The University of Sydney, Sydney Medical School, Camperdown, NSW, Australia.
Heart Research Group, Murdoch Children's Research Institute, Melbourne, VIC, Australia.
Cardiol Young. 2024 Feb;34(2):238-249. doi: 10.1017/S1047951123004389. Epub 2024 Jan 23.
The population of people living with a Fontan circulation are highly heterogenous, including both children and adults, who have complex health issues and comorbidities associated with their unique physiology throughout life. Research focused on nutritional considerations and interventions in the Fontan population is extremely limited beyond childhood. This review article discusses the current literature examining nutritional considerations in the setting of Fontan physiology and provides an overview of the available evidence to support nutritional management strategies and future research directions. Protein-losing enteropathy, growth deficits, bone mineral loss, and malabsorption are well-recognised nutritional concerns within this population, but increased adiposity, altered glucose metabolism, and skeletal muscle deficiency are also more recently identified issues. Emergencing evidence suggets that abnormal body composition is associated with poor circulatory function and health outcomes. Many nutrition-related issues, including the impact of congenital heart disease on nutritional status, factors contributing to altered body composition and comorbidities, as well as the role of the microbiome and metabolomics, remain poodly understood.
患有 Fontan 循环的人群高度异质化,包括儿童和成人,他们在整个生命过程中都存在复杂的健康问题和合并症,与他们独特的生理状况有关。除了儿童时期,针对 Fontan 人群的营养考虑因素和干预措施的研究极其有限。本文综述了目前研究 Fontan 生理学中营养考虑因素的文献,并概述了现有证据,以支持营养管理策略和未来的研究方向。蛋白丢失性肠病、生长缺陷、骨矿物质丢失和吸收不良是该人群中公认的营养问题,但越来越多的肥胖、葡萄糖代谢改变和骨骼肌缺乏也是最近发现的问题。新出现的证据表明,异常的身体成分与循环功能和健康结果不良有关。许多与营养相关的问题,包括先天性心脏病对营养状况的影响、导致身体成分改变和合并症的因素,以及微生物组和代谢组学的作用,仍然知之甚少。