. Oono 2 , S. Ugawa 2 , Y. Tanabe 1 ,
K. Nakamura 2 , H. Morita 2 , K. Kusano 2 , H. Ito 2 . 1 Okayama University Hospital,
Okayama, Japan; 2 Okayama University, Department of Cardiovascular Medicine,
Okayama, Japan
Conclusion: Quantitative MCE revealed patients with PAH have impaired RV
myocardial perfusion, and this correlated well with the RV longitudinal myocardial
Right ventricular function in asymptomatic type 2 diabetic
patients. A conventional and tissue doppler echocardiographic
imaging study
W. Ammar, S. Abdallah, H. El Hossary, M. Abdel Ghani. Cairo University
Hospitals, Department of Cardiovascular Medicine, Cairo, Egypt
Introduction: Cardiac adverse effects influenced by diabetes have been demonstrated thus far for the left ventricle. Right ventricular dysfunction is relevant in a
variety of disease states affecting both the course and prognosis. Therefore assessment of right ventricular performance is also an important issue in diabetic
Aim of the work: To study right ventricular systolic and diastolic functions using conventional and tissue Doppler echocardiographic imaging in asymptomatic
type 2 diabetic patients and to asses the relationship of RV functions to diabetes
severity and complications.
Patients and methods: Fifty patients with type 2 diabetes with no cardiac diseases were prospectively enrolled. The control group included fifteen healthy volunteers. Patients with hypertension, valvular or coronary heart disease were excluded. Full clinical evaluation including fundus examination, laboratory work up
including (FBS, HbA1C, hs-CRP, S. creatinine and albuminuria), 12 lead ECG
and full conventional and tissue Doppler echocardiographic assessment of both
Results: Right ventricular diastolic function was abnormal in diabetic group as evidenced by significantly lower values of peak early diastolic velocity Em (p<0.001)
and prolonged IVRT (p<0.001) compared to control group. A significant reverse
relationship between FBS and RV systolic function assessed using fractional area
change (r= -0.280, p=0.049). RV diastolic dysfunction was significantly correlated
with severity and complications of type 2 DM. 1- FBS and RV regional IVRT, (r=
0.352, p=0.012). 2- HbA1c and Em of RV, (r= -0.403, p=0.004). 3- Albuminuria
and RV regional IVRT, (p=0.009). 4- hs-CRP and Am of RV, (r= 0.357, p=0.011).
Conclusions: Type 2 diabetes mellitus affects right ventricular diastolic function
in the presence of normal right ventricular systolic function. Impairment was evident mainly by TDI- derived indices and correlated significantly with severity and
complications of type 2 diabetes mellitus.
P1181 | SPOTLIGHT 2013
Right ventricular function by 3D-echocardiography and 2D-speckle
tracking in scleroderma patients in absence of pulmonary
D. Peluso 1 , D. Muraru 1 , U. Cucchini 1 , L. Dal Bianco 1 , E. Pigatto 2 , E. Zanatta 2 ,
L. Punzi 2 , F. Cozzi 2 , L.P. Badano 1 , S. Iliceto 1 . 1 University Hospital of Padua,
Department of Cardiac, Thoracic and Vascular Sciences, Padua, Italy; 2 Medicine,
Rheumatology Unit, University of Padua, Padua, Italy
Purpose: Right ventricular (RV) involvement in Systemic Sclerosis (SSc) is usually related to the appearance of pulmonary hypertension (PH) and it’s unknown
if SSc can directly affect RV function in absence of PH. Recently, new more accurate echocardiographic techniques, as 3D-echocardiography (3DE) and 2Dspeckle tracking (2DSTE), have been introduced for better assessment of heart
chamber function and mechanics. The aim of this study was to use 3DE and
2DSTE, in both SSc patients and sex and age-matched healthy subjects, to identify early echo markers of RV impairment.
Methods: 31 SSc patients (29 females, age 56±11 years, SSc duration 13.6±9.4
years), without known heart disease and PH, were studied. 21 patients were affected by limited and 10 by diffuse cutaneous form of SSc. 36 age and gendermatched healthy subjects were enrolled as controls. All subjects underwent a
complete echocardiogram, including RV volumes and ejection fraction measured
by 3DE dataset. In addition, we measured global and regional longitudinal strain
by 2D-speckle tracking method from a dedicated apical view of the RV.
Results: RV end-diastolic area and fractional area change were similar in SSc
and control subjects; conversely, end-systolic area was larger in SSc patients
(9±2.2 vs 8.1±1.4 cm2 , p=0.045). TAPSE (23±3 vs 26±3 mm, p=0.001) and
S wave of RV free wall (13±2 vs 15±3 cm, p=0.004) were lower in SSc patients than in controls. Pulmonary artery systolic pressure (28±6 vs 22±5 mmHg,
p=0.001) and pulmonary vascular resistance (1.7±0.3 vs 1.5±0.3 UW, p=0.011)
were higher in SSc patients than in controls, without reaching cut-off values for
PH diagnosis. Upper limits of RV end-diastolic volume (EDV) (114 ml) and endsystolic volume (ESV) (45 ml) and lower limit of RV ejection fraction (EF) (51%)
were obtained by 3DE in control subjects. EDV was similar in SSc and controls
(88±22 vs 85±14 ml, p=0.53); conversely, end-systolic volume was larger (43±11
vs 33±7 ml) and ejection fraction was lower (51±5% vs 61±6%) in SSc compared
to controls (p<0.0001 for both). However, using 2DSTE we found no differences
about RV global (-25.6±2.9% vs -25.5±2.8%, p=0.84) and free wall longitudinal
strain (-30.3±4.9% vs -31.1±3.6%, p=0.51) between SSc patients and controls.
Conclusions: 3DE evaluation showed an impairment of RV pump function, in
term of ejection fraction, in SSc patients, without impairment of myocardial mechanics as assessed by longitudinal strain. Our data suggest that RV ejection
fraction impairment in SSc patients without PH could be related to slightly increased afterload, in presence of preserved RV myocardial function.
Gender differences in cardiovascular causes of sudden cardiac
death in the young
S. Rizzo, K. Pilichou, E. Carturan, G. Thiene, C. Basso. University of Padua,
Department of Cardiac, Thoracic and Vascular Sciences, Pathological Anatomy,
Padua, Italy
Purpose: Sudden cardiac death (SCD) in the young is due to a wide spectrum
of cardiovascular causes but few studies specifically addressed their prevalence
and characteristics in the female (F) vs. male (M) gender.
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Background: Little is known about right ventricular (RV) dysfunction in patient
with pulmonary arterial hypertension (PAH). Recently, RV ischemia has been reported to be a contributory factor in the development of RV dysfunction. The aim
of this study is to evaluate RV myocardial perfusion and assess its relationship
with RV dysfunction.
Methods: This prospective study consisted of 10 consecutive patients (7 female,
51±17 years) with PAH (6 idiopathic PAH (IPAH), and 4 chronic thromboembolic pulmonary hypertension (CTEPH)), and 6 healthy controls (3 females, 37±6
years). Quantitative myocardial contrast echocardiography (MCE) was performed
using Philips iE33 imaging system with an S5-1 probe. We depicted the apical
four chamber view. A solution of Levovist (300mg/ml) was inravenously administered at a rate of 3ml/min with a volumetric pump. End-systolic images were
obtained every four heart beats. The MCE images were analyzed off-line using
VoluMap-445 system. The RV myocardium was divided into six segments, at each
myocardial segment, calibrated contrast intensity (CI) was determined as the difference (dB) between the mean myocardial CI and cavity CI. 2D speckle tracking
echocardiographic study was also performed using Vivid E9 ultrasound system.
Results: Among 96 RV myocardial segments, quantitative CI analysis was possible in 86 segments (90%). RV fractional area change was 46% (healthy), 34%
(CTEPH) and 25% (IPAH). Mean calibrated CI of RV free wall was -13.4dB, 19.3dB and -22.8dB, respectively (p=0.01). We found good inverse correlation
between calibrated CI and longitudinal strain of RV free wall (r=-0.5, p=0.0013).
Advances in cardiomyopathies
Methods: In the time interval 1980-2011, 591 SCDs in young people aged 1-40
yrs (mean age 259 yrs) have been prospectively studied according to a uniform
pathology protocol in our referral pathology Centre.
Results: They were 406 M (69%) and 185 F (31%), mean age 268 vs. 2410,
p=0.01. Major causes included atherosclerotic coronary artery disease (CAD
108, 18%), myocarditis (72, 12%), arrhythmogenic right ventricular cardiomyopathy (ARVC 58, 10%), hypertrophic cardiomyopathy (HCM 55, 9%), dilated cardiomyopathy (22, 4%), non ath-CAD (41, 7%) and mitral valve prolapse (MVP 45,
7.5%). In 37 cases SCD was mechanical (6%) and in 101 (17%) the heart was
structurally normal (unexplained SCD).
Among cardiomyopathies, myocarditis, HCM and DCM show an almost equal
prevalence in M and F (11%, 10% and 4% vs. 15%, 7% and 3.5%, respectively,
p=NS). Atherosclerotic CAD (24% vs. 6%) and ARVC (13% vs. 3%) are the leading causes in M SCD victims. On the contrary, spontaneous coronary artery dissection (5% vs. 0.2%), MVP (14% vs. 5%) and mechanical causes (9% vs. 5%)
are more typical of F SCD victims. SCD remains unexplained in 25% of F vs.
13.5% of M. Competitive sport activity is more frequent in M than F (17% vs. 3%).
All p are statistically significant. Among F cases aged 18 yrs, 12% died suddenly
during pregnancy or in the peri-partum period.
Conclusion: One third of young SCD victims are women. In the F gender, SCD
remains unexplained in one forth of cases and major cardiovascular causes are
represented by subtle substrates such as myocarditis and MVP. Athletic activity
is underrepresented as compared to M, possibly explaining the low prevalence of
ARVC among F SCD victims at difference from other inherited cardiomyopathies.
P1184 | BENCH
Usefulness of right ventricle 2D strain in arrythmogenic right
ventricle dysplasia/cardiomyopathy
A. Healy-Brucker, F. Pousset, S. Almeida, E. Gandjbakhch, G. Duthoit,
J.L. Hebert, L. Boubrit, N. Hammoudi, R. Isnard, F. Hidden-Lucet. AP-HP Hospital Pitie-Salpetriere, Department of Cardiology, Paris, France
Mutations and polymorphisms of the alpha-galactosidase A gene
in patients with hypertrophic cardiomyopathy
S. Pfaffenberger 1 , N. Lajic 1 , M. Gaggl 2 , A. Jallitsch-Halper 2 , R. Rosenhek 1 ,
T. Voigtlaender 3 , R. Sunder-Plassmann 4 , G. Sunder-Plassmann 2 ,
G. Mundigler 1 . 1 Medical University of Vienna, Department of Internal Medicine
II, Division of Cardiology, Vienna, Austria; 2 Medical University of Vienna,
Department of Internal Medicine III, Division of Nephrology, Vienna, Austria;
3 Medical University of Vienna, Institute of Neurology, Vienna, Austria; 4 Medical
University of Vienna, Department of Laboratory Medicine, Vienna, Austria
Aims: Anderson-Fabry disease (AFD) is a rare cause of hypertrophic cardiomyopathies (HCM). Clinical signs alone do not allow distinguishing a cardiac manifestation of AFD from sarcomeric forms of HCM. Enzyme activity analysis and
genetic testing of the α-galactosidase A (α-GAL) gene (GLA) are therefore important for diagnosis and verification of disease causing mutations.
Methods and results: We enrolled 108 adults (76 males, 32 females) with a
referral diagnosis of HCM with a left ventricular wall thickness of ≥15 mm on
echocardiography. Laboratory analyses included the measurement of α-GAL activity in leukocytes and sequencing of the GLA gene in all patients. Symptoms
were evaluated using a specified questionnaire.
Mutation analyses confirmed AFD in two patients (p.G35R and g.5092A>G;
1.9%). One female patient (0.98%) had a rare polymorphism (p.D313Y) previously described as disease causing for AFD. In 31 patients (28.7%) various
combinations of polymorphisms were detected. The g.1170C>T polymorphism
was found in 9 patients (7 males) and was associated with a significantly decreased α-GAL activity in leukocytes among male subjects compared to the wild
type GLA gene (p=0.003). Clinically these patients showed higher frequencies of
non-sustained ventricular tachycardias (nsVTs, p=0.009).
Conclusion: Specific testing of the GLA gene in patients with HCM confirmed
AFD in two patients and revealed various polymorphisms in almost one third of
patients. The g.1170C>T polymorphism was found in 8% and is associated with
a reduced α-GAL activity and a higher incidence of nsVTs in males.
How to determine effectiveness of alcohol septal ablation
immediately after intervention?
E. Kretov. State Research Institute of Circulation Pathology, Novosibirsk, Russian
Conclusion: RV 2D strain is significantly lower in affected ARVD/C patients compared with normal normal, but not with borderline, especially in mid lateral and
basals segments. The presence of a RV strain impairment may be useful for the
diagnosis of ARVD/C in the early course of the disease.
Acute effect of adjunctive breast cancer radiotherapy on right
ventricular systolic and diastolic function
S. Tuohinen 1 , T. Skytta 2 , P.L. Kellokumpu-Lehtinen 2 , V. Virtanen 1 , T. Luukkaala 3 ,
P. Raatikainen 1 . 1 Tampere University Hospital, Heart Center, Department of
Cardiology, Tampere, Finland; 2 University of Tampere, Medical School, Tampere,
Finland; 3 University of Tampere, Department of Health Sciences, Tampere,
Aim: Breast cancer radiotherapy has been shown to depress left ventricular (LV)
Aims: In many clinical scenarios, technical limitations of echocardiography and
heterogeneous phenotypic expression made evaluation after intervention very difficult, and cardiac magnetic resonance has emerged as a useful adjunctive imaging modality to evaluate results after alcohol septal ablation. The purpose of the
present investigation was to determine the anatomical sequelae of alcohol septal
ablation using cardiac magnetic resonance.
Methods and results: We performed alcohol septal ablation in 114 patients with
hypertrophic obstructive cardiomyopathy. The average age was 51 years ± 9.7;
males -55 (48.2%), females -59 (51.8%). The average pressure gradient at the
output of the left ventricle was 72.5±20.5 mm. Hg. Cardiovascular magnetic resonance imaging was performed before ablation and again 1 month after the procedure in all patients. We investigated localization and size of myocardial infarction by cardiac magnetic resonance. After the procedure pressure gradient of left
ventricular outflow tract decreased from 72.5±20.5 mm. Hg to 30.7±14.1 mm
Hg. There was no mortality. Atrioventricular block occurred in one patient (1%).
Contrast-enhanced cardiac magnetic resonance showed no infarct-related hyperenhancement outside the target septal area. Left ventricle mass decreased from
320±72 g at baseline to 314±69 g at one month follow-up. Data obtained by cardiac magnetic resonance showed that septal infarct was localized in the basal part
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Background: Diagnosis of Arrhythmogenic Right Ventricular (RV) Dysplasia/Cardiomyopathy (ARVD/C) is based on International Task Force Criteria
(ITFC). But the diagnosis remains challenging especially in the early course of
the disease.The purpose of this study was to assess if the RV 2D strain analysis
could be an accurate tool to detect RV abnormalities in patient with suspicion of
Methods: We enrolled 95 patients with suspicion of ARVD/C referred to our institution. ECG, SAECG, echocardiography and RV angiography were performed
in all patients. Based on ITFC and a final experts’agreement, the patients were
classified as follows: affected ARVD/C (n=50), borderline ARVD/C (n=9) or normal (n=36). In addition, RV longitudinal strain was measured in 6 RV segments
obtained from the RV focused apical 4-chamber view. Analysis was performed
offline and blinded of the final diagnosis.
Results: Compared to normal, affected patients had only a mild decrease in
RV fractional area change (FAC) (37±9% vs 44±7% p=0.0002) and an mild
increase RV outflow tract diameter (RVOT) (35±6mm vs 32±5mm, p=0.002).
Borderline had normal FAC and increase RVOT. Affected patients displayed a
reduced global RV strain (RV-GS) (-20.8±4.8% vs-24.8±3.3% p<0.0001). Segmental strain analysis showed the reduction was major in the lateral-basal segment (RV-LBasS), the lateral-mid segment (RV-LMidS) and the septum-basal
segment (RV-SBasS). Bordeline’s patients had no significant altered RV strain
compared to normals or affected patients.
function, but its effects of right ventricular (RV) function are largely unknown.
Reduced RV systolic performance correlates with poor prognosis across broad
spectrum of diseases. The aim of this prospective single center study was to investigate whether conformal 3D breast cancer radiotherapy has negative effect
on RV systolic and diastolic function in the acute phase.
Methods: Forty consecutive patients with early left-side breast cancer were evaluated before and immediately after radiotherapy. A comprehensive 2D echocardiographic examination was performed at each visit. Several measurements of
RV function were performed including tricuspidal annular plane systolic excursion
(TAPSE), pulsed tissue Doppler peak velocity at the lateral RV wall (S’), RV inflow
and outflow analysis, valvular assessment and venous flow analysis.
Results: Radiotherapy reduced TAPSE from a baseline value of 24.9±4.1 mm
to 22.7±4.0 mm (p< 0.001). In keeping with this S’ declined from 13.0±5.0 m/s
to 12.3±4.4 m/s (p=0.086) and pulmonary flow velocity time integral (VTI) from
16.7±3.3 to15.9±2.5 (p=0.089), respectively. These changes were not related
to LV systolic or diastolic changes. According to multivariate analysis the use of
angiotensin-converting enzyme inhibitor or angiotensin II receptor blocker may
protect against these changes (p= 0.097).
Conclusions: Modern 3D radiotherapy reduced RV systolic function. These early
changes might progress in time, and continuous follow-up of cardiac function is
warranted in radiotherapy patients. As a readily available and sensitive measurement TAPSE is as a practical tool for this purpose.