Search results for "synnynnäiset sydänviat"
showing 4 items of 4 documents
Arterial Stiffness and Its Relationship to Cardiorespiratory Fitness in Children and Young Adults with a Fontan Circulation
2019
There are no previous studies on arterial stiffness and its associations with cardiorespiratory fitness in young Fontan patients. Therefore, we examined the arterial stiffness and its relationship to cardiorespiratory fitness in children and young adults with a Fontan circulation. Altogether, 17 Fontan patients and 26 healthy controls (16 females and 27 males aged 8–40 years) participated in this cross-sectional study. The cardiorespiratory fitness was assessed by cardiopulmonary exercise testing on a cycle ergometer and was defined as the standard deviation scores (SDS) of peak oxygen uptake per body mass (VO2peak/kg) based on the national reference values and assessed with cardiopulmonary…
Critical Structural Defects Explain Filamin A Mutations Causing Mitral Valve Dysplasia
2019
Mitral valve diseases affect approximately 3% of the population and are the most common reasons for valvular surgery because no drug-based treatments exist. Inheritable genetic mutations have now been established as the cause of mitral valve insufficiency, and four different missense mutations in the filamin A gene (FLNA) have been found in patients suffering from non-syndromic mitral valve dysplasia (MVD). The FLNA protein is expressed, in particular, in endocardial endothelia during fetal valve morphogenesis and is key in cardiac development. The FLNA-MVD causing mutations are clustered in the N-terminal region of FLNA. How the mutations in FLNA modify its structure and function, have mos…
Exercise as therapy for neurodevelopmental and cognitive dysfunction in people with a Fontan circulation: A narrative review
2023
People with a Fontan circulation are at risk of neurodevelopmental delay and disability, and cognitive dysfunction, that has significant implications for academic and occupational attainment, psychosocial functioning, and overall quality of life. Interventions for improving these outcomes are lacking. This review article discusses current intervention practices and explores the evidence supporting exercise as a potential intervention for improving cognitive functioning in people living with a Fontan circulation. Proposed pathophysiological mechanisms underpinning these associations are discussed in the context of Fontan physiology and avenues for future research are recommended.