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1. Specific Morphology of Coronary Artery Aneurysms in Mainly White Patients With Kawasaki Disease: Initial Data From the Cardiac Catheterization in Kawasaki Disease Registry

Author

Julia Weisser, Leonie Arnold, Wolfgang Wällisch, Daniel Quandt, Bernd Opgen‐Rhein, Frank‐Thomas Riede, Florentine Gräfe, Jörg Michel, Raoul Arnold, Heike Schneider, Daniel Tanase, Ulrike Herberg, Christoph Happel, Mali Tietje, Gleb Tarusinov, Jochen Grohmann, Johanna Hummel, André Rudolph, Nikolaus Haas, and André Jakob

Subjects
cardiac catheterization, coronary artery pathology, Kawasaki disease, long‐term cardiac care, White, Diseases of the circulatory (Cardiovascular) system, RC666-701
Abstract

Background Patients with Kawasaki disease (KD) with coronary artery involvement require long‐term cardiac care. Although respective evidence‐based recommendations are missing, cardiac catheterization is still considered the gold standard for diagnosing detailed coronary pathology. Therefore, to better understand coronary artery pathology development, we conducted a survey to document and evaluate cardiac catheterization data in a European population. Methods and Results We retrospectively analyzed cardiac catheterization data from KD children from the year 2010 until April 2023. This registry covers basic acute‐phase clinical data, and more importantly, detailed information on morphology, distribution, and the development of coronary artery pathologies. A total of 164 mainly White patients (65% boys) were included. A relevant number of patients had no coronary artery aneurysm (CAA) at the cardiac catheterization, indicating that distal CAAs were almost exclusively detected alongside proximal CAAs. Patients with multiple CAAs revealed a significant positive correlation between the number of CAAs and their dimensions in diameter and in length. Location of the CAA within the coronary artery, age at onset of KD, or natal sex did not significantly influence CAA diameters, but CAAs were longer in older children and in boys. Conclusions That distal CAAs were only present together with proximal ones will hopefully reduce diagnostic CCs in patients with KD without echocardiographically detected proximal CAAs. Furthermore, this study gives valuable insights into dimensional specifics of CAAs in patients with KD. As an ongoing registry, future analyses will further explore long‐term outcomes and performed treatments, helping to refine clinical long‐term strategies for patients with KD. REGISTRATION URL: https://drks.de/; Unique Identifier: DRKS00031022.

Published
2024
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2. Relationship between molecular pathogen detection and clinical disease in febrile children across Europe: a multicentre, prospective observational studyResearch in context

Author

Priyen Shah, Marie Voice, Leonides Calvo-Bado, Irene Rivero-Calle, Sophie Morris, Ruud Nijman, Claire Broderick, Tisham De, Irini Eleftheriou, Rachel Galassini, Aakash Khanijau, Laura Kolberg, Mojca Kolnik, Aleksandra Rudzate, Manfred G. Sagmeister, Nina A. Schweintzger, Fatou Secka, Clare Thakker, Fabian van der Velden, Clementien Vermont, Katarina Vincek, Philipp K.A. Agyeman, Aubrey J. Cunnington, Ronald De Groot, Marieke Emonts, Katy Fidler, Taco W. Kuijpers, Marine Mommert-Tripon, Karen Brengel-Pesce, Francois Mallet, Henriette Moll, Stéphane Paulus, Marko Pokorn, Andrew Pollard, Luregn J. Schlapbach, Ching-Fen Shen, Maria Tsolia, Effua Usuf, Michiel van der Flier, Ulrich von Both, Shunmay Yeung, Dace Zavadska, Werner Zenz, Victoria Wright, Enitan D. Carrol, Myrsini Kaforou, Federico Martinon-Torres, Colin Fink, Michael Levin, Jethro Herberg, Irene Rivero Calle, Manfred Sagmeister, Nina Schweintzger, Fabian Van der Velden, Taco Kuijpers, Michiel Van der Flier, Ulrich Von Both, Lucas Baumard, Evangelos Bellos, Lachlan Coin, Giselle D'Souza, Dominic Habgood-Coote, Shea Hamilton, Cllive Hoggart, Sara Hourmat, Heather Jackson, Naomi Lin, Stephanie Menikou, Samuel Nichols, Ivonne Pena Paz, Oliver Powell, Ortensia Vito, Clare Wilson, Amina Abdulla, Ladan Ali, Sarah Darnell, Rikke Jorgensen, Ian Maconochie, Sobia Mustafa, Salina Persand, Ben Walsh, Molly Stevens, Nayoung Kim, Eunjung Kim, Benjamin Pierce, Julia Dudley, Vivien Richmond, Emma Tavliavini, Ching-Chuan Liu, Shih-Min Wang, Fernando Álves González, Cristina Balo Farto, Ruth Barral-Arca, María Barreiro Castro, Xabier Bello, Mirian Ben García, Sandra Carnota, Miriam Cebey-López, María José Curras-Tuala, Carlos Durán Suárez, Luisa García Vicente, Alberto Gómez-Carballa, Jose Gómez Rial, Pilar Leboráns Iglesias, Nazareth Martinón-Torres, José María Martinón Sánchez, Belén Mosquera Pérez, Jacobo Pardo-Seco, Lidia Piñeiro Rodríguez, Sara Pischedda, Sara Ray Vázquez, Carmen Rodríguez-Tenreiro, Lorenzo Redondo-Collazo, Miguel Sadiki Ora, Antonio Sallas, Sonia Serén Fernández, Cristina Serén Trasorras, Marisol Vilas Iglesias, Anda Balode, Arta Bãrdzdina, Dãrta Deksne, Dace Gardovska, Dagne Grãvele, Ilze Grope, Anija Meiere, Ieve Nokalna, Jana Pavãre, Zanda Pučuka, Katrīna Selecka, Dace Svile, Urzula Nora Urbãne, Kalifa Bojang, Syed M.A. Zaman, Suzanne Anderson, Anna Roca, Isatou Sarr, Momodou Saidykhan, Saffiatou Darboe, Samba Ceesay, Umberto D'alessandro, Dorine M. Borensztajn, Nienke N. Hagedoorn, Chantal Tal, Joany Zachariasse, W. Dik, Christoph Aebi, Christoph Berger, Verena Wyss, Mariama Usman, Eric Giannoni, Martin Stocker, Klara M. Posfay-Barbe, Ulrich Heininger, Sara Bernhard-Stirnemann, Anita Niederer-Loher, Christian Kahlert, Giancarlo Natalucci, Christa Relly, Thomas Riedel, Elizabeth Cocklin, Rebecca Jennings, Joanne Johnson, Simon Leigh, Karen Newall, Sam Romaine, Maria Tambouratzi, Antonis Marmarinos, Marietta Xagorari, Kelly Syggelou, Nikos Spyridis, Jennifer Blackmore, Rebekah Harrison, Benno Kohlmaier, Daniela S. Kohlfürst, Christoph Zurl, Alexander Binder, Susanne Hösele, Manuel Leitner, Lena Pölz, Glorija Rajic, Sebastian Bauchinger, Hinrich Baumgart, Martin Benesch, Astrid Ceolotto, Ernst Eber, Siegfried Gallisti, Gunther Gores, Harald Haidl, Almuthe Hauer, Christa Hude, Markus Keldorfer, Larissa Krenn, Heidemarie Pilch, Andreas Pfleger, Klaus Pfurtscheller, Gudrun Nordberg, Tobias Niedrist, Siegfried Rödl, Andrea Skrabl-Baumgartner, Matthias Sperl, Laura Stampfer, Volker Strenger, Holger Till, Andreas Trobisch, Sabine Löffler, Juan Emmanuel Dewez, Martin Hibberd, David Bath, Alec Miners, Elizabeth Fitchett, Catherine Wedderburn, Anne Meierford, Baptiste Leurent, Marien I. De Jonge, Koen van Aerde, Wynand Alkema, Bryan van den Broek, Jolein Gloerich, Alain J. Van Gool, Stefanie Henriet, Martijn Huijnen, Ria Philipsen, Esther Willems, G.P.J.M. Gerrits, M. Van Leur, J. Heidema, L. De Haan, C.J. Miedema, C. Neeleman, C.C. Obihara, G.A. Tramper-Stranders, Rama Kandasamy, Michael J. Carter, Daniel O'Connor, Sagida Bibi, Dominic F. Kelly, Meeru Gurung, Stephen Throson, Imran Ansari, David R. Murdoch, Shrijana Shrestha, Zoe Oliver, Emma Lim, Lucille Valentine, Karen Allen, Kathryn Bell, Adora Chan, Stephen Crulley, Kirsty Devine, Daniel Fabian, Sharon King, Paul McAlinden, Sam McDonald, Anne McDonell, Alisa Pickering, Evelyn Thomson, Amanda Wood, Diane Wallia, Phil Woodsford, Frances Baxter, Ashley Bell, Mathew Rhodes, Rachel Agbeko, Christine Mackerness, Bryan Baas, Lieke Kloosterhuis, Wilma Oosthoek, Tasnim Arif, Joshua Bennet, Kalvin Collings, Ilona Van der Giessen, Alex Martin, Aqeela Rashid, Emily Rowlands, Joshua Soon, Gabriella De Vries, Mike Martin, Ravi Mistry, Manuela Zwerenz, Judith Buschbeck, Christoph Bidlingmaier, Vera Binder, Katharina Danhauser, Nikolaus Haas, Matthias Griese, Matthias Kappler, Eberhard Lurz, Georg Muench, Karl Reiter, Carola Schoen, Alexandre Pachot, Marine Mommert, Tina Plankar Srovin, Natalija Bahovec, Petra Prunk, Veronika Osterman, Tanja Avramoska, Ilse Jongerius, J.M. van den Berg, D. Schonenberg, A.M. Barendregt, D. Pajkrt, M. van der Kuip, A.M. van Furth, Evelien Sprenkeler, Judith Zandstra, G. van Mierlo, and J. Geissler

Subjects
Molecular diagnostics, Diagnostic, Febrile illness, Infectious disease, Bacterial, Viral, Public aspects of medicine, RA1-1270
Abstract

Summary: Background: The PERFORM study aimed to understand causes of febrile childhood illness by comparing molecular pathogen detection with current clinical practice. Methods: Febrile children and controls were recruited on presentation to hospital in 9 European countries 2016–2020. Each child was assigned a standardized diagnostic category based on retrospective review of local clinical and microbiological data. Subsequently, centralised molecular tests (CMTs) for 19 respiratory and 27 blood pathogens were performed. Findings: Of 4611 febrile children, 643 (14%) were classified as definite bacterial infection (DB), 491 (11%) as definite viral infection (DV), and 3477 (75%) had uncertain aetiology. 1061 controls without infection were recruited. CMTs detected blood bacteria more frequently in DB than DV cases for N. meningitidis (OR: 3.37, 95% CI: 1.92–5.99), S. pneumoniae (OR: 3.89, 95% CI: 2.07–7.59), Group A streptococcus (OR 2.73, 95% CI 1.13–6.09) and E. coli (OR 2.7, 95% CI 1.02–6.71). Respiratory viruses were more common in febrile children than controls, but only influenza A (OR 0.24, 95% CI 0.11–0.46), influenza B (OR 0.12, 95% CI 0.02–0.37) and RSV (OR 0.16, 95% CI: 0.06–0.36) were less common in DB than DV cases. Of 16 blood viruses, enterovirus (OR 0.43, 95% CI 0.23–0.72) and EBV (OR 0.71, 95% CI 0.56–0.90) were detected less often in DB than DV cases. Combined local diagnostics and CMTs respectively detected blood viruses and respiratory viruses in 360 (56%) and 161 (25%) of DB cases, and virus detection ruled-out bacterial infection poorly, with predictive values of 0.64 and 0.68 respectively. Interpretation: Most febrile children cannot be conclusively defined as having bacterial or viral infection when molecular tests supplement conventional approaches. Viruses are detected in most patients with bacterial infections, and the clinical value of individual pathogen detection in determining treatment is low. New approaches are needed to help determine which febrile children require antibiotics. Funding: EU Horizon 2020 grant 668303.

Published
2023
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3. Impact of infection on proteome-wide glycosylation revealed by distinct signatures for bacterial and viral pathogens

Author

Esther Willems, Jolein Gloerich, Anouk Suppers, Michiel van der Flier, Lambert P. van den Heuvel, Nicole van de Kar, Ria H.L.A. Philipsen, Maurice van Dael, Myrsini Kaforou, Victoria J. Wright, Jethro A. Herberg, Federico Martinon Torres, Michael Levin, Ronald de Groot, Alain J. van Gool, Dirk J. Lefeber, Hans J.C.T. Wessels, Marien I. de Jonge, Amina Abdulla, Christoph Aebi, Koen van Aerde, Rachel Agbeko, Philipp Agyeman, Umberto D’alessandro, Ladan Ali, Wynand Alkema, Karen Allen, Fernando Álvez González, Suzanne Anderson, Imran Ansari, Tasnim Araf, Tanja Avramoska, Bryan Baas, Natalija Bahovec, Cristina Balo Farto, Anda Balode, A.M. Barendregt, Ruth Barral-Arca, María Barreiro Castro, Arta Bārzdiņa, David Bath, Sebastian Bauchinger, Lucas Baumard, Hinrich Baumgart, Frances Baxter, Ashley Bell, Kathryn Bell, Xabier Bello, Evangelos Bellos, Martin Benesch, Mirian Ben García, Joshua Bennet, Christoph Berger, J.M. van den Berg, Sara Bernhard-Stirnemann, Sagida Bibi, Christoph Bidlingmaier, Alexander Binder, Vera Binder, Kalifa Bojang, Dorine M. Borensztajn, Ulrich von Both, Karen Brengel-Pesce, Bryan van den Broek, Judith Buschbeck, Leo Calvo-Bado, Sandra Carnota, Enitan D. Carrol, Michael J. Carter, Miriam Cebey-López, Samba Ceesay, Astrid Ceolotto, Adora Chan, Elizabeth Cocklin, Kalvin Collings, Stephen Crulley, Aubrey Cunnington, María José Curras-Tuala, Katharina Danhauser, Saffiatou Darboe, Sarah Darnell, Tisham De, Dārta Deksne, Kirsty Devine, Juan Emmanuel Dewez, Julia Dudley, Carlos Durán Suárez, Ernst Eber, Irini Eleftheriou, Marieke Emonts, Daniel Fabian, Tobias Feuchtinger, Katy Fidler, Colin Fink, A.M. van Furth, Rachel Galassini, Siegfried Gallistl, Luisa García Vicente, Dace Gardovska, J. Geissler, G.P.J.M. Gerrits, Eric Giannoni, Ilona van der Giessen, Alberto Gómez-Carballa, Jose Gómez Rial, Gunther Gores, Dagne Grāvele, Matthias Griese, Ilze Grope, Meeru Gurung, L. de Haan, Nikolaus Haas, Dominic Habgood-Coote, Nienke N. Hagedoorn, Harald Haidl, Shea Hamilton, Almuthe Hauer, J. Heidema, Ulrich Heininger, Stefanie Henriet, Jethro Herberg, Clive Hoggart, Susanne Hösele, Sara Hourmat, Christa Hude, Martijn Huijnen, Heather Jackson, Rebecca Jennings, Joanne Johnston, Ilse Jongerius, Rikke Jorgensen, Christian Kahlert, Rama Kandasamy, Matthias Kappler, Julia Keil, Markus Keldorfer, Dominic F. Kell, Eunjung Kim, Sharon King, Lieke Kloosterhuis, Daniela S. Kohlfürst, Benno Kohlmaier, Laura Kolberg, Mojca Kolnik, Larissa Krenn, Taco Kuijpers, M. van der Kuip, Pilar Leboráns Iglesias, Simon Leigh, Manuel Leitner, M. van Leur, Emma Lim, Naomi Lin, Ching-Chuan Liu, Sabine Löffler, Eberhard Lurz, Ian Maconochie, Christine Mackerness, François Mallet, Federico Martinón-Torres, Antonis Marmarinos, Alex Martin, Mike Martin, José María Martinón Sánchez, Nazareth Martinón-Torres, Paul McAlinden, Anne McDonnell, Sam McDonald, C.J. Miedema, Anija Meiere, Stephanie Menikou, G. van Mierlo, Alec Miners, Ravi Mistry, Henriëtte A. Moll, Marine Mommert, Belén Mosquera Pérez, David R. Murdoch, Sobia Mustafa, Giancarlo Natalucci, C. Neeleman, Karen Newall, Samuel Nichols, Tobias Niedrist, Anita Niederer-Loher, Ruud Nijman, Ieva Nokalna, Urzula Nora Urbāne, Gudrun Nordberg, C.C. Obihara, Daniel O'Connor, Wilma Oosthoek, Veronika Osterman, Alexandre Pachot, D. Pajkrt, Jacobo Pardo-Seco, Stéphane Paulus, Jana Pavāre, Ivonne Pena Paz, Salina Persand, Andreas Pfleger, Klaus Pfurtscheller, Ria Philipsen, Ailsa Pickering, Benjamin Pierce, Heidemarie Pilch, Lidia Piñeiro Rodríguez, Sara Pischedda, Tina Plankar Srovin, Marko Pokorn, Andrew J. Pollard, Lena Pölz, Klara M. Posfay-Barbe, Petra Prunk, Zanda Pučuka, Glorija Rajic, Aqeela Rashid, Lorenzo Redondo-Collazo, Christa Relly, Irene Rivero Calle, Sara Rey Vázquez, Mathew Rhodes, Vivien Richmond, Thomas Riedel, Anna RocaIsatou Sarr, Siegfried Rödl, Carmen Rodríguez-Tenreiro, Sam Romaine, Emily Rowlands, Miguel Sadiki Ora, Manfred G. Sagmeister, Momodou Saidykhan, Antonio Salas, Luregn J. Schlapbach, D. Schonenberg, Fatou Secka, Katrīna Selecka, Sonia Serén Fernández, Cristina Serén Trasorras, Priyen Shah, Ching-Fen Shen, Shrijana Shrestha, Aleksandra Sidorova, Andrea Skrabl-Baumgartner, Giselle D’Souza, Matthias Sperl, Evelien Sprenkeler, Nina A. Schweintzger, Laura Stampfer, Molly Stevens, Martin Stocker, Volker Strenger, Dace Svile, Kelly Syggelou, Maria Tambouratzi, Chantal Tan, Emma Tavliavini, Evelyn Thomson, Stephen Thorson, Holger Till, G.A. Tramper-Stranders, Andreas Trobisch, Maria Tsolia, Effua Usuf, Lucille Valentine, Clementien L. Vermont, Marisol Vilas Iglesias, Katarina Vincek, Marie Voice, Gabriella de Vries, Diane Wallia, Shih-Min Wang, Clare Wilson, Amanda Wood, Phil Woodsford, Victoria Wright, Marietta Xagorari, Shunmay Yeung, Joany Zachariasse, Dace Zavadska, Syed M.A. Zaman, Judith Zandstra, Werner Zenz, Christoph Zurl, and Manuela Zwerenz

Subjects
Health sciences, Glycobiology, Immunology, Glycomics, Science
Abstract

Summary: Mechanisms of infection and pathogenesis have predominantly been studied based on differential gene or protein expression. Less is known about posttranslational modifications, which are essential for protein functional diversity. We applied an innovative glycoproteomics method to study the systemic proteome-wide glycosylation in response to infection. The protein site-specific glycosylation was characterized in plasma derived from well-defined controls and patients. We found 3862 unique features, of which we identified 463 distinct intact glycopeptides, that could be mapped to more than 30 different proteins. Statistical analyses were used to derive a glycopeptide signature that enabled significant differentiation between patients with a bacterial or viral infection. Furthermore, supported by a machine learning algorithm, we demonstrated the ability to identify the causative pathogens based on the distinctive host blood plasma glycopeptide signatures. These results illustrate that glycoproteomics holds enormous potential as an innovative approach to improve the interpretation of relevant biological changes in response to infection.

Published
2023
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4. Transient liver elastography in the follow-up of Fontan patients: results of a nation wide survey in Germany

Author

Zora Meyer, Nikolaus Haas, Richard Mühlberg, Annabell Braun, Markus Fischer, and Guido Mandilaras

Subjects
Fontan, liver, elaslography, fibrosis, patient, Pediatrics, RJ1-570
Abstract

IntroductionFontan-palliated patients are at risk for the development of Fontan-associated liver disease (FALD). Currently, there is no consensus on how to stage FALD. Transient elastography (TE) is a rapid, non-invasive method to assess FALD and liver fibrosis.MethodTo assess the availability and conditions of using TE to monitor liver disease in Fontan patients in german centers for pediatric cardiology and to propose the introduction of a standardized national protocol for the monitoring of liver disease, we developed a questionnaire.ResultsIn total, 95 valid questionnaires were collected. Only 20% of the centers offer the TE investigation directly. Most of the centers transfer the patients to another department or center (40%) or didńt offer TE (40%). In only 2.6% of the centers TE is performed directly by the cardiologist. Most of the centers transfer the patients to a other department. In 29.2% TE is performed only at a certain age of the patients and in 27.7% it is performed if the patients present symptoms of failing Fontan. In only 13.9% of the centers TE is proposed in all the Fontan patients on a routine basis. Most often TE is performed only from the beginning of the adolescence. In the majority of answers it was not known if the patients are fasting for the examination (68%) or not and if the TE examination had to be performed in a specific breathing phase during TE (Inspiration/Expiration, 90%). In the majority, TE is not offered routinely (46.9%).DiscussionTo date in Germany, TE is only used in a few numbers of centers specialized in Fontan follow-up. A standardized protocol to use TE is currently not existing. With regard to the feasibility of the examination, it is evident that TE is a quick, cheap and easy method to distinguish between cases with and without progressive FALD. This makes TE a useful and prognostic tool for screening of liver disease and to failing Fontan circulation.ConclusionWe propose a systematic TE evaluation of possible liver congestion and fibrosis, as a part of the routine follow-up of Fontan patients.

Published
2023
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5. Case report: Paracorporeal lung assist device for 215 days as a bridge-to-lung transplantation in a patient with bronchopulmonary dysplasia and severe pulmonary hypertension

Author

Sebastian G. Michel, Maja Hanuna, Joseph Pattathu, Jelena Pabst von Ohain, Christian Schneider, Theresa Kauke, Nikolaus Kneidinger, Juergen Behr, Katrin Milger, Juergen Barton, Tobias Veit, Christine Kamla, Christoph Mueller, Robert Dzieciol, Lauren Christen, Michael Irlbeck, Roland Tomasi, Jan Abicht, Patrick Scheiermann, Matthias Feuerecker, Robert Dalla-Pozza, Marcus Fischer, Andre Jakob, Matthias Hermann, Nikolaus Haas, Christian Hagl, and Jürgen Hörer

Subjects
lung assist device, lung transplantation, pulmonary hypertension, bridge-to-lung transplant, ECMOextracorporeal membrane oxygenation, Specialties of internal medicine, RC581-951
Abstract

Pulmonary hypertension (PH) is a known and life limiting complication of preterm born young adults with bronchopulmonary dysplasia (BPD), ultimately leading to progressive right ventricular (RV) failure. Prognosis remains poor, especially in patients unresponsive to modern vasoactive pharmacotherapy. Therefore, lung transplantation presents the treatment of choice to avert cardiac failure. With limited donor organ availability and long waiting times, the implantation of a paracorporeal lung assist device (PLAD) is a way to bridge the patient as an alternative to veno-arterial ECMO. Herein, we present the case of a prematurely born 23-year-old female, who developed severe PH due to BPD and consequently experienced therapy refractory RV failure. Urgent PLAD implantation was performed and the patient successfully underwent double-lung transplantation after 215 days of PLAD support. No major PLAD-associated complications occurred and full recovery of RV function could be observed after double-lung transplantation.

Published
2023
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6. Case report: Central venous catheter thrombosis complicated by chronic thromboembolic disease/pulmonary hypertension in two children requiring parenteral nutrition

Author

Maja Hanuna, Joseph Pattathu, Joscha Buech, Christine Kamla, Nikolaus Kneidinger, Juergen Behr, Katrin Milger, Tobias Veit, Marina Nagel, Jan Abicht, Robert Dalla-Pozza, Marcus Fischer, Andre Jakob, Matthias Hermann, Rene Schramm, Laura L. Rosenthal, Nikolaus Haas, Jürgen Hörer, Christian Hagl, and Sebastian G. Michel

Subjects
hickmann-catheter, pulmonary embolism, chronic thromboembolic pulmonary hypertension, pulmonary endarterectomy, parenteral nutrition (PN), Diseases of the circulatory (Cardiovascular) system, RC666-701
Abstract

Chronic thromboembolic pulmonary hypertension is a rare but life-threatening complication of long-term central venous catheters (CVC) in children. However, evidence in terms of potential treatment strategies and outcome data remains scarce. We describe two cases of CVC-related thrombosis (Hickman-catheter) complicated by recurrent pulmonary emboli. One patient experienced a complete thromboembolic obstruction of the right pulmonary artery with normal pulmonary pressures and the second patient suffered from a central thromboembolic obstruction of both pulmonary arteries associated with severe pulmonary hypertension. Both patients successfully underwent surgical thromboendarterectomy with deep hypothermic circulatory arrest.

Published
2023
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7. Case report: Heart Mate III for systemic right ventricular support in a patient with hypoplastic left heart syndrome

Author

Maja Hanuna, Jelena Pabst von Ohain, Nikolaus Haas, Christoph S. Mueller, Robert Dalla-Pozza, Marcus Fischer, Frank Born, Christine Kamla, Andre Jakob, Christian Hagl, Jürgen Hörer, and Sebastian G. Michel

Subjects
failing Fontan, mechanical circulatory support, VAD—ventricular assist device, right ventricular (RV) failure, hypoplastic left heart syndrome, case report, Diseases of the circulatory (Cardiovascular) system, RC666-701
Abstract

Ventricular assist device implantation presents a possible bridge to heart transplantation for patients with failing Fontan physiology. However, evidence regarding outcome and possible pitfalls associated with the Fontan circulation is still insufficient. We describe the course of a 13-year-old male, who was born with hypoplastic left heart syndrome and underwent HeartMate III implantation due to refractory failure of the systemic right ventricle.

Published
2023
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8. Aminoterminal Pro B-Type Natriuretic Peptide (NT-proBNP) Levels for Monitoring Interventions in Paediatric Cardiac Patients with Stenotic Lesions

Author

Eva Welisch, Knut Kleesiek, Nikolaus Haas, Kambiz Norozi, Ralf Rauch, and Guido Filler

Subjects
Pediatrics, RJ1-570
Abstract

Background. Serum concentration of NT-proBNP correlates well with the severity of cardiac disease in adults. Few studies have been performed on the applicability of NT-proBNP for monitoring children with congenital heart disease. Objective. To assess the potential of NT-proBNP for monitoring the success of interventions in children with stenotic cardiac lesions. Methods. NT-proBNP was measured in 42 children aged 1 day to 17 years (y) before and 6 to 12 weeks after surgical or interventional correction of obstructive lesions of the heart. Comparison is made with the clinical status and echocardiographic data of the child. Results. NT-proBNP levels (median 280, range 10–263,000 pg/mL) were above the reference value in all but 6 patients (pts) prior to the intervention. Higher levels were found in more compromised patients. The 35 children with clinical improvement after the procedure showed a decline of their NT-proBNP level in all but 4 patients, whose levels remained unchanged. Five patients with unchanged gradients despite a therapeutic intervention also demonstrated unchanged NT-proBNP levels after the intervention. Thus, the success rate of the procedure correlated well to clinical and echocardiographic findings. Conclusion. NT-proBNP can be used to assess the efficiency of an intervention.

Published
2009
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9. 14/w mit Synkope

Author

Joseph Pattathu, Nikolaus Haas, and André Jakob

Subjects
Pediatrics, Perinatology and Child Health, Surgery
Published
2023
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14. 'Popcorn' in the head

Author

Sabine Diepenbruck, Mathias Kunz, Christoph Funk, and Nikolaus Haas

Subjects
Pediatrics, Perinatology and Child Health, Surgery
Published
2022
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15. Case report: Heart Mate III for systemic right ventricular support in a patient with hypoplastic left heart syndrome

Author

Maja Hanuna, Jelena Pabst von Ohain, Nikolaus Haas, Christoph S. Mueller, Robert Dalla-Pozza, Marcus Fischer, Frank Born, Christine Kamla, Andre Jakob, Christian Hagl, Jürgen Hörer, and Sebastian G. Michel

Subjects
Cardiovascular Medicine, failing Fontan, mechanical circulatory support, VAD_ventricular assist device, right ventricular (RV) failure, hypoplastic left heart syndrome, case report, Cardiology and Cardiovascular Medicine, ddc
Abstract

Ventricular assist device implantation presents a possible bridge to heart transplantation for patients with failing Fontan physiology. However, evidence regarding outcome and possible pitfalls associated with the Fontan circulation is still insufficient. We describe the course of a 13-year-old male, who was born with hypoplastic left heart syndrome and underwent HeartMate III implantation due to refractory failure of the systemic right ventricle.

Published
2022

16. Long-Term Follow-up of Transcatheter Ventricular Septal Defect Closure in Children, and Comparison of Single-Hole Versus Multi-holes Ventricular Septal Defects

Author

Hojjat Mortezaeian, Mina Farshidgohar, Ahmad Vesal, Behzad Alizadeh, Yasaman Khalili, Mahmood Meraji, and Nikolaus Haas

Subjects
Pediatrics, Perinatology and Child Health
Abstract

Background: Evaluation of complications after transcatheter ventricular septal defect (VSD) closure in long-term follow-up and large samples of children is limited. Objectives: We compared the residual shunt after transcatheter closure in VSDs with a single hole and multiple holes, a new task that has not been done so far. Methods: This retrospective study included all patients who underwent transcatheter device closure for VSD in a tertiary cardiovascular center from 2009 to 2020. Follow-up evaluation using transthoracic echocardiography (TTE) and electrocardiogram (ECG) was performed at 1, 6, 12 months, and annually after the procedure. Results: A total of 409 patients underwent transcatheter VSD closure. The mean age was 7 years (2 - 15 years), and the median follow-up duration was 48 months (1 - 10 years). The number of patients with a singular VSD was 259 (63.4%), and those with multiple exit holes were 150 (36.6%). The incidence of a residual shunt immediately after implantation was significantly higher in VSDs with multiple holes than those with a single hole (P = 0.008). During the follow-up, the residual shunts decreased in the group of VSDs with a single hole. Forty-five patients (11%) and 16 patients (4%) had a new-onset of mild and moderate tricuspid regurgitation (TR), respectively, and it decreased dramatically over time. Only 1 patient showed a new-onset mild aortic regurgitation (AR). The most crucial complication shown in 2 patients was a persistent complete heart block. Conclusions: Ventricular septal defects with multiple exit holes are a risk factor for a residual shunt. After transcatheter VSD closure, the residual shunt in patients with a multiple-hole VSD was significantly higher (P = 0.008). Although TR may increase during the procedure, it decreases dramatically over time. Interestingly, patients who had pre-procedure tricuspid or aortic regurgitation disappeared after 2 years of the procedure. The most important complication was a complete heart block in 2 patients.

Published
2022
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18. Tricuspid Annular Plane Systolic Excursion (TAPSE) correlates with mean pulmonary artery pressure especially 10 years after pediatric heart transplantation

Author

Morgana Michalski, Nikolaus Haas, Robert Dalla Pozza, Sebastian Michel, Marcus Fischer, Anja Lehner, Laura Rosenthal, Andre Jakob, Madeleine Orban, and Sarah Ulrich

Subjects
Transplantation
Abstract

Tricuspid annular plane systolic excursion (TAPSE) is important in the noninvasive echocardiographic assessment of right heart function. This retrospective observational study shows correlations of TAPSE with invasive right heart catheterization parameters after pediatric heart transplantation (HTx). The study included patients after pediatric HTx with cardiac catheterizations in 2018/2019 and measurement of TAPSE (n = 52 patients with 57 examinations; 50.9% adults, 52.6% female, median age: 18.54 years). TAPSE was compared with normal values. Stepwise, linear and multiple regression were used to show influencing variables on TAPSE. Mean TAPSE z-score was -3.48 (SD: 2.25) and 68.4% of HTx-recipients showed abnormally reduced TAPSE (z-score ←2) compared to normal values. Multiple regression (p-value0.001; corrected R

Published
2022

19. Congenital Heart Diseases: The Broken Heart : Clinical Features, Human Genetics and Molecular Pathways

Author

Silke Rickert-Sperling, Robert G. Kelly, Nikolaus Haas, Silke Rickert-Sperling, Robert G. Kelly, and Nikolaus Haas

Subjects
Congenital heart disease
Abstract

This 2nd edition has been extensively updated and provides comprehensive and current insight into congenital heart diseases, from embryonic development through to clinical features, including human genetics and our current knowledge of the underlying molecular pathways.The book is divided into three parts: an introduction to the development of the heart and its vessels, an overview of the molecular pathways affecting the development of various cardiovascular structures, and a main section focusing on the different types of structural and nonstructural congenital heart diseases, including their clinical features, underlying genetic alterations and insights from animal models and pathways. All chapters have been updated and new chapters added on state-of-the-art approaches including stem cells and organoids, cardiac metabolism, single cell transcriptomics and 3D reconstruction of human heart development. The clinical chapters have been extended and include new sections on diagnostic imaging techniques. Taken together, the book, written by and for clinicians and researchers, provides an integrated and up-to-date resource for all those who want to learn more about both the clinical aspects and the genetic and molecular basis of congenital heart disease.

Published
2024

20. Bridging patients in cardiogenic shock with a paracorporeal pulsatile biventricular assist device to heart transplantation-a single-centre experience

Author

Sebastian Michel, Stefan Buchholz, Joscha Buech, Tobias Veit, Thomas Fabry, Jan Abicht, Nikolaus Thierfelder, Christoph Mueller, Laura Lily Rosenthal, Jelena Pabst von Ohain, Nikolaus Haas, Jürgen Hörer, and Christian Hagl

Subjects
Pulmonary and Respiratory Medicine, Adult, Heart Failure, Shock, Cardiogenic, General Medicine, Treatment Outcome, Heart Transplantation, Humans, Surgery, Female, Heart-Assist Devices, Cardiology and Cardiovascular Medicine, Child, Retrospective Studies
Abstract

OBJECTIVES We evaluated the outcome of patients in cardiogenic shock receiving a paracorporeal pulsatile biventricular assist device as a bridge to transplantation. METHODS We performed a retrospective single-centre analysis of all patients who received a Berlin Heart Excor® at our institution between 2004 and 2019. RESULTS A total of 97 patients (90 adults, 7 paediatric) were analysed. Eighty-four patients were in Interagency Registry for Mechanically Assisted Circulatory Support level 1 (80 adults, 4 paediatric). Diagnoses were dilated cardiomyopathy (n = 41), ischaemic cardiomyopathy (n = 17) or myocardial infarction (n = 4), myocarditis (n = 15), restrictive cardiomyopathy (n = 2), graft failure after heart transplant (n = 7), postcardiotomy heart failure (n = 5), postpartum cardiomyopathy (n = 3), congenital heart disease (n = 1), valvular cardiomyopathy (n = 1) and toxic cardiomyopathy (n = 1). All patients were in biventricular heart failure and had secondary organ dysfunction. The mean duration of support was 63 days (0–487 days). There was a significant decrease in creatinine values after assist device implantation (from 1.83 ± 0.79 to 1.12 ± 0.67 mg/dl, P = 0.001) as well as a decrease in bilirubin values (from 3.94 ± 4.58 to 2.65 ± 3.61 mg/dl, P = 0.084). Cerebral stroke occurred in 16 patients, bleeding in 15 and infection in 13 patients. Forty-eight patients died on support, while 49 patients could be successfully bridged to transplantation. Thirty-day survival and 1-year survival were 70.1% and 41.2%, respectively. CONCLUSIONS A pulsatile biventricular assist device is a reasonable therapeutic option in cardiogenic shock, when immediate high cardiac output is necessary to rescue the already impaired kidney and liver function of the patient.

Published
2021

27. Vorwort

Author

Ulrich Kleideiter, Robert Dalla Pozza, and Nikolaus Haas

Published
2019
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