Nutritional Management of Patients with Fontan Circulation: A Potential for Improved Outcomes from Birth to Adulthood

Abstract

Fontan circulation (FC) is a surgically achieved palliation state offered to patients affected by a wide variety of congenital heart defects (CHDs) that are grouped under the name of univentricular heart. The procedure includes three different surgical stages. Malnutrition is a matter of concern in any phase of life for these children, often leading to longer hospital stays, higher mortality rates, and a higher risk of adverse neurodevelopmental and growth outcomes. Notwithstanding the relevance of proper nutrition for this subset of patients, specific guidelines on the matter are lacking. In this review, we aim to analyze the role of an adequate form of nutritional support in patients with FC throughout the different stages of their lives, in order to provide a practical approach to appropriate nutritional management. Firstly, the burden of faltering growth in patients with univentricular heart is analyzed, focusing on the pathogenesis of malnutrition, its detection and evaluation. Secondly, we summarize the nutritional issues of each life phase of a Fontan patient from birth to adulthood. Finally, we highlight the challenges of nutritional management in patients with failing Fontan.

Nutritional considerations for people living with a Fontan circulation: a narrative review

“…The nutritional management of children prior to the completion of Fontan surgery has been described in detail elsewhere. 14,77,78 The pre-Fontan circulation stage is recognised to overlap a sensitive period for growth and development, whereby disturbances in nutrition can significantly impact growth trajectories later in life and the risk of adverse outcomes (i.e., length of stay, quality of life, mortality, wound healing, morbidity). 12,14,23,[79][80][81] Abnormalities often originate in the prenatal phase and manifest as fetal growth restriction (previously known as intrauterine growth restriction).…”

“…14,80,[82][83][84][85][86][87] Newborn weight for those with single-ventricle physiology is reported to be within the normal range for their gestational age, but comparatively lower than the general population matched for age. 12,77,80,81,87,88 However, a child's birth weight is not a predictor of their weight gain trajectory; children who are born with low birth weight are more likely to demonstrate greater gains in weight than those who were born at higher birth weight. 88 The nutritional efforts (i.e., oral intake, enteral nutrition) for growth catch-up, particularly for weight, may influence body composition leading to an increased accumulation of adiposity rather than lean muscle mass later in life.…”

Advances in Pediatric Cardiology Nutrition

“…Specific guidelines on the nutritional management of FC patients are still lacking despite the fact that malnutrition has significant implications for survival, quality of life and short- and long-term complications in these children. Baldini et al conducted an extensive review on the nutritional management of these patients [ 4 ]. The authors reported the current evidence-based literature on the nutritional interventions in these patients, from the assessment of the nutritional status to the dietary interventions that should be taken into account throughout the different stages of these children’s lives.…”

“…The papers by Palleri et al and Baldini et al definitively show that proper nutritional management is crucial in children with CHDs [ 3 , 4 ]. Samanidis et al investigated another aspect of the complex relationship between nutrition and CHD, particularly the role of nutritional interventions in the management of postoperative complications [ 5 ].…”

“…Palleri et al, Baldini et al and Samanidis et al show the need for a shared consensus on the optimal nutritional management of children with CHDs at every stage of these patients’ lives, given the crucial role of nutritional status in this broad spectrum of diseases [ 3 , 4 , 5 ].…”