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

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 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

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 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

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