Abstract
Fontan procedures are radical surgical reconstructions performed for children born with only one effective ventricle, or two that cannot be separated functionally. They entail the connection of the pulmonary vascular resistance downstream of the systemic vascular resistance, flow through both being delivered, in series, by the one ventricle, but at the cost of elevated systemic venous pressure (Fig. 10.1). This aims to eliminate shunting and the associated ventricular volume loading, and to achieve full pulmonary oxygenation.
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10.1 Electronic supplementary Material
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218193_1_En_10_MOESM1_ESM.avi
Three types of Fontan connection illustrated by CMR cine images, (a) atrio-pulmonary connection, (band Movie 10.1) lateral tunnel and (c) extracardiac conduit. (b, c) Can both be called total cavo-pulmonary connection (TCPC). As a transitional stage to Fontan physiology, a limited residual shunt may be left in the form of a fenestration between the IVC pathway and the low-pressure atrial cavity to slightly alleviate systemic venous pressure. The TCPC avoids the progressive right atrial distension which can predispose to atrial arrhythmias, stagnation and thrombosis, and the coronary sinus drains to the low pressure part of the right atrium, which is marked *(AVI 3351 KB)
218193_1_En_10_MOESM2_ESM.avi
Complications of early atrio-pulmonary Fontan procedures. This sagittal cine image shows the dilated right atrium (RA) with a large thrombus attached to its floor. The solidified, ineffective leaflets of homograft atrial inflow valves can be seen in this case, mildly restricting inflow from the SVCand IVC. For this reason, atrial inflow valves were not included in later variants of the operation (AVI 2583 KB)
Complications of early atrio-pulmonary Fontan procedures. The dilated right atrium (RA) upstream of an atrio-pulmonary Fontan connection causing compression of the right lower pulmonary vein (arrow), which then tends to exacerbates right atrial pressure and distension (AVI 1722 KB)
Desaturating shunts in three different patients. (a, Movie 10.4) A diastolic leak through the detachment of a patch placed across the right atrio-ventricular valve of a patient with double inlet left ventricle and an atrio-pulmonary Fontan connection. (b) Magnetic resonance contrast angiogram showing evidence of right pulmonary arterio-venous malformations (arrows) in a patient after Kawashima operation in whom hepatic venous return was flowing to the left lung, but not the right. (c, d) Subcutaneous (black arrow) and intra-thoracic (white arrows) branches of systemic venous to pulmonary venous collateral veins (AVI 319 KB)
Movie 10.1
Three types of Fontan connection illustrated by CMR cine images, (a) atrio-pulmonary connection, (band Movie 10.1) lateral tunnel and (c) extracardiac conduit. (b, c) Can both be called total cavo-pulmonary connection (TCPC). As a transitional stage to Fontan physiology, a limited residual shunt may be left in the form of a fenestration between the IVC pathway and the low-pressure atrial cavity to slightly alleviate systemic venous pressure. The TCPC avoids the progressive right atrial distension which can predispose to atrial arrhythmias, stagnation and thrombosis, and the coronary sinus drains to the low pressure part of the right atrium, which is marked *(AVI 3351 KB)
Movies 10.2
Complications of early atrio-pulmonary Fontan procedures. This sagittal cine image shows the dilated right atrium (RA) with a large thrombus attached to its floor. The solidified, ineffective leaflets of homograft atrial inflow valves can be seen in this case, mildly restricting inflow from the SVCand IVC. For this reason, atrial inflow valves were not included in later variants of the operation (AVI 2583 KB)
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Seale, A.N., Kilner, P.J. (2012). Single Ventricle and Fontan Procedures. In: Syed, M., Mohiaddin, R. (eds) Magnetic Resonance Imaging of Congenital Heart Disease. Springer, London. https://doi.org/10.1007/978-1-4471-4267-6_10
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DOI: https://doi.org/10.1007/978-1-4471-4267-6_10
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