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1. A Lumped Parameter Model to Study Atrioventricular Valve Regurgitation in Stage 1 and Changes Across Stage 2 Surgery in Single Ventricle Patients

Author

Catriona Baker, Irene E. Vignon-Clementel, Chiara Corsini, Tain-Yen Hsia, Giancarlo Pennati, Sanjay Pant, College of Engineering [Swansea], Swansea University, Laboratory of biological Structure Mechanics (LaBS), Politecnico di Milano [Milan] (POLIMI), Great Ormond Street Hospital for Children [London] (GOSH), Cardiac Unit, Institute of Child Health (UCL), University College of London [London] (UCL), Numerical simulation of biological flows (REO), Sorbonne Université (SU)-Inria de Paris, Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria)-Laboratoire Jacques-Louis Lions (LJLL (UMR_7598)), and Université Paris Diderot - Paris 7 (UPD7)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS)

Subjects
medicine.medical_specialty, 0206 medical engineering, Heart Valve Diseases, Lumped parameter model, Biomedical Engineering, Hemodynamics, 02 engineering and technology, 030204 cardiovascular system & hematology, Cardiovascular, Hypoplastic left heart syndrome, 03 medical and health sciences, 0302 clinical medicine, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Models, Prolapse, Atrioventricular valve, medicine, Humans, [PHYS.MECA.BIOM]Physics [physics]/Mechanics [physics]/Biomechanics [physics.med-ph], cardiovascular flow, business.industry, incomplete leaflet closure, Cardiovascular flow, Models, Cardiovascular, Infant, Incomplete leaflet closure, Stroke volume, Clinical literature, Valve regurgitation, medicine.disease, Heart Valves, 020601 biomedical engineering, Surgery, medicine.anatomical_structure, hypoplastic left heart syndrome, lumped parameter model, prolapse, valve regurgitation, Hypoplastic Left Heart Syndrome, Ventricle, Single ventricle physiology, Pulmonary venous hypertension, business
Abstract

Goal: This manuscript evaluates atrioventric-ular valve regurgitation (AVVR) in babies born with an already very challenging heart condition, i.e., with single ventricle physiology. Although the second surgery that single ventricle patients undergo is thought to decrease AVVR, there is much controversy in the clinical literature about AVVR treatment. Methods: The effect of AVVR on Stage 1 haemodynamics and resulting acute changes from conversion to Stage 2 circulation in single ventricle patients are analyzed through lumped parameter models. Several degrees of AVVR severity are analyzed, for two types of valve regurgitation: incomplete leaflet closure and valve prolapse. Results: The models show that increasing AVVR in Stage 1 induces the following effects: first, higher stroke volume and associated decrease in ventricular end-systolic volume; second, increase in atrial volumes with V-loop enlargement in pressure-volume curves; third, pulmonary venous hypertension. The Stage 2 surgery results in volume unloading of the ventricle, thereby, driving a decrease in AVVR. However, this effect is offset by an increase in ventricular pressures resulting in a net increase in regurgitation fraction (RF) of approximately 0.1 (for example, in severe AVVR, the preoperative RF increases from $\sim$ 60% to $\sim$ 70% postoperatively). Moreover, despite some improvements to sarcomere function early after Stage 2 surgery, it may deteriorate in cases of severe AVVR. Conclusion: In patients with moderate to severe AVVR, restoration of atrioventricular valve competence prior to, or at the time of, Stage 2 surgery would likely lead to improved haemodynamics and clinical outcome as the models suggest that uncorrected AVVR can worsen across Stage 2 surgery. This was found to be independent of the AVVR degree and mechanisms.

Published
2018
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2. Dystrophin quantification in Duchenne and Becker muscular dystrophy: correlation between dystrophin protein and clinical phenotype

Author

Deborah M. Eastwood, Matthew Ellis, Anirban Majumdar, Joana Domingos, Kathryn Urankar, Jennifer E. Morgan, Gisèle Bonne, Francesco Muntoni, S. Torelli, Giorgio Tasca, Valentina Sardone, Rahul Phadke, Helen Roper, Enzo Ricci, F. Leturcq, A. Jones, R. Barresi, R.B. Yaour, V. Straub, Caroline Sewry, F. Maqsood, Met Office Hadley Centre for Climate Change (MOHC), United Kingdom Met Office [Exeter], Department of Animal Sciences, University of Illinois System, Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de recherche en Myologie – U974 SU-INSERM, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Institut de Myologie, Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Association française contre les myopathies (AFM-Téléthon)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Lunar and Planetary Laboratory [Tucson] (LPL), University of Arizona, Department of Mathematical Sciences, Department of Mathematical Sciences, NMSU, Virologie humaine, École normale supérieure de Lyon (ENS de Lyon)-IFR128-Institut National de la Santé et de la Recherche Médicale (INSERM), Immunité infection vaccination (I2V), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-IFR128-Institut National de la Santé et de la Recherche Médicale (INSERM), Hospices Civils de Lyon (HCL), Università cattolica del Sacro Cuore = Catholic University of the Sacred Heart [Roma] (Unicatt), Cardiac Unit, Institute of Child Health (UCL), and University College of London [London] (UCL)

Subjects
Pathology, medicine.medical_specialty, biology, business.industry, [SDV]Life Sciences [q-bio], medicine.disease, Dystrophine, Neurology, [SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics, Pediatrics, Perinatology and Child Health, medicine, biology.protein, Neurology (clinical), Muscular dystrophy, Clinical phenotype, Dystrophin, business, Genetics (clinical)
Published
2018
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3. First results from the international LMNA -related congenital and childhood onset muscular dystrophy retrospective natural history study

Author

Pomi Yun, Ivana Dabaj, Gina Norato, Hui Xiong, Anna Sarkozy, Anne Rutkowski, Andrés Nascimento, Soledad Monges, Marta Bertoli, Susana Quijano-Roy, Edmar Zanoteli, Francesco Muntoni, Eugenio Mercuri, Hirofumi Komaki, Carsten G. Bönnemann, R. Ben Yaou, Gisèle Bonne, Lorenzo Maggi, K. Bushby, Institut de Myologie, Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Association française contre les myopathies (AFM-Téléthon)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Centre de recherche en Myologie – U974 SU-INSERM, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), National Institutes of Health [Bethesda] (NIH), Laboratoire de Mécanique des Contacts et des Structures [Villeurbanne] (LaMCoS), Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Centre National de la Recherche Scientifique (CNRS), Avignon Université (AU), Cardiac Unit, Institute of Child Health (UCL), University College of London [London] (UCL), Service de Pédiatrie, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Raymond Poincaré [AP-HP], and Hôpital Raymond Poincaré [AP-HP]

Subjects
0301 basic medicine, Pediatrics, medicine.medical_specialty, Lamin A/C, [SDV.GEN]Life Sciences [q-bio]/Genetics, business.industry, [SDV]Life Sciences [q-bio], dystrophie musculaire, medicine.disease, Clinical neurology, LMNA, 03 medical and health sciences, 030104 developmental biology, Neurology, [SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics, Pediatrics, Perinatology and Child Health, Medicine, Neurology (clinical), Muscular dystrophy, business, Genetics (clinical), Natural history study
Published
2017
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4. Statistical shape modelling to aid surgical planning: associations between surgical parameters and head shapes following spring-assisted cranioplasty

Author

David Dunaway, Greg James, Xavier Pennec, Alessandro Borghi, Jan L. Bruse, Juling Ong, Silvia Schievano, N. U. Owase Jeelani, Herman Vercruysse, Naiara Rodriguez-Florez, Great Ormond Street Hospital for Children [London] (GOSH), Cardiac Unit, Institute of Child Health (UCL), University College of London [London] (UCL), Université de Neuchâtel (UNINE), Analysis and Simulation of Biomedical Images (ASCLEPIOS), Inria Sophia Antipolis - Méditerranée (CRISAM), and Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria)

Subjects
Male, medicine.medical_specialty, Statistical shape modelling, medicine.medical_treatment, Population, Biomedical Engineering, Health Informatics, Surgical planning, Craniosynostosis, Partial least squares regression, 03 medical and health sciences, Craniosynostoses, 0302 clinical medicine, Imaging, Three-Dimensional, [INFO.INFO-IM]Computer Science [cs]/Medical Imaging, Medicine, Humans, Radiology, Nuclear Medicine and imaging, 3D scanning, education, Craniofacial surgery, Craniotomy, Orthodontics, education.field_of_study, business.industry, Skull, Infant, General Medicine, Plastic Surgery Procedures, medicine.disease, Computer Graphics and Computer-Aided Design, Cranioplasty, Sagittal plane, Computer Science Applications, Surgery, Clinical decision Support, medicine.anatomical_structure, 030220 oncology & carcinogenesis, Female, Original Article, Computer Vision and Pattern Recognition, business, Tomography, X-Ray Computed, 030217 neurology & neurosurgery
Abstract

Purpose Spring-assisted cranioplasty is performed to correct the long and narrow head shape of children with sagittal synostosis. Such corrective surgery involves osteotomies and the placement of spring-like distractors, which gradually expand to widen the skull until removal about 4 months later. Due to its dynamic nature, associations between surgical parameters and post-operative 3D head shape features are difficult to comprehend. The current study aimed at applying population-based statistical shape modelling to gain insight into how the choice of surgical parameters such as craniotomy size and spring positioning affects post-surgical head shape. Methods Twenty consecutive patients with sagittal synostosis who underwent spring-assisted cranioplasty at Great Ormond Street Hospital for Children (London, UK) were prospectively recruited. Using a nonparametric statistical modelling technique based on mathematical currents, a 3D head shape template was computed from surface head scans of sagittal patients after spring removal. Partial least squares (PLS) regression was employed to quantify and visualise trends of localised head shape changes associated with the surgical parameters recorded during spring insertion: anterior–posterior and lateral craniotomy dimensions, anterior spring position and distance between anterior and posterior springs. Results Bivariate correlations between surgical parameters and corresponding PLS shape vectors demonstrated that anterior–posterior (Pearson’s \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$r=0.64, p=0.002$$\end{document}r=0.64,p=0.002) and lateral craniotomy dimensions (Spearman’s \documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$\rho =0.67, p

Published
2017
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5. Looks Do Matter! Aortic Arch Shape After Hypoplastic Left Heart Syndrome Palliation Correlates With Cavopulmonary Outcomes

Author

Jan L. Bruse, Elena Cervi, Kristin McLeod, Giovanni Biglino, Maxime Sermesant, Xavier Pennec, Andrew M. Taylor, Silvia Schievano, Tain-Yen Hsia, Andrew Taylor, Sachin Khambadkone, Marc de Leval, T.-Y. Hsia, Edward Bove, Adam Dorfman, G. Hamilton Baker, Anthony Hlavacek, Francesco Migliavacca, Giancarlo Pennati, Gabriele Dubini, Alison Marsden, Irene Vignon-Clementel, Richard Figliola, Cardiac Unit, Institute of Child Health (UCL), University College of London [London] (UCL), Analysis and Simulation of Biomedical Images (ASCLEPIOS), Inria Sophia Antipolis - Méditerranée (CRISAM), Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria), Institute of Child Health [London], and Great Ormond Street Hospital for Children [London] (GOSH)

Subjects
Pulmonary and Respiratory Medicine, Aortic arch, Male, medicine.medical_specialty, Ventricular Ejection Fraction, Magnetic Resonance Imaging, Cine, Aorta, Thoracic, 030204 cardiovascular system & hematology, Fontan Procedure, Hypoplastic left heart syndrome, 03 medical and health sciences, 0302 clinical medicine, Imaging, Three-Dimensional, medicine.artery, Internal medicine, Ascending aorta, Hypoplastic Left Heart Syndrome, medicine, [INFO.INFO-IM]Computer Science [cs]/Medical Imaging, Humans, Arch, ComputingMilieux_MISCELLANEOUS, Retrospective Studies, Aorta, Ejection fraction, [SHS.STAT]Humanities and Social Sciences/Methods and statistics, business.industry, Palliative Care, Stroke Volume, medicine.disease, Surgery, Treatment Outcome, Descending aorta, Child, Preschool, Cardiology, Female, Cardiology and Cardiovascular Medicine, business, 030217 neurology & neurosurgery, Follow-Up Studies
Abstract

BACKGROUND: Aortic arch reconstruction after hypoplastic left heart syndrome (HLHS) palliation can vary widely in shape and dimensions between patients. Arch morphology alone may affect cardiac function and outcome. We sought to uncover the relationship of arch three-dimensional shape features with functional and short-term outcome data after total cavopulmonary connection (TCPC).METHODS: Aortic arch shape models of 37 patients with HLHS (age, 2.89 ± 0.99 years) were reconstructed from magnetic resonance data before TCPC completion. A novel, validated statistical shape analysis method was used to compute a three-dimensional anatomic mean shape from the cohort and calculate the deformation vectors of the mean shape toward each patient's specific anatomy. From these deformations, three-dimensional shape features most related to ventricular ejection fraction, indexed end-diastolic volume, and superior cavopulmonary pressure were extracted by partial least-square regression analysis. Shape patterns relating to intensive care unit and hospital lengths of stay after TCPC were assessed.RESULTS: Distinct deformation patterns, which result in an acutely mismatched aortic root and ascending aorta, and a gothic-like transverse arch, correlated with increased indexed end-diastolic volume and higher superior cavopulmonary pressure but not with ejection fraction. Specific arch morphology with pronounced transverse arch and descending aorta mismatch also correlated with longer intensive care unit and hospital lengths of stay after TCPC completion.CONCLUSIONS: Independent of hemodynamically important arch obstruction, altered aortic morphology in HLHS patients appears to have important associations with higher superior cavopulmonary pressure and with short-term outcomes after TCPC completion as highlighted by statistical shape analysis, which could act as adjunct to risk assessment in HLHS.

Published
2016
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6. Voxelwise atlas rating for computer assisted diagnosis: Application to congenital heart diseases of the great arteries

Author

Ninon Burgos, Alex F. Mendelson, Andrew M. Taylor, Maria A. Zuluaga, Sebastien Ourselin, Centre for Medical Image Computing (CMIC), University College of London [London] (UCL), Great Ormond Street Hospital for Children [London] (GOSH), Cardiac Unit, Institute of Child Health (UCL), and UCL Institute of Neurology, Queen Square, London

Subjects
Computer science, Morphological similarity, Transposition of Great Vessels, education, Health Informatics, Similarity measure, computer.software_genre, Sensitivity and Specificity, Cross-validation, Article, Congenital heart diseases, 030218 nuclear medicine & medical imaging, Pattern Recognition, Automated, 03 medical and health sciences, 0302 clinical medicine, Voxel rating, Atlas (anatomy), Voxel, Image Interpretation, Computer-Assisted, medicine, [INFO.INFO-IM]Computer Science [cs]/Medical Imaging, Humans, Radiology, Nuclear Medicine and imaging, Computer vision, Radiological and Ultrasound Technology, business.industry, Reproducibility of Results, Atlases, Image synthesis, Computer-aided diagnosis, Image Enhancement, Computer Graphics and Computer-Aided Design, medicine.anatomical_structure, Great arteries, Radiology Nuclear Medicine and imaging, Subtraction Technique, Artificial intelligence, Computer Vision and Pattern Recognition, Mr images, business, computer, 030217 neurology & neurosurgery, Algorithms, Magnetic Resonance Angiography
Abstract

Highlights • This paper presents a voxelwise atlas rating approach for computer-aided diagnosis. • The method relies on multiple atlas databases, but does not require annotated images. • The method reports an accuracy of 97.3%, which is higher than other state-of-the-art methods., Graphical abstract, Atlas-based analysis methods rely on the morphological similarity between the atlas and target images, and on the availability of labelled images. Problems can arise when the deformations introduced by pathologies affect the similarity between the atlas and a patient’s image. The aim of this work is to exploit the morphological dissimilarities between atlas databases and pathological images to diagnose the underlying clinical condition, while avoiding the dependence on labelled images. We propose a voxelwise atlas rating approach (VoxAR) relying on multiple atlas databases, each representing a particular condition. Using a local image similarity measure to assess the morphological similarity between the atlas and target images, a rating map displaying for each voxel the condition of the atlases most similar to the target is defined. The final diagnosis is established by assigning the condition of the database the most represented in the rating map. We applied the method to diagnose three different conditions associated with dextro-transposition of the great arteries, a congenital heart disease. The proposed approach outperforms other state-of-the-art methods using annotated images, with an accuracy of 97.3% when evaluated on a set of 60 whole heart MR images containing healthy and pathological subjects using cross validation.

Published
2015
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7. The EuroBioBank Network: 10 years of hands-on experience of collaborative, transnational biobanking for rare diseases

Author

Mirella Filocamo, Alessandra Renieri, Corrado Angelini, Marina Mora, Peter Schneiderat, Luisa Politano, Stefano Goldwurm, Stephen Lynn, Anne Mary Bodin, Safaa Saker, Yann Lecam, Monica Ensini, David Gurwitz, Hanns Lochmüller, Marco Crimi, Mojgan Reza, Veronika Karcagi, Lucia Monaco, Elena Pegoraro, Alex E. Felice, Kurt Zatloukal, Franca Dagna Bricarelli, C. Baldo, Fabrizia Bignami, Diana Johnson, Maurizio Moggio, Jack Puymirat, Manuel Posada de la Paz, Cécile Jaeger, Giuseppe Merla, Thomas Voit, Francesco Muntoni, Barbara Garavaglia, Jeanne Hélène di Donato, Marija Meznaric, Sciences Economiques et Sociales de la Santé & Traitement de l'Information Médicale (SESSTIM - U912 INSERM - Aix Marseille Univ - IRD), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Università degli Studi di Padova = University of Padua (Unipd), GlaxoSmithKline, Glaxo Smith Kline, EURORDIS - Plateforme Maladies Rares [Paris], Fondazione Telethon, 3 C-R, University of Malta [Malta], Potsdam Institute for Climate Impact Research (PIK), National Institute of Environmental Health Sciences [Durham] (NIEHS-NIH), National Institutes of Health [Bethesda] (NIH), Newcastle University [Newcastle], University of Ljubljana, Università degli Studi di Milano = University of Milan (UNIMI), Istituto Nazionale di Fisica Nucleare, Sezione di Milano (INFN), Istituto Nazionale di Fisica Nucleare (INFN), Second University of Naples-Caserta, University of Naples Federico II = Università degli studi di Napoli Federico II, CIBER de Enfermedades Raras (CIBERER), Approches génétiques intégrées et nouvelles thérapies pour les maladies rares (INTEGRARE), École Pratique des Hautes Études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université d'Évry-Val-d'Essonne (UEVE)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Généthon, Ludwig-Maximilians University [Munich] (LMU), Fondazione IRCCS Istituto Neurologico 'Carlo Besta', Tel Aviv University (TAU), Cardiac Unit, Institute of Child Health (UCL), University College of London [London] (UCL), CHU de Québec–Université Laval, Université Laval [Québec] (ULaval), Aalto University, CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université Paris Cité (UPCité), E.O. Ospedali Galliera, Istituti Clinici di Perfezionamento, Istituto di Ricovero e Cura a Carattere Scientifico, Ospedale Casa Sollievo della Sofferenza [San Giovanni Rotondo] (IRCCS), Università degli Studi di Siena = University of Siena (UNISI), Medical University Graz, Istituto G.Gaslini, Mora, M, Angelini, C, Bignami, F, Bodin, Am, Crimi, M, Di Donato, Jh, Felice, A, Jaeger, C, Karcagi, V, Lecam, Y, Lynn, S, Meznaric, M, Moggio, M, Monaco, L, Politano, Luisa, Posada de la Paz, M, Saker, S, Schneiderat, P, Ensini, M, Garavaglia, B, Gurwitz, D, Johnson, D, Muntoni, F, Puymirat, J, Reza, M, Voit, T, Baldo, C, Dagna Bricarelli, F, Goldwurm, S, Merla, G, Pegoraro, E, Renieri, A, Zatloukal, K, Filocamo, M, Lochmüller, H., Unión Europea. Comisión Europea. 5 Programa Marco, Unión Europea. Comisión Europea. 6 Programa Marco, Unión Europea. Comisión Europea. 7 Programa Marco, Medical Research Council (Reino Unido), Institut de Recherche pour le Développement (IRD)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Aix Marseille Université (AMU), University of Padova [Padova, Italy], University of Milan, University of Naples Federico II, Université d'Évry-Val-d'Essonne (UEVE)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Généthon-École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL), Ludwig Maximilians University of Munich, Tel Aviv University [Tel Aviv], Université de Paris (UP), Padova University, University of Siena (University of Siena), HAL, Univ Évry, and École pratique des hautes études (EPHE)

Subjects
Quality Control, Service (systems architecture), Knowledge management, Human dna, Best practice, International Cooperation, [SDV]Life Sciences [q-bio], Biology, Phase (combat), 03 medical and health sciences, 0302 clinical medicine, Consolidation (business), Rare Diseases, Genetics, Humans, European commission, Registries, Genetics (clinical), 030304 developmental biology, Biobank, Biological Specimen Banks, Computational Biology, Europe, 0303 health sciences, business.industry, Health care, Professional standards, [SDV] Life Sciences [q-bio], Policy, EBB, business, 030217 neurology & neurosurgery
Abstract

The EuroBioBank (EBB) network (www.eurobiobank.org) is the first operating network of biobanks in Europe to provide human DNA, cell and tissue samples as a service to the scientific community conducting research on rare diseases (RDs). The EBB was established in 2001 to facilitate access to RD biospecimens and associated data; it obtained funding from the European Commission in 2002 (5th framework programme) and started operation in 2003. The set-up phase, during the EC funding period 2003-2006, established the basis for running the network; the following consolidation phase has seen the growth of the network through the joining of new partners, better network cohesion, improved coordination of activities, and the development of a quality-control system. During this phase the network participated in the EC-funded TREAT-NMD programme and was involved in planning of the European Biobanking and Biomolecular Resources Research Infrastructure. Recently, EBB became a partner of RD-Connect, an FP7 EU programme aimed at linking RD biobanks, registries, and bioinformatics data. Within RD-Connect, EBB contributes expertise, promotes high professional standards, and best practices in RD biobanking, is implementing integration with RD patient registries and 'omics' data, thus challenging the fragmentation of international cooperation on the field. EBB thanks EURORDIS for administrative support, Fondazione Telethon for administrative and financial support, the EC for providing funds (grants: FP5 EuroBioBank, contract N. 02769; FP6 TREAT-NMD, contract N. 036825; and FP7 RD-Connect, grant agreement N. 305444), and AFM for the vision of the network and for encouraging the network to get together. EBB gratefully acknowledges patients and collaborating clinicians/researchers for contributing samples and data to the network, and biobank staffs for running the EBB biobanks. The MRC support to the biobank in London and Newcastle is also gratefully acknowledged. Sí

Published
2015
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8. Evidence-based provisional clinical classification criteria for autoinflammatory periodic fevers

Author

Federici S., Sormani M. P., Ozen S., Lachmann H. J., Amaryan G., Woo P., Kone-Paut I., Dewarrat N., Cantarini L., Insalaco A., Uziel Y., Rigante D., Quartier P., Demirkaya E., Herlin T., Meini A., Fabio G., Kallinich T., Martino S., Butbul A. Y., Olivieri A., Kuemmerle-Deschner J., Neven B., Simon A., Ozdogan H., Touitou I., Frenkel J., Hofer M., Martini A., Ruperto N., Gattorno M., Espada G., Russo R., De Cunto C., Boros C., Borzutzky A., Jelusic-Drazic M., Dolezalova P., Nielsen S., Hentgen V., Schwarz T., Berendes R., Jansson A., Horneff G., Papadopoulou-Alataki E., Tsitsami E., Tsakalidou F. K., Gallizzi R., Obici L., Barone P., Cimaz R., Alessio M., Nishikomori R., Stanevicha V., Hoppenreijs E., Wolska-Kusnierz B., Iagaru N., Nikishina I., Al-Mayouf S. M., Sewairi, Susic G., Toplak N., Modesto C., Elorduy M. J. R., Anton J., Bou R., Federici, S., Sormani, M. P., Ozen, S., Lachmann, H. J., Amaryan, G., Woo, P., Kone-Paut, I., Dewarrat, N., Cantarini, L., Insalaco, A., Uziel, Y., Rigante, D., Quartier, P., Demirkaya, E., Herlin, T., Meini, A., Fabio, G., Kallinich, T., Martino, S., Butbul, A. Y., Olivieri, A., Kuemmerle-Deschner, J., Neven, B., Simon, A., Ozdogan, H., Touitou, I., Frenkel, J., Hofer, M., Martini, A., Ruperto, N., Gattorno, M., Espada, G., Russo, R., De Cunto, C., Boros, C., Borzutzky, A., Jelusic-Drazic, M., Dolezalova, P., Nielsen, S., Hentgen, V., Schwarz, T., Berendes, R., Jansson, A., Horneff, G., Papadopoulou-Alataki, E., Tsitsami, E., Tsakalidou, F. K., Gallizzi, R., Obici, L., Barone, P., Cimaz, R., Alessio, M., Nishikomori, R., Stanevicha, V., Hoppenreijs, E., Wolska-Kusnierz, B., Iagaru, N., Nikishina, I., Al-Mayouf, S. M., Sewairi, Susic, G., Toplak, N., Modesto, C., Elorduy, M. J. R., Anton, J., Bou, R., Istituto Giannina Gaslini, Genova, Immunologia Clinica e Sperimentale, University of Genoa (UNIGE), Hacettepe University = Hacettepe Üniversitesi, National Amyloidosis Centre, University College London Medical School, University College of London [London] (UCL), 'ARABKIR' JOINT MEDICAL CENTER & INSTITUTE OF CHILD AND ADOLESCENT HEALTH, Cardiac Unit, Institute of Child Health (UCL), Centre de Référence des Maladies Auto-Inflammatoires et des Amyloses [CH Versailles] (CeRéMAIA - Hôpital André Mignot), Centre Hospitalier de Versailles André Mignot (CHV), University Hospital Center (CHUV) and University of Lausanne (UNIL), Lausanne, Università degli Studi di Siena = University of Siena (UNISI), Children's Hospital Bambino Gesù IRCCS [Rome], Meir Medical Centre, Università cattolica del Sacro Cuore [Roma] (Unicatt), Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Ankara University School of Medicine [Turkey], Aarhus University Hospital, Università degli Studi di Brescia [Brescia], IRCCS Istituto Nazionale dei Tumori [Milano], Charité - UniversitätsMedizin = Charité - University Hospital [Berlin], Università degli studi di Torino (UNITO), Rambam Medical Health Center, Israel., Universita degli studi di Napoli 'Parthenope' [Napoli], Universitätsklinikum Tübingen - University Hospital of Tübingen, Eberhard Karls Universität Tübingen = Eberhard Karls University of Tuebingen, Radboud University Medical Centre [Nijmegen, The Netherlands], Cerrahpasa Faculty of Medicine, Istanbul University, CHU Montpellier, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB), Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), University Medical Center [Utrecht], Geneva University Hospital (HUG), Universita degli studi di Genova, and Olivieri, Alma Nunzia

Subjects
Male, Pediatrics, Multivariate analysis, [SDV]Life Sciences [q-bio], Fever Syndromes, Familial Mediterranean fever, Immunology and Allergy, Mevalonate Kinase Deficiency/classification/diagnosis, Registries, Child, ddc:618, Evidence-Based Medicine, Middle Aged, Pharyngitis, 3. Good health, Familial Mediterranean Fever, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Child, Preschool, Autoinflammation, Familial Mediterranean Fever/classification/diagnosis, Female, medicine.symptom, Periodic fever syndrome, Inflammatory diseases Radboud Institute for Molecular Life Sciences [Radboudumc 5], Adult, medicine.medical_specialty, Fever, Adolescent, Immunology, Cryopyrin-Associated Periodic Syndromes/classification/diagnosis, Sensitivity and Specificity, General Biochemistry, Genetics and Molecular Biology, Young Adult, Inflammation, Rheumatology, medicine, Humans, Preschool, Hereditary Autoinflammatory Diseases/classification/diagnosis, Receiver operating characteristic, business.industry, Hereditary Autoinflammatory Diseases, Case-control study, Cryopyrin-associated periodic syndrome, Infant, Gold standard (test), medicine.disease, Case-Control Studies, Cryopyrin-Associated Periodic Syndromes, Mevalonate Kinase Deficiency, ROC Curve, business
Abstract

Item does not contain fulltext The objective of this work was to develop and validate a set of clinical criteria for the classification of patients affected by periodic fevers. Patients with inherited periodic fevers (familial Mediterranean fever (FMF); mevalonate kinase deficiency (MKD); tumour necrosis factor receptor-associated periodic fever syndrome (TRAPS); cryopyrin-associated periodic syndromes (CAPS)) enrolled in the Eurofever Registry up until March 2013 were evaluated. Patients with periodic fever, aphthosis, pharyngitis and adenitis (PFAPA) syndrome were used as negative controls. For each genetic disease, patients were considered to be 'gold standard' on the basis of the presence of a confirmatory genetic analysis. Clinical criteria were formulated on the basis of univariate and multivariate analysis in an initial group of patients (training set) and validated in an independent set of patients (validation set). A total of 1215 consecutive patients with periodic fevers were identified, and 518 gold standard patients (291 FMF, 74 MKD, 86 TRAPS, 67 CAPS) and 199 patients with PFAPA as disease controls were evaluated. The univariate and multivariate analyses identified a number of clinical variables that correlated independently with each disease, and four provisional classification scores were created. Cut-off values of the classification scores were chosen using receiver operating characteristic curve analysis as those giving the highest sensitivity and specificity. The classification scores were then tested in an independent set of patients (validation set) with an area under the curve of 0.98 for FMF, 0.95 for TRAPS, 0.96 for MKD, and 0.99 for CAPS. In conclusion, evidence-based provisional clinical criteria with high sensitivity and specificity for the clinical classification of patients with inherited periodic fevers have been developed.

Published
2015
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9. Corticosteroid treatment in early-onset lamin A/C related muscular dystrophies

Author

M. Lorenzo, F. Muntoni, Akihiko Ishiyama, Anne Rutkowski, Emmanuelle Lagrue, Thomas Sejersen, James J. Dowling, Susana Quijano-Roy, Juliana Gurgel-Giannetti, Karin Kleinsteuber, Ivana Dabaj, Soledad Monges, Andrés Nascimento, R. Erazo Torricelli, R. Ben Yaou, C. Casteglioni, Gisèle Bonne, Carsten G. Bönnemann, K. Bushby, Institut de Myologie, Commissariat à l'énergie atomique et aux énergies alternatives (CEA)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Association française contre les myopathies (AFM-Téléthon)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Centre National de la Recherche Scientifique (CNRS), Centre de recherche en Myologie – U974 SU-INSERM, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), National Institutes of Health [Bethesda] (NIH), Avignon Université (AU), Cardiac Unit, Institute of Child Health (UCL), University College of London [London] (UCL), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), UMR 1253 IBrain Imagerie & Cerveau Equipe 3 'Imagerie, Biomarqueurs & Thérapie' (IBT), Imagerie et cerveau (iBrain - Inserm U1253 - UNIV Tours ), Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Commonwealth Scientific and Industrial Research Organisation [Canberra] (CSIRO), Université de Strasbourg (UNISTRA), Service de Pédiatrie, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Raymond Poincaré [AP-HP], and Hôpital Raymond Poincaré [AP-HP]

Subjects
0301 basic medicine, Lamin A/C, medicine.medical_specialty, business.industry, [SDV]Life Sciences [q-bio], Corticosteroid treatment, 03 medical and health sciences, 030104 developmental biology, Endocrinology, Neurology, Internal medicine, Pediatrics, Perinatology and Child Health, medicine, Neurology (clinical), business, Genetics (clinical), Lamin, Early onset
Abstract

International audience

Published
2017
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10. Screening, intervention and outcome in autism and other developmental disorders: the role of randomized controlled trials

Author

Elisabeth Fernell, David Taylor, Helen Minnis, Thomas Bourgeron, Philip Wilson, Brian Neville, Christopher Gillberg, Nouchine Hadjikhani, University of Gothenburg (GU), University of Aberdeen, Athinoula A. Martinos Center for Biomedical Imaging, Massachusetts General Hospital [Boston]-Harvard Medical School [Boston] (HMS), Génétique humaine et fonctions cognitives - Human Genetics and Cognitive Functions (GHFC (UMR_3571 / U-Pasteur_1)), Institut Pasteur [Paris]-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS), Cardiac Unit, Institute of Child Health (UCL), University College of London [London] (UCL), University of Manchester [Manchester], University of Glasgow, Harvard Medical School [Boston] (HMS)-Massachusetts General Hospital [Boston], and Institut Pasteur [Paris] (IP)-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS)

Subjects
medicine.medical_specialty, Referral, Developmental Disabilities, Autism, [SDV]Life Sciences [q-bio], Population, MESH: Autistic Disorder, Intervention, Outcome (game theory), law.invention, 03 medical and health sciences, [SCCO]Cognitive science, 0302 clinical medicine, Randomized controlled trial, law, Intervention (counseling), mental disorders, Developmental and Educational Psychology, Medicine, Humans, Mass Screening, 0501 psychology and cognitive sciences, MESH: Mass Screening, Autistic Disorder, education, Psychiatry, Randomized Controlled Trials as Topic, MESH: Treatment Outcome, education.field_of_study, MESH: Humans, business.industry, Public health, 05 social sciences, Developmental disorders, medicine.disease, 3. Good health, Treatment Outcome, MESH: Randomized Controlled Trials as Topic, MESH: Developmental Disabilities, [SDV.MHEP.PSM]Life Sciences [q-bio]/Human health and pathology/Psychiatrics and mental health, Screening, Randomized controlled trials, business, 030217 neurology & neurosurgery, Screening intervention, 050104 developmental & child psychology, Clinical psychology
Abstract

International audience; We draw attention to a number of important considerations in the arguments about screening and outcome of intervention in children with autism and other developmental disorders. Autism screening in itself never provides a final clinical diagnosis, but may well identify developmental deviations indicative of autism-or of other developmental disorders-that should lead to referral for further clinical assessment. Decisions regarding population or clinic screening cannot be allowed to be based on the fact that prospective longitudinal RCT designs over decades could never be performed in complex developmental disorders. We propose an alternative approach. Early screening for autism and other developmental disorders is likely to be of high societal importance and should be promoted and rigorously evaluated.

Published
2014
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11. An integrated approach to patient-specific predictive modeling for single ventricle heart palliation

Author

Ethan Kung, Tain Yen Hsia, Giancarlo Pennati, Alessia Baretta, Catriona Baker, Chiara Corsini, Giovanni Biglino, Adam L. Dorfman, Gabriele Dubini, Alison L. Marsden, Francesco Migliavacca, Irene E. Vignon-Clementel, Silvia Schievano, Gregory Arbia, Andrew M. Taylor, Laboratory of biological Structure Mechanics (LaBS), Politecnico di Milano [Milan] (POLIMI), Great Ormond Street Hospital for Children [London] (GOSH), Cardiac Unit, Institute of Child Health (UCL), University College of London [London] (UCL), Department of Mechanical and Aerospace Engineering [La Jolla] (UCSD), University of California [San Diego] (UC San Diego), University of California-University of California, Numerical simulation of biological flows (REO), Laboratoire Jacques-Louis Lions (LJLL), Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS)-Inria Paris-Rocquencourt, Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria), Institute of Child Health [London], Department of Pediatrics, University of Michigan [Ann Arbor], University of Michigan System-University of Michigan System, Department of Mechanical and Aerospace Engineering [Univ California San Diego] (MAE - UC San Diego), and University of California (UC)-University of California (UC)

Subjects
medicine.medical_specialty, Palliative care, Heart disease, medicine.medical_treatment, Heart Ventricles, 0206 medical engineering, Biomedical Engineering, Bioengineering, 02 engineering and technology, computational fluid dynamics, 030204 cardiovascular system & hematology, Fontan Procedure, lumped parameter network, Surgical planning, Article, Fontan procedure, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, Computer Simulation, Stage (cooking), business.industry, Palliative Care, Hemodynamics, Models, Cardiovascular, Infant, General Medicine, Patient specific, Integrated approach, [INFO.INFO-NA]Computer Science [cs]/Numerical Analysis [cs.NA], medicine.disease, 020601 biomedical engineering, congenital heart disease, Computer Science Applications, Surgery, Human-Computer Interaction, medicine.anatomical_structure, Ventricle, business, virtual surgery, multi-scale model
Abstract

International audience; In patients with congenital heart disease and a single ventricle (SV), ventricular support of the circulation is inadequate, and staged palliative surgery (usually 3 stages) is needed for treatment. In the various palliative surgical stages individual differences in the circulation are important and patient-specific surgical planning is ideal. In this study, an integrated approach between clinicians and engineers has been developed, based on patient-specific multi-scale models, and is here applied to predict stage 2 surgical outcomes. This approach involves four distinct steps: (1) collection of pre-operative clinical data from a patient presenting for SV palliation, (2) construction of the pre-operative model, (3) creation of feasible virtual surgical options which couple a three-dimensional model of the surgical anatomy with a lumped parameter model (LPM) of the remainder of the circulation and (4) performance of post-operative simulations to aid clinical decision making. The pre-operative model is described, agreeing well with clinical flow tracings and mean pressures. Two surgical options (bi-directional Glenn and hemi-Fontan operations) are virtually performed and coupled to the pre-operative LPM, with the hemodynamics of both options reported. Results are validated against postoperative clinical data. Ultimately, this work represents the first patient-specific predictive modeling of stage 2 palliation using virtual surgery and closed-loop multi- scale modeling.

Published
2014
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12. Numerical blood flow simulation in surgical corrections: what do we need for an accurate analysis?

Author

Gregory, Arbia, Chiara, Corsini, Mahdi, Esmaily Moghadam, Alison L, Marsden, Francesco, Migliavacca, Giancarlo, Pennati, Tain-Yen, Hsia, Irene E, Vignon-Clementel, John, McGregor, Numerical simulation of biological flows (REO), Laboratoire Jacques-Louis Lions (LJLL), Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS)-Inria Paris-Rocquencourt, Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria), Laboratory of biological Structure Mechanics (LaBS), Politecnico di Milano [Milan] (POLIMI), Department of Mechanical and Aerospace Engineering [La Jolla] (UCSD), University of California [San Diego] (UC San Diego), University of California-University of California, Great Ormond Street Hospital for Children [London] (GOSH), Cardiac Unit, Institute of Child Health (UCL), University College of London [London] (UCL), Multi-scale Modeling of Single Ventricle Hearts for Clinical Decision Support, CARDIO, Department of Mechanical and Aerospace Engineering [Univ California San Diego] (MAE - UC San Diego), and University of California (UC)-University of California (UC)

Subjects
Heart Defects, Congenital, Computer science, Pulsatile flow, Computational fluid dynamics, Patient specific modeling, Complex geometry, CFD, Numerical methods, Code comparison, Single ventricle palliation, Applied mathematics, Humans, Computer Simulation, Boundary value problem, Patient-Specific Modeling, business.industry, Numerical analysis, Models, Cardiovascular, Blood flow, Solver, [INFO.INFO-NA]Computer Science [cs]/Numerical Analysis [cs.NA], Pulsatile Flow, Blood Circulation, Hydrodynamics, Surgery, business
Abstract

International audience; Background: Computational fluid dynamics has been increasingly used in congenital heart surgery to simulate pathophysiological blood flow, investigate surgical options, or design medical devices. Several commercial and research computational or numerical codes have been developed. They present different approaches to numerically solve the blood flow equations, raising the question whether these numerical codes are equally reliable to achieve accurate simulation results. Accordingly, we sought to examine the influence of numerical code selection in several complex congenital cardiac operations. Material and methods: The main steps of blood flow simulations are detailed (geometrical mesh, boundary conditions, and solver numerical methods) for congenital cardiac operations of increasing complexity. The first case tests different numerical solutions against an analytical, or exact, solution. In the second case, the three-dimensional domain is a patient-specific superior cavopulmonary anastomosis. As an analytical solution does not exist in such a complex geometry, different numerical solutions are compared. Finally, a realistic case of a systemic-to-pulmonary shunt is presented with both geometrically and physiologically challenging conditions. For all, solutions from a commercially available code and an open-source research code are compared. Results: In the first case, as the mesh or solver numerical method is refined, the simulation results for both codes converged to the analytical solution. In the second example, velocity differences between the two codes are greater when the resolution of the mesh were lower and less refined. The third case with realistic anatomy reveals that the pulsatile complex flow is very similar for both codes. Conclusions: The precise setup of the numerical cases has more influence on the results than the choice of numerical codes. The need for detailed construction of the numerical model that requires high computational cost depends on the precision needed to answer the biomedical question at hand and should be assessed for each problem on a combination of clinically relevant patient-specific geometry and physiological conditions.

Published
2013
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13. Respiratory effects on hemodynamics in patient-specific CFD models of the Fontan circulation under exercise conditions

Author

Chiara Corsini, Gabriele Dubini, Alison L. Marsden, Giancarlo Pennati, Tain-Yen Hsia, Irene E. Vignon-Clementel, Alessia Baretta, Francesco Migliavacca, Laboratory of biological Structure Mechanics (LaBS), Politecnico di Milano [Milan] (POLIMI), Department of Mechanical and Aerospace Engineering [Univ California San Diego] (MAE - UC San Diego), University of California [San Diego] (UC San Diego), University of California (UC)-University of California (UC), Numerical simulation of biological flows (REO), Laboratoire Jacques-Louis Lions (LJLL), Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7)-Centre National de la Recherche Scientifique (CNRS)-Inria Paris-Rocquencourt, Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria), Great Ormond Street Hospital for Children [London] (GOSH), Cardiac Unit, Institute of Child Health (UCL), University College of London [London] (UCL), Leducq foundation, Modeling Of Congenital Hearts Alliance, Department of Mechanical and Aerospace Engineering [La Jolla] (UCSD), and University of California-University of California

Subjects
medicine.medical_specialty, 0206 medical engineering, General Physics and Astronomy, Hemodynamics, 02 engineering and technology, 030204 cardiovascular system & hematology, Anastomosis, Computational fluid dynamics, Inferior vena cava, [SPI.MECA.MEFL]Engineering Sciences [physics]/Mechanics [physics.med-ph]/Fluids mechanics [physics.class-ph], Lumped-parameter, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Medicine, [PHYS.MECA.MEFL]Physics [physics]/Mechanics [physics]/Fluid mechanics [physics.class-ph], Respiratory system, [PHYS.MECA.BIOM]Physics [physics]/Mechanics [physics]/Biomechanics [physics.med-ph], Multiscale model, Mathematical Physics, Congenital heart disease, Mathematical models, business.industry, [SPI.MECA.BIOM]Engineering Sciences [physics]/Mechanics [physics.med-ph]/Biomechanics [physics.med-ph], Mechanics, 020601 biomedical engineering, Right pulmonary artery, 3. Good health, medicine.anatomical_structure, medicine.vein, Ventricle, Cardiology, Venae cavae, business, Lower limbs venous ultrasonography
Abstract

MOCHA Investigators: Andrew Taylor, MD, Alessandro Giardini, MD, Sachin Khambadkone, MD, Silvia Schievano, PhD, Marc de Leval, MD, and T.-Y. Hsia, MD (Institute of Child Health, London, United Kingdom); Edward Bove, MD, and Adam Dorfman, MD (University of Michigan, Ann Arbor, MI); G. Hamilton Baker, MD, and Anthony Hlavacek (Medical University of South Carolina, Charleston, SC); Francesco Migliavacca, PhD, Giancarlo Pennati, PhD, and Gabriele Dubini, PhD (Politecnico di Milano, Milan, Italy); Alison Marsden, PhD (University of California, San Diego, CA); Jeffrey Feinstein, MD (Stanford University, Stanford, CA) Irene Vignon-Clementel (National Institute of Research in Informatics and Automation, Paris, France); Richard Figliola, PhD, and John McGregor, PhD (Clemson University, Clemson, SC).; International audience; Single ventricle malformations are complex congenital heart defects which require a three-stage surgical treatment, starting from the very first days of life, to separate the systemic and pulmonary circulations, and restore the serial circuit occurring in normal patients. The final surgery results in a total cavopulmonary connection (TCPC), where both the superior and the inferior vena cava are connected to the right pulmonary artery. Several clinical and computational studies have been done to optimize the geometry of the TCPC, with the aim of minimizing energy losses and improving surgical outcomes. To date, only few modeling studies have taken into account respiration and exercise as important factors to quantify the performance of a Fontan geometry. The objective of this work is to test the dependence of fluid dynamic variables and energy efficiency on respiration in patient-specific models of Fontan circulation, when subjected to exercise tests.|A closed-loop multiscale approach was used, including a simple respiration model that modulates the extravascular pressures in the thoracic and abdominal cavities, to generate physiologic time-varying flow conditions. A lumped parameter network (LPN) representing the whole circulation was coupled to a patient-specific 3D finite volume model of the preoperative bidirectional cavo-pulmonary anastomosis (BCPA) with detailed pulmonary anatomy. Subsequently, three virtual TCPC alternatives were coupled to the LPN and investigated in terms of both local and global hemodynamics. In particular, a T-junction of the venae cavae to the pulmonary arteries, a design with an offset between the venae cavae and a Y-graft design were compared under exercise conditions.|Results showed that the BCPA model is able to realistically capture oscillations due to both cardiac and respiratory effects, when compared to the venous Doppler velocity tracings acquired preoperatively on the patient.|The differences in hemodynamics between the three investigated TCPC options were minimal and similar to those obtained without inclusion of respiratory effects. Hence, the three surgical options result to be equivalent according to the analyzed parameters. Moreover, although the simulation of the Fontan circulation with a respiratory model requires a longer computational time, the developed framework allows for a more physiologic method to incorporate respiratory effects that was not possible using other methods.

Published
2012
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