376 Congenital heart disease: aortic disease P2112 | BEDSIDE Features of systemic inflammation and tissue remodeling in patients with bicuspid aortic valve disease combined with ascending aorta aneurysm I. Bilavka, T. Solomenchuk. Danylo Halytsky Lviv National Medical University, Lviv, Ukraine Purpose: To explore the features of cytokine profile and C-reactive protein (CRP) of patients with bicuspid aortic valve disease (BAV) combined with ascending aorta aneurysm (AAA). Materials and methods: The study involved 108 patients aged 17 to 75 years, 31 of them in the course of disease had complications with aneurysm of the ascending aorta. Average age - 52±1,2 years. Control group - 26 healthy individuals. Serum concentrations of cytokines, transforming growth factor B1 (TGF B1), interleukin-4 (IL-4) and CRP were determined by enzyme immunoassay. Results: Formation of AAA on the background of the BAV disease accompanied the highest serum concentrations of TGF β1, IL-4 and CRP compared with patients without AAA and individuals of the control group. The average level of TGF β1 was 22,04±4,26 pg/ml of patients with AAA, 16,12±2,12 pg/ml without AAA and 4,37±1,66 pg/ml - in the control group (p<0.001). The level of IL-4 was 8,91±1,02 pg/ml in patients with AAA, 6,62±2,37 pg/ml - without AAA and 0,75±1,51 pg/ml in controls (p<0,001). Significant differences were found in the study of CRP of patients with the BAV disease and AAA and BAV disease without AAA, compared with the control group: 13,12±2,87 mg/ml, 11,52±2,76 mg/ml and 6,95±2,03 mg/ml, respectively (p<0,05). Conclusions: When combined BAV disease with AAA showed a significant increase in TGF β1, IL-4 and CRP compared with patients without AAA and the control group. Thus, activation of cytokine profile underlies remodeling of outcellular matrix, improving processesof fibrosis valve that accelerates the development of stenosis or aortic valve insufficiency. R. Van Kimmenade 1 , M. Kempers 2 , M. De Boer 3 , B. Loeys 4 , J. Timmermans 3 . 1 University Medical Center Utrecht, Department of Cardiology, Utrecht, Netherlands; 2 Radboud University Nijmegen Medical Centre, Department of Clinical Genetics, Nijmegen, Netherlands; 3 Radboud University Nijmegen Medical Centre, Department of Cardiology, Nijmegen, Netherlands; 4 Antwerp University Hospital & University of Antwerp, Centre for Medical Genetics, Antwerpen, Belgium Purpose: Aortic root diameter dilatation expressed as a Z-score>2.0 is according to the new "revised Ghent criteria" a major criterium for diagnosing Marfan syndrome. Besides the classic equation (Z1), two new equations were recently introduced (Z2 & Z3). The Z1 equation is based on 135 subjects and discriminates between subjects < and ≥ 40 years old. The Z2 and Z3 equation were recently derived from >1200 subjects and correct for body surface area (BSA) (Z2) or body height (Z3). We investigated the differences between the equations with respect to clinical parameters such as age, obesity and the absolute cut-point of 40 mm (considered abnormal) for aortic root evaluation. Methods: We studied the effects of obesity, age and the absolute cut point of 40 mm on Z-score equations using 2674 echocardiographic measurements performed in 260 Marfan patients seen at our specialized Marfan out-patient clinic. Z1: – In subjects age 20-40: Mean predicted diameter (cm) for BSA = 0.97 + 1.12*BSA Z = (Measured diameter – predicted diameter)/SD with a SD of = 0.24 cm –In subjects age >40: Mean predicted diameter (cm) for BSA = 1.92 + 0.74*BSA Z = (Measured diameter – predicted diameter)/SD with a SD of = 0.37 cm Z2: Mean predicted diameter (cm) for BSA = 2.423 + (age*0.009) + (BSA*0.461) – (sex*0.267) Z = (Measured diameter – predicted diameter)/SD with a SD of = 0.261 cm Sex: male = 1, female = 2. Z3: Mean predicted diameter (cm) for length = 1.519 + (age*0.010) + (H*0.10) – (sex*0.247) Z = (Measured diameter – predicted diameter)/SD with a SD of = 0.215 cm Sex: male = 1, female = 2. Results: Comparing Z-scores between patients with a body mass index (BMI)<25 kg/m2 (group A) and BMI ≥25 kg/m2 (group B), median Z1 scores differed between groups (Z1=3.00 in group A, Z1=1.78 in group B; p=0.012) while Z2 (Z2=2.82 in group A, Z2=2.47 in group B; p=0.52) and Z3 scores (Z3=2.72 in group A, Z2=3.12 in group B; p=0.32) did not differ. Diameters ≥ 40mm were associated with Z1 scores <2.0 in 109 measurements (11.0%; 35 patients), Z2scores<2.0 in 37 measurements (3.8%; 13 patients) and Z3-scores <2.0 in 24 measurements (2.4%; 11 patients). Mean diameters increased after the 40th birthday: 42.0 mm (37.3-44.8IQR) to 42.5 mm (39.0-45.0IQR); p=NS). Mean Z1 scores decreased from 3.60 to 2.17 (p<0.01) whereas Z2 and Z3 scores tended to increase (Z2: 3.04 to 3.27; Z3: 3.39 to 3.55; p=NS for both). Conclusion: Z1 scores are inferior to Z2 and Z3 scores in the diagnostic evaluation for Marfan syndrome. Especially the Z3 score, correcting aortic root diameter for body height, seems excellent and clinically preferable R. Franken 1 , A.W. Hartog 1 , L. Van De Riet 1 , J. Timmermans 2 , A.J. Scholte 3 , M.P. Van Den Berg 4 , A.H. Zwinderman 5 , M. Groenink 1 , J.W. Yip 6 , B.J.M. Mulder 1 . 1 Academic Medical Center, University of Amsterdam, Department of Cardiology, Amsterdam, Netherlands; 2 Radboud University Nijmegen Medical Centre, Department of Cardiology, Nijmegen, Netherlands; 3 Leiden University Medical Center, Department of Cardiology, Leiden, Netherlands; 4 University Medical Center Groningen, Department of Cardiology, Groningen, Netherlands; 5 Academic Medical Center, Department of Clinical Epidemiology Biostatistics & Bioinformatics, Amsterdam, Netherlands; 6 National University Hospital, Singapore, Singapore Background: Prevention of aortic dissection and sudden death in Marfan syndrome (MFS) requires accurate diagnosis. MFS is diagnosed by the Ghent criteria, which are based on clinical features of primarily Caucasian populations. We determined whether the Ghent criteria are applicable to Oriental populations by comparing clinical features between an Oriental and Caucasian Marfan population. Methods: Clinical features of the cardiovascular, ocular and skeletal system of 273 adult Marfan patients according to the Ghent criteria of 2010 were compared between a Caucasian and Oriental population. Because genetic testing was unavailable in the Oriental population, Caucasian patients dependent on their mutation to fulfill the Ghent criteria were excluded in subgroup-analysis. Results: The Oriental Marfan population had a smaller body surface area (BSA: 1.75m2 vs. 2.01m2 , p<0.001), more severely affected aortic root diameter corrected for age and BSA (42.8mm vs. 43.8mm, p=0.46; Z-score: 4.8 vs. 3.4, p=0.008), more aortic dissections (15.4% vs. 2.3%, p=0.01) and more often a positive systemic score (76.9% vs. 58.8%, p=0.01), but less frequently ectopia lentis (23.1% vs. 45.2%, p=0.002) compared to the Caucasian population. Exclusion of Caucasian patients depending on their mutation to fulfill the Ghent criteria did not influence outcomes. Conclusion: The Oriental Marfan population differs significantly from the Caucasian by having more severely affected cardiovascular and skeletal systems and less frequently ectopia lentis. The results of this study reveal that the Ghent criteria are not applicable to Oriental Marfan populations and justify more research to optimize the Ghent criteria diagnosis of MFS for Oriental populations. P2115 | BEDSIDE Aortic tortuosity predicts cardiovascular events in Marfan syndrome R. Franken 1 , A.W. Den Hartog 1 , A. El Morabit 2 , J. Timmermans 3 , A.J. Scholte 4 , M.P. Van Den Berg 5 , H.A. Marquering 6 , A.H. Zwinderman 7 , B.J.M. Mulder 1 , M. Groenink 1 . 1 Academic Medical Center, University of Amsterdam, Department of Cardiology, Amsterdam, Netherlands; 2 VU University Medical Center, Department of Cardiology, Amsterdam, Netherlands; 3 Radboud University Nijmegen Medical Centre, Department of Cardiology, Nijmegen, Netherlands; 4 Leiden University Medical Center, Department of Cardiology, Leiden, Netherlands; 5 University Medical Center Groningen, Department of Cardiology, Groningen, Netherlands; 6 Academic Medical Center, Department of Radiology, Amsterdam, Netherlands; 7 Academic Medical Center, Department of Clinical Epidemiology Biostatistics & Bioinformatics, Amsterdam, Netherlands Purpose: Marfan syndrome (MFS) is a connective tissue disorder with highly variable aortic disease leading to cardiovascular events and sudden death. Increased tortuosity might be a marker for vascular fragility. The purpose of this study was to define the prognostic role of aortic tortuosity in the whole trajectory of the aorta on cardiovascular events in MFS. Methods: In this prospective multicentre study, 143 adults with MFS underwent a gadolinium enhanced three dimensional magnetic resonance imaging of the aorta. Analysis software (3mensio) constructed a stretched vessel view of the aorta from the supra aortic ridge to the beginning of the aortic bifurcation. Aortic tortuosity was calculated by aortic length/geometric length. Aortic volume was measured by 3mensio. Combined clinical endpoint comprised aortic dissection and elective aortic surgery. Results: At baseline, aortic tortuosity (1.9±0.2) was significantly correlated with aortic volume (235±64 ml, r2 =0.17, p<0.001). After a mean follow-up of 49.3±8.8 months, a total of 35 events (28 elective aortic root replacements, 2 distal aortic graft surgery and 5 aortic type B dissections) occurred. Patients with an event had higher aortic tortuosity (2.0±0.2 vs. 1.9±0.2, p=0.01) than patients without Kaplan Meier curve Downloaded from by guest on October 21, 2014 P2113 | BEDSIDE A clinical appraisal of different Z-score equations for aortic root assessment in the diagnostic evaluation of Marfan syndrome P2114 | BEDSIDE The Ghent criteria for Marfan syndrome are not applicable to Oriental populations