18 year old male with a history of hypoplastic right ventricle who under went BT shunt as infant followed by take down of BT anastomosis, creation of bidirectional Glenn shunt, enlargement of atrial septostomy, and followed by lateal tunnel Fontan with fenestrations who is referred for cardiac MRI for further evaluation to evaluate for hypoxia. Overall AnatomyHypoplastic right ventricle s/p bidirectional Glenn shunt and Fontan (lateral tunnel) procedure. The interatrial septum has been resected. There is tricuspid valve atresia with hypoplastic right ventricle. The left ventricle is dominant and is the systemic ventricle.Systemic Venous Return (Fontan)A Glenn shunt connects the SVC to the right pulmonary artery. There is a conduit connecting the IVC to the right pulmonary artery. There are no obstruction or leak noted in or around the Glenn shunt. The possibility of a stenosis or a Fontan leak in the distal part of the lateral tunnel can not be excluded due to imaging artifact in that territory. Systemic Ventricle (i.e. Left Ventricle)The left ventricle is normal in size with low normal systolic function. The overall LV ejection fraction is 50%, the LV end diastolic volume index is 92 ml/m2 (normal range: 74+/-15), the LVEDV is 156 ml (normal range 142+/-34), the LV end systolic volume index is 46 ml/m2 (normal range 25+/-9), the LVESV is 78 ml (normal range 47+/-19). There are no regional wall motion abnormalities present. There is no late gadolinium enhancement to suggest the presence of an underlying fibrosing, infiltrative, or inflammatory process.Pulmonary Venous ReturnThere are four pulmonary veins which seem to drain into the common atrium; unfortunately, the anatomy is not optimally defined due to large artifact related to surgical material posterior to the heart. Right VentricleThe right ventricle is hypoplastic.Common AtriumThe interatrial septum has been resected resulting in a common atrium. Aortic ValveThe aortic valve opens widely and there is no significant aortic regurgitation.Mitral ValveThe mitral valve is not well seen due to the above described imaging artifact.Tricuspid ValveThe tricuspid valve is atretic.AortaThere is a left sided aortic arch with a normal brachiocephalic branching pattern. There is no thoracic aortic aneurysm or thoracic aortic dissection. Left and Right Pulmonary ArteryThe left pulmonary artery has a diameter of ~10mm. The right pulmonary artery has a diameter of ~9mm.PericardiumThere is no obvious pericardial disease.Extracardiac FindingsThis study is not designed for the specific evaluation of extra-cardiac findings; therefore, the differentiation between artifacts and real extracardiac pathology may be difficult. If clinically indicated, a separate dedicated evaluation should be considered.
1. Glenn shunt and lateral tunnel Fontan noted. The Glenn shunt is without obstruction or obvious other abnormality. The IVC is dilated and the possibility of a stenosis or leak in the distal portion of the Fontan can not be excluded due to imaging artifact. 2. Normal left ventricular size with low normal systolic function (LVEF 50%).3. Hypoplastic right ventricle/ tricuspid atresia4. Coronary sinus drains into the "common" atrium. I personally reviewed the Images and/or procedure with the Resident/Fellow and agree with this report.