Cardiac Mass and Mass-Like Structures

2017-11-16

KSE 2017 Basic Echo Review Course (4) Nov 26, 2017

Cardiac Mass and Mass-like Structures

Sun Hwa Lee, MD, PhD Chonbuk National University Hospital & Medical School

Introduction

✓ Although cardiac tumors are rare, they represent an important group of cardiovascular abnormalities because an early and accurate diagnosis may permit a curative procedure or in some circumstances, may even avoid unnecessary surgery ✓ In addition, echocardiographic evaluation of intracardiac masses is critically dependent on the ability to distinguish normal from abnormal findings

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Cardiac Mass

✓ Cardiac tumors ✓ Non-tumors - Epidemiology - Intracardiac thrombi - Primary benign tumors - Vegetations • Myxoma • Papillary fibroelastoma ✓ Normal variants and • Lipoma benign conditions • Rhabdomyoma • Fibroma - Primary malignant tumors - Metastatic tumors

Cardiac Mass

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Epidemiology

✓ Primary cardiac tumors: 0.001~0.03% in autopsy series ✓ Secondary involvement of the heart by extracardiac tumors is 20-40 times more common than by primary cardiac tumors

Type %

Myxoma 30 Secondary Lipoma 10 Papillary fibroelastoma 8 (90~95%) Benign Rhabdomyoma 6 (70%) Primary Fibroma 3 (5~10%) Hemangioma 2 Teratoma 1

Angiosarcoma 8 Rhabdomyosarcoma 5 Malignant Fibrosarcoma 3 (30%) Mesothelioma 3 Lymphoma 2 Leiomyosarcoma 1 Feigenbaum’s echocardiography

Cardiac Mass

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Primary Benign Tumors Myxoma ✓ ≈30% of all primary cardiac tumors ✓ ≈70% of primary benign cardiac tumors in adults ✓ Mean age at Dx: 50 years ✓ F>M (60-70%) ✓ 80% in LA, 15% in RA - Can occur in ventricles ✓ Average size: 5-6 cm ✓ 90% solitary, 7% Carney complex ✓ Friable/villous surface (1/3) → emboli ✓ Histology - Mesenchymal cells in mucopolysaccharide stroma

Primary Benign Tumors Myxoma ✓ Clinical manifestations - Factors: size, anatomic location

✓ Classic triad of symptoms - Intracardiac obstruction

• Dyspnea, orthopnea, pulmonary edema

• Presyncope/syncope - Systemic embolization

• CVA, retinal artery emboli, emboli to extremities - Constitutional symptoms: fever, fatigue, weight loss, …

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Primary Benign Tumors Myxoma ✓ Echo Findings - Prolapsing mass across MV/TV - Mobile, distensible tumor connected to the limbus of fossa ovalis by a narrow stalk (pedicle)

#961008 67/M

Primary Benign Tumors Myxoma High embolic risk friable villous polypoid prolapsing RA

Low embolic risk round smooth

#593238 53/M, #834110 60/M

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Primary Benign Tumors Myxoma

Mean PG = 14 mmHg

#909813 79/M

Primary Benign Tumors Myxoma ✓ Treatment - Resection

• Including surrounding septum at attachment - Surgical mortality <5% - Risk for atrial arrhythmias - Recurrence in 2-5% - Recurrence in Carney complex 12-22%

- After surgical excision, surveillance echocardiograms should be obtained annually for several years

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Primary Benign Tumors Papillary Fibroelastoma ✓ ≈10% of all primary cardiac tumors ✓ Mean age: 60 years ✓ Mean size: 9 mm (2-70 mm) ✓ The most often valve tumors - 80~90% on valvular endocardium

• AV 36% > MV 29% > TV 11% > PV 7% - Downstream side ✓ Clinical manifestations - 1/3: asymptomatic - Embolization: tumor or thrombus

• CVA/pulmonary/peripheral embolization - MI, angina, sudden cardiac death, syncope

Primary Benign Tumors Papillary Fibroelastoma ✓ Echo Findings - 5~20 mm in diameter - Homogeneous echogenicity - Usually attached to the downstream side of the valve by a small pedicle - Often confused with vegetations

#KSE

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Primary Benign Tumors Papillary Fibroelastoma

#660585 73/F

Primary Benign Tumors Papillary Fibroelastoma

#1120705 62/M

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Primary Benign Tumors Lipoma ✓ Slow-growing ✓ Mature adipose tissue ✓ Location - LV > RA > IAS - Sub-endocardial (50%), myocardial (25%), sub-epicardial (25%) - Valvular attachment rare ✓ Clinical manifestations - Most asymptomatic

• Invasive into tissue → arrhythmias, conduction block

• ↑Size → obstruction ✓ Treatment - Resection recommended (continued growth)

Primary Benign Tumors Lipoma

✓ Echo findings - Sessile or polypoid - Low mobility - Homogeneous & increased echogenicity

#1059177 62/M

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Primary Benign Tumors Lipomatous Hypertrophy of the IAS

✓ Older, obese women ✓ Rarely associated with clinical manifestations ✓ Echo findings: spares fossa ovalis → “dumbbell-shaped”

#1120799 66/M

Cardiac Mass

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Primary Malignant Tumors

✓ Overall 15~30% of primary cardiac tumors ✓ Sarcomas: most common (95%) - Angiosarcoma - Sarcomas with myo- or fibroblastic differentiation - Rhabdomyosarcoma Type % ✓ Suggestive echo findings - Right-sided Myxoma 30 Lipoma 10 - Originated from myocardium → growth Papillary fibroelastoma 8 Benign Rhabdomyoma 6 into ventricular/atrial/pericardial (70%) cavities Fibroma 3 Hemangioma 2 - Broad-based attachment Teratoma 1 - Ill-defined margins - Tissue inhomogeneity / heterogeneous Angiosarcoma 8 contrast enhancement Rhabdomyosarcoma 5 Malignant Fibrosarcoma 3 - Size >5 cm (30%) Mesothelioma 3 - Pericardial effusion Lymphoma 2 Leiomyosarcoma 1

Primary Malignant Tumors Angiosarcoma ✓ Most common primary malignant tumor ✓ 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 ✓ Treatment - Resection + chemotherapy • ↑Survival with complete resection - Transplantation

• Echo: mural type

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Primary Malignant Tumors Rhabdomyosarcoma

• Echo findings: intramural type

#1396137 45/M

Primary Malignant Tumors Lymphoma ✓ ≈5% of primary malignant cardiac tumors ✓ Increasing in immunocompromised patients ✓ Mean age: 38 years ✓ Echo findings: RA, pericardial effusion ✓ Treatment - Chemotherapy

#KSE

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Cardiac Mass

Metastatic Tumors

✓ 1.5~20.6% (≈6%) of oncology patients at autopsy - Most clinically silent ✓ Most common: lung cancer, melanoma

✓ Tumors can spread to heart by propagation through venous ✓ Manifest as system - Mass - Pericardial disease (effusion common) - Myocardial involvement ✓ Multiple masses suggestive

direct extension hematogenous spread

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Metastatic Tumors to the Heart: Sources and Cardiac Manifestations

Origin of source Cardiac effect

Lung Direct extension, often via pulmonary veins, effusion

Breast Hematogenous or lymphatic spread; effusion

Lymphoma Lymphatic spread, varied manifestations

Gastrointestinal Variable manifestations

Melanoma Intracardiac or myocardial involvement

Renal cell carcinoma IVC to RA to RV; confused with thrombus

Carcinoid Tricuspid and pulmonic valve thickening

Feigenbaum’s echocardiography

Metastatic Tumors

Venous Spread From Lung Cancer via Pulmonary Vein

#697431 54/F

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Metastatic Tumors Venous Spread From HCC via IVC

#1477014 66/F

Metastatic Tumors Venous Spread From Thymoma via SVC

Tumor SVC

#1604696 62/F

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Metastatic Tumors Pericardial & Myocardial Involvement from HCC

2010.6 2010.9

2010.11 #558871 84/M

Metastatic Tumors Direct Pericardial Invasion From Breast Cancer

Subcutaneous tumor infiltration

#1573672 53/F

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Cardiac Mass

Intracardiac Thrombi

✓ Risk factors - Dilated cavity - Decreased contractility - Low flow & blood stasis ✓ LV - Recent MI - LV aneurysm - Significant LV dysfunction: DCM ✓ LA - ↑Risk: MS, atrial fibrillation, LV failure - ↓Risk: significant MR, ↑flow velocity within the LA during systole

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Intracardiac Thrombi Sensitivity: 75~95% LV Specificity: 86~88%

Acute MI DCM

DCM Acute myocarditis

#1372875 54/M, #1347604 72/M, #1380658 41/F, #1596441 54/M

Intracardiac Thrombi LV

Standard A4C view Off-axis views

5 MHz transducer 8 MHz transducer Use of contrast agent

#1372875 54/M, #1584636 30/M

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Intracardiac Thrombi DDx LV Thrombi

Thrombus Myxoma

Eosinophilic endomyocarditis Isolated LV noncompaction #1347604 72/M, #KSE, #1570966 51/M, #1432733 33/F

Intracardiac Thrombi LA TTE: sensitivity <50% TEE: sensitivity & specificity 95~100%

A2C

no thrombus

PSAX

#1463994 78/M

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Intracardiac Thrombi LA

#1330198 73F

Intracardiac Thrombi RA

#1577655 65/F, #1536312 62/M, #528042 68/F

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Vegetations

#660472 59/M, #1602804 70/F, #1506962 44/M

Cardiac Mass

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Artifacts: Near-field Clutter

#1819638 46/M

Normal Variants and Benign Conditions

RA Chiari network RV Moderator band Eustachian valve Muscle bundles/trabeculations Crista terminalis Catheters and pacemaker leads Catheter LV False chords (tendons) Pacemaker leads Papillary muscles Lipomatous hypertrophy of interatrial septum LV trabeculations Pectinate muscles Fatty material (surrounding tricuspid annulus) Aorta Brachiocephalic vein Innominate vein LA Suture line following transplant Pleural effusion Fossa ovalis Calcified mitral annulus Valves Lambl’s excrescence Coronary sinus Flail or prolapsing leaflet Ridge between LUPV and LAA Severed MV apparatus after MVR Lipomatous hypertrophy of interatrial septum Fatty TV annulus Pectinate muscles Mitral annular calcification Transverse sinus

Feigenbaum’s echocardiography

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RA: Chiari Network

#1480470 20/M, #KSE

RA: Eustachian Valve

#1076905 45/M

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RA/RV: Pacemaker Lead / Catheter

#1180316 70/F, #KSE

RV: Moderator Band

#1619914 17/F

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LV: False Tendon

#720178 73/F, #1248339 40/M

LA: Coumadin Ridge (Q Tip)

#1597238 38/F

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AV: Lambl’s Excrescence

#1597238 38/F

MV: Mitral Annular Calcification

#1621828 70/F

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Take Home Message

✓ Primary cardiac tumors: rare and usually benign - Myxoma - Clinical presentation varies by location and size of mass ✓ Secondary cardiac metastatic tumors: more common ✓ Echo (TTE and TEE) remains the initial imaging test ✓ Conditions mimic cardiac tumors - Intracardiac thrombi / vegetations - Iatrogenic sources: pacemaker leads or catheters - Normal variant and benign conditions • Recognition of normal variants depends on image quality, technique, and experience

Thank You For Your Attention!