A 21 year old female with a history of Trisomy 21, left dominant unbalanced AV septal defect with hypoplastic right heart, over-riding AV valve, and bilateral SVC. At age 2, patient underwent AV septal defect patch closure, Left AV valve repair, and a modified Glenn shunt (right SVC to right PA). At age 8, the patient developed recurrent cyanosis and was found to have recurrent VSD. At that time, her Glenn shunt was taken down and her RPA and SVC were reconstructed. Her VSD and mitral valve were re-repaired. She had a cardiac MRI performed in 2009 and another in 2013. She is referred to cardiac MRI for further follow-up. Overall anatomy. See aboveLeft VentricleThe left ventricle is mildly dilated with mildly reduced systolic function. The LVEF is 49%, the LV end diastolic volume index is 105 ml/m2 (normal range 69+/-14), the LVEDV is 211 ml (normal range 110+/-24), the LV end systolic volume index is 54 ml/m2 (normal range 22+/-8), and the LVESV is 108 ml (normal range 35+/-13). There is hypokinesis of the apical lateral wall. The LV apical lateral wall has prominent trabeculations. There is no late gadolinium enhancement present to suggest underlying myocardial fibrosis, inflammation, or infiltration. No significant difference compared to the CMR of 2013. No intracardiac thrombus.Right VentricleThe right ventricle is mildly dilated with mildly reduced systolic function. There is abnormal motion of the apical free wall. The overall RV ejection fraction is 48%, the RV end diastolic volume index is 114 ml/m2 (normal range 69+/-14), the RVEDV is 228 ml (normal range 110+/-24), the RV end systolic volume index is 59 ml/m2 (normal range 22+/-8), and the RVESV is 119 ml (normal range 35+/-13). No significant difference compared to the CMR of 2013. Shunts. An ASD and VSD patch are noted. On first pass contrast imaging, there is no evidence of right to left or left to right shunting. There is evidence of a muscular VSD which has "spontaneously" closed. No evidence of Fontan anatomy. No significant difference compared to the CMR of 2013. Left AtriumThe left atrium is mild to moderately dilated (112ml). The left-sided SVC (see below) forms a "belt" around the mid-level of the left atrium, separating it into 2 portions. Three right pulmonary veins and two left pulmonary veins drain into the proximal portion of the left atrium and the effectively tunnelled to the distal portion of the left atrium. No significant difference compared to the CMR of 2013. Right AtriumThe right atrium is severely enlarged (136ml). The right atrium directly receives drainage from the right-sided SVC, left-sided SVC, coronary sinus, and a single IVC. The right-sided SVC has a stenosis with a minimum diameter of 10mm, just before entering the right atrium. No significant difference compared to the CMR of 2013. Aortic ValveThe aortic valve opens widely and there is no significant aortic regurgitation.Pulmonic ValveThe pulmonic valve opens widely. There is no significant pulmonic regurgitation.Atrio-Ventricular valvesIt appears that there may have been a single over-riding Atrio-Ventricular valve, which was then reconstructed into two separate AV valves, with portions of the previous common Atrio-Ventricular valve indistinguishable from the VSD patch.The mitral valve is opens widely and has moderate to severe MR. The mitral valve leaflets are thickened.The tricuspid valve annulus is hypoplastic (diameter 29x35mm). The tricuspid valve opens widely. There is mild tricuspid regurgitation.AortaThere is a left sided aortic arch with a normal brachiocephalic branching pattern. The maximum diameter of the ascending aorta is 20mm. There is no thoracic aortic aneurysm or thoracic aortic dissection. There is no coarctation of the aorta. Pulmonary Arteries The main pulmonary artery has an upper normal diameter (27mm). There are no proximal stenoses of the left and right pulmonary arteries; however, their caliber is small. The left pulmonary artery measures 10mm. The right pulmonary artery measures 10mm.PericardiumThere is no pericardial effusion or enhancement.Extracardiac FindingsThere is a midline sternotomy. Stable segmentation defect (butterfly vertebrae) of a mid thoracic vertebral body. The spleen measures 13 mm in craniocaudal dimension, upper limits of normal, stable.
1. No evidence of Fontan circulation. No evidence of residual ASD or VSD. No significant difference compared to the CMR of 2013.2. The left ventricle is mildly dilated with mildly reduced systolic function (LVEF 49%). There is hypokinesis of the apical lateral wall which is also thinned and associated with prominent trabeculation. There is no underlying myocardial fibrosis, inflammation, or infiltration. No significant difference compared to the CMR of 2013.3. The right ventricle is mildly dilated with mildly reduced systolic function (RVEF 48%). There is abnormal motion of the apical free wall.4. There is a persistent left-sided SVC which drains directly into the right atrium and forms a tightened "belt" around the mid level of the left atrium. No significant difference compared to the CMR of 2013.5. The right-sided SVC has a stenosis with a minimum diameter of 10mm, just before entering the right atrium. No significant difference compared to the CMR of 2013.6. The right atrium is severely dilated. No significant difference compared to the CMR of 2013.7. It appears that there may have been a single over-riding AV valve, which was then reconstructed into two separate AV valves. No significant difference compared to the CMR of 2013. 8. The mitral valve leaflets are thickened and somewhat distorted and there is moderate to severe mitral regurgitation. No significant difference compared to the CMR of 2013.9. The tricuspid valve annulus is hypoplastic (diameter 29x35mm). No significant difference compared to the CMR of 2013.10. The main pulmonary artery has an upper normal, the left and right pulmonary arteries are relatively small in caliber.I personally reviewed the Images and/or procedure with the Resident/Fellow and agree with this report.