WK1: Cardiac Masses + Sources of Embolism & Systemic Diseases

Define Cardiac Masses

Abnormal lesions/structure(s) w/in o around the heart.

Very rare.

Types of Cardiac Masses

• tumors

• Thrombus

• Vegetations: endocarditis

• Normal structures or artifacts mistaken for masses

Which are more common, benign or malignant cardiac tumors?

Benign

Most common benign cardiac Tumor:

Myxoma

Most common malignant cardiac tumor:

Angiosarcoma

Traits of Papillary Fibroelastomas:

• Benign cardiac tumor

• common on aortic valve & papillary muscles

• Usually on arterial side of AoV

• Small, mobile, pedunculated

• May appear like Lambl’s excrescences

Traits of Lipomas

• benign cardiac tumor

• Well-encapsulated

• Can occur in any heart layer

• Mostly sessile (NOT pedunculated/attached by a stalk)

Traits of rhabdomyomas

• benign cardiac tumor

• Most common tumor in children

• Yellowish-gray color

• Invades ventricular myocardium

• May cause arrhythmias

• Associated w/ tuberous sclerosis

Traits of Fibromas

• benign cardiac tumor

• Bulky

• Embedded in the myocardium of any chamber

  • Usually ventricular

• Usually intramural

• Age range: 2 - 57

• Difficult to resect d/t size in symptomatic pts

  • Typically results in transplant

Traits of Angiosarcomas

• malignant cardiac tumor

• Most common primary malignant tumor

• Mostly occur in the RA

• Infiltrates into cardiac tissue

• Large, mural mass

  • May extend into the pericardium

Traits of rhabdomyosarcomas

• malignant cardiac tumor

• 2nd most common primary malignant tumor

• Can occur in any layer of the heart

• 25% found in those <20 y/o

Primary benign cardiac tumors:

• papillary fibroelastoma

• Lipoma

• Rhabdomyoma

• Fibroma

Primary malignant cardiac tumors

• angiosarcoma

• Rhabdomyosarcoma

• Other (rare) ones: fibrosarcoma, lymphoma, sarcoma, mesothelioma

Non-primary/secondary cardiac tumors are also called _________.

Metastatic

Which type of cardiac tumor is more common, primary or secondary?

Secondary are 20-40x more common than primary tumors

Where do secondary cardiac tumors most often metastasize to?

Which side of the heart do they occur most often (right or left)?

The pericardium (w/ associated effusion). May also have myocardial infusion & intracavitary extension

They occur most often in the Right heart.

Examples of secondary cardiac tumors:

• renal cell carcinoma (enter the RA via the IVC)

• Carcinoid: a deposition of serotonin on the endocardium, causing stiffness. Mostly on the right heart.

Ways that metastatic cancer can enter the heart:

• lymphatic system

• Pulmonary veins to the LA

• IVC to the RA

Indications & Signs/Symptoms of cardiac tumors:

• Family hx

  • Carney Syndrome (myxomas)

• Dyspnea, syncope, palpitations, chest pain, fever, weight loss, TIA, dizziness

• Malignant tumors:

  • Obstruction to blood flow

  • Interference of heart function

  • Local invasion: arrhythmias/effusions

  • Embolisms

Where is cardiac thrombus most often located?

• LV

• LAA

• IVC/RA

What are some potential causes of cardiac thrombus?

• reduced LV function

• Afib

• Hemodynamic stasis:

  • Dilated CMO

  • MI

  • Aneurysm

• Foreign medical device

  • Pacer wires

  • PICC lines

Typical appearance of cardiac thrombus:

• layered/laminar: distinct layers of different echogenicities

• Single

• Multilobulated

• Pedunculated

Different LAA shapes:

Treatments for cardiac thrombus:

• anti-coagulation

  • NOAC/DOAC: apixiban

  • Heparin

• Thrombolysis

  • EKOS: Ultrasound assisted catheter-delivered thrombolysis. Thrombus “opens up” d/t ultrasound and allows more medication into the thrombus

  • Oral

• Thrombectomy

  • Angiovac

  • Surgical

What are some indications to use EKOS?

When should it be turned off/not used?

• most commonly used for pulmonary embolisms

• Can be used for critical DVTs

• MUST be turned off during echocardiograms

Complications of cardiac masses:

• risk stratification for surgeries/procedures

• Embolism

• Progression of malignant processes

• Hemodynamic consequences

Predisposing conditions for a cardiac source of embolism:

• PFO

• IAS aneurysm

• Apical aneurysm

• Prosthetic valves

• Aortic atheroma

What is the Ridge of Coumadin?

a part of the left atrium that lies between the left atrial appendage

Amyloid is what type of particle?

1. Hormone

2. Protein

3. Amino Acid

4. Body Cell

Protein

What is amyloidosis?

A heterogenous disease (multiple etiologies)

The deposition of extracellular proteins in various organs, including the myocardium

Types of amyloidosis

1. AL (Primary): abnormal antibody production in bone marrow

2. AA 9secondary): triggered by an inflammatory response to disease

3. Hereditary (Familial): Inherited, most commonly from the liver making abnormal proteins

4. Senile: amyloid deposition d/t advanced age

Consequences of amyloidosis on the heart:

• thickened myocardium

• Diastolic dysfunction (early)

• systolic dysfunction (late)

• Conduction disorders

• Embolic events

Signs & Symptoms of amyloidosis:

• symptoms of congestive heart failure: SOB, edema

• Peripheral neuropathy

• Other cardiomyopathies

• Arrhythmias

• Hepatomegaly

Amyloidosis Treatments

• Heart failure medications

• Organ transplant

• Chemotherapy

• Anti-inflammatory meds

• Stem cell transplant

• Cardiac transplantation

What is the typical strain pattern for amyloidosis? Is it specific for this disease?

• Apical Sparing: a preservation of contractility in the apical segments, with a decrease in the basal & mid segments

• It is NOT specific for amyloidosis

Echo features of amyloidosis

• increased ventricle wall thickness

• → Ground glass or granular myocardium (sparkling appearance)

• Atrial dilation (diastolic dysfunction)

• Pericardial/pleural effusion

• Significant diastolic dysfunction

• → Altered strain pattern (apical sparing)

• Thickened valves w/ regurgitation (usually mild)

• Thickened IAS

• Thickened papillary muscles

What is carcinoid heart disease (CaHD)

• generally malignant, metastatic endocrine tumor

• Cardiac manifestations

  • Hepatic metastases release tumor substances to the right heart

  • Fibrous white place deposits lead to fibrosis & valve dysfunction

• Predominance on the right heart (TV, PV)

Which valve is most commonly involved in CaHD?

the TV

Etiology of CaHD

• when neuroendocrine tumors metastasize to the liver & begin secreting serotonin

• Usually originate in the small intestine & appendix

  • Some originate in the lungs

• 50% of carcinoid tumors have cardiac involvement

  • Worsened prognosis

Echo features of CaHD

• TV involvement: severe TR w/ minimal TS

  • Restricted TV movement

• Varying PI & PS

  • Usually PS

• R Heart Failure (volume overload)

• Potential MV & AoV Involvement

• Myocardial Metastases

• Pericardial effusion

Clinical Presentation of CaHD:

• Carcinoid Syndrome:

  • Face flushing

  • Hypotension

  • Diarrhea

  • Bronchospasm

  • Wheezing

• Heart failure (late)

Treatments of CaHD

• chemotherapy

• Surgical removal of tumor

• Valve replacement

• Medications to decrease serotonin levels

• Heart failure meds

Which organ system is primarily affected in sarcoidosis?

Lungs (pulmonary)

What is sarcoidosis?

• Inflammatory disease; abnormal collections of inflammatory cells, congregate into masses (granulomas)

  • Invade organ tissue, can cause dysfunction/failure

• Most commonly affects the lungs

  • May impact the heart & skin

• Unknown etiology (idiopathic)

Clinical presentation of cardiac manifestations of sarcoidosis:

• asymptomatic

  • Depends on size & quantity of granulomas

• Sudden death

• Arrhythmias & conduction abnormalities

• LV dysfunction & CHF

• Typically presents before age 40

Echo features of sarcoidosis:

• enlarged LV

  • Decreased systolic function

• RWMA

• RV & RA enlargement/hypertrophy

  • PHTN from lung sarcoidosis

• Septal wall thinning

  • W/ or w/out aneurysm

• Diastolic dysfunction

• Pericardial effusion

Treatments for sarcoidosis:

• None, if asymptomatic

• Anti-inflammatory meds (corticosteroids)

• Immune suppression (methotrexate)

• Pacemaker/ICD implantation for arrhythmias

• Transplant

What is hyperesinophilic syndrome?

• abnormal amount of eosinophils in the blood stream

• Infiltrative disease, may impact multiple organs

  • Cardiac, skin, renal

• Confirmed by blood testing (>1500 eosinophils/mm³ for >6mo)

What are the stages of hypereosinophilic syndrome?

3 stages

1. Necrotic Stage: eosinophils damage the endocardium

2. Thrombotic Stage: thrombus forms on the damaged endocardium

   1. Concern w/systemic embolization.

3. Fibrotic Stage: the endocardium becomes fibrotic & scarred.

   1. LV filling becomes restrictive

What is Loeffler’s Cardiomyopathy?

• A form of restrictive CMO

• Caused by significant endocardial damage from hypereosinophilic syndrome

Echo features of hypereosinophilic syndrome

• normal LV/RV size & function

• Atrial enlargement

• Restrictive physiology (decreased LV compliance)

Treatment of hypereosinophilic syndrome

• treat CHF

• Anticoagulants

• Treat the hypereosinophilic (steroids)

• Endocardial stripping for fibrosed tissue

What is hemochromatosis?

• Excess deposition of iron in the blood, affection organ/tissue function

• Rare before age 20

  • Peaks in the 5th decade

Types of Hemochromatosis:

1. Primary (hereditary): excess absorption of dietary iron

2. Secondary (acquired): transfusion, iron therapy

Clinical Presentation of hemochromatosis

• males >40 y/o

• Liver & heart failure

• Hyperpigmentation of the skin

Echo appearance of hemochromatosis

• dilated cardiac chambers

  • Normal wall thickness

• Global systolic dysfunction

• Progressive diastolic dysfunction

What is HIV/AIDS? What is its involvement in the cardiovascular system?

• A virus that attacks the immune system that reduces its effectiveness & leads to susceptibility to disease and infections

• Develop intramuscular plaque at a greater rate

• 2x risk of cardiovascular events

Although rare, with the advent of antiretrovirals - this is a common finding with late stage AIDS:

1. Kaposi sarcoma

2. Skin rush

3. Systemic HTN

4. Thrush

1. Kaposi sarcoma: a type of cancer that forms in the lining of blood vessels and lymph vessels

Cardiac concerns in patients w/ HIV/AIDS:

• DCM

• Pericarditis

• RV dysfunction

• Endocarditis

• CAD

• Cardiac tumors (Kaposi’s sarcoma)

Echo features in pts w/ HIV/AIDS:

• dilated LV and/or RV

  • Systolic dysfunction

• Varying diastolic dysfunction

• MR

• Endocarditis (bacterial & NBTE)

• Pericardial effusion

• Pleural effusion

• Constrictive pericarditis

• Cardiac neoplasm

• PHTN

Treatments for HIV/AIDS

• Antiretroviral therapy

  • Cause lower risks of complications

• CHF medications

• Antibiotics (endocarditis)

1. What is Rheumatoid Arthritis (RA)?

2. What is the clinical presentation?

3. What is its cardiac involvement?

1. Chronic inflammatory disease primary impacting the joints

2. Clinical Presentation:

   1. Common in women

   2. Fatigue, myalgias, fever, limited joint motion

3. Effusion, MI, valvular disease

1. What is antiphospholipid syndrome?

2. What is its clinical presentation?

3. What is its cardiac involvement?

1. A hypercoagulability syndrome.

2. Can cause PHTN from PE

3. Causes cardiac dysfunction w/ potential thrombosis of coronary arteries

1. What is scleroderma?

2. What is its clinical presentation?

3. What is its cardiac involvement?

1. Excessive connective tissue that accumulates in various body tissues.

2. Can cause PHTN, systemic HTN

3. Presents w/ conduction abnormalities

1. What is systemic lupus erythematosus (SLR)?

2. What is its clinical presentation?

3. What is its cardiac involvement?

• an autoimmune disease

• Prevalent in women

• Cardiac manifestations:

  • Valvular: Libyan-sacks endocarditis (w/ regurgitation)

  • Pericardial effusion

  • Global LV dysfunction

  • PHTN w/ pulmonary involvement

  • Diffuse leaflet thickening

1. What is Marfan’s Syndrome?

2. What are common cardiac manifestations?

3. What are common echo features?

1. Hereditary (autosomal dominant) connective tissue disorder

2. Most common cause of death is Ao dissection

   1. MV disease

   2. Dilated Asc Ao

   3. AI

   4. Ao Dissection

3. Echo features:

   1. Ao root dilatation

   2. Dilated asc Ao

   3. Annuloaortic ectasia

   4. Ao dissection

   5. Myxomatous MV w/ prolapse

Types of vasculitis that may have cardiac involvement:

• Giant cell Arteritis: Ao aneurysms & dissections

• Takayasu Arteritis: granulamatous pan arteritis of large vessels (AR, Ao Dilatation)

• Kawasaki Disease: acute systemic vasculitis of unknown origin

• Churg-Strauss Syndrome: Pericardial effusion, valve regurgitation

• Wegner’s Granulomatosis: pericarditis, mass lesions

Kawasaki Disease is also called….

Mucocutaneous Lymph Node Syndrome

Causes swelling of mucous membranes & lymph node glands

What is Kawasaki’s Disease? What are common cardiac findings?

• acute systemic vasculitis of unknown origin

• Seen <5 y/o

• Cardiac involvement:

  • Coronary artery dilation/aneurysm

  • LV dysfunction

  • MR

  • Pericardial effusion

Endocrine diseases with potential cardiac involvement:

• hyperthyroidism

• Hypothyroidism

• Pheochromocytoma: excessive catecholamines

• Acromegaly: secretion of excessive growth hormone (usually pituitary adenoma)