Day 1 :
Creighton University School of Medicine, USA
Keynote: Impact of pre-procedural simulation and planning using 3D resin (solid) and photopolymer (flexible) models on interventional cardiac procedures
Time : 10:00-10:40
Randy Richardson is the Chairman of radiology at St. Joseph's Hospital and Medical Center and the Associate Dean for Creighton University School of Medicine Phoenix Campus. He is Professor of radiology for the Creighton Schools of Medicine and Adjunct Professor at Arizona State University School of Biological and Health Systems Engineering. He is the author of a textbook, 31 book chapters, 11 syllabi, 44 scientific exhibits, 24 scientific papers, 22 peer reviewed journal articles, 3 websites and 14 multimedia presentations. His area of expertise and research has been in the field of cardiac MRI and CT imaging in children.
Pre-surgical planning using imaging in pediatric congenital and acquired heart disease has played a vital role in patient outcomes for many decades. With advances in 3D printing technology we are now able to take patient specific imaging data and create 3D patient specific physical models. These models can be used to for pre-procedural patient specific simulations of cardiac procedures. We have successfully performed pre-procedure simulated procedures on physical models in a patient with aortic stenosis undergoing per-cutaneous aortic valve placement where the size of the valve was in question, two patients with VSD from myocardial infarction that needed a device closure and to predict compatibility of donors and recipients for heart transplants. In all cases the simulated procedure had a significant impact on the actual procedure resulting in decreased time of procedure compared to standards, influenced the choice of the device chosen and was key in predicting correct sizing of implant. All though the numbers are small the impact seems to be significant.
Université Pierre et Marie Curie, France
Time : 10:40-11:20
Guy Fontaine has made 15 original contributions at the inception of cardiac pacemakers in the mid-60s. He has published more than 900 scientific papers including 201 book chapters. He is included in the Profiles in Cardiology (W Hurst 2003) book of the 216 individuals who have made a significant contribution to the study of cardiovascular diseases since the 14th century. He has been included in the book “500 greatest Geniuses of the 21st century” of the American Biographical Institute (ABI 2005). He was the reviewer of 17 journals both in clinical and basic Science. He served during 5 years as a Member of the Editorial Board of Circulation. He has been invited to give 11 master lectures of 90 minutes each during three weeks in the top universities of China (2014).
A 5 years old child died suddenly beside his father watching television. The immediate appeal of the EMS and Fire Brigade did not allow resuscitation practiced under ideal conditions. The child has a bipolar, epicardial, dual-chamber PM, for Complete AV Block detected in utero. A fracture led to unipolarized atrial lead system because a fracture was discovered during follow-up. However, another lead fracture was also visible at the bifurcation of the ventricular lead system. Nevertheless pacing of both atrial and ventricular chambers was OK. LVEF was borderline lower limit can be explained by abnormal area of contraction near the LV apex. The case is still in Court after failure of two conciliation committees. Two university hospitals, eight lawyers with one PM technician, 17 doctors and cardiologists, four experts including two super-experts (including GF) were involved. Up to now this case has been presented to 25 doctors and cardiologists who have not found the solution. Seven half-days to study a 500-pages file were necessary to completely elucidate the mechanism of this irreversible death. The conclusion which needs knowledge in Medicine, Cardiology, PM technology and heart Pathology (despite absence of autopsy) suffers no alternative. The case will be presented step by step asking participation of the audience. The first document is the last standard ECG recorded before the catastrophe; the second are laboratory data concerning an asymptomatic lupus detected in the mother by specific antibodies which may explain AV block. The third is the standard X-Ray showing the two leads fracture. The last is the post-mortem interrogation of PM memories. Analysis and interconnection of all these elements have led a final diagnosis explaining the catastrophe which has no alternative. Up to now no correct solution has been proposed by 212 Drs and cardiologists who attended the presentation of this case.
Pomeranian Medical University, Poland
Time : 11:40-12:20
Krzysztof Piotrowski, a specialist In Obstetrics, Gynecology and Clinical genetics, completed his PhD with a dissertation on fetal echocardiography. Putting his knowledge into practice, he performs about 3,000 USG investigations of gravidas annually for prenatal diagnosis. He has published many scientific papers and chapters covering prenatal diagnosis. Having introduced the BACs-on-BEADs TM technology to Polish diagnostics, at present he is focused on applying molecular genetics prenatally. For the last nine years he was the Manager of Cytogenetic Unit for Pomeranian Medical University, Szczecin, Poland. Lately, he has founded a new independent genetic centre, DIAGEN – PREDIAG.
Congenital heart diseases are the most common malformations both as an isolated form and as part of genetic syndromes. Extraordinarily fast development of molecular genetics confirms that almost all CHD are genetically dependent in terms of micro-aberrations in different regions of a chromosome or single gene mutations. On the other hand, CHD are an important component of diverse genetic diseases, including monogenic, metabolic and mitochondrial disorders, most often as secondary cardiomyopathies. The genes participating therein are located nearly on each chromosome, mainly on pathways, along with ligand genes and co-factors, transcription factors or individually. Many mechanisms on heart development are based on the balance between apoptosis, proliferation and migration. Crucial genes controlling fetal development, including the creation of heart tube and the forming of left and right ventricular outflow are primary “homeobox” genes grouped in 4 clusters HOX1-4. Other genes condition the forming of different structures. Moreover, in numerous functional disorders, for example the arrhythmia or block, the reason is also genetic, namely the mutation of ion- channel gene placed in 6 chromosomes. Many genes of cardio-genesis were identified, thanks to the investigation of other genetic disorders, for example PTPN11 gene in Noonan syndrome. The gene is also responsible for the development of pulmonary valves or TBX5 gene in Holt-Oram Syndrome. Heart development is also affected by the imprinting (about 30 genes) and the inactivation of the X chromosome in day 21 stage of embryo. We propose that a practical classification could refer to specific CHD characteristic of particular disorders, which might prove helpful in daily practice, because in prenatal diagnosis CHD is often the sole syndrome confirmed by USG scan, which may depend on truly isolated nature or non-specific mild ultrasound co-markers.