International Conference and Exhibition on Pediatric Cardiology August 25-27, 2015 Valencia, Spain, Europe

David Coman is a Paediatrician, Metabolic Physician and a Clinical Geneticist working in clinical practise in Brisbane Australia. He has a public appointment at the RoyalrnChildren’s Hospital in Brisbane, and is the Medical Director of Paediatrics at the Wesley Private Hospital in Brisbane. He is interested in teaching and is the Academic leadrnfor Paediatrics in the Uniting Care Clinical School, Brisbane. He has a particular research interest in disorders associated with aberrant post-translational modification,rnepigenetics modification of single gene disorders, and novel disease discovery. He has active interests in rare disease advocacy, and runs philanthropic general paediatricrnclinics to the Western provinces of the Solomon Islands.

The inborn errors of metabolism (IEM) exhibit protean clinical manifestations. Cardiac disease either in isolation or as part of arnwider multisystem disease process, are frequently observed in the IEM. Cardiac presentations associated with the IEM include,rnarrhythmias, dilated cardiomyopathy, hypertrophic cardiomyopathy, non-compaction, valvular disease, endocardial fibroelastosis,rnand aortic root pathology. IEM groups commonly associated with cardiac disease include the mitochondrial respiratory chain defects,rnfatty acid oxidation defects, glycogen storage diseases, lysosomal storage diseases, defects of organic acid and amino acid metabolism,rnand defects of post-translational modification. This presentation will provide an update of the IEM disease process associated withrncardiac disease, outlining a simple clinical approach to delineating an overarching diagnosis; especially in disease processes where arnrapid diagnosis is essential, e.g., Pompe Disease.

Aarti Bavare has completed her Pediatric Residency at the Children’s Hospital Boston and Boston University combined pediatric residency program. She has receivedrnfellowship training in Pediatric Critical Care and Pediatric Cardiac Critical Care at Texas Children’s Hospital and Baylor College of Medicine. She is currently an AssistantrnProfessor of Pediatrics at Baylor College of Medicine and is the Medical Director of the house-wide Pediatric Rapid Response System at Texas Children’s Hospital. Herrnresearch interests include critical communication, resource utilization to serve critical needs of children and simulation medicine.

The Rapid Response (RR) system is a multidisciplinary modality to detect, trigger and provide response to clinical decompensationrnof patients outside the Intensive Care Unit (ICU). There is growing evidence to support that early detection of physiologicrndeterioration and prompt response can decrease adverse outcomes i.e. cardiopulmonary arrest or mortality. Pediatric cardiac patientsrnare highly vulnerable to decompensation needing emergent intervention and treatment. We sought to explore the frequency andrnpattern of utilization of RR by these patients and their outcomes. We conducted a retrospective review study at a large tertiary carernreferral pediatric heart center. We reviewed 1906 RR events that occurred in our center over a period of three years. Out of thesern152 occurred in cardiac patients. We reviewed the charts of the patients involved in these events with respect to the demographicrncharacteristics, reason for RR event, interventions needed and outcomes thereafter. The results provide useful insight into causesrnof acute clinical deterioration in pediatric cardiac patient population. The important observations made during this research willrnhopefully be a useful guide to direct initiatives to improve care and resource allocation to this high risk population.

Halimic M is working in Paediatric clinic of University Clinical Centre Sarajevo at Bosnia and Herzegovina. His main reserch interest are genetic epidemiology, internal\r\nmedicine (general medicine) and cardiology.

Introduction: Despite recent advances in anesthesia, cardiopulmonary bypass and surgical techniques, children undergoing\r\ncongenital heart surgery require postoperative mechanical ventilation. Early extubation was defined as ventilation shorter than 12\r\nhours.\r\nAim: To identify markers associated with successful early extubation after pediatric cardiac surgery.\r\nMethods: Retrospective-prospective clinical study was performed in Paediatric Clinic and Heart Center Clinical Centre Univerity\r\nof Sarajevo from 01.01.2006 to 01.01.2011. Study included 100 children up to 5 years of age with congenital heart disease, left–right\r\nshunt and opstructive congenital heart disease. Patients were devided into two groups: I Group-54 patients extubated within 12 hours\r\nafter surgery and Group II- 46 patients extubated more than 12 hours after surgery.\r\nResults: The most frequently encountered preopeartive variables were age with odds ratio 4% 95% CI(1-7%), Down’s syndrome\r\n8.5 95% CI (1.6-43.15), failure to thrive 4.3 95% CI(1-18). Statisticly significant postoperative data included lung disease (reactive\r\nairways, pneumonia, atelectasis, pneumothorax) and with odds ratio 35.1 95% CI (4-286) and blood transfusion with odds ratio 4.6\r\n95% CI(2-12). Proven markers were, age with cut of 21.5 months (sensitivity 74% and specificity 70%) and extracorporeal circulation\r\n(ECC) with cut of 45.5 minutes (sensitivity 71% and specificity 65%).\r\nConclusion: Younger age and prolonged time ECC are markers associated with prolonged mechanical ventilatio

Patricia Bastero is currently involved with The Congenital Heart Surgery project in Mexico, in collaboration with TCH international services. She participated in manyrninternationals ECMO courses bringing a good “hands on” experience with high fidelity simulation for ECMO. Her main research interest is based on education researchrnat the moment. Her past research experience involves her PhD thesis, a prospective investigation of the relationship between the vitamin D receptor gene and estrogenrnreceptor gene polymorphisms, and their relation to the growth pattern in healthy children from birth to 7 years of age.

Background: Acute care inpatient units, such as cardiology floors, are high acuity and highly specialized areas with an increasedrnpotential for codes and other emergency events. Simulation based educational programs are a very effective and efficient wayrnto train health care providers on emergency situations, and improving their confidence and comfort when treating high riskrnpatients. We present the results of the first in-situ simulation room in our cardiology floor, the learning opportunities created,rnthe cost and the impact of this new regular activity in our staff.rnMethods: We installed our simulation equipment, including a camera and a mock crash cart, in one of the free procedurernrooms in our cardiology floor. Five simulation instructors with experience on cardiology emergencies and/or CRM skillsrnparticipate in the program. We run twice weekly, mostly, “just in time” simulation scenarios. We use the small conference/rnfamily room on the cardiology floor for our debriefings. The scenarios are run for 10 minutes, debriefings for 20 minutes.rnEvaluations are filled post-simulation activities every time, by every participant.rnResults: We created over 994 multidisciplinary educational opportunities, training 97% of the residents, 100% of the cardiologyrnfellows, 67% of the NPs and 90% of the RNs, on our cardiology floor. Over 85% of the scenarios were run by the same threerninstructors and 80% of the scenarios were conducted during daytime hours. The total cost was $37,205. The evaluations werernoverall >4/5 when assessing for self reported ability to perform as part of a team during high-stakes events, increasing thernvigilance to patient safety during crisis and self confidence in handling critical situations.rnDiscussion: It is feasible to create an interesting and engaging “in situ” educational program with minimal equipment andrncost, and limited manpower that improves team and personal performance. We need further research to evaluate whetherrnthis educational method helps improving patient safety,

Rubin S Cooper, MD Specialty is Pediatric Cardiology, Pediatrics. He is a Chief - CCMC Division of Pediatric Cardiology and Professor, Hofstra North Shore-LIJ School of\r\nMedicine, USA.

Introduction: This study evaluated endothelial function in patients with anorexia nervosa (AN) using Endothelial Pulse Amplitude\r\nTesting (Endo-PAT) and correlated findings with the patients’ history and biochemical data.\r\nMethod: Twenty-one patients age 13-21 years diagnosed with AN by the Division of Adolescent Medicine at Cohen Children’s Medical\r\nCenter of NY between 6/1/2012 and 5/31/2013 were studied along with 19 healthy controls similar in age and gender distribution.\r\nDigital pulse amplitude was examined using Endo-PAT. Raw data were automatically transferred into a reactive hyperemia index\r\n(RHI) and the natural log transformation of RHI (LnRHI). Subjects’ and controls’ electrocardiograms and biochemical markers were\r\nobtained.\r\nResults: AN and controls had similar RHI (P=0.75) and LnRHI (P=0.95). AN had lower mean weight (P<0.001), height (P=0.02), BMI\r\n(P<0.001), resting HR (P<0.001), systolic (P<0.001) and diastolic BP (P=0.01). AN also had lower mean HR during EndoPAT testing\r\n(P<0.001), triiodothyronine (T3) (P<0.001), luteinizing hormone (LH) (P=0.006) and estradiol (E2) (P=0.005). Total cholesterol\r\n(Chol) (P<0.001) was higher in AN subjects. No correlation was observed between RHI and other parameters.\r\nConclusion: No significant differences in RHI or LnRHI found between the two groups. There were significantly higher Chol and\r\nlower HR, T3, LH and E2 levels in the AN group compared to controls. There were no correlations of these parameters to RHI.