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

Anne-Marie Anagnostopoulos, MDNon-Invasive ConferenceApril 8, 2009

Outline

Clinical Presentation Echocardiographic Evaluation and

Normal Variants Primary Cardiac Tumors Metastatic Disease in the Heart Cardiac Thrombus Summary

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

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

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

Eustatian Valve

Chiari Network

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

Benign Primary Cardiac Tumors

Braunwald’s, 7th Edition, page 1746

Cardiac Myxomas

75% are found in Left Atrium Site of attachment almost alwaysthe 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

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

Cardiac Myxoma - TTE

Cardiac Myxoma - TEE

Cardiac Myxoma- 3D echo

Cardiac Myxoma

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

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%

Cardiac Myxomas

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

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

Papillary Fibroelastoma – TTE

Papillary Fibroelastoma - TEE

? MRI PF

CMR same patient

CMR same patient

Papillary Fibroelastoma – Less Common Site

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

Papillary Fibroelastoma

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

Cardiac Lipoma – CMR Imaging

After fat suppression turned on:

Lipomatous Hypertrophy of Interatrial Septum

Lipomatous Hypertrophy of Interatrial Septum

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

Rhabdomyomas and Fibromas

Cardiac Fibroma

Malignant Primary Cardiac Tumors

Braunwald’s, 7th Edition, page 1746

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

Cardiac Angiosarcoma

No consensus on treatment

Surgery, chemotherapy and radiation have been used

Prognosis is poor – survival about 1 year after diagnosis

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

Angiosarcoma on MRI

T2 weighted image

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

Cardiac Lymphoma - TTE

Cardiac Lymphoma - TEE

Cardiac Lymphoma - TEE

Cardiac Lymphoma – CT scan

Cardiac Lymphoma - CMR

Cardiac Tumor Imaging

Braunwald’s 7th Edition

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

Metastatic Disease to the HeartPrimary 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

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

Metastatic Melanoma

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

Metastatic Renal Cell Carcinoma

Metastatic Renal Cell Carcinoma

Metastatic Renal Cell Carcinoma

CMR – Renal Cell Carcinoma

CMR – Renal Cell Carcinoma

Metastasis by Direct Extension: Lung Cancer Common

Metastatic Lymphoma

CT Scan CMR

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

Metastatic Carcinoid

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)

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

LV Thrombus – Echo Features Sensitivity of TTE to detect LV thrombus

is 75-95% 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

LV Thrombus - TTE

LV Thrombus – TTE with contrast

LV thrombus

Multiple Intracardiac Thrombi

LV thrombus on CMR

LV Thrombus on Delayed Enhancement Imaging - CMR

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

LA Appendage Thrombus

LA Thrombus

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

References

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