33 year old man with history of complex congenital disease with multiple previous surgeries, admitted for hypoxia and atrial fibrillation with suspected RA clot on CT, who is now referred for cardiac MRI to evaluate morphology and function.Surgical history is as follow:At birth: formation of PDA and atrial septal defectAt 6 months: Blalock-Taussig shunt At 4 years: RVOT patch At 6 years: Closure of BT shunt and ASD; Glenn shunt performed Overall AnatomyThere is pulmonary atresia with the right main pulmonary artery not well visualized, atretic or absent. Right pulmonary arteries are visualized at the right hilum at lobar level which branch to small segmental and subsegmental arteries. A Glenn shunt is present with the superior vena cava providing flow to the origin of the right pulmonary artery at the lobar level. The inferior vena cava maintains conventional anatomy and connects to the floor of the right atrium which is severely dilated.The right ventricle is normal in size and a tricuspid valve is present. The left ventricle is normal in size and is the systemic ventricle.Systemic Venous ReturnA Glenn shunt connects the SVC to the right pulmonary artery. The IVC empties in the right atrium. There are no obstructions or stenoses in either the SVC or IVC.Right VentricleThe right ventricle is normal in size with moderately reduced systolic function. The overall RV ejection fraction is 42%, the RV end diastolic volume index is 58 ml/m2 (normal range 82+/-16), the RVEDV is 127 ml (normal range 142+/-31), the RV end systolic volume index is 34 ml/m2 (normal range 31+/-9), and the RVESV is 74 ml (normal range 54+/-17).Pulmonary Venous ReturnThere are four pulmonary veins which drain into the left atrium. There are no obvious obstructions of the pulmonary veins.Left VentricleThe left ventricle is normal size. The overall LV ejection fraction is 56%, the LV end diastolic volume index is 51 ml/m2 (normal range: 74+/-15), the LVEDV is 111 ml (normal range 142+/-34), the LV end systolic volume index is 22 ml/m2 (normal range 25+/-9), the LVESV is 49 ml (normal range 47+/-19), the LV mass index is 39 g/m2 (normal range 85+/-15), and the LV mass is 85 g (normal range 164+/-36). There is interventricular septal flattening throughout the entire cardiac cycle.There is high signal on the delayed viability sequences at the basal interventricular septum which corresponds to expected membranous VSD patch. On the four-chamber cine images, this area demonstrates slight increased mobility than the muscular VSD. There is no associated jet on either side of this patch.Left Atrium: Normal size. There are 4 pulmonary veins on the right and 2 on the left that drain into the left atrium.Right Atrium: The right atrium is severely enlarged. There is a low signal nodular structure in the right atrium which appears to be adherent to the superior interatrial septum measuring 2.6 x 4.6cm. It approaches the ostium of the right atrial appendage and occupies the atrial chamber, not prolapsing into the tricuspid valve. It does not perfuse on first pass imaging or enhance, consistent with thrombus. The coronary sinus is dilated. No gross defect of the interatrial septum is visualized on cine views.Contrast injection was performed via antecubital fossa with initial opacification of the left chambers due to the Glen shunt. Therefore, possible resting shunt from right to left was not excluded. Aortic ValveThe aortic valve opens widely and there is trivial aortic regurgitation. Regurgitant fraction is 6%.Mitral ValveThe mitral valve appears normal in structure with trivial mitral regurgitation.Tricuspid ValveThe tricuspid valve opens widely. There is no significant tricuspid regurgitation. The septal leaflet is faintly visualized and appears to be displaced apically by 10 mm relative to the anterior leaflet of the mitral valve.AortaThe ascending thoracic aorta is located posterior to the pulmonary trunk. The ascending aorta is dilated (41mm x 41mm at the level of the main PA). There is a left sided aortic arch with a normal brachiocephalic branching pattern. There is post ductal diffuse narrowing of the aorta without obvious coarctation. The descending thoracic aorta measures 1.3 x 1.7cm at the level of the coronary sinus. There is no thoracic aortic dissection. Pulmonary Artery: There is a patch in the RVOT consistent with previous surgery. The main pulmonary artery is small and atretic. The proximal right PA is not visualized. The left pulmonary artery diameter is 26mm.PericardiumThere is no pericardial effusion.Extracardiac FindingsSternotomy wires are present. The liver appears nodular with dilated suprahepatic IVC and intrahepatic veins, consistent with clinical history of cirrhosis.
1. Anatomy consistent with pulmonary atresia, with absent or atretic right main pulmonary artery. 2. The right ventricle is normal in size with moderately reduced function (EF 42%). There is a tricuspid valve present. 3. The left ventricle is the systemic ventricle, normal in size with normal systolic function (EF 57%).4. A Glenn shunt is seen connecting the SVC to a lobar branch of the right pulmonary artery. The IVC empties into the right atrium, which is severely enlarged.5. Due to contrast injection, unable to visualize the right sided chambers therefore cannot exclude a right to left shunt on this study.6. There is a large thrombus occupying the right atrium which measures 2.6 x 4.6cm. 7. Dilated ascending aorta (41 x 41mm) at the level of the main PA. There is post ductal narrowing without coarctation.8. Liver appearance consistent with cirrhosis, possibly related to reflux hepatopathy. I personally reviewed the Images and/or procedure with the Resident/Fellow and agree with this report.