1. Evaluation of Cardiac Masses
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 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 %

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
Primary Malignancy
Cardiac Effect
Lung
Direct extension, effusion
Breast
Hematogenous/lymphatic spread, effusion
Lymphoma
Lymphatic spread, variable effects GI
Variable
Melanoma
Intracardiac and myocardial Involvement
Renal Cell Carcinoma
IVC-RA-RV extension, can look like thrombus
Carcinoid
Tricuspid and pulmonic valve abnormalities

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

51. Metastatic Melanoma

52. Metastatic Renal Cell Carcinoma
Commonly spreads by intravascular extension from IVC to RA
RA mass seen on echo can be first presentation and should be distinguished from thrombus or other benign mass
May need supplemental imaging with CT and MRI

53. Metastatic Renal Cell Carcinoma

54. Metastatic Renal Cell Carcinoma

55. Metastatic Renal Cell Carcinoma

56. CMR – Renal Cell Carcinoma

57. CMR – Renal Cell Carcinoma

58. Metastasis by Direct Extension: Lung Cancer Common

59. Metastatic Lymphoma
CT Scan
CMR

60. Metastatic Carcinoid
Tricuspid and pulmonic valves affected by vasoactive substances released by carcinoid tumors when mets present in liver
Results in valve thickening and fibrosis
On echo: the valves can be thick, retracted and immobile
Effect on TV: severe regurgitation
Effect on PV (when involved): stenosis

61. Metastatic Carcinoid

62. Intracardiac Thrombus
Intracardiac source of emboli account for approximately 15-20% of strokes
TEE is imaging modality of choice for evaluation of intracardiac thrombus and source of emboli (except for LV apex)
Major sources: LA (45%), LV apex, aorta, valve prosthesis, abnormal interatrial septum (aneurysm)

63. Imaging Intracardiac Thrombus
Transthoracic Echo with/without contrast – best for LV thrombi associated with aneurysm or akinesis of the apex
TEE – best for all other locations of thrombus
MRI – excellent way to identify thrombus; usually identified on spin echo and gadolinium enhanced images with delayed enhancement

64. LV Thrombus – Echo Features
Sensitivity of TTE to detect LV thrombus is %
Associated with myocardial infarction that results in akinesis of the apex or dilated cardiomyopathy resulting in slow flow
May be multiple, mobile
Texture usually distinct from myocardium
Risk factors for embolism: large size, mobility, and protrusion into LV cavity
TTE used to follow LV thrombi over time

65. LV Thrombus - TTE

66. LV Thrombus – TTE with contrast

67. LV thrombus

68. Multiple Intracardiac Thrombi

69. LV thrombus on CMR

70. LV Thrombus on Delayed Enhancement Imaging - CMR

71. LA Thrombus – Echo Features
LA appendage is most likely site
Associated conditions: Atrial Fibrillation, mitral stenosis, LV failure
The LAA can be multi-lobed in up to 70% of patients
Sensitivity of TEE to detect an LA thrombus approaches 95%, with equally high specificity
TEE evaluates size, mobility, emptying velocity, extension into LA, and interatrial aneurysm if present
Can also assess spontaneous echo contrast

72. LA Appendage Thrombus

73. LA Thrombus

74. Summary Primary Cardiac tumors are rare and usually benign
Clinical presentation based on location and size of mass
Echo (TTE and TEE) remains the initial imaging test
CMR is a useful modality to further characterize intracardiac masses (especially lipomas, angiosarcomas and thrombi) and narrow the differential diagnosis
Treatment usually involves surgery for tumors

75. References Braunwald’s 7th Edition NEJM case records Feigenbaum
Uptodate
Imaging teaching files