1. Clinical aspects and pathophysiological mechanisms of (systemic) right ventricular failure
- Author
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Zandstra, T.E., Schalij, M.J., Jongbloed, M.R.M., Kiès, P., Blom, N.A., Winter, R.J. de, Ebels, T., Bootsma, M., Kuipers, I.M., and Leiden University
- Subjects
Echocardiography ,Sympathetic nervous system ,Parasympathetic nervous system ,Heart failure ,Transposition of great vessels ,Congenital heart disease ,Atrial switch - Abstract
Adult patients with congenital heart disease and a systemic right ventricle are prone to develop late complications such as rhythm and conduction disorders and heart failure. This thesis aims to clarify the pathophysiology of this condition and to explore diagnostic and treatment options. Chapter 2 describes asymmetry and heterogeneity in cardiac autonomic innervation. Chapters 3 and 4 describe ambulatory ECG-derived measures of cardiac autonomic function in patients with a systemic right ventricle, and show that these measures are related to outcomes such as supraventricular arrhythmias. In chapter 5, echocardiographic measures of systemic right ventricular function are compared with cardiac MRI. Chapter 6 describes the clinical course of patients long after atrial switch correction. Chapter 7 describes the first results of treatment of systemic right ventricular failure with sacubitril/valsartan: a.o. a small but significant improvement in systemic right ventricular function was seen. Chapter 8 describes the experience of the application of eHealth smart technology in the titration of sacubitril/valsartan in the same cohort. Chapter 9 reports the first two cases of implantation of a ventricular assist device in patients with systemic right ventricular failure. This thesis may form the base for ongoing research to improve outcome in this vulnerable group.
- Published
- 2023