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3. Common Genetic Variants Contribute to Risk of Transposition of the Great Arteries

Author

Škorić-Milosavljević, Doris, Tadros, Rafik, Bosada, Fernanda M., Tessadori, Federico, van Weerd, Jan Hendrik, Woudstra, Odilia I., Tjong, Fleur V.Y., Lahrouchi, Najim, Bajolle, Fanny, Cordell, Heather J., Agopian, A.J., Blue, Gillian M., Barge-Schaapveld, Daniela Q.C.M., Gewillig, Marc, Preuss, Christoph, Lodder, Elisabeth M., Barnett, Phil, Ilgun, Aho, Beekman, Leander, van Duijvenboden, Karel, Bokenkamp, Regina, Müller-Nurasyid, Martina, Vliegen, Hubert W., Konings, Thelma C., van Melle, Joost P., van Dijk, Arie P.J., van Kimmenade, Roland R.J., Roos-Hesselink, Jolien W., Sieswerda, Gertjan T., Meijboom, Folkert, Abdul-Khaliq, Hashim, Berger, Felix, Dittrich, Sven, Hitz, Marc-Phillip, Moosmann, Julia, Riede, Frank-Thomas, Schubert, Stephan, Galan, Pilar, Lathrop, Mark, Munter, Hans M., Al-Chalabi, Ammar, Shaw, Christopher E., Shaw, Pamela J., Morrison, Karen E., Veldink, Jan H., van den Berg, Leonard H., Evans, Sylvia, Nobrega, Marcelo A., Aneas, Ivy, Radivojkov-Blagojević, Milena, Meitinger, Thomas, Oechslin, Erwin, Mondal, Tapas, Bergin, Lynn, Smythe, John F., Altamirano-Diaz, Luis, Lougheed, Jane, Bouma, Berto J., Chaix, Marie-A., Kline, Jennie, Bassett, Anne S., Andelfinger, Gregor, van der Palen, Roel L.F., Bouvagnet, Patrice, Clur, Sally-Ann B., Breckpot, Jeroen, Kerstjens-Frederikse, Wilhelmina S., Winlaw, David S., Bauer, Ulrike M.M., Mital, Seema, Goldmuntz, Elizabeth, Keavney, Bernard, Bonnet, Damien, Mulder, Barbara J., Tanck, Michael W.T., Bakkers, Jeroen, Christoffels, Vincent M., Boogerd, Cornelis J., Postma, Alex V., and Bezzina, Connie R.

Published
2022
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5. Cardiac Fibrosis and Innervation State in Uncorrected and Corrected Transposition of the Great Arteries: A Postmortem Histological Analysis and Systematic Review

Author

Engele, Leo J., primary, van der Palen, Roel L. F., additional, Egorova, Anastasia D., additional, Bartelings, Margot M., additional, Wisse, Lambertus J., additional, Glashan, Claire A., additional, Kiès, Philippine, additional, Vliegen, Hubert W., additional, Hazekamp, Mark G., additional, Mulder, Barbara J. M., additional, Ruiter, Marco C. De, additional, Bouma, Berto J., additional, and Jongbloed, Monique R. M., additional

Published
2023
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6. Contemporary Management Strategies of Baffle Leaks in Adults with a Failing Systemic Right Ventricle Late after Atrial Switch: A Case Series and Literature Overview

Author

Neijenhuis, Ralph M. L., primary, Regeer, Madelien V., additional, van der Kley, Frank, additional, Vliegen, Hubert W., additional, Jongbloed, Monique R. M., additional, Kiès, Philippine, additional, Schalij, Martin J., additional, Jukema, J. Wouter, additional, and Egorova, Anastasia D., additional

Published
2023
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7. Anomalous aortic origin of the right coronary artery: invasive hemodynamic assessment in adult patients with high-risk anatomical features.

Author

Verheijen, Diederick B.H., primary, Egorova, Anastasia D., additional, Jongbloed, Monique R.M., additional, van der Kley, Frank, additional, Koolbergen, Dave R., additional, Hazekamp, Mark G., additional, Lamb, Hildo J., additional, Jukema, J. Wouter, additional, Kiès, Philippine, additional, and Vliegen, Hubert W., additional

Published
2023
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9. A comprehensive analysis of the intramural segment in interarterial anomalous coronary arteries using computed tomography angiography

Author

Team Medisch, Circulatory Health, Koppel, Claire J, Verheijen, Diederick B H, Kiès, Philippine, Egorova, Anastasia D, Lamb, Hildo J, Voskuil, Michiel, Jukema, J Wouter, Koolbergen, Dave R, Hazekamp, Mark G, Schalij, Martin J, Jongbloed, Monique R M, Vliegen, Hubert W, Team Medisch, Circulatory Health, Koppel, Claire J, Verheijen, Diederick B H, Kiès, Philippine, Egorova, Anastasia D, Lamb, Hildo J, Voskuil, Michiel, Jukema, J Wouter, Koolbergen, Dave R, Hazekamp, Mark G, Schalij, Martin J, Jongbloed, Monique R M, and Vliegen, Hubert W

Published
2022

10. Type D Personality Associated With Increased Risk for Mortality in Adults With Congenital Heart Disease

Author

Team Medisch, Circulatory Health, Kauw, Dirkjan, Schoormans, Dounya, Sieswerda, Gertjan Tj, Van Melle, Joost P, Vliegen, Hubert W, Van Dijk, Arie P J, Hulsbergen-Zwarts, Mariët S, Post, Marco C, Ansink, Tieneke J, Mulder, Barbara J M, Bouma, Berto J, Schuuring, Mark J, Team Medisch, Circulatory Health, Kauw, Dirkjan, Schoormans, Dounya, Sieswerda, Gertjan Tj, Van Melle, Joost P, Vliegen, Hubert W, Van Dijk, Arie P J, Hulsbergen-Zwarts, Mariët S, Post, Marco C, Ansink, Tieneke J, Mulder, Barbara J M, Bouma, Berto J, and Schuuring, Mark J

Published
2022

11. Medication in adults after atrial switch for transposition of the great arteries: clinical practice and recommendations

Author

Team Medisch, Circulatory Health, Cardiologie, Woudstra, Odilia I, Kuijpers, Joey M, Jongbloed, Monique R M, van Dijk, Arie P J, Sieswerda, Gertjan Tj, Vliegen, Hubert W, Egorova, Anastasia D, Kiès, Philippine, Duijnhouwer, Anthonie L, Robbers-Visser, Daniëlle, Konings, Thelma C, Zwinderman, Aeilko H, Meijboom, Folkert J, Mulder, Barbara J M, Bouma, Berto J, Team Medisch, Circulatory Health, Cardiologie, Woudstra, Odilia I, Kuijpers, Joey M, Jongbloed, Monique R M, van Dijk, Arie P J, Sieswerda, Gertjan Tj, Vliegen, Hubert W, Egorova, Anastasia D, Kiès, Philippine, Duijnhouwer, Anthonie L, Robbers-Visser, Daniëlle, Konings, Thelma C, Zwinderman, Aeilko H, Meijboom, Folkert J, Mulder, Barbara J M, and Bouma, Berto J

Published
2022

12. Atrioventricular Block Necessitating Chronic Ventricular Pacing After Tricuspid Valve Surgery in Patients With a Systemic Right Ventricle: Long-Term Follow-Up

Author

Nederend, Marieke, primary, Jongbloed, Monique R. M., additional, Kiès, Philippine, additional, Vliegen, Hubert W., additional, Bouma, Berto J., additional, Regeer, Madelien V., additional, Koolbergen, Dave R., additional, Hazekamp, Mark G., additional, Schalij, Martin J., additional, and Egorova, Anastasia D., additional

Published
2022
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13. A comprehensive analysis of the intramural segment in interarterial anomalous coronary arteries using computed tomography angiography

Author

Koppel, Claire J, primary, Verheijen, Diederick B H, additional, Kiès, Philippine, additional, Egorova, Anastasia D, additional, Lamb, Hildo J, additional, Voskuil, Michiel, additional, Jukema, J Wouter, additional, Koolbergen, Dave R, additional, Hazekamp, Mark G, additional, Schalij, Martin J, additional, Jongbloed, Monique R M, additional, and Vliegen, Hubert W, additional

Published
2022
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16. Grand Round: Case report of the broad spectrum of late complications in an adult patient with univentricular physiology palliated by the Fontan circulation

Author

Nederend, Marieke, primary, Egorova, Anastasia D, additional, Vliegen, Hubert W, additional, Roest, Arno A W, additional, Ruijter, Bastian N, additional, Korteweg, Tijmen, additional, Ninaber, Maarten K, additional, Zeppenfeld, Katja, additional, Hazekamp, Mark G, additional, Kiès, Philippine, additional, and Jongbloed, Monique R M, additional

Published
2022
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20. Secundum atrial septal defect is associated with reduced survival in adult men

Author

Kuijpers, Joey M., van der Bom, Teun, van Riel, Annelieke C. M. J., Meijboom, Folkert J., van Dijk, Arie P. J., Pieper, Petronella G., Vliegen, Hubert W., Waskowsky, W. Marc, Oomen, Toon, Zomer, A. Carla, Wagenaar, Lodewijk J., Heesen, Wilfred F., Roos-Hesselink, Jolien W., Zwinderman, Aeilko H., Mulder, Barbara J. M., and Bouma, Berto J.

Published
2015
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22. Applicability of the Leiden Convention and the Lipton Classification in Patients with a Single Coronary Artery in the Setting of Congenital Heart Disease

Author

Katekaru-Tokeshi, Diana Isabel, primary, Jiménez-Santos, Moisés, additional, Koppel, Claire J., additional, Vliegen, Hubert W., additional, Díaz-Zamudio, Mariana, additional, Castillo-Castellón, Francisco, additional, Jongbloed, Monique R. M., additional, and Kimura-Hayama, Eric, additional

Published
2021
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23. Computed Tomography Derived Coronary Triangulated Orifice Area—Deduction of a New Parameter for Follow-up After Surgical Correction of Anomalous Aortic Origin of Coronary Arteries and Call for Validation

Author

Meijer, Fleur M. M., primary, Kiès, Philippine, additional, Verheijen, Diederick B. H., additional, Vliegen, Hubert W., additional, Jongbloed, Monique R. M., additional, Hazekamp, Mark G., additional, Lamb, Hildo J., additional, and Egorova, Anastasia D., additional

Published
2021
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24. Common Genetic Variants Contribute to Risk of Transposition of the Great Arteries

Author

Skoric-Milosavljevic, Doris, Tadros, Rafik, Bosada, Fernanda M, Tessadori, Federico, van Weerd, Jan Hendrik, Woudstra, Odilia Ilse, Tjong, Fleur V Y, Lahrouchi, Najim, Bajolle, Fanny, Cordell, Heather J, Agopian, A J, Blue, Gillian M, Barge-Schaapveld, Daniela Qcm, Gewillig, Marc H, Preuss, Christoph, Lodder, Elisabeth M, Barnett, Phil, Ilgun, Aho, Beekman, Leander, van Duijvenboden, Karel, Bokenkamp, Regina, Müller-Nurasyid, Martina, Vliegen, Hubert W, Konings, Thelma C, van Melle, Joost P, van Dijk, Arie, van Kimmenade, Roland Rj, Roos-Hesselink, Jolien W, Sieswerda, Gertjan, Meijboom, Folkert, Abdul-Khaliq, Hashim, Berger, Felix, Dittrich, Sven, Hitz, Marc-Phillip, Moosmann, Julia, Riede, Frank-Thomas, Schubert, Stephan, Galan, Pilar, Lathrop, G Mark, Munter, Hans Markus, Al-Chalabi, Ammar, Shaw, Christopher E, Shaw, Pamela J, Morrison, Karen E, Veldink, Jan H, van den Berg, Leonard H, Evans, Sylvia M, Nobrega, Marcelo A, Aneas, Ivy, Radivojkov-Blagojevic, Milena, Meitinger, Thomas, Oechslin, Erwin, Mondal, Tapas, Bergin, M Lynn, Smythe, John F, Altamirano-Diaz, Luis, Lougheed, Jane, Bouma, Berto J, Chaix, Marie A, Kline, Jennie, Bassett, Anne Susan, Andelfinger, Gregor, van der Palen, Roel Lf, Bouvagnet, Patrice, Clur, Sally-Ann B, Breckpot, Jeroen, Kerstjens-Frederikse, Wilhelmina S, Winlaw, David S, Bauer, Ulrike, Mital, Seema, Goldmuntz, Elizabeth, Keavney, Bernard D, Bonnet, Damien, Mulder, Barbara J, Tanck, Michael, Bakkers, Jeroen, Christoffels, Vincent M, Boogerd, Cornelis J, Postma, Alex V, Bezzina, Connie R, Skoric-Milosavljevic, Doris, Tadros, Rafik, Bosada, Fernanda M, Tessadori, Federico, van Weerd, Jan Hendrik, Woudstra, Odilia Ilse, Tjong, Fleur V Y, Lahrouchi, Najim, Bajolle, Fanny, Cordell, Heather J, Agopian, A J, Blue, Gillian M, Barge-Schaapveld, Daniela Qcm, Gewillig, Marc H, Preuss, Christoph, Lodder, Elisabeth M, Barnett, Phil, Ilgun, Aho, Beekman, Leander, van Duijvenboden, Karel, Bokenkamp, Regina, Müller-Nurasyid, Martina, Vliegen, Hubert W, Konings, Thelma C, van Melle, Joost P, van Dijk, Arie, van Kimmenade, Roland Rj, Roos-Hesselink, Jolien W, Sieswerda, Gertjan, Meijboom, Folkert, Abdul-Khaliq, Hashim, Berger, Felix, Dittrich, Sven, Hitz, Marc-Phillip, Moosmann, Julia, Riede, Frank-Thomas, Schubert, Stephan, Galan, Pilar, Lathrop, G Mark, Munter, Hans Markus, Al-Chalabi, Ammar, Shaw, Christopher E, Shaw, Pamela J, Morrison, Karen E, Veldink, Jan H, van den Berg, Leonard H, Evans, Sylvia M, Nobrega, Marcelo A, Aneas, Ivy, Radivojkov-Blagojevic, Milena, Meitinger, Thomas, Oechslin, Erwin, Mondal, Tapas, Bergin, M Lynn, Smythe, John F, Altamirano-Diaz, Luis, Lougheed, Jane, Bouma, Berto J, Chaix, Marie A, Kline, Jennie, Bassett, Anne Susan, Andelfinger, Gregor, van der Palen, Roel Lf, Bouvagnet, Patrice, Clur, Sally-Ann B, Breckpot, Jeroen, Kerstjens-Frederikse, Wilhelmina S, Winlaw, David S, Bauer, Ulrike, Mital, Seema, Goldmuntz, Elizabeth, Keavney, Bernard D, Bonnet, Damien, Mulder, Barbara J, Tanck, Michael, Bakkers, Jeroen, Christoffels, Vincent M, Boogerd, Cornelis J, Postma, Alex V, and Bezzina, Connie R

Abstract

Background: Dextro-transposition of the great arteries (D-TGA) is a severe congenital heart defect which affects approximately 1 in 4,000 live births. While there are several reports of D-TGA patients with rare variants in individual genes, the majority of D-TGA cases remain genetically elusive. Familial recurrence patterns and the observation that most cases with D-TGA are sporadic suggest a polygenic inheritance for the disorder, yet this remains unexplored. Methods: We sought to study the role of common single nucleotide polymorphisms (SNPs) in risk for D-TGA. We conducted a genome-wide association study in an international set of 1,237 patients with D-TGA and identified a genome-wide significant susceptibility locus on chromosome 3p14.3, which was subsequently replicated in an independent case-control set (rs56219800, meta-analysis P=8.6x10-10, OR=0.69 per C allele). Results: SNP-based heritability analysis showed that 25% of variance in susceptibility to D-TGA may be explained by common variants. A genome-wide polygenic risk score derived from the discovery set was significantly associated to D-TGA in the replication set (P=4x10-5). The genome-wide significant locus (3p14.3) co-localizes with a putative regulatory element that interacts with the promoter of WNT5A, which encodes the Wnt Family Member 5A protein known for its role in cardiac development in mice. We show that this element drives reporter gene activity in the developing heart of mice and zebrafish and is bound by the developmental transcription factor TBX20. We further demonstrate that TBX20 attenuates Wnt5a expression levels in the developing mouse heart. Conclusions: This work provides support for a polygenic architecture in D-TGA and identifies a susceptibility locus on chromosome 3p14.3 near WNT5A. Genomic and functional data support a causal role of WNT5A at the locus.

Published
2021

25. Efficacy and safety of a 12-week outpatient pulmonary rehabilitation program in Post-PE Syndrome

Author

Boon, Gudula J A M, Janssen, Steffi M J, Barco, Stefano; https://orcid.org/0000-0002-2618-347X, Bogaard, Harm Jan, Ghanima, Waleed, Kroft, Lucia J M, Meijboom, Lilian J, Ninaber, Maarten K, Nossent, Esther J, Spruit, Martijn A, Symersky, Petr, Vliegen, Hubert W, Noordegraaf, Anton Vonk, Huisman, Menno V, Siegerink, Bob, Abbink, Jannie J, Klok, Frederikus A, Boon, Gudula J A M, Janssen, Steffi M J, Barco, Stefano; https://orcid.org/0000-0002-2618-347X, Bogaard, Harm Jan, Ghanima, Waleed, Kroft, Lucia J M, Meijboom, Lilian J, Ninaber, Maarten K, Nossent, Esther J, Spruit, Martijn A, Symersky, Petr, Vliegen, Hubert W, Noordegraaf, Anton Vonk, Huisman, Menno V, Siegerink, Bob, Abbink, Jannie J, and Klok, Frederikus A

Abstract

BACKGROUND The Post-Pulmonary Embolism Syndrome (PPES) comprises heterogeneous entities, including chronic thromboembolic disease with/without pulmonary hypertension (CTEPH/CTEPD), and deconditioning. OBJECTIVES To assess underlying physiological determinants of PPES, and efficacy and safety of rehabilitation training in these patients. METHODS 56 consecutive PE patients with persistent dyspnea and/or functional limitations despite ≥3 months of anticoagulation underwent standardized diagnostic work-up including exercise testing as part of routine practice. All diagnostic (imaging and cardiopulmonary function) tests were interpreted by a core group of experienced clinicians. A subgroup of patients without CTEPH or other treatable conditions was referred for a 12-week personalized rehabilitation program, studying changes in physical condition and patient-reported outcome measures. RESULTS Persistent vascular occlusions were observed in 21/56 patients (38%) and CTEPH was confirmed in ten (18%). Regarding those without CTEPH, impaired cardiopulmonary responses were evident in 18/39 patients with available CPET data (46%), unrelated to chronic thrombi. Rehabilitation was completed by 27 patients after excluding 29 (patients with CTEPH or treatable comorbidities, refusal, ineligibility, or training elsewhere). Training intensity, PE-specific quality of life (PEmb-QoL) and fatigue (CIS) improved with a median difference of 20 W (p = 0.001), 3.9 points (p < 0.001) and 16 points (p = 0.003), respectively. Functional status (Post-VTE Functional Status Scale) improved ≥1 grade in 18 (67%) patients, and declined in one (3.7%). CONCLUSIONS Our findings suggest that abnormal cardiopulmonary responses to exercise are common in patients with PPES and are not limited to those with chronic thrombi. Offering pulmonary rehabilitation to patients not treated otherwise seems safe and promising.

Published
2021

26. Clinical course long after atrial switch: A novel risk score for major clinical events

Author

Team Onderzoek, Team Medisch, Regenerative Medicine and Stem Cells, Circulatory Health, Cardiologie, Woudstra, Odilia I., Zandstra, Tjitske E., Vogel, Rosanne F., van Dijk, Arie P.J., Vliegen, Hubert W., Kiès, Philippine, Jongbloed, Monique R.M., Egorova, Anastasia D., Doevendans, Pieter A.F.M., Konings, Thelma C., Mulder, Barbara J.M., Tanck, Michael W.T., Meijboom, Folkert J., Bouma, Berto J., Team Onderzoek, Team Medisch, Regenerative Medicine and Stem Cells, Circulatory Health, Cardiologie, Woudstra, Odilia I., Zandstra, Tjitske E., Vogel, Rosanne F., van Dijk, Arie P.J., Vliegen, Hubert W., Kiès, Philippine, Jongbloed, Monique R.M., Egorova, Anastasia D., Doevendans, Pieter A.F.M., Konings, Thelma C., Mulder, Barbara J.M., Tanck, Michael W.T., Meijboom, Folkert J., and Bouma, Berto J.

Published
2021

29. Validation and Feasibility of Echocardiographic Assessment of Systemic Right Ventricular Function: Serial Correlation With MRI

Author

Zandstra, Tjitske E., primary, Jongbloed, Monique R. M., additional, Widya, Ralph L., additional, ten Harkel, Arend D. J., additional, Holman, Eduard R., additional, Mertens, Bart J. A., additional, Vliegen, Hubert W., additional, Egorova, Anastasia D., additional, Schalij, Martin J., additional, and Kiès, Philippine, additional

Published
2021
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30. Clinical Course Long After Atrial Switch: A Novel Risk Score for Major Clinical Events

Author

Woudstra, Odilia I., primary, Zandstra, Tjitske E., additional, Vogel, Rosanne F., additional, van Dijk, Arie P. J., additional, Vliegen, Hubert W., additional, Kiès, Philippine, additional, Jongbloed, Monique R. M., additional, Egorova, Anastasia D., additional, Doevendans, Pieter A. F. M., additional, Konings, Thelma C., additional, Mulder, Barbara J. M., additional, Tanck, Michael W. T., additional, Meijboom, Folkert J., additional, and Bouma, Berto J., additional

Published
2021
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34. Myocardial Deformation in the Systemic Right Ventricle: Strain Imaging Improves Prediction of the Failing Heart

Author

Team Onderzoek, Cardiologie, Team Medisch, Circulatory Health, Woudstra, Odilia I, van Dissel, Alexandra C, van der Bom, Teun, de Bruin-Bon, Rianne H A C M, van Melle, Joost P, van Dijk, Arie P J, Vliegen, Hubert W, Mulder, Barbara J M, Tanck, Michael W T, Meijboom, Folkert J, Bouma, Berto J, Team Onderzoek, Cardiologie, Team Medisch, Circulatory Health, Woudstra, Odilia I, van Dissel, Alexandra C, van der Bom, Teun, de Bruin-Bon, Rianne H A C M, van Melle, Joost P, van Dijk, Arie P J, Vliegen, Hubert W, Mulder, Barbara J M, Tanck, Michael W T, Meijboom, Folkert J, and Bouma, Berto J

Published
2020

38. The Leiden Convention coronary coding system: translation from the surgical to the universal view

Author

Koppel, Claire J, primary, Vliegen, Hubert W, additional, Bökenkamp, Regina, additional, ten Harkel, A Derk Jan, additional, Kiès, Philippine, additional, Egorova, Anastasia D, additional, Jukema, J Wouter, additional, Hazekamp, Mark G, additional, Schalij, Martin J, additional, Gittenberger-de Groot, Adriana C, additional, and Jongbloed, Monique R M, additional

Published
2021
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41. Leiden Convention coronary coding system: translation from the surgical to the universal view.

Author

Koppel, Claire J, Vliegen, Hubert W, Bökenkamp, Regina, Harkel, A Derk Jan ten, Kiès, Philippine, Egorova, Anastasia D, Jukema, J Wouter, Hazekamp, Mark G, Schalij, Martin J, Groot, Adriana C Gittenberger-de, and Jongbloed, Monique R M

Subjects
CORONARY arterial radiography, PULMONARY valve, ECHOCARDIOGRAPHY, CARDIOLOGISTS, THORACIC surgery, CONGENITAL heart disease, MAGNETIC resonance imaging, DIAGNOSTIC imaging, CORONARY angiography, CORONARY arteries, COMPUTED tomography
Abstract

Aims The Leiden Convention coronary coding system structures the large variety of coronary anatomical patterns; isolated and in congenital heart disease. It is widely used by surgeons but not by cardiologists as the system uses a surgeons' cranial view. Since thoracic surgeons and cardiologists work closely together, a coronary coding system practical for both disciplines is mandatory. To this purpose, the 'surgical' coronary coding system was adapted to an 'imaging' system, extending its applicability to different cardiac imaging techniques. Methods and results The physician takes place in the non-facing sinus of the aortic valve, oriented with the back towards the pulmonary valve, looking outward from the sinus. From this position, the right-hand sinus is sinus 1, and the left-hand sinus is sinus 2. Next, a clockwise rotation is adopted starting at sinus 1 and the encountered coronary branches described. Annotation of the normal anatomical pattern is 1R-2LCx, corresponding to the 'surgical' coding system. The 'imaging' coding system was made applicable for Computed Tomography (CT), Magnetic Resonance Imaging (MRI), echocardiography, and coronary angiography, thus facilitating interdisciplinary use. To assess applicability in daily clinical practice, images from different imaging modalities were annotated by cardiologists and cardiology residents and results scored. The average score upon evaluation was 87.5%, with the highest scores for CT and MRI images (average 90%). Conclusion The imaging Leiden Convention is a coronary coding system that unifies the annotation of coronary anatomy for thoracic surgeons, cardiologists, and radiologists. Validation of the coding system shows it can be easily and reliably applied in clinical practice. [ABSTRACT FROM AUTHOR]

Published
2022
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45. Mortality in adult congenital heart disease

Author

Verheugt, Carianne L., Uiterwaal, Cuno S.P.M., van der Velde, Enno T., Meijboom, Folkert J., Pieper, Petronella G., van Dijk, Arie P.J., Vliegen, Hubert W., Grobbee, Diederick E., and Mulder, Barbara J.M.

Published
2010
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47. Case report of the broad spectrum of late complications in an adult patient with univentricular physiology palliated by the Fontan circulation.

Author

Nederend, Marieke, Egorova, Anastasia D, Vliegen, Hubert W, Roest, Arno A W, Ruijter, Bastian N, Korteweg, Tijmen, Ninaber, Maarten K, Zeppenfeld, Katja, Hazekamp, Mark G, Kiès, Philippine, and Jongbloed, Monique R M

Subjects
CONGENITAL heart disease, VENA cava inferior, VENA cava superior, PULMONARY circulation, CARDIAC patients, TRANSPOSITION of great vessels
Abstract

Background At the most severe end of the spectrum of congenital heart disease are patients with an univentricular physiology. They comprise a heterogeneous group of congenital heart malformations that have the common characteristic that the cardiac morphology is not equipped for sustaining a biventricular circulation. Case summary Here, we present a case of an adult patient after Fontan palliation, illustrative of the complex clinical course and the broad spectrum of complications that can be encountered during follow-up, highlighting the need for a multidisciplinary approach in the clinical care for these patients. Discussion During the surgical Fontan procedure, the inferior vena cava is connected to the pulmonary circulation, after prior connection of the superior vena cava to the pulmonary arterial circulation. The resulting cavopulmonary connection, thus lacking a subpulmonic ventricle, provides non-pulsatile passive flow of oxygen-poor blood from the systemic venous circulation into the lungs, and the functional monoventricle pumps the oxygen-rich pulmonary venous return blood into the aorta. With an operative mortality of <5% and current 30-year survival rates up to 85%, the adult population of patients with a Fontan circulation is growing. This increase in survival is, however, inevitably accompanied by long-term complications affecting multiple organ systems, resulting in decline in cardiovascular performance. Conclusion For optimal treatment, the evaluation in a multidisciplinary team is mandatory, using the specific expertise of the team members to timely detect and address late complications and to support quality of life. [ABSTRACT FROM AUTHOR]

Published
2022
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48. Menstrual Cycle and its Disorders in Women with Congenital Heart Disease

Author

Drenthen, Willem, Hoendermis, Elke S., Moons, Philip, Heida, Karst Y., Roos-Hesselink, Jolien W., Mulder, Barbara J.M., Van Dijk, Arie P.J., Vliegen, Hubert W., Sollie, Krystyna M., Berger, Rolf M.F., Lely, A. Titia, Canobbio, Mary M., and Pieper, Petronella G.

Published
2008

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