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- Author
-
A.C. van Dissel
- Title
- Adult congenital heart disease
- Subtitle
- When left seems right and right is wrong
- Supervisors
-
B.J.M. Mulder
C.S. Broberg - Co-supervisors
- Award date
- 27 September 2022
- Number of pages
- 279
- ISBN
- 9789463617406
- Document type
- PhD thesis
- Faculty
- Faculty of Medicine (AMC-UvA)
- Abstract
-
Congenital heart disease (CHD) is the most common birth defect and affects approximately 9 per 1,000 newborns. Major advances in the management of children with CHD has yielded a dramatic change in age distribution, with more adults living with CHD nowadays than children. With increased longevity, caregivers need to be prepared to diagnose and treat late complications of this growing and aging patient population, who —in contrast to acquired heart disease— more commonly have right-sided (RV) complications. In this thesis, we addressed the role of the RV in adult CHD in the following clinical settings: in the subpulmonic position in pulmonary arterial hypertension (PAH-CHD), in the subaortic position in transposition of the great arteries (TGA), and when subjected to hemodynamic stress during exercise training.
Effective collaborations at both national and international levels have enabled us to challenge the existing paradigm that RV failure is inevitable in CHD. In TGA patients, we observed slow disease progression to end-stage heart failure or death. Several drugs (i.e. selexipag and valsartan) showed less beneficial effects in patients with PAH-CHD or TGA respectively than expected from landmark trials in PAH and LV failure. Exercise training however seemed to be safe, even in those with severe CHD. Lastly, we developed several simplified prediction tools for adverse outcome to help identify those at greatest risk.
Better understanding of the risk factors and treatment options for the RV in patients with CHD is highly relevant and must continue to be the focus of intensive research in future. - Note
- Please note that the acknowledgements section is not included in the thesis downloads.
- Persistent Identifier
- https://hdl.handle.net/11245.1/2e3c942a-e0c8-4da8-826a-85d7d8a5da57
- Downloads
-
Thesis
Front matter
Chapter 1: Introduction, aim and thesis outline
Chapter 2: The changing landscape of pulmonary arterial hypertension in the adult with congenital heart disease
Chapter 3: Selexipag for pulmonary arterial hypertension in a wide range of adult congenital heart disease
Chapter 4: Prognostic value of multiple repeated biomarkers in pulmonary arterial hypertension associated with congenital heart disease
Chapter 5: Improved risk prediction using a modified European guidelines instrument in pulmonary arterial hypertension related to congenital heart disease
Chapter 6: Long-term clinical outcomes of valsartan in patients with a systemic right ventricle: Follow-up of a multicenter randomized controlled trial
Chapter 7: Long-term outcomes after atrial switch operation for transposition of the great arteries
Chapter 8: Heart failure outcomes in congenitally corrected transposition of the great arteries: A multicenter study
Chapter 9: Cardiac MRI predictors of adverse outcomes in adults with a systemic right ventricle
Chapter 10: The effect of exercise training in symptomatic patients with grown-up congenital heart disease: A review
Chapter 11: Safety and effectiveness of home-based, self-selected exercise training in symptomatic adults with congenital heart disease: A prospective, randomised, controlled trial
Chapter 12: Summary / Nederlandse samenvatting
Chapter 13: Concluding remarks and future perspectives
List of abbreviations; List of publications; Contributing authors; Curriculum vitae; Portfolio
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