Cardiovascular drug therapy in special populations 1051 represents the prototype of a restrictive cardiomyopathy. A vast majority of af- fected patients present with advanced heart failure and face significant morbidity and mortality. However, an effective therapy is still lacking and a diagnosis of CA precludes patients from participation in standard heart failure clinical trials. The soluble guanylate cyclase - stimulator riociguat, already approved for the treatment of pre-capillary pulmonary hypertension, has also been shown to have favorable hemodynamic effects in heart failure. Purpose: We aimed to test the safety and efficacy of riociguat in a case-series of patients with TTR CA. Methods: TTR CA was diagnosed either by histological assessment of endomy- ocardial biopsy samples with Congo red staining and subsequent immunohisto- chemical typing or non-invasively in accordance with current recommendations. Parameters of interest were change in invasively measured hemodynamics, ex- ercise capacity, quality of life as well as safety and tolerability. Results: Between August 2014 and June 2017, 11 patients with wild-type TTR CA and 2 patients with mutations of the TTR gene (His108Arg) were included into our study. 2 patients discontinued with the study and the remaining 11 patients completed all procedures. Median age of the study population was 75.0 years (IQR: 69.0–83.0) and 9 (81.8%) were male. The majority of the patients were in New York Heart As- sociation (NYHA) class ≥III (n=6, 54.6%), and NT-proBNP values were markedly elevated with a median of 2923pg/mL (IQR: 1773–7912). Median 6-MWD was 370m (IQR: 330–526). Cardiac output improved significantly from 4.3L/min (IQR: 3.8–5.1) to 4.5L/min (IQR: 4.2–5.1) (p=0.022, Figure 1A) whereas diastolic pressure gradient de- creased (baseline: 0.0mmHg, IQR: -2.0–3.0; follow-up: -1.0mmHg, IQR: -3.0– 1.0; p=0.049). 6-MWD increased from 370m (IQR: 330–526) at baseline to 400m (IQR: 350–570) at follow-up (p=0.045) Correspondingly, NYHA class im- proved significantly (baseline: NYHA class ≥III: n=6, 54.6%; follow-up: n=0, 0.0%; p=0.031). However, NT-proBNP did not change from baseline 2923pg/mL (IQR: 1773–7912) to follow-up: 2584pg/mL (IQR: 1804–7255) (p=0.929). Overall health status improved significantly from 50% (IQR: 40.0–50.0) at baseline to 60% (IQR: 50.0–75.0) at follow-up (p=0.021). Figure 1 Conclusion: The present case series of TTR CA patients suggests beneficial ef- fects of riociguat administration in this patient population. However, further studies of stronger design are warranted to explore the therapeutic potential of riociguat in TTR CA. 5071 The monocentric, double-blind, placebo-controlled research of sildenafil impact in pediatric patients with secondary pulmonary hypertension I. Palii 1 , E. Vataman 2 , M. Vataman 2 . 1 Mother and Child Institute, Chisinau, Moldova Republic of; 2 Institute of Cardiology, Chisinau, Moldova Republic of Purpose: Pulmonary hypertension (PH) caused by congenital cardiac disease continues to be one of the most problematic pediatric diseases to cure. Moreover, PH ultimately faces to RV remodeling and its dysfunction. Sildenafil takes a part of selective inhibitors of phosphodiesterase type 5 class, is famous as an efficient pulmonary vasodilator with insignificant side effects. We have investigated the effectiveness of the aforementioned drug in children with advanced secondary PH CCD with shunts simple (18 ps), mixed (28 ps) and complex (27 ps). Methods: In this study, we performed a monocentric, double-blind, placebo- controlled comparison. 69 children with advanced PAH (39 with repaired congeni- tal SP shunts and persistent PH, 21 with the palliative procedure and 9 inoperable patients) were randomly selected to placebo or sildenafil treatment. The study protocol was accepted by the competent ethical review committee. The patient information and informed consent were provided by all the parents of each child. There study included two groups: first – 34 children (mean age 26,3±7,06 months), and the second one – 35 patients (mean age 23,5±7,85 months). The initial treatment with Sildenafil began with 0,5 mg/kg dose, then – 1 and 2 mg/kg/day every 6 hours for 12–24 weeks. The research agreement included: clinical signs; transcutaneous O2 saturation; 6-minute walk test; transthoracic echocardiogram, right cardiac catheterization, measuring pulmonary vascular re- sistance. Also, we tracked all the adverse reactions in a special questionnaire. All the contested patients and their parents completed cardiac module of PedsQL questionnaire. Results: Most of the patients from Sildenafil group attended positive progress of cardiac function NYHA/Ross from 3,2±0,1 to 1,6±0,1 (p<0,001); O2 satu- ration from 92, 9±0,7 to 96,7% (p<0,001); an effort tolerance estimated by 6- minute walk test from 286,73±17,35 to 486,688±14,99 (p<0,001); the decreasing of mean PAP from 53,76±2,4 to 34,2±1,95 mmHg (p<0,01); the modification of the PVR from 6,558±0,321 to 3,824±0,272 UW/m 2 (p<0,01); the elevation of the systolic function, TAPSE from 16,82±0,39 to 23,44±0,52 mm (p<0,01) and global function of RV (the performance Tei index) from 0,489±0,012 to 0,323±0,011 (p<0,001). No death among the sildenafil group was registered, in opposite to 4 cases in the placebo group. Conclusions: The research outcome is proving the efficacy of in treating sec- ondary PH to congenital systemic-to-pulmonary shunts and RV dysfunction, but even more effective in corrected surgical shunts. Sildenafil boosts CF (p<0,01), systemic O2 saturation (p<0,01), tolerability at effort (p<0,05), systolic function – TAPSE (p<0,001), global function of RV – Tei index (p<0,01), decreasing PAPm (P<0,01) and PVR (p<0,001) comparing with placebo. This drug has good tolera- bility, with few and minimal adverse effects, avoiding severe complications, having an excellent consequence on the quality of life of these children. 5072 Obesity paradox outcomes after acute myocardial infarction in Japanese is due to optimal medical therapy in overweight patients K. Sakamoto 1 , K. Tsujita 1 , K. Kaikita 1 , K. Nakao 2 , Y. Ozaki 3 , K. Kimura 4 , J. Ako 5 , T. Noguchi 6 , S. Yasuda 6 , S. Suwa 7 , K. Fujimoto 8 , K. Nishimura 6 , Y. Miyamoto 6 , H. Ogawa 6 , M. Ishihara 9 . 1 Kumamoto University Hospital, Department of Cardiovascular Medicine, Kumamoto, Japan; 2 Saiseikai Kumamoto Hospital, Division of Cardiology, Kumamoto, Japan; 3 Fujita Health University, Department of Cardiology, Toyoake, Japan; 4 Yokohama City University Medical Center, Cardiovascular Center, Yokohama, Japan; 5 Kitasato University, Department of Cardiovascular Medicine, Sagamihara, Japan; 6 National Cerebral and Cardiovascular Center, Department of Cardiovascular Medicine, Suita, Japan; 7 Juntendo University Shizuoka Hospital, Department of Cardiology, Izunokuni, Japan; 8 National Hospital Organization Kumamaoto Medical Center, Department of Cardiology, Kumamoto, Japan; 9 Hyogo College of Medicine, Division of Coronary Artery Disease, Nishinomiya, Japan Background: Although the counter-intuitive association between obesity and mortality has been termed the obesity paradox, the evidence in patients with coronary artery disease have been inconsistent, in contrast to those with con- gestive heart failure. The mechanisms for the paradox have yet to be clarified. We hypothesized that a different factor, in addition to the patient background, which concerned with obesity, may have an impact on adverse events in acute myocardial infarction (AMI) patients. Methods: To elucidate the impact of obesity, we analyzed data from the Japanese registry of acute Myocardial INfarction diagnosed by Universal dEfiniTion (J- MINUET). This was a prospective and multicenter registry consisting of 3,283 AMI patients, who were hospitalized within 48 hrs from onset, from July 2012 to March 2014. In total, 3,167 patients with known body parameters were stratified into 3 groups according to their degree of adiposity assessed by BMI (kg/m 2 ), utilizing WHO classification, under 18.5 kg/m 2 for Underweight, 18.5–24.9 kg/m 2 for Normal, and over 24.9 kg/m 2 for Overweight. Follow-up data was obtained for 3 years. The primary endpoint was a composite of all-cause death, non-fatal MI, non-fatal stroke, cardiac failure and urgent revascularization for unstable angina. The secondary endpoint was all-cause death. Results: There were 348 deaths (11.0%) during the 3-year follow up period. Regarding both the primary and secondary endpoints, we observed the obesity paradox which declines with increasing BMI (Figure). Ratio of female, older age, higher Killip classification, coexistence of atrial fibrillation, history of stroke, pe- ripheral artery disease, and multi-vessel disease were higher in Underweight pa- tients. Diabetes, hypertension, dyslipidemia, and current smoking were more fre- quent in Overweight patients. Overweight was an independent negative predictor for both endpoints. Kaplan-Meier analysis showed significant differences among the groups. Regarding medication at onset, usage rates of dual antiplatelet ther- apy (DAPT), calcium channel blocker (CCB), β-blockade (BB), and Nitrates were comparable among the groups, except for higher usage rate of RAAS blockades (25.8% vs. 30.5% vs. 33.9%, p=0.029) and Statins (15.7% vs. 24.1% vs. 24.0%, p=0.018) in Overweight patients. From the view point of medication at discharge, more optimal medical therapy (OMT) was observed in Overweight patients, as Downloaded from https://academic.oup.com/eurheartj/article/39/suppl_1/ehy566.5071/5081491 by guest on 01 March 2023