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. P1179 | BEDSIDE Impaired right ventricular myocardial perfusion and right ventricular dysfunction in patients with pulmonary arterial hypertension: a study of contrast echocardiography and strain imaging 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 dysfunction. P1180 | BEDSIDE 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 patients. 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 ventricles. 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 hypertension 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. ADVANCES IN CARDIOMYOPATHIES P1183 | BEDSIDE 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. Downloaded from by guest on October 21, 2014 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). 209 210 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 P1186 | BEDSIDE 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. P1187 | BEDSIDE How to determine effectiveness of alcohol septal ablation immediately after intervention? E. Kretov. State Research Institute of Circulation Pathology, Novosibirsk, Russian Federation 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. P1185 | BEDSIDE 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, Finland 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 Downloaded from by guest on October 21, 2014 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 ARVD/C. 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.