Defining long term morbidity and mortality in adult survivors of congenital heart disease

R Medicine (General);RC Internal medicine

Dobson, Richard ; Tzemos, Nikolaos

University:University of Glasgow

Department:Institute of Cardiovascular and Medical Sciences

关键词: Adult congenital heart disease, cardiac MRI, cardiopulmonary exercise testing, Tetralogy of Fallot, systemic right ventricle.;   

Others  :  http://theses.gla.ac.uk/8526/1/2017Dobson.MD.pdf

来源: University of Glasgow

【 摘 要 】

Over the past thirty years there has been a dramatic improvement in the survival of children with congenital heart disease, and the vast majority can now expect to reach adulthood. However it is rare that a lesion can truly be described as "cured" even with everything that modern surgery can offer. Often lesions are palliated instead, and this subjects patients to a variety of complications in later life. These include residual haemodynamic lesions necessitating further surgical or percutaneous intervention, cardiac arrhythmia, and heart failure. All of these are potentially lethal, and result in a steady decline in survival, functional status and quality of life in later years.In order to care for these patients a new medical subspecialty has emerged - that of Adults with Congenital Heart Disease (ACHD). Although the provision of highly specialist management for this population has allowed many significant clinical improvements to be made, healthcare practitioners working in this field face significant challenges. The long term prognosis for these patients is uncertain, making it difficult to know how to counsel youngpeople who wish for employment, a family, and a future. Whilst powerful tools for the non-invasive assessment of cardiovascular structure and function exist, their role in clinical decision making is often poorly defined, and the nature and timing of repeat intervention are frequently based on expert opinion rather than robust evidence from large well conductedclinical trials, and consequently the true benefit of such procedures is unclear. Furthermore, to my knowledge, there has never been a serious attempt to characterise outcomes in ACHD patients in Scotland. Because of the sheer heterogeneity of congenital cardiac conditions Idecided to focus on outcomes in two distinct, relatively easily defined cohorts of adult patients with repaired congenital heart disease - patients with a systemic right ventricle, and patients with repaired Tetralogy of Fallot (TOF), both characterised by the presence of an abnormal right ventricle. In chapter 3 I outline what is currently known about mortality andmorbidity in each condition, based on what has been reported globally by other congenital cardiac centres. I then report the survival and adverse outcomes experienced by patients followed up by our centre: for patients with a systemic right ventricle in chapter 4, and for patients with repaired TOF in chapter 5. I assess these outcomes both in relation to the published literature internationally, and also to the general Scottish population. Finally, in chapter 6 I assess the clinical utility of parameters derived from cardiac MRI and cardiopulmonary exercise testing to establish whether this can improve clinical risk stratification for patients with repaired TOF.