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52. 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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64. 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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70. 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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71. 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

72. 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

73. 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

85. 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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87. Improved ECG detection of presence and severity of right ventricular pressure load validated with cardiac magnetic resonance imaging

Author

Henkens, Ivo R., Mouchaers, Koen T.B., Vonk-Noordegraaf, Anton, Boonstra, Anco, Swenne, Cees A., Maan, Arie C., Man, Sum-Che, Twisk, Jos W.R., van der Wall, Ernst E., Schalij, Martin J., and Vliegen, Hubert W.

Subjects
Electrocardiogram -- Methods, Electrocardiography -- Methods, Magnetic resonance imaging -- Methods, Heart ventricle, Right -- Medical examination, Pulmonary hypertension -- Physiological aspects, Heart enlargement -- Physiological aspects, Biological sciences
Abstract

The study aimed to assess whether the 12-lead ECG-derived ventricular gradient, a vectorial representation of ventricular action potential duration heterogeneity directed toward the area of shortest action potential duration, can improve ECG diagnosis of chronic right ventricular (RV) pressure load. ECGs from 72 pulmonary arterial hypertension patients recorded 1 with R > 0.5 mV in V1, and QRS axis >90[degrees]) had a sensitivity of 89% and a specificity of 93% for presence of chronic RV pressure load. However, the ventricular gradient not only had a higher diagnostic accuracy for chronic RV pressure load by receiver operating characteristic analysis [areas under the curve (AUC) = 0.993, SE 0.004 vs. AUC = 0.945, SE 0.021, P < 0.05], but also discriminated between mild-to-moderate and severe RV pressure load. CMR identified an inverse relation between the ventricular gradient and RV mass, and a trend toward a similar relation with RV volume. In conclusion, chronically increased RV pressure load is electrocardiographically reflected by an altered ventricular gradient associated with RV remodeling-related changes in ventricular action potential duration heterogeneity. The use of the ventricular gradient allows ECG detection of even mildly increased RV pressure load. hypertension; pulmonary; right ventricular hypertrophy; diagnosis; ventricular gradient; electrocardiogram

Published
2008

89. Early changes in rat hearts with developing pulmonary arterial hypertension can be detected with three-dimensional electrocardiography

Author

Henkens, Ivo R., Mouchaers, Koen T.B., Vliegen, Hubert W., van der Laarse, Willem J., Swenne, Cees A., Maan, Arie C., Draisma, Harmen H.M., Schalij, Ingrid, van der Wall, Ernst E., Schalij, Martin J., and Vonk-Noordegraaf, Anton

Subjects
Heart enlargement -- Physiological aspects, Electrocardiogram -- Usage, Electrocardiography -- Usage, Pulmonary hypertension -- Development and progression, Physiological research, Biological sciences
Abstract

The study aim was to assess three-dimensional electrocardiogram (ECG) changes during development of pulmonary arterial hypertension (PAH). PAH was induced in male Wistar rats (n = 23) using monocrotaline (MCT; 40 mg/kg sc). Untreated healthy rats served as controls (n = 5). ECGs were recorded with an orthogonal three-lead system on days O, 14, and 25 and analyzed with dedicated computer software. In addition, left ventricular (LV)-to-right ventricular (RV) fractional shortening ratio was determined using echocardiography. Invasively measured RV systolic pressure was 49 (SD 10) mmHg on day 14 and 64 (SD 10) mmHg on day 25 vs. 25 (SD 2) mmHg in controls (both P < 0.001). Baseline ECGs of controls and MCT rats were similar, and ECGs of controls did not change over time. In MCT rats, ECG changes were already present on day 14 but more explicit on day 25: increased RV electromotive forces decreased mean QRS-vector magnitude and changed QRS-axis orientation. Important changes in action potential duration distribution and repolarization sequence were reflected by a decreased spatial ventricular gradient magnitude and increased QRS-T spatial angle. On day 25, LV-to-RV fractional shortening ratio was increased, and RV hypertrophy was found, but not on day 14. In conclusion, developing PAH is characterized by early ECG changes preceding RV hypertrophy, whereas severe PAH is marked by profound ECG changes associated with anatomical and functional changes in the RV. Three-dimensional ECG analysis appears to be very sensitive to early changes in RV afterload. fight ventricular hypertrophy; monocrotaline; electrocardiogram

Published
2007

90. 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

95. Cardiovascular magnetic resonance in the follow-up of patients with corrected tetralogy of Fallot: A review

Author

Oosterhof, Thomas, Mulder, Barbara J.M., Vliegen, Hubert W., and De Roos, Albert

Subjects
Tetralogy of Fallot, Genetic disorders, Cardiac patients, Health
Abstract

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.ahj.2005.03.058 Byline: Thomas Oosterhof (a)(c), Barbara J.M. Mulder (c), Hubert W. Vliegen (b), Albert de Roos (a) Abstract: Cardiovascular magnetic resonance (CMR) is becoming an important tool in the clinical management of patients with congenital heart disease. Because of the diverse problems patients may face after initial correction for tetralogy of Fallot and the large amount of CMR techniques that can be applied, creating a patient-orientated imaging protocol is a difficult issue. Although it is still not certain what the impact of some parameters, provided by CMR, should be on clinical decision making, new techniques are being developed and applied. In this report, we review the current clinical issues in patients with tetralogy of Fallot and review the current implication and limitations of CMR in this patient category. Author Affiliation: (a) Department of Radiology, Leiden University Medical Center, Leyden, The Netherlands (b) Department of Cardiology, Leiden University Medical Center, Leyden, The Netherlands (c) Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands Article History: Received 10 February 2005; Accepted 26 March 2005

Published
2006

96. Non-invasive early exclusion of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism: the InShape II study

Author

Boon, Gudula J A M, primary, Ende-Verhaar, Yvonne M, additional, Bavalia, Roisin, additional, El Bouazzaoui, Lahassan H, additional, Delcroix, Marion, additional, Dzikowska-Diduch, Olga, additional, Huisman, Menno V, additional, Kurnicka, Katarzyna, additional, Mairuhu, Albert T A, additional, Middeldorp, Saskia, additional, Pruszczyk, Piotr, additional, Ruigrok, Dieuwertje, additional, Verhamme, Peter, additional, Vliegen, Hubert W, additional, Vonk Noordegraaf, Anton, additional, Vriend, Joris W J, additional, and Klok, Frederikus A, additional

Published
2021
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97. 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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98. 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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