30 year old woman with complex congenital disease s/p repair as follows: hypoplastic RV and tricuspid and pulmonary artery atresia with Glen shunt, ASD and previous Blalock-Taussig shunt placement transferred here for hemoptysis, now referred for cardiac MRI to better delineate her anatomy. Overall AnatomyHypoplastic right ventricle s/p Glenn shunt. The interatrial septum has been resected with effectively a large ASD present. There is tricuspid valve atresia with hypoplastic right ventricle. The left ventricle is dominant and is the systemic ventricle.Systemic Venous Return (Fontan)A Glenn shunt connects the SVC to a small caliber right pulmonary artery. There are no obstructions or stenoses in either the SVC or IVC. Systemic Ventricle (i.e. Left Ventricle)The left ventricle is severely dilated with moderately reduced systolic function. The overall LV ejection fraction is 40%, the LV end diastolic volume index is 120 ml/m2 (normal range: 65+/-11), the LVEDV is 185 ml (normal range 109+/-23), the LV end systolic volume index is 72 ml/m2 (normal range 18+/-5), the LVESV is 111 ml (normal range 31+/-10), the LV mass index is 53 g/m2 (normal range 67+/-11), and the LV mass is 81 g (normal range 114+/-24). Wall motion is dyssynchronous consistent with the patient's history of congenital heart disease and previous cardiac surgery.Pulmonary Venous ReturnThere are four pulmonary veins which drain into the common atrium. There are no obvious obstructions of the pulmonary veins.Right VentricleThe right ventricle is hypoplastic.Common AtriumThe interatrial septum has been resected resulting in a common atrium. Aortic ValveThe aortic valve is trileaflet. The valve opens widely and there is trace aortic regurgitation.Mitral ValveThe mitral valve opens widely and there is no significant mitral regurgitation.Tricuspid ValveThe tricuspid valve is atretic.AortaThe aorta is located anterior. There is a left sided aortic arch with no left subclavian noted. There is no thoracic aortic aneurysm or thoracic aortic dissection. The maximum diameter of the aorta is 30mm. The sinus of Valsalva measures 36mm. Left and Right Pulmonary ArteryThe main pulmonary artery is not seen. The left pulmonary artery is not seen. The right pulmonary artery is small in caliber and measures 12 mm. PericardiumThere is no obvious pericardial disease.Extracardiac FindingsLarge intraabdominal mass with associated liver metastases. Left lower lobe lung changes consistent with bronchiectasis. There is peripheral enhancement of the lung fields on MRA consistent with extensive chest wall collateralization. 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. Hypoplastic RV with tricuspid and pulmonary artery atresia. 2. Severely dilated LV (systemic ventricle) with moderately reduced EF of 40%. Wall motion is dyssynchronous. 3. Glen shunt intact (SVC to RPA). 4. Extensive chest wall collateralization. 5. Large intraabdominal mass with liver metastases. 