Transcript 4/10 Cardiac Masses

Connie Tsao
Non-invasive Conference
April 7, 2010
Outline

Non-tumors



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
Normal Variants
Catheters
Thrombotic disease
Infective endocarditis
Cardiac tumors
 Epidemiology
 Clinical Manifestations
 Primary Cardiac Tumors
○ Benign
○ Malignant
 Metastatic Tumors
Non-tumors
Normal Variants

Structural variants
 False tendon: fibrous/fibromuscular
 Eustachian valve
 Chiari network

Prosthetic material
 Catheters
 Pacing wires
 Cardiac assist devices
Arrhythmogenic potential?


Series of 15 patients
with idiopathic LV
tachycardia vs.
controls referred for
echo
All ILVT had false
tendon from IL wallseptum
 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
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Associated with:
 Lower BMI
 Innocent murmur
 ECG-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 2001
Schuchlenz HW, JASE 2004
Chiari Network


Hans Chiari, 1897:
11 pts, fibrous network
in RA
Remnant of right valve
of sinus venosus
 Directed 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
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PFO present in:
 24/29 (83%) with Chiari
 44/160 (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 thrombi
 Post-MI
 Significant 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
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Pathologic correlation in 8 pts, LV thrombus in 5
 All 5 detected by LGE-CMR
 2 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-CMR
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 Larger infarcts, aneurysm, lower LVEF
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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
Primary cardiac tumors
Majority (>75%) are benign
 Rare; incidence of <0.001-0.03% in
autopsy studies

Primary Benign Tumors
Braunwald’s Heart Disease, 7th Ed.
Classic Triad of Symptoms

Intracardiac obstruction:
 Dyspnea, orthopnea, pulmonary edema
 Presyncope/syncope
 Angina, claudication

Systemic embolization:
 CVA, retinal artery emboli
 Emboli 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
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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
loss

 Anemia, elevated ESR, leukocytosis
 ↑IL-6, inflammatory factors
Imaging

Echo
 Prolapsing mass across MV/TV
 Identification of point of attachment

CMR
 Heterogeneous appearance on T1W, T2W
images
 Patchy LGE

CT
 Low attenuation mass, no enhancement
 Calcification in 10-15%
T1W post gadolinium
T2W
58 year old man with dyspnea
Treatment

Resection
 Including surrounding septum at attachment
Surgical mortality <5%
 Risk for atrial arrhythmias
 Recurrence in 2-5%
 Recurrence in Carney complex 12-22%
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Papillary Fibroelastoma
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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 thrombus
 CVA/TIA
 PE
 Peripheral 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
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 Well-circumscribed nodule on T1W, T2W
 LGE reported

Distinction from vegetation
 No significant valvular regurgitation
 Location 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:
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 Any embolic events
 Highly mobile
 >1 cm

No recurrences known
Sun JP et al, Circ 2001
Lipoma
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
Slow-growing
Mature adipose tissue
Sub-endocardial (50%)
 Broad based attachment
 Growth into adjacent chambers
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Myocardial (25%)
Sub-epicardial (25%)
 Narrow attachment point
 Growth into pericardial space
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Valvular attachment rare
Lipomatous hypertrophy of IAS
 Older, obese
 Associated 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
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 Bright on T1W + T2W images
 Uniform suppression by fat sat
 No soft tissue component/ LGE
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CT
 Homogenous 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
children
 Most <1 year of age
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80-90% association with tuberous
sclerosis
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Most regress spontaneously
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Arrhythmias
 Heart 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
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CMR:
 Low signal on T1W, T2W
 Hypovascular on 1st pass perfusion
 Homogeneous on LGE

CT
 Mildly enhancing
 Up to 50% calcification
32 yo F with recurrent syncope, VT
T1W BB
SSFP
T2W BB
LGE
Hoey ED et al, Clin Radiol 2009
Primary Malignant Tumors
Braunwald’s Heart Disease, 7th Ed.
Overview


Overall 15% of primary cardiac tumors
Sarcomas most common
 Angiosarcoma
 Sarcomas with myo- or fibroblastic differentiation
 Rhabdomyosarcoma
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Suggestive imaging findings:


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Right-sided
Broad-based attachment
Ill-defined margins
Tissue inhomogeneity/ heterogeneous contrast
enhancement
 Size >5 cm
 Pericardial 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 failure
 Tamponade

Metastases in 66-89%  lungs/brain/bone/liver
Imaging
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CMR
 T1 isointense, T2 hyperintense
 Flow voids = vascular channels
 Prominent LGE “sunray appearance”

CT
 Low attenuation/ irregular
 Heterogenous enhancement
25 year old woman with dyspnea
T1W BB
T2W, fat suppression
Hoey ED et al, Clin Radiol 2009
T1W BB
LGE
O’Donnell DH et al, Am J Roentol 2009
63 year old man with chest pain
Treatment

Resection + chemotherapy
 ↑ survival with complete resection

Transplantation
 Sarcoma in 15/21 malignancies
 Mean survival 12 months
 7 patients with mean survival 27 mos
Gowdamarajan A et al, Curr Opin Cardiol 2000;

Autotransplantation
 8 sarcomas resected
○ 7 atrial, 1 ventricular
 Median survival 18.5
mos
Reardon MJ et al, Ann Thorac Surg 1999, 2006
Lymphoma

Majority aggressive B-cell lymphomas
 Commonly 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
 CMR

 Isointense on T1W, or hyperintense on T2W
 Heterogeneous enhancement on LGE

CT
 Isointense relative to myocardium
T1W
T2W
T1W LGE
LGE
54 yo F with CP, DOE, palpitations
Metastatic Tumors
Overview
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Up to 12% of oncology pts at autopsy
 Most clinically silent
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
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Most common: lung cancer, melanoma
Pericardial effusion common
Multiple masses suggestive
Imaging characteristics
 Hypointense on T1W (except melanoma:
paramagnetic effect of melanin)
 Hyperintense on T2W
 Enhancement after gadolinium administration
 Soft 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