anne-marie anagnostopoulos, md non-invasive conference april 8, 2009
TRANSCRIPT
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