Evaluation of Cardiac Masses

1. Anne-Marie Anagnostopoulos, MD Non-Invasive Conference April 8, 2009 Evaluation of Cardiac Masses

2. Outline • Clinical Presentation • Echocardiographic Evaluation and Normal Variants • Primary Cardiac Tumors • Metastatic Disease in the Heart • Cardiac Thrombus • Summary

3. Presentation • Cardiac tumors are often misdiagnosed because they are rare • Examples of confusion include: RHD, endocarditis, myocarditis, pulmonary embolism, PHTN, vasculitis • Can present with heart failure, arrhythmia, or embolic phenomena

4. Presentation • Heart Failure: Due to obstruction of outflow tract or cavity filling or dysfunction due to myocardial involvement • Arrythmias: More often occur with intramural involvement; SVT’s with atrial masses, PVC/VT/VF with ventricular myocardial involvement and conduction problems with AV node involvement • Emboli: Right and left sided phenomena

5. Normal Variants on Echo • Many benign findings on echo often misinterpreted as pathologic • Chiari network, Eustatian valve, Catheters, crista terminalis • Suture line, coronary sinus, moderator band, muscle bundles • False chords, trabeculations, Brachiocephalic vein, pleural effusion • Other non-cardiac findings

6. Eustatian Valve

7. Chiari Network

8. Primary Cardiac Tumors • The vast majority are benign – 75% • In an autopsy series, incidence was only found to be 0.02 % • TTE can identify masses/tumors accurately and is useful in follow up • CT can define myocardial infiltration, calcification and surrounding structures • Cardiac MRI offers the best soft tissue characterization and correlates well with pathological findings • T1 images good for soft tissue, T2 for tissue contrast and fluid components (useful for heterogeneous masses) • Can suppress fat signals (useful for lipomas) • Gadolinium enhancement can define myocardial infiltration, vascularity of mass, and differentiate between mass and thrombus

9. Benign Primary Cardiac Tumors Braunwald’s, 7th Edition, page 1746

10. Cardiac Myxomas • 75% are found in Left Atrium • Site of attachment almost always the limbus of the fossa ovalis • 15-20% in the right atrium, less often in right and left ventricles • 90% are solitary, average size 5-6cm (range 1-15 cm) • Average age of presentation is 50 years old

11. Cardiac Myxomas – Echo Features • Mobile Tumor • Narrow Stalk connected to fossa ovalis • Heterogenous with hypo/hyper-echoic foci • Lucent areas and areas of calcification • If appearance is typical, TTE is diagnostic • TEE and 3D echo can supplement characterization of myxomas

12. Cardiac Myxoma - TTE

13. Cardiac Myxoma - TEE

14. Cardiac Myxoma- 3D echo

15. Cardiac Myxoma

16. Cardiac Myxomas – CT and MRI Features • Contrast enhanced CT: usually demonstrates well defined mass with lobular contours that does not enhance • CMR findings of Heterogeneous mass with heterogeneous enhancement • Primarily isointense on T1, and hyperintense on T2 images

18. Cardiac Myxomas - Treatment • Treatment is surgical with en bloc resection including rim of septum around base • Recurrence in about 1-5% of cases (incomplete resection, implantation from first tumor etc) - therefore annual surveillance recommended • In the familial Carney complex (combination of myxomas, pigmented skin lesions, and endocrine neoplasia)– risk of recurrence 12-22%

19. Cardiac Myxomas

20. Papillary Fibroelastomas • Benign papilloma of endocardium • Average age of detection is 60 years old • Found equally in men and women • Many are clinically silent but can result in emboli

21. Papillary Fibroelastoma – Echo Features • 90% are single, with median diameter of 8mm • Most commonly found on downstream side of valves (can be confused for vegetations) • Less common locations: Papillary muscle, chordae tendenae or atria • Irregularly shaped with delicate frond-like surface • Mobility is common and risk factor for embolization • Valvular regurgitation is rare • Controversial if they are distinct from Lambl’s excrescences (acellular deposits covered by endothelium on valves, often at closure margins) • Because of small size – difficult to see on CT or MRI

22. Papillary Fibroelastoma – TTE

23. Papillary Fibroelastoma - TEE

24. ? MRI PF CMR same patient

25. CMR same patient

26. Papillary Fibroelastoma – Less Common Site

27. Papillary Fibroelastoma –Treatment • Most recommend resection, especially for left sided lesions • Risk of embolism can be up to 25% over 3 years and 6% in asymptomatic patients in whom the fibroelastoma was found incidentally • Surgery can usually be valve-sparing • Recurrences have not been reported

28. Papillary Fibroelastoma

29. Cardiac Lipomas • Uncommon benign tumor, usually small and found on epicardial surface • True lipomas are rare, more often present as lipomatous hypertrophy of the interatrial septum • Highly echogenic • Usually present in inferior and superior portions of the septum with sparing of fossa ovalis  “dumbell-shaped” • Associated with atrial arrhythmias • No enhacement on MRI, decreased signal with fat suppression • True lipomas  resection • Lipomatous hypertrophy  surgery only if SVC obstructed or significant arrhythmias

30. Cardiac Lipoma – CMR Imaging After fat suppression turned on:

31. Lipomatous Hypertrophy of Interatrial Septum

32. Lipomatous Hypertrophy of Interatrial Septum

33. Rhabdomyomas and Fibromas • Most common cardiac tumor in children • Rhabdomyomas occur within a cavity or embedded within myocardium, usual small and multiple; often regress on own • Fibromas are well-demarcated, echogenic masses that can extend into cavity and result in obstruction and arrhythmia; often found in free wall of LV • On MRI rhabomyomas are hyperintense on T2, while fibromas are hypointense on T2 and iso-intense after gadolinium

34. Rhabdomyomas and Fibromas

35. Cardiac Fibroma

36. Malignant Primary Cardiac Tumors Braunwald’s, 7th Edition, page 1746

37. Malignant Primary Cardiac Tumors – Echo Assessment • Much less common than metastatic disease • Malignant tumors tend to invade/replace myocardial tissue with disruption of normal anatomy • Heart can appear teathered • Associated pericardial effusion is common • Angiosarcoma often involves right atrium • Rhabdomyosarcoma can occur anywhere

38. Cardiac Angiosarcoma • No consensus on treatment • Surgery, chemotherapy and radiation have been used • Prognosis is poor – survival about 1 year after diagnosis

39. Malignant Cardiac Tumors – CT and MRI assessment • Angiosarcoma on CT: low attenuation, irregular or nodular with contrast enhacement • Angiosarcoma on MRI: heterogeneous signal intensity on T2 images due to blood filled spaces in neoplasm; heterogeneous enhancement with gadolinium; late enhancement due to fibrosis

40. Angiosarcoma on MRI T2 weighted image

41. Primary Cardiac Lymphoma • Rare, especially in immunocompetent patients • Median age of presentation is 64 years old, 3:1 male:female • Often aggressive B-cell lymphomas associated with EBV • Typically present with right sided heart failure, fever, arrhythmias, tamponade • Most commonly arises from Right atrium and half have pericardial effusions (often large) • TTE only moderate sensitivity, MRI has best sensitivity; biopsy is diagnostic • Survival approximately 1 year, with chemotherapy treatment

42. Cardiac Lymphoma - TTE

43. Cardiac Lymphoma - TEE

44. Cardiac Lymphoma - TEE

45. Cardiac Lymphoma – CT scan

46. Cardiac Lymphoma - CMR

47. Cardiac Tumor Imaging Braunwald’s 7th Edition

48. Metastatic Disease to the Heart • Metastases can manifest in the heart as a mass, pericardial disease, myocardial involvement • Tumors can spread to heart by: direct invasion, spread through venous system or hematongenously • Cardiac involvement is often established at autopsy in patients with otherwise widely metastatic disease

49. Metastatic Disease to the Heart

50. Metastatic Melanoma • Metastasizes to myocardium or pericardium and involves the heart 50% of the time • Often presents as intracardiac mass • Best visualized on TTE after contrast injection • Differentiated from thrombus by intact apical wall motion