Systemic and CT disorders U2 L3

What is rheumatoid arthritis?

An autoimmune, chronic inflammatory disease that primarily affects the joints.

Can rheumatoid arthritis have a gradual or sudden onset?

Yes

List the 8 systemic signs and symptoms of rheumatoid arthritis.

1. Fatigue

2. Myalgias

3. Weight loss

4. Fever

5. Pain

6. Malaise

7. Anorexia

8. Loss of skeletal function/motion

How common is rheumatoid arthritis in the United States?

About 2.5 million people.

What 2 demographic factors are associated with rheumatoid arthritis?

• Age related

• More common in women than men

What is the etiology of rheumatoid arthritis?

It is idiopathic with no known cure and may be related to genetics.

How common is cardiovascular involvement in rheumatoid arthritis?

Symptomatic cardiac manifestations are somewhat rare, but mild or severe cardiovascular involvement is seen in up to half of patients.

What can cause the cardiac manifestations of rheumatoid arthritis?

Inflammation

List the 3 pericardial manifestations of rheumatoid arthritis.

• Pericarditis

• Pericardial effusion

• Constriction

List the 6 myocardial manifestations of rheumatoid arthritis.

1. CAD

2. Myocarditis

3. Global LV dysfunction

4. RWMAs — rare

5. Diastolic dysfunction

6. Myocardial nodules

List the 3 valvular findings associated with rheumatoid arthritis.

• May affect the aortic or pulmonic valve

• Valve thickening and regurgitation

• Valvular lesions/nodules

How common is pulmonary hypertension in rheumatoid arthritis?

Rare

What can cause conduction abnormalities in rheumatoid arthritis?

Inflammation of the AV node/bundle

What is systemic lupus erythematosus?

An autoimmune systemic disease characterized by antinuclear antibodies causing inflammation of various organs

What is the etiology of SLE, and who is it more common in?

• Idiopathic with no known cure

• More common in women

List the 8 clinical manifestations of SLE.

1. Butterfly rash

2. Photosensitivity

3. Joint pain

4. Fatigue

5. Fever

6. Weight loss

7. Pleurisy

8. Renal disease

List the 6 valvular manifestations of SLE.

1. Libman-Sacks endocarditis

2. Valvulitis

3. Leaflet thickening

4. Regurgitation

5. Stenosis — rare

6. MVP

Which 2 valves are more commonly involved in Libman-Sacks endocarditis?

• Aortic valve

• Mitral valve

List the 2 additional non-pericardial/non-myocardial cardiac manifestations of SLE.

• Conduction abnormalities

• Premature CAD

List the 2 pericardial manifestations of SLE.

• Pericardial effusion

• Constriction — rare

List the 4 myocardial/pulmonary manifestations of SLE.

• Myocarditis

• LV dysfunction

• RWMAs

• Pulmonary hypertension

What is Libman-Sacks endocarditis?

Non-bacterial thrombotic endocarditis (NBTE).

How common is Libman-Sacks endocarditis in SLE?

6–11%

Can Libman-Sacks endocarditis be active or healed?

Yes

List the 2 findings of active Libman-Sacks endocarditis.

• Recent disease onset

• Focal necrosis and fibrinous clumps

What finding is associated with healed Libman-Sacks endocarditis?

Valve calcification

What is antiphospholipid syndrome?

An autoimmune disorder causing hypercoagulability

What autoimmune disease is APS associated with?

SLE

List the 4 clinical features of APS.

• Recurrent vascular thrombosis

• Pregnancy loss

• Thrombocytopenia

• High anticardiolipin antibody — IgG or IgM

What ventricular functional abnormality may occur with APS?

Ventricular dysfunction.

List the 3 thrombotic cardiac/systemic findings associated with APS.

• Coronary thrombosis

• Intracardiac thrombi

• Systemic thrombosis

What ventricular structural abnormality may occur with APS?

Ventricular hypertrophy.

Which side of the heart and which valve are mainly affected by valvular lesions in APS?

Left-sided valves, especially the mitral valve

List the 2 mitral valve findings associated with APS.

• NBTE

• Focal or diffuse leaflet thickening

Does leaflet thickening in APS usually cause significant stenosis?

No. It is usually without significant stenosis

What is scleroderma?

An autoimmune connective-tissue disorder with excessive collagen deposition.

Who is scleroderma more common in, and what is the typical age of diagnosis?

More common in women, usually diagnosed between 30–50 years old.

What does CREST stand for in scleroderma? List 5

1. Calcinosis

2. Raynaud’s

3. Esophageal dysmotility

4. Sclerodactyly

5. Telangiectasia

How common is pericardial disease in scleroderma?

In up to 78% of patients.

List the 2 pericardial findings associated with scleroderma.

• Pericardial effusion

• Inflammation

What pulmonary vascular complication has a high rate in scleroderma?

Pulmonary hypertension, including PAH

List the 2 additional mechanisms that can cause pulmonary hypertension in scleroderma.

1. Myocardial fibrosis or restrictive physiology

2. Interstitial lung disease U2 L3 Systemic and CT disorders

What is Marfan syndrome?

The most common hereditary systemic connective-tissue disorder.

What is the inheritance pattern of Marfan syndrome?

Autosomal dominant.

List the 3 systems affected by Marfan syndrome.

• Skeletal

• Ocular

• Cardiovascular

List the 6 clinical features of Marfan syndrome.

1. Tall, thin stature

2. Bony overgrowth

3. Scoliosis

4. Pectus deformities

5. Joint and skin laxity

6. Arachnodactyly

List the 5 cardiac manifestations of Marfan syndrome.

1. Mitral valve prolapse

2. Mitral regurgitation

3. Aortic root dilation

4. Aortic regurgitation

5. Risk of aortic dissection

What does the slide state about Marfan syndrome and aortic insufficiency requiring AVR?

It is the 3rd most common cause of AI leading to AVR.

What is Ehlers-Danlos syndrome?

An inherited connective-tissue disorder.

List the 2 main clinical manifestations of Ehlers-Danlos syndrome.

• Joint hyperflexibility

• Hyperelasticity

How do the cardiac manifestations of Ehlers-Danlos compare with Marfan syndrome?

They are similar to Marfan syndrome.

List the 2 cardiac manifestations specifically listed for Ehlers-Danlos syndrome.

• Aortic pathologies

• Mitral valve prolapse

What is Loeys-Dietz syndrome?

An inherited autosomal dominant connective-tissue disorder.

How do the cardiac manifestations of Loeys-Dietz syndrome compare with Marfan syndrome?

They are similar to Marfan syndrome.

What major cardiovascular risk is even higher in Loeys-Dietz syndrome?

Aortic rupture.

What is ankylosing spondylitis?

A chronic systemic inflammatory disorder with a pathogenesis that is not entirely known but has genetic components. U2 L3 Systemic and CT disorders

What area does ankylosing spondylitis primarily affect?

The axial skeleton, including the hips and shoulders.

List the 4 musculoskeletal abnormalities associated with ankylosing spondylitis.

• Inflammation and fusion of joints

• Arthritis of the hips and shoulders

• Osteoporosis

• Spinal deformity and fracture

Who is typically affected by ankylosing spondylitis?

Young men

List the 7 cardiac manifestations of ankylosing spondylitis.

1. Ascending aortitis

2. Aortic root dilation with possible dissection

3. Thickened aortic valve with aortic regurgitation

4. Conduction disturbances

5. Mitral valve disease

6. Cardiomyopathy

7. Pericarditis

What is Churg-Strauss syndrome now called?

Eosinophilic granulomatosis with polyangiitis (EGPA)

What type of disease is Churg-Strauss syndrome?

An autoimmune inflammatory condition causing multisystem necrotizing vasculitis of small and medium-sized vessels

List the 6 clinical features of Churg-Strauss syndrome.

1. Asthma/allergic rhinitis

2. Eosinophilia

3. Extravascular granuloma formation

4. Vasculitis of multiple organ systems

5. Coronary arteritis

6. Intestinal ischemia

List the 3 echo findings associated with Churg-Strauss syndrome.

• Pericarditis with pericardial effusion

• DCM from myocarditis

• Endomyocardial fibrosis

What triad defines Wegner’s granulomatosis?

• Small-vessel vasculitis

• Granulomatous inflammation

• Necrosis

List the 4 clinical findings of Wegner’s granulomatosis.

1. Sinusitis

2. Pulmonary infiltrates

3. Glomerulonephritis

4. Ocular involvement U2 L3 Systemic and CT disorders

List the 7 echo/cardiac findings of Wegner’s granulomatosis.

• Pericarditis/pericardial effusion

• Myocarditis with DCM

• Global hypokinesis

• Valvulitis with valvular regurgitation

• Arteritis

• Mass lesions/granulomas with LV RWMAs

• Arrhythmias

What is another name for Osler-Weber-Rendu?

Hereditary hemorrhagic telangiectasia.

What is the inheritance pattern and primary abnormality in Osler-Weber-Rendu?

It is an autosomal dominant disorder affecting the development of vasculature.

List the 3 cardiac/vascular manifestations of Osler-Weber-Rendu.

• High cardiac output

• Coronary AVMs

• Pulmonary AVMs

What bubble-study finding is associated with pulmonary AVMs in Osler-Weber-Rendu?

A late positive bubble study.

List the 4 general functions influenced by thyroid hormone.

• Growth and maturation of tissues

• Cell respiration and energy expenditure

• Turnover of substrates, vitamins, and hormones

• Metabolism

List the 4 cardiovascular effects influenced by thyroid hormone.

• Contractility force and frequency

• Extracellular volume

• Tissue metabolism

• Catecholamine effects

What is hyperthyroidism?

Overproduction of thyroid hormone

What disease is listed as a cause/association of hyperthyroidism?

Graves disease

List the 5 clinical features of hyperthyroidism.

• Insomnia

• Nervousness

• Weight loss

• Bulging eyes

• Heat intolerance

List the 4 hyperdynamic/functional cardiac effects of hyperthyroidism.

• Increased stroke volume

• Increased cardiac output

• Increased LV mass

• Hyperdynamic systolic function

List the 4 additional cardiovascular manifestations of hyperthyroidism.

• Diastolic dysfunction

• Pulmonary hypertension

• Tachycardia/tachyarrhythmias

• Decreased peripheral vascular resistance

What effect can hyperthyroidism have on preexisting heart conditions?

It may aggravate them

What is hypothyroidism?

Thyroid hormone deficiency.

List the 3 clinical features of hypothyroidism.

1. Fatigue

2. Dry skin

3. Brittle hair and nails U2 L3 Systemic and CT disorders

List the 5 cardiac manifestations of hypothyroidism.

• Accelerated atherosclerosis

• Bradycardia with decreased cardiac output

• Diastolic dysfunction

• Pericardial effusion

• Dilated cardiomyopathy/heart failure

What is Type 1 diabetes mellitus?

An autoimmune disorder that destroys pancreatic beta cells.

What is Type 2 diabetes mellitus?

Acquired insulin resistance associated with lifestyle factors.

What is gestational diabetes?

Diabetes that develops during pregnancy.

List the 5 clinical features of diabetes mellitus.

• Dry mouth

• Frequent urination

• Blurred vision

• Numbness/tingling in the peripheries

• Fatigue

How much does Type 2 diabetes increase the risk of CVD/CAD?

2–4 times.

List the 3 additional cardiovascular manifestations of diabetes mellitus.

• Diastolic dysfunction/diastolic heart failure

• Atrial fibrillation or ventricular arrhythmias

• Stroke and PAD

What causes acromegaly?

Excessive growth hormone secretion, almost always from a pituitary adenoma in adults.

How common are cardiac manifestations in acromegaly?

In about 1/3 of patients

List the 4 cardiac manifestations of acromegaly.

• LVH

• Diastolic dysfunction

• Systolic dysfunction

• CHF

What is hepatopulmonary syndrome?

Hypoxemia from dilated intrapulmonary vasculature in the presence of liver disease or portal hypertension.

What happens to hypoxemia with oxygen supplementation in hepatopulmonary syndrome?

In worsening cases, hypoxemia does not respond to oxygen supplementation; milder cases may respond when vasodilation is less severe

What 2 clinical findings are associated with hepatopulmonary syndrome?

• Dyspnea

• Hypoxemia in the setting of advanced liver disease

What echo finding is associated with hepatopulmonary syndrome?

A late positive bubble study from dilated pulmonary vasculature.

When should a bubble study be performed for possible hepatopulmonary syndrome?

In patients with an indication related to liver disease, such as:

1. Pre-TIPS

2. Transplant evaluation

What is the only treatment option listed for hepatopulmonary syndrome?

Liver transplant, if indicated.

What survival is listed for hepatopulmonary syndrome?

10.5 months.

What is a pheochromocytoma?

A rare adrenal tumor that secretes catecholamines

What severe cardiovascular problem can occur with pheochromocytoma?

Acute, severe hypertension with potentially life-threatening cardiovascular events.