Cardiovascular Cases 26-50

1. Cardiovascular Cases 26-50

2. Case directory

3. Case 26

4. Case directory Scimitar syndrome • Components of scimitar syndrome: • PAPVR (infracardiac) from the right lung to the IVC • Hypoplasia of right lung with dextroposition of heart; • Hypoplasia of right pulmonary artery • Systemic arterial supply of the right lower lobe from the abdominal aorta • Not all components need to be present

5. Case 27

6. Case directory Aortic insufficiency • Case findings: • Flow jet emanating from the aortic root into the left ventricle represents aortic regurgitation • No flow jet into aortic root to suggest aortic stenosis

7. Case 28

8. Transposition of great vessels (TGV) • Case findings: • MR: aorta is anterior right of pulmonary artery • Complete TGV: • Cardiac chambers are in normal position • Connection between left and right heart (VSD, ASD) necessary for life • Congenitally corrected transposition • Cardiac chambers are also switched (compatible with life) • Aorta is located on the left and arises from RV • RV lies posterior and to the left of the LV • Anatomic right ventricle • Triangular configuration • Muscular band around the outflow tract • Coarse trabecular pattern of the apical septum • Moderator band at its apex • Arteriovenous valve and semilunar valve separated by muscle • Jatene (arterial switch): pulmonary artery straddles aorta on axial images • Mustard/Senning: atrial baffle

9. Case directory Jatene (arterial switch) for TGV

10. Case 29

11. Case directory Tricuspid atresia • Case findings: • Solid bar of fat and muscle between dilated RA and hypoplastic RV • Large secundum-type ASD • RV outflow tract stenosis, infundibular stenosis (not shown) • Tricuspid valve and inflow portion of RV are absent • Hypoplastic RV consists of conus portion, which appears as an outpouching of LV • Associated with ventricular septal defect, pulmonary stenosis, and pulmonary atresia • Classified by associated cardiac anomalies: • Normally related great arteries (70%) • TGV (25%) • Corrected TGV (5%) • Treated with Fontan and Glenn procedures to bypass the RV

12. Case 30

13. PAPVR • Case findings: • MIP: drainage of the right upper lobe pulmonary vein into SVC • Types: • Supracardiac: draining into SVC, brachiocephalic vein, or azygous vein • Cardiac: draining into RA or coronary sinus • Infracardiac: draining into IVC • Called scimitar syndrome • Associated with hypogenetic right lung • MC is right upper pulmonary vein draining into SVC • Associated with sinus venosus ASD

14. Case directory PAPVR (supracardiac) Left upper lobe pulmonary vein (arrow) into a left vertical vein, which drains into the left brachiocephalic vein

15. Case 31

17. Polysplenia syndrome • Case findings: • XR: • Ambiguous position of the liver • Dextroposition of the heart • MR: • Situs ambiguous with the liver on both the right and left side • At least two small spleens • Stomach is on the right • Persistent left SVC drains into the pulmonary venous atrium • Not shown: • Interruption of the IVC with azygous continuation • Aorta and pulmonary artery arise from a common single ventricle • Bilateral pulmonary arteries are seen passing over bilateral bronchi consistent with bilateral left-sidedness

18. Case directory Polysplenia syndrome • Bilateral left-sidedness with bilateral hyparterial bronchi • Situs ambiguous, multiple small spleens • Cardiac manifestations: • Interruption of the IVC with azygous continuation (70%) • Persistent left SVC (50%) • Partial or total anomalous pulmonary venous connection (50%) • Atrial septal defect (80%) • Ventricular septal defect (70%) • Single ventricle (5%) • Malposition of the great arteries (30%)

19. Case 32 Axial SE T1 Axial SE T1 FS post Gd

20. Pericardial hematoma • Case findings: • Mass in the left atrioventricular groove, compressing the LA and LV • Acute angle of pericardial interface with the mass, indicating an intrapericardial location • Signal is intermediate T1, without enhancement • Not shown: • Dark foci internally and dark rim on GRE images (central and peripheral calcifications) • Low T2

21. Pericardial hematoma • DDX pericardial mass: • MC pericardial hematoma • Pseudoaneurysm • Pericardial cyst • Low T1, homogeneous high T2, no enhancement • Neoplasm

22. Case directory Pericardial hematoma Pericardial hematoma with heterogeneous SI was located in right atrioventricular groove

23. Case 33 Coronal SE T1 Coronal SE T1 with Gd

24. Lymphoma • Case findings: • Mediastinal mass that encases the distal trachea and bilateral mainstem bronchi • Mass invades the roof of the left atrium • Enhances homogeneously • DDX: • Thrombus • Thrombus is darker on GRE sequences than muscle • Tumor on GRE cine images is similar to, or higher than, the myocardium • Tumor enhances with gadolinium, whereas clot does not • Angiosarcoma • MC primary cardiac tumor (MC in RA) • Unlikely to be centered around the carina • Fibrosing mediastinitis: should not invade the heart • Metastasis: MC small cell carcinoma of the lung

25. Case directory Lymphoma (different patient)

26. Case 34 Axial SE T2 Coronal SE T1 with Gd

27. Coronal SE T1 with Gd and FS

28. Case directory Atrial lipoma • Case findings: • Homogeneously high T2 mass in LA • Mass intrinsically bright on T1 (not shown), and does not enhance after gadolinium • SI of mass homogeneously reduced with FS • MC benign primary tumor of the heart • MC in RA (this case was a LA lipoma)

29. Case 35

30. Case directory Angiosarcoma • Case findings: • Focally enlarged and lobulated interventricular septum mass • Mass demonstrates enhancement • DDX of thickened interventricular septum: • Asymmetric hypertrophic cardiomyopathy • MC malignant primary intracardiac tumor • Other primary malignant tumors: • Rhabdomyosarcoma • Leiomyosarcoma • Liposarcoma • Lymphoma

31. Case 36 Axial SE T1 and T2

32. Axial SE T1 with Gd

33. Case directory Pericardial cyst • Case findings: • Cystic mass conforming to the contour of the heart and the main pulmonary artery • Low T1, high T2, no enhancement • Benign developmental lesion formed when part of the embryonic percardium is pinched off • MC found in the right anterior cardiophrenic angle behind the right atrium • DDX of benign mediastinal cyst: • Bronchogenic cyst • Thymic cyst • Pericardial cyst: cyst conforms to the contour of other mediastinal structures

34. Case 37 Axial SE T1

35. Constrictive pericarditis Pericardial fat Epicardial (subpericardial) fat

36. Constrictive pericarditis • Case findings: • Pericardium is thickened over the RA and TV • Pericardium is clearly identified between two layers of fat (pericardial fat and epicardial/subpericardial fat) • Etiology: • Cardiac surgery, radiation therapy • Tuberculosis, post-viral pericarditis • Collagen vascular disease • Infiltration of the pericardium by neoplasm

37. Case directory Constrictive pericarditis • Clinically difficult to differentiate between constrictive pericarditis and restrictive cardiomyopathy • Patients with constrictive pericarditis may benefit from pericardiodectomy whereas those with restrictive cardiomyopathy would not • Restrictive cardiomyopathy • Pericardium is of normal thickness (< 2 mm) • Constrictive pericarditis • Pericardium is > 4 mm thick • DDX thickened pericardium (use clinical history to help diagnose) • Constrictive pericarditis • After cardiac surgery • Uremic pericarditis • Pericardial effusion

38. Case 38

39. Axial SE T1 Axial SE T1 with Gd and FS

40. Enhancing and thickened pericardium Pericardial effusion in pericardial space

41. Case directory Tuberculous pericarditis • Uncommon cause of pericarditis • Features: • Thickening of the pericardium and/or pericardial effusion • Enhancement of the pericardium seen in tuberculous pericarditis and may be due to granulation tissue in the pericardium

42. Case 39

43. Left ventricular true aneurysm (arrow)

44. Ventricular aneurysm • Case findings: • Pericardial effusion with high T1 (serosanguinous) • Left ventricular true aneurysm • True ventricular aneurysm • Large segment of nonviable myocardium • Focal wall thinning and is deformed during diastole and dilates during systole • MC located in the anterolateral and apical aspect of LV, wide ostium • False ventricular aneurysm • Cavity that communicates with ventricular lumen, but not formed by myocardium • Results from a contained rupture of LV after myocardial infarction • MC in posterior and diaphragmatic aspect of LV, narrow ostium

45. Case directory False ventricular aneurysm

46. Case 40

47. Case directory Arrhythmogenic right ventricular dysplasia (ARVD) • Case findings: • High T1 in myocardium of RV anterior free wall, corresponding to fatty infiltration • NB: signal from the posterior portion of the heart was suppressed with a posterior saturation band to reduce artifact • Right ventricular dysplasia • Results from replacement RV myocardium with fat or LC fibrous tissue • Results in right ventricular arrhythmias, which may be provoked by exercise and complicated by syncope or sudden cardiovascular collapse • MC men at a young or middle age • DDX: • Right ventricular outflow tract tachycardia (no evident structural abnormality)

48. Case 41

49. Case 42

50. Case 43