15 year old female with a history of pulmonary atresia with ventricular septal defect and major aorto-pulmonary artery collaterals, status post unifocalization and right ventricle to pulmonary artery conduit. Referred for a limited non-contrast cardiac MRI to evaluate right ventricular volume and pulmonary regurgitant fraction as part of a research protocol. Systemic Venous AnatomyThe SVC and IVC are normal in size and drain normally into the right atrium. Right AtriumThe right atrium is mildly dilated. Tricuspid ValveThe tricuspid valve opens widely. There is mild tricuspid regurgitation.Right VentricleThe right ventricle is severely dilated with mildly reduced systolic function. There is systolic flattening of the interventricular septum, suggesting elevated right ventricular systolic pressure. The overall RV ejection fraction is 41%, the RV end diastolic volume index is 137 ml/m2 (normal range 69+/-14), the RVEDV is 196 ml (normal range 110+/-24), the RV end systolic volume index is 81 ml/m2 (normal range 22+/-8), and the RVESV is 117 ml (normal range 35+/-13). Pulmonic ValveThere is a prosthetic pulmonary valve with mild insufficiency. Pulmonary valve regurgitant fraction is 26%. Peak velocity through the pulmonary valve is 2.7 m/s, suggesting a peak pressure gradient of 29 mmHg.Pulmonary ArteryThere is a valved conduit from the right ventricle to the pulmonary artery: 35 x 31mmThe right pulmonary artery has long segment distal stenosis, with narrowest dimension 8 x 5 mm. The right pulmonary artery receives approximately 50% of forward flow.The left pulmonary artery is normal in size, measuring 11 x 16mm, and receives approximately 50% of forward flow.Pulmonary VeinsAll four pulmonary veins drain normally into the left atrium.Left AtriumThe left atrium is normal in size. Mitral ValveThe mitral valve opens widely and there is no significant mitral regurgitation.Left VentricleThe left ventricle is normal in size and systolic function. The overall LV ejection fraction is 58%, the LV end diastolic volume index is 84 ml/m2 (normal range: 65+/-11), the LVEDV is 120 ml (normal range 109+/-23), the LV end systolic volume index is 35 ml/m2 (normal range 18+/-5), the LVESV is 50 ml (normal range 31+/-10), the LV mass index is 45 g/m2 (normal range 67+/-11), and the LV mass is 64 g (normal range 114+/-24). There are no regional wall motion abnormalities present. No intracardiac thrombus.Aortic ValveThe aortic valve opens widely and there is mild aortic regurgitation. The aortic valve overrides a VSD which is closed by patch technique. AortaAortic root is mild to moderately dilated. Left aortic arch with normal brachiocephalic branching pattern.Aortic Valve: 25 x 25 mm, Z-score +3.5Aortic Sinuses: 29 x 33 mm, Z-score +2.5ST Junction: 31 x 31 mm, Z-score +3.5PericardiumThere is no pericardial effusion.
1. Right ventricle is severely dilated with mildly reduced systolic function. RVEF 41%. RVEDV indexed 137 mL/m2.2. Normal left ventricular size and systolic function. LVEF 58%.3. Systolic flattening of the interventricular septum, suggesting elevated RV systolic pressure.4. Mild regurgitation of the prosthetic valve. Regurgitant fraction 26%.5. Long segment distal narrowing of the right pulmonary artery (8 x 5 mm)6. Mild to moderate aortic root dilation7. Mild aortic regurgitation.8. Mild mitral regurgitation.9. Mild tricuspid regurgitation.