Connie Tsao

Non-invasive Conference

April 7, 2010

Outline Non-tumors

Normal VariantsCathetersThrombotic diseaseInfective endocarditis

Cardiac tumorsEpidemiologyClinical ManifestationsPrimary Cardiac Tumors

○ Benign○ Malignant

Metastatic Tumors

Non-tumors

Normal Variants

Structural variantsFalse tendon: fibrous/fibromuscularEustachian valveChiari network

Prosthetic materialCathetersPacing wiresCardiac assist devices

Arrhythmogenic potential? Series of 15 patients

with idiopathic LV tachycardia vs. controls referred for echo

All ILVT had false tendon from IL wall-septum 2/3 of these >2 mm

34/671 (5%) of controls had false tendon• Oriented across LV• <2 mm

Thakur RK, Circ 1996

Epidemiology In FHS Original and Offspring cohort:

101 participants with LV false tendons (2% of population)

Kenchaiah S et al, JASE 2009

Associated with:Lower BMIInnocent murmurECG-LVH

Not associated with ventricular ectopy, or other ECG abnormalities

No excess mortality in 7.7±1.6 yrs follow-up

Kenchaiah S et al, JASE 2009

Eustachian valve

Persistent Eustachian valve Case reports of association

between Eustachian valve and PFO

In 306 pts referred for TEE (211 for cryptogenic CVA): 143/211 (68%) of cryptogenic

stroke group had EV 31/95 (33%) of controls had

EV 70% of pts with EV had PFO

? Effect of flow on increasing patency of PFO

Strotmann JM, Heart 2001Schuchlenz HW, JASE 2004

Chiari Network Hans Chiari, 1897:

11 pts, fibrous network in RA

Remnant of right valve of sinus venosusDirected IVC flow

through fossa ovalis to LA

Incomplete resorption

1-4% in autopsy studies

Chiari network and PFO 1436 pts consecutive pts referred for TEE Prevalence 29/1436 (2%) Chiari network present in:

24/522 (4.6%) referred for paradoxical embolus 5/913 (0.5%) controls

PFO present in: 24/29 (83%) with Chiari44/160 (28%) controls

Significant R-L shunt by agitated saline in 1/3 with Chiari

Schneider B, et al, JACC 1995

Prosthetic Material

Impella

Intracardiac Thrombi Accounts for 15-20% strokes

Major source: LA thrombi (>45% cases)○ LA thrombi detected by TEE:

Acute AF: 14%Chronic AF: 27%AF with clinical thromboembolism: 43%

Other: Aorta, valve prostheses, inter-atrial septum aneurysm

LV thrombiPost-MISignificant LV dysfunction

Stoddard MF et al, JACC 1995; Manning WJ et al, Ann Int Med 1995

LAA masses

LV Thrombus

Same patient, LGE

LV Thrombus: Value of LGE-CMR 784 consecutive pts with LVEF <50% Thrombus detection:

37 (4.7%) by cine-CMR 55 (7%) by LGE-CMR

Pathologic correlation in 8 pts, LV thrombus in 5All 5 detected by LGE-CMR2 detected by cine-CMR

Cine CMR missed small intracavity and mural thrombi

Weinsaft JW et al, JACC 2008

Weinsaft JW et al, JACC 2008

LV Thrombus: Contrast Echo vs CMR

121 pts post MI or clinical heart failure TTE, contrast-TTE, LGE-CMR

LV thrombus in 24 pts by LGE-CMRLarger infarcts, aneurysm, lower LVEF

TTE sensitivity 33%, Contrast TTE: 61%Low LVEF predictor of thrombus detection by CMR

Thrombi detected by DE-CMR vs contrast echo: mural, small apical

Close agreement with contrast echo (k=0.79)

Weinsaft JW et al, JACC Imaging 2009

Asymptomatic 50 year old man

SSFP First pass perfusion

Hoey ED et al, Clin Radiol 2009

Cardiac Tumors

Majority (>75%) are benign Rare; incidence of <0.001-0.03% in

autopsy studies

Primary cardiac tumors

Primary Benign Tumors

Braunwald’s Heart Disease, 7th Ed.

Classic Triad of Symptoms Intracardiac obstruction:

Dyspnea, orthopnea, pulmonary edemaPresyncope/syncopeAngina, claudication

Systemic embolization: CVA, retinal artery emboliEmboli to extremities

Constitutional symptoms: fever, fatigue, weight loss, arthalgia

Myxoma

Mean age 50 years at diagnosis F>M (60-70%) 80% in left atrium, 15% in right atrium

Can occur in ventricles

90% solitary, 7% Carney complex Average size 5-6 cm Attachment to fossa ovalis

Pedunculated, gelatinous

Friable/villous surface (1/3) emboli

Histology:Mesenchymal cells in

mucopolysaccharide stroma

Production of VEGF angiogenesis

Clinical manifestations Factors: size, anatomic location Pulmonary venous or mitral valve

obstruction Stroke/neurologic deficits Systemic embolization Constitutional symptoms: fever, weight

lossAnemia, elevated ESR, leukocytosis↑IL-6, inflammatory factors

Imaging Echo

Prolapsing mass across MV/TVIdentification of point of attachment

CMR Heterogeneous appearance on T1W, T2W

imagesPatchy LGE

CTLow attenuation mass, no enhancement Calcification in 10-15%

T1W post gadolinium

T2W

58 year old man with dyspnea

Treatment

ResectionIncluding surrounding septum at attachment

Surgical mortality <5% Risk for atrial arrhythmias Recurrence in 2-5% Recurrence in Carney complex 12-22%

Papillary Fibroelastoma Incidence 0.002-0.33% in

autopsies Mean age 60 years Mean size 9 mm (2-70 mm) 80-90% on valvular

endocardium, AV 36%> MV 29%> TV 11% > PV 7% Downstream side

Histology: fibromyxoid core, rim of elastic fibers covered by endothelial cells Distinction from Lambl’s

excrescence

Clinical manifestations

Embolization: tumor or thrombusCVA/TIAPEPeripheral embolization

MI, angina Sudden cardiac death Syncope 1/3 of patients asymptomatic

Imaging

TTE can miss due to size CMR not ideal due to high mobility

Well-circumscribed nodule on T1W, T2WLGE reported

Distinction from vegetationNo significant valvular regurgitationLocation away from valvular free edge

29 year old woman with incidentally discovered mass…

Parthenakis F et al, Cardiovasc Ultrasound 2009

Treatment

Observation: small, nonmobile tumors Surgical resection:

Any embolic events Highly mobile>1 cm

No recurrences known

Sun JP et al, Circ 2001

Lipoma Slow-growing Mature adipose tissue Sub-endocardial (50%)

Broad based attachmentGrowth into adjacent chambers

Myocardial (25%) Sub-epicardial (25%)

Narrow attachment pointGrowth into pericardial space

Valvular attachment rare Lipomatous hypertrophy of IAS

Older, obeseAssociated with CAD (Chaowalit N et al, Chest 2007)

Clinical manifestations/Treatment Most asymptomatic

Invasion into tissue arrhythmias, conduction block

↑size obstruction

Resection recommended (continued growth)Lipomatous hypertrophy of IAS: no resection

unless significant clinical sxs

Imaging Echo: variable appearance

Spares fossa ovalis CMR + CT: corresponds to fat signal CMR

Bright on T1W + T2W imagesUniform suppression by fat satNo soft tissue component/ LGE

CTHomogenous fat attenuation

Lipoma

Leu HB et al, Eur Heart J 2004

35 yo woman with AF, mass on TTE

T2W BB T1W BB

Lack of LGE T2W fat sat

Hoey ED et al, Clin Radiol 2009

Rhabdomyoma

Most common primary cardiac tumor in childrenMost <1 year of age

80-90% association with tuberous sclerosis Most regress spontaneously

ArrhythmiasHeart block, VT

Fibroma

2nd most common pediatric cardiac tumor

Fibroblasts interwoven with collagen Arise in myocardial free wall/septum LV:RV 5:1 Heart failure: obstruction, valvular

dysfunction

Fibroma- Imaging

CMR: Low signal on T1W, T2WHypovascular on 1st pass perfusionHomogeneous on LGE

CTMildly enhancing Up to 50% calcification

32 yo F with recurrent syncope, VT

Hoey ED et al, Clin Radiol 2009

T1W BBT2W BB

SSFP LGE

Primary Malignant Tumors

Braunwald’s Heart Disease, 7th Ed.

Overview Overall 15% of primary cardiac tumors Sarcomas most common

AngiosarcomaSarcomas with myo- or fibroblastic differentiationRhabdomyosarcoma

Suggestive imaging findings:Right-sidedBroad-based attachmentIll-defined marginsTissue inhomogeneity/ heterogeneous contrast

enhancementSize >5 cmPericardial effusion

Angiosarcoma

Highly aggressive, anaplastic epithelial cells, vascular channels

M>F, peak incidence in 40s RA involved in 75% RV, pericardium Clinical symptoms

Right heart failureTamponade

Metastases in 66-89% lungs/brain/bone/liver

Imaging

CMRT1 isointense, T2 hyperintenseFlow voids = vascular channelsProminent LGE “sunray appearance”

CTLow attenuation/ irregularHeterogenous enhancement

25 year old woman with dyspnea

T1W BB T2W, fat suppression

Hoey ED et al, Clin Radiol 2009

O’Donnell DH et al, Am J Roentol 2009

T1W BB LGE

63 year old man with chest pain

Treatment

Resection + chemotherapy↑ survival with complete resection

TransplantationSarcoma in 15/21 malignanciesMean survival 12 months7 patients with mean survival 27 mos

Gowdamarajan A et al, Curr Opin Cardiol 2000;

Autotransplantation8 sarcomas resected

○ 7 atrial, 1 ventricularMedian survival 18.5

mos

Reardon MJ et al, Ann Thorac Surg 1999, 2006

Lymphoma

Majority aggressive B-cell lymphomasCommonly in immunocompromised

Disseminated non-Hodgkin’s lymphoma more common

Firm, nodular aggregates of lymphoid tissue

Mean age 38 years Treatment: anthracyclines, monoclonal

anti-CD20 antibody

Imaging

Echo characteristic features: RA, pericardial effusion

CMRIsointense on T1W, or hyperintense on T2WHeterogeneous enhancement on LGE

CTIsointense relative to myocardium

T1W LGET1W

T2W LGE

54 yo F with CP, DOE, palpitations

Metastatic Tumors

Overview Up to 12% of oncology pts at autopsy

Most clinically silent Most common: lung cancer, melanoma Pericardial effusion common Multiple masses suggestive Imaging characteristics

Hypointense on T1W (except melanoma: paramagnetic effect of melanin)

Hyperintense on T2WEnhancement after gadolinium administrationSoft tissue attenuation on CT

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

Braunwald’s Heart Disease, 7th Ed.

Melanoma

Direct Extension Tumors

Lung cancer

Hepatocellular carcinoma

Renal Cell Carcinoma

Braunwald’s Heart Disease, 7th Ed.

Summary Many conditions mimic cardiac masses Primary cardiac tumors are rare and

usually benign Clinical presentation varies by location

and size of mass TTE and CMR with gadolinium helpful to

narrow differential diagnoses Treatment: surgical resection for bulky

tumors/ chemotherapy