10 year old female with Down's syndrome, s/p repair of AV canal with tetralogy of Fallot in 2006, bilateral pulmonary artery stents (2013) with differential perfusion to lungs (Left 34%). Referred for cardiac MRI for re-assessment. SitusLevocardia with normal cardiac and abdominal situs.Left VentricleThe left ventricle is normal in size with mildly reduced systolic function. The overall LV ejection fraction is 44%. The LVEDV is 81 ml (normal range 55-95), LV end diastolic volume index is 72 ml/m2 (normal range: 65+/-11), LVESV is 46 ml (normal range 15-35), and LV end systolic volume index is 40 ml/m2 (normal range 18+/-5). 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.There is no LV thrombus. The ventricular septal patch is intact.Left AtriumThe left atrium is normal in size. Atrial septal patch is intact.Right VentricleThe right ventricle is severely dilated with mildly reduced systolic function. The overall RV ejection fraction is 43%, the RVEDV is 157 ml, the RV end diastolic volume index is 139 ml/m2 (normal range 76 +/- 18), RVESV is 91 ml, and the RV end systolic volume index is 80 ml/m2 (normal range 27 +/-10).Right AtriumThe right atrium is moderately dilated. Aortic ValveThe aortic valve is trileaflet, opens widely and there is no significant aortic regurgitation.Left atrio-ventricular valveMild left atrio-ventricular valve regurgitation.Pulmonary arteries and pulmonary valveThere is thinning and bulging and of the RVOT suggestive of previous transannular patch type repair. The pulmonic valve leaflets are not clearly visible and there is free regurgitation through the RV outflow tract. Regurgitant fraction is 55%. The main and both proximal pulmonary arteries are focally obscured by blooming artifact from the PA stents. Right atrio-ventricular valveMild-moderate right atrio-ventricular valve regurgitation.AortaThere is a left sided aortic arch with normal brachiocephalic branching pattern. The aortic root is normal in size.Pulmonary VeinsAll four pulmonary veins drain normally into the left atrium. Venous AnatomyThe SVC and IVC drain normally into the right atrium. PericardiumThere is no obvious pericardial disease.Extracardiac FindingsLeft lower lobe subsegmental atelectasis. Suggestion of antero-posterior collapse of the left mainstem bronchus. This 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.Morphologic changes of repaired AV canal and Tetralogy of Fallot. Bilateral pulmonary artery stents present.2.Severe right atrial and ventricular dilation with mildly reduced RV function. RVEF 43%, RVEDV 157 ml, RVEDVi 139 ml/m2.3.Free pulmonary regurgitation with no clearly appreciable pulmonary valve leaflets. Regurgitant fraction is 55%.4.Normal left ventricular size with mildly reduced left ventricular function. 5.No residual atrial or ventricular septal defects.6.Mild-moderate right atrio-ventricular valve regurgitation and mild left atrio-ventricular valve regurgitation.7.There is no late gadolinium enhancement to suggest the presence of an underlying fibrosing, infiltrative, or inflammatory process.