We describe an in vitro model of the Fontan circulation with respiration to study subdiaphragmatic venous flow behavior. The venous and arterial connections of a total cavopulmonary connection (TCPC) test section were coupled with a physical lumped parameter (LP) model of the circulation. Intrathoracic and subdiaphragmatic pressure changes associated with normal breathing were applied. This system was tuned for two patients (5 years, 0.67 m2; 10 years, 1.2 m2) to physiological values. System function was verified by comparison to the analytical model on which it was based and by consistency with published clinical measurements. Overall, subdiaphragmatic venous flow was influenced by respiration. Flow within the arteries and veins increased during inspiration but decreased during expiration with retrograde flow in the inferior venous territories. System pressures and flows showed close agreement with the analytical LP model (p < 0.05). The ratio of the flow rates occurring during inspiration to expiration were within the clinical range of values reported elsewhere. The approach used to setup and control the model was effective and provided reasonable comparisons with clinical data.

Despite the recent advances in computational modeling, experimental simulation of the circulation with congenital heart defect using mock flow circuits remains an important tool for device testing, and for detailing the probable flow consequences resulting from surgical and interventional corrections. Validated mock circuits can be applied to qualify the results from novel computational models. New mathematical tools, coupled with advanced clinical imaging methods, allow for improved assessment of experimental circuit performance relative to human function, as well as the potential for patient-specific adaptation. In this review, we address the development of three in vitro mock circuits specific for studies of congenital heart defects. Performance of an in vitro right heart circulation circuit through a series of verification and validation exercises is described, including correlations with animal studies, and quantifying the effects of circuit inertiance on test results. We present our experience in the design of mock circuits suitable for investigations of the characteristics of the Fontan circulation. We use one such mock circuit to evaluate the accuracy of Doppler predictions in the presence of aortic coarctation.

Each year, a small fraction of children are born with univentricular hearts, causing the lethal blue baby syndrome. Several preliminary operations are required to buy time until the child’s blood vessels grow to sufficient size. Once the child reaches an age of 3–5 years, the blood vessels have grown enough for the Stage 3 Fontan operation, in which the superior and inferior vena cavae are coupled directly to the pulmonary arteries in a cruciform junction [2,3]. After this operation, the heart is only pumping blood to the systemic circulation. Only residual pressure in the systemic veins and intrathoracic pressure change with respiration drive the flow into the lungs [5]. This circulation decreases the load on the heart, allowing the patients to survive with normal blood oxygen levels. This circulation decreases the load on the heart, allowing the patients to survive into their 20s and 30s. An aim of this study is to develop an experimental model of the Fontan circulation that can be readily adapted to simulate patient specific anatomies so as to assist in potential surgical decisions. Of interest is the study of chronic venous hypertension, a result of the Fontan circulation having no heart “vacuum” at the end of the vena cavae; it is known to cause liver failure. We also intend to examine the hypothesis of Hsia et al. [6] that decreasing sub-diaphragmatic venous flow reversal will improve functional outcome of the Fontan.

The Fontan circulation is the result of a series of operations performed on children born with univentricular circulations (1). These congenital heart defects are uniformly fatal if left alone. After birth, an operation is performed to assure that the child receives enough blood flow to the lungs, but not too much in order to avoid pulmonary vascular disease. Once the child reaches 2–4 years of age, the child’s blood vessels are sufficiently large for the Fontan operation. The Fontan operation connects the great systemic veins directly to the pulmonary arteries, bypassing the right ventricle entirely. One method of the Fontan procedure, which is known as the total cavopulmonary connection (TCPC), achieves venous return to the pulmonary circulation without a ventricular power source. The load on the heart is reduced, and these patients can lead a normal life into adulthood; although late complications continue to prevent normal lifespan. One unique feature of the Fontan circulation is reliance of the inferior vena cava (IVC) flow on respiration, and flow reversal in the IVC and hepatic vein during expiratory phase of breathing (2). Hsia et al. (3) suggest that reducing flow reversal in the hepatic vein will improve the outcome of the Fontan operation. The goal of this study is to model experimentally the Fontan circulation for a variety of different patients using an adjustable mock circulatory system, which for the first time includes the influence of respiration.