Ebstein's disease has so wide spectrum in age and severity, that therapeutic strategy including surgical procedures has also wide spectrum from uni-ventricular repair to bi-ventricular repair depending on the performance of the right ventricle, the tricuspid valve and so on.
Most of the elder children and adults can perform bi-ventricular repair, and the tricuspid valve replacement can give a reasonable outcome for the elder group, however the tricuspid valve repair may or must brings a better outcome to the child group.
On the other hand, neonates and early infants with severe clinical condition including low performance of the right ventricle and the tricuspid valve can no always perform bi-ventricular repair, and the results have not been acceptable even now. In this post-graduate seminar, the basic information about surgical strategy including the surgical procedures will be provided.

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Atrioventricular septum is an organization that separates the right atrium and the left ventricle and stands between the interatrial septum and interventricular septum. The atrioventricular septum is derived from the endocardial cushion in an atrioventricular canal. When a primitive heart tube is looping, and an atrium and the ventricle become morphologically apparent, an extracellular matrix, or cardiac jelly, is remaining and increasing in an atrioventricular canal. The mesenchymal cells that were separated and transformed from the endocardial cells through a process called “epithelial-mesenchymal transformation”, invade into the cardiac jelly and form an endocardial cushion. An endocardial cushion mainly upheaves and develops from a superior part (dorsal side) and an inferior part (ventral side). The atrioventricular septum is formed of the coalescence in the central part of the superior and inferior endocardial cushion that divides an atrioventricular canal into the right (tricuspid) and left (mitral) atrioventricular valve orifice. Superior and inferior endocardium cushions extend also in the perpendicular direction to an atrioventricular canal, and participate in closing the ostium primum and formation of membranous interventricular septum. Many congenital heart diseases result from the developmental anomaly of atrioventricular septum, interventricular septum, and interatrial septum.

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Cardiac magnetic resonance imaging (C-MRI) has been progressed in hardware and software techniques past two decades. Clinical application has been done in diagnosis of cardiovascular morphology, function, ischemia and infarction with recent improvement. Cine MRI using steady-state free precession (SSFP) sequence has clear the contrast between the cardiac lumen and the muscle. At 3.0T magnets, multi-phasic 3D contrast-enhanced angiography with breath-holding has established to get continuously. This article discusses the current state of 3D contrast-enhanced angiography, cine MRI by using SSFP, phase contrast, cardiac perfusion and late enhancement in clinical practice.

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Currently, a variety of modalities (radiograph, electrocardiogram, echocardiogram, cardiac catheterization, cardiac angiography, Computed Tomography (CT), magnetic resonance imaging (MRI), etc.) are used for the diagnosis of congenital heart disease. Echocardiography, CT, and MRI have become more important methods in the diagnosis of congenital heart disease in a three-dimensional display. This paper briefly describes the development of CT and the application of the CT to congenital heart disease.

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Objective: To evaluate the surgical outcomes of patients with a functional single ventricle accompanied by coarctation of the aorta or an interrupted aortic arch (CoA/IAA) after staged palliation.
Methods: We compared surgical outcomes and cardiac functions between patients with a functional single ventricle and CoA/IAA after bilateral pulmonary artery banding as the first palliation (n = 13) or aortic arch repair with pulmonary trunk banding (n = 11).
Results: One patient in the bilateral pulmonary artery banding group with asplenia syndrome and severe common atrioventricular valve regurgitation died three months after palliation, seven completed the Fontan procedure as the 3^rd stage of repair and five others who underwent the Norwood-Glenn operation as the interim procedure after the initial palliation await the completion of the Fontan operation. A good end-diastolic volume index and ejection fraction were maintained in these patients while waiting for the Fontan operation.
Three patients in the aortic arch repair with pulmonary trunk banding group died and two others dropped out from BDG/Fontan track because subaortic stenosis progressed, and the first palliation exacerbated atrioventricular valve regurgitation. End-diastolic volumes increased over time in the remaining six patients during BDG/Fontan track.
Conclusions: The surgical outcomes for patients with a functional single ventricle and CoA/IAA in whom subaortic stenosis progressed after aortic arch repair with pulmonary trunk banding were unsatisfactory. Bilateral pulmonary artery banding as the first palliation followed by the Norwood-Glenn operation reduced the risk of subaortic stenosis and atrioventricular valve regurgitation, and promoted hemodynamic stability and candidacy for the Fontan operation.

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