34 year old male with a history of D-transposition of the great vessels s/p Mustard procedure at 18 months, sinus node dysfunction, recurrent SVT, s/p CTI for atrial flutter who was admitted for persistent junctional rhythm and atypical chest pain. He was referred for cardiac MRI for further evaluation. Overall AnatomyD-TGA. There is a Mustard baffle that connects the SVC and IVC to the sub-pulmonic ventricle (i.e. morphologic left ventricle) via the mitral valve. The morphologic left ventricle communicates with main pulmonary artery. The pulmonary venous flow returns to the systemic ventricle (i.e. morphologic right ventricle) via the common atrium that surrounds the baffle via the tricuspid valve. The morphologic right ventricle communicates with the aorta.Systemic Venous ReturnThere is a baffle that connects the SVC and IVC to the sub-pulmonic ventricle (i.e. morphologic left ventricle) via the mitral valve. There is significant tubular narrowing within the the SVC and IVC baffle limbs. The SVC limb is ~8 mm at its narrowest transverse dimension. The IVC limb is ~10 mm at its narrowest transverse dimension. The common portion of the baffle limb is ~6 mm at its narrowest dimension. There is no evidence of flow acceleration within the baffle. The IVC is dilated. The SVC is normal in size. There is no obvious baffle leak noted. Systemic Ventricle (i.e. Morphologic Right Ventricle)The right ventricle is significantly dilated and hypertrophied with moderate to severely reduced systolic function. The overall RV ejection fraction is 30%, the RV end diastolic volume index is 113 ml/m2 (normal range 82+/-16), the RVEDV is 206 ml (normal range 142+/-31), the RV end systolic volume index is 79 ml/m2 (normal range 31+/-9), and the RVESV is 62 ml (normal range 54+/-17). There is late gadolinium enhancement noted at the anterior and inferior insertion points of the right ventricle. Pulmonary Venous ReturnThere are four pulmonary veins which drain into the common atrium and then through the tricuspid valve. There are no obvious obstructions of the pulmonary veins.Sub-Pulmonary Ventricle (i.e. Morphologic Left Ventricle)The left ventricle is normal in size with normal systolic function. The overall LV ejection fraction is 63%, the LV end diastolic volume index is 58 ml/m2 (normal range: 74+/-15), the LVEDV is 106 ml (normal range 142+/-34), the LV end systolic volume index is 22 ml/m2 (normal range 25+/-9), the LVESV is 40 ml (normal range 47+/-19). The interventricular septum bows into the sub-pulmonic left ventricle causing systolic anterior motion of the mitral valve leaflets and flow acceleration in the outflow tract below the pulmonary valve. Common AtriumThe interatrial septum has been resected resulting in a common atrium. The common atrium is significantly dilated.Aortic ValveThe aortic valve opens widely and there is no significant aortic regurgitation.Mitral ValveThe mitral valve opens widely and there is mild mitral regurgitation. There is systolic anterior motion of the mitral leaflets causing sub-pulmonic outflow obstruction as described above. Pulmonic ValveThe pulmonic valve opens widely. There is no significant pulmonic regurgitation.Tricuspid ValveThe tricuspid valve opens widely. There is moderate tricuspid regurgitation.Great VesselsThe aorta is located anterior and to the right of the pulmonary trunk. There is a left sided aortic arch with a normal brachiocephalic branching pattern. There is no thoracic aortic aneurysm or thoracic aortic dissection. PericardiumThere is no obvious pericardial disease.Extracardiac FindingsThis 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. D-Transposition of the Great Vessels status post Mustard atrial switch procedure. 2. There is significant narrowing within the SVC and IVC baffle. No obvious baffle leaks were detected.3. Significantly dilated and hypertrophied systemic right ventricle. RV systolic function is moderate to severely reduced (RVEF 30%).4. The subpulmonic ventricle (left ventricle) is normal in size with normal systolic function (LVEF 63%). 5. The interventricular septum bows into the sub-pulmonic left ventricle causing systolic anterior motion of the mitral valve leaflets and flow acceleration in the outflow tract below the pulmonary valve.6. Moderate tricuspid regurgitation associated with significant atrial dilation. 7. IVC is dilated. When compared to the prior study, the systemic ventricle has a slightly lower ejection fraction.I personally reviewed the Images and/or procedure with the Resident/Fellow and agree with this report.