43 year old female with a VSD, ASD (secundum) and a PDA s/p surgical repair at age 2y, RV dysfunction, s/p supravalvular pulmonic plasty and coiling of an aorto-left pulmonary artery fistula (alternatively a residual PDA) 1/8/14. CMR today for pre-operative evaluation prior to shoulder surgery. Left VentricleThe left ventricle is normal in size with a normal systolic function. The overall LV ejection fraction is 58%, the LV end diastolic volume index is 66 ml/m2 (normal range: 65+/-11), the LVEDV is 122 ml (normal range 109+/-23), the LV end systolic volume index is 28 ml/m2 (normal range 18+/-5), the LVESV is 51 ml (normal range 31+/-10), the LV mass index is 41 g/m2 (normal range 67+/-11), and the LV mass is 75 g (normal range 114+/-24). Ventricular septal patch repair is noted. The presence of residual VSD can not be excluded (see below). There is systolic septal flattening consistent with RV pressure overload. There late gadolinium enhancement of the inferior right ventricular insertion point suggesting the presence of underlying myocardial fibrosis. This pattern is commonly seen in RV pressure overload states such as pulmonary hypertension and congenital heart disease. Left AtriumThe left atrium is normal in size. Right VentricleThe right ventricle is severely dilated with a mildly reduced systolic function. The overall RV ejection fraction is 43%, the RV end diastolic volume index is 129 ml/m2 (normal range 69+/-14), the RVEDV is 238 ml (normal range 110+/-24), the RV end systolic volume index is 74 ml/m2 (normal range 22+/-8), and the RVESV is 102 ml (normal range 35+/-13). There is no obstruction in the RVOT.Right AtriumThe right atrium is severely dilated. Aortic ValveThe aortic valve opens widely and there is no significant aortic regurgitation.Mitral ValveThe mitral valve opens widely and there is no significant mitral regurgitation.Pulmonic ValveThe pulmonic valve opens widely. There is no significant pulmonic regurgitation.Tricuspid ValveThe tricuspid valve opens widely. There is likely moderate tricuspid regurgitation with an eccentric jet; however, the possibility that the jet represents a residual ventricular septal defect can not be completely excluded.AortaThe aortic root is normal in size. There is a left sided aortic arch with a normal brachiocephalic branching pattern.Pulmonary VeinsThe right-sided pulmonary veins form a common antrum and join with the left atrium. The left sided pulmonary veins are not visible.Pulmonary ArteryThe main pulmonary artery is severely dilated (43x37mm). The proximal portion of the main pulmonary artery is narrowed (16mm) but without evidence of significant flow acceleration. The right pulmonary artery is normal in size (17mm). The left pulmonary vein is small in caliber (10mm). Venous AnatomyThe SVC is normal in size and drains normally into the right atrium. The IVC is massively dilated and drains normally into the right atrium. PericardiumThere is no obvious pericardial disease.Extracardiac FindingsThere are diffusely increased interstitial markings in the left lung.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. Normal LV size and systolic function (LVEF 58%) with evidence of fibrosis along the right ventricular inferior insertion point, which is commonly seen in RV pressure overload states.2. Severe RV dilation with mildly reduced systolic function (RVEF 43%). There is systolic flattening of the interventricular septum suggesting that the RV systolic pressure is increased.3. The IVC is massively dilated.4. The left pulmonary artery is significantly diminished in caliber. The left-sided pulmonary veins are not well seen. The left lung is hypoperfused relative to the right lung. There are diffusely increased interstitial markings in the left lung.5. The main pulmonary artery is severely dilated.6. There is likely moderate tricuspid regurgitation with an eccentric jet; however, the possibility that the jet represents a residual ventricular septal defect can not be completely excluded.7. When compared to the prior study, there has been mild improvement in RV function.I personally reviewed the Images and/or procedure with the Resident/Fellow and agree with this report.