Background: Reduced exercise capacity is nearly universal among Fontan patients, though its etiology is not yet fully understood. While previous computational studies have attempted to model Fontan exercise, they did not fully account for global physiologic mechanisms nor directly compare results against clinical and physiologic data. Methods: In this study, we developed a protocol to simulate Fontan lower-body exercise using a closed-loop lumped-parameter model describing the entire circulation. We analyzed clinical exercise data from a cohort of Fontan patients, incorporated previous clinical findings from literature, quantified a comprehensive list of physiological changes during exercise, translated them into a computational model of the Fontan circulation, and designed a general protocol to model Fontan exercise behavior. Using inputs of patient weight, height, and if available, patient-specific reference heart rate (HR) and oxygen consumption, this protocol enables the derivation of a full set of parameters necessary to model a typical Fontan patient of a given body-size over a range of physiologic exercise levels. Results: In light of previous literature data and clinical knowledge, the model successfully produced realistic trends in physiological parameters with exercise level. Applying this method retrospectively to a set of clinical Fontan exercise data, direct comparison between simulation results and clinical data demonstrated that the model successfully reproduced the average exercise response of a cohort of typical Fontan patients. Conclusion: This work is intended to offer a foundation for future advances in modeling Fontan exercise, highlight the needs in clinical data collection, and provide clinicians with quantitative reference exercise physiologies for Fontan patients. [DOI: 10.1115/1.4027271]
IntroductionSingle ventricle physiology is one of the most severe forms of congenital heart disease, in which an infant has only one functional pumping chamber. The condition is not compatible with life without treatment and these infants require immediate surgical intervention after birth, typically followed by two additional palliative procedures within the first few years of life. The Fontan procedure, in its various technical modifications, represents the final stage of the multistaged surgery that achieves complete separation of the deoxygenated and oxygenated blood in the circulation. The procedure involves directly connecting the superior venae cavae and inferior venae cavae (SVC and IVC) to the pulmonary arteries, thus bypassing the heart and resulting in the total venous return flowing passively into the pulmonary circulation.Exercise intolerance is among the myriad complications that contribute to the gradual attrition of post-Fontan patients in the years following surgery. Other causes of decreased survival include ventricular dysfunction, atrial arrhythmias, protein-losing enteropathy, arteriovenous malformations, thrombotic complications, and poor neurodevelopmental outcomes [1]. While ...