23 year old woman with double inlet left ventricle s/p Fontan, s/p coiling of innominate vein to pulmonary vein collateral vessel, and s/p SVT ablation referred for evaluation of hypoxia. Overall AnatomyDoublet inlet left ventricle s/p bidirectional Glenn shunt and Fontan procedure. There are side-by-side, malposed great vessels. The interatrial septum has been resected. The tricuspid and mitral valves both drain into the left ventricle. There is a hypoplastic right ventricle which communicates with the left ventricle via a large ventricular septal defect. The main pulmonary artery is ligated and forms a blind pouch. Systemic Venous Return (Fontan)A Glenn shunt connects the SVC to the right pulmonary artery. There is a lateral tunnel conduit connecting the IVC to the right pulmonary artery. There are no obstructions or stenoses in either the SVC or IVC. The IVC is dilated. The presence of a communication between the lateral tunnel conduit and right atrial appendage can not be excluded. The communication is suggested on the non-contrast MRA and first pass contrast imaging, but not on cine CMR or contrast enhanced MRA. Systemic Ventricle (i.e. Left Ventricle)The left ventricle is normal in size with normal systolic function. The overall LV ejection fraction is 54%, the LV end diastolic volume index is 76 ml/m2 (normal range: 65+/-11), the LVEDV is 125 ml (normal range 109+/-23), the LV end systolic volume index is 35 ml/m2 (normal range 18+/-5), and the LVESV is 58 ml (normal range 31+/-10). Pulmonary Venous ReturnThere are four pulmonary veins which drain into the common atrium. There are no obvious obstructions of the pulmonary veins.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 opens widely into the left ventricle and there is no significant mitral regurgitation.Tricuspid ValveThe tricuspid valve opens widely into the left ventricle and there is mild tricuspid regurgitation. Great VesselsThere are side-by-side, malposed great vessels. There is a left sided aortic arch with a normal brachiocephalic branching pattern. There is no thoracic aortic aneurysm or thoracic aortic dissection. The main pulmonary artery is ligated and forms a blind pouch emptying into the right ventricle. There are no proximal stenoses of the left and right pulmonary arteries. The left pulmonary artery diameter is 11mm. The right pulmonary artery diameter is 16mm.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. Double inlet left ventricle with hypoplastic right ventricle s/p tunneled Fontan2. No Fontan/ Glenn obstruction. Can not rule out small communication between intra-cardiac tunnel and right atrial appendage. 3. Normal left ventricular size and systolic function (LVEF 54%). 4. Main pulmonary artery is ligated and forms blind pouch.5. Dilated inferior vena cava.6. Left pulmonary artery is 2/3 of the size of right pulmonary artery. I personally reviewed the Images and/or procedure with the Resident/Fellow and agree with this report.