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Anne-Marie Anagnostopoulos, MD Non-Invasive Conference April 8, 2009.

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Presentation on theme: "Anne-Marie Anagnostopoulos, MD Non-Invasive Conference April 8, 2009."— Presentation transcript:

1 Anne-Marie Anagnostopoulos, MD Non-Invasive Conference April 8, 2009

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, 7 th 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

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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

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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

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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, 7 th 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

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45 Cardiac Lymphoma – CT scan

46 Cardiac Lymphoma - CMR

47 Cardiac Tumor Imaging Braunwald’s 7 th 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 MalignancyCardiac Effect LungDirect extension, effusion BreastHematogenous/lymphatic spread, effusion LymphomaLymphatic spread, variable effects GIVariable MelanomaIntracardiac and myocardial Involvement Renal Cell CarcinomaIVC-RA-RV extension, can look like thrombus CarcinoidTricuspid 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

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56 CMR – Renal Cell Carcinoma

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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 7 th Edition  NEJM case records  Feigenbaum  Uptodate  Imaging teaching files


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