Cardiac Manifestations of Systemic Diseases&Rheumatic Heart Disease

Question 1

Systemic Disease

Answer 1

multi-organ involvement that can affect the heart by:

↑ demands on the heart
Cause arrhythmias
Enable coronary arterial disease → ischemic heart disease
Distort cardiac structure: pericardium, myocardium, endocardium (valves)

Question 2

Cardiac Manifestations of Systemic Diseases– DM

Answer 2

Independent risk factor for CAD, PAD, CHF, MI

CAD is the most common cause of death in DM 1 and 2

Prognosis is worst than for non-DM
Have larger infarct size
Greater CAD burden
Greater post-infract complications (CHF, death)

Duration and control of hyperglycemia correlates w/incidence

DM Pts have greater risk for MI due to high CAD burden
Greater CAD burden = greater MI area = greater post-infarct complications
HF
Shock
Death

Question 3

Worrisome “angina equivalent” symptoms (6)

Answer 3

Nausea
Dyspnea
Pulmonary edema
Arrhythmias
Heart block
Syncope

Question 4

Why does CAD happen (DM)?

Answer 4

↑ Insulin resistance → endothelial dysfunction and ↑ plasminogen activator inhibitors-1 →↑ coagulation and thrombosis formation and platelet dysfunction

Question 5

Why does cardiomyopathy happen (DM)?

Answer 5

DM has ↑ intra-ventricular collagen/fibrosis/inflammation →↓ mechanical compliance during diastole →↓ myocardial relaxation → diastolic HF seen in early failure

Question 6

Treatment Approach for DM with Endocrine disease and Cardiac Manifestations

Answer 6

Maintain A1C ~ 7%
Physiologically normal Insulin concentration slows cardiac pathology
Dyslipidimia
Hypertension
<130/80 mmHg (Pts with Nephropathy, CVD)

Question 7

People with Diabetes that get CAD Treatment approach

Answer 7

Revascularization

Percutaneous coronary intervention (PCI) restenosis
Coronary Artery Bypass Grafting (CABG) for multi-vessel disease

Question 8

Malnutrition

Answer 8

Vitamins and minerals are essential in biochemical reactions as co-enzymes

Dietary proteins are essential for amino acid protein synthesis

~50% of Internal Medicine/Surgical Pts have negative protein balance
Low serum albumin

When caloric intake is scarce some amino acids can be used for energy = gluconeogenesis (alanine)

Too little protein consumption = negative protein (negative nitrogen) balance
Protein Energy Malnutrition (PEM)

Question 9

Cardiac Manifestations of Protein Energy Malnutrition (PEM) Syndromes:

Answer 9

Myocardial (myofibrillar) atrophy

Ventricular hypokenesis =↓ Cardiac Output and Systolic BP

↓ Pulse pressure

Generalized edema due to:
↓ oncotic pressure
Ventricular hypokenesis

Question 10

Cardiac Manifestations of Protein Energy Malnutrition (PEM) Syndromes Treatment

Answer 10

Nutritional rehabilitation
Total parental nutrition
Jejunostomy tube

Question 11

Thiamine deficiency (beriberi, B1 deficiency)

Answer 11

Found in vegetables, beef, yeast, nuts, whole grains

Deficiency found in 20-90% of U.S. Heart Failure Pts
Diuretic induced renal excretion
Decreased PO intake

Common in alcoholics
 Due to:
 malnutrition
Malabsorption from EtOH
Impaired cellular B1 utilization and ↓ tissue oxygenation

Anorexia Nervosa
Poor dietary intake is the main cause

Question 12

Clinical presentation: Thiamine deficiency

Answer 12

Tachycardia
High out-put heart failure 
Definition: increased cardiac output w/o meeting metabolic needs
Wide pulse pressure (↑SBP-DBP)
Third heart sound—S3
Apical systolic murmur

Question 13

Wernike-Korsakoff syndrome

Answer 13

is the most common complication of alcohol related B1 deficiency = encephalopathy, oculomotor, ataxia then anterograde-retrograde amnesia.

Question 14

B6, B12 and Folate

Answer 14

Co-factors in metabolism of Homocysteine

Folate (Folic Acid)
Leafy green vegetables

B6 (Pyridoxine)
Co-factor in > 100 enzymes involved in amino acid metabolism
Present in all food groups

B12 (cobalamin)
Clinical signs of deficiency will be seen after a yr or more
Causes of deficiency:
Chronic gastric atrophy
Auto-antibody formation to gastric intrinsic factor
Gastrectomy

Question 15

Homocysteine

Answer 15

Elevated homocysteine is an independent risk factor for atherosclerotic vascular disease

Homocysteine triggers formation of atheromas
Creates endothelial oxidative stress
Is Prothrombotic

Plasma deficiencies of Folate, B12 and B6 are inversely related to Homocysteine levels—especially B12, Folate

Question 16

Treatment for Folate, B6, and B12

Answer 16

Replace Folate: 1-5mg/day x 3 months until levels normalize
Replace B12: 1000mg/day x 3 months until levels normalize
Replace B6: according to age and sex until levels normalize

Question 17

Obesity is associated with? (6)

Answer 17

Obesity was declared a “disease” in 2013 for the 1st time

> 68% of U.S. adults are overweight (BMI > 25%)

Obesity is associated with:
↑ glucose intolerance, DM
↑ HTN
↑ atherosclerosis, CAD
↓ adiponectin 
Anti-atherogenic in vascular endothelium
CAD (can also lead to HF)
Heart failure (obesity as an independent risk factor)

Question 18

Obesity Cardiac Manifestations

Answer 18

Obese Pts have greater central and total blood volume to address excess adipose tissue
The heart is over burdened by having to pump blood against an ↑ total body mass system = after-load.
Greater blood volume →↑ Cardiac Output →↑right and left ventricular filling pressure
Greater CO supports blood demand of excess adiposity

Question 19

Chronic blood volume overload leads to? (Obesity)

Answer 19

HTN
Right and Left ventricular hypertrophy with dilatation
Poor exercise resistance due to overworked cardiopulmonary system even at rest (base-line)
Poor cardiac reserve = heart failure

Question 20

Pulmonary congestion with obese patients

Answer 20

Seen with increased left ventricular and atrial pressures and pulmonary venous circulation back up

Question 21

Treatment for Obesity

Answer 21

Weight reduction is the most effective and safest approach and tailored exercise

↓ blood volume, CO, HTN, Hypertrophy

Question 22

Cardiac Manifestations of Thyropathy

Answer 22

Thyroid hormone is an essential determinant of metabolic activity:

O2 consumption 
Exerts influence on cardiovascular system
Increases/decreases cardiac work load
Exerts effect on cardiac activity:
Inotropic
Chronotopic
Dromotropic

Question 23

Clinical Signs for Cardiac Manifestations of Thyroid Disease

Answer 23

Hyper-dynamic precordium
Widened pulse pressure
Loud S1 (MT)
Pleuropericardial friction rub

Question 24

Clinical Presentation of Hyperthyroidism with Cardiac Manifestations of Thyropathy

Answer 24

Clinical presentation:
Sinus tachycardia
Palpitations
Atrial fibrillation
HTN
Fatigue

Question 25

Clinical presentation and Signs of Hypothyroidism with Cardiac Manifestations of Thyopathy

Answer 25

Clinical presentation:
Pathologically: Myofibrillar edema, interstitial fibrosis = ↓ CO
↓ CO → ↓ SV, ↓ Pulse pressure and ↓ HR

Signs:
Pericardial effusion develops subacutely, slower than acute
Distant heart sounds
Weak arterial pulses
ECG: low voltage, sinus bradycardia and prolonged QT interval
CXR: “water bottle” cardiac silhouette (pericardial effusion), cardiomegaly
Hypercholesterolemia, high triglycerides

Question 26

Treatment of Thyroid Disease for Cardiac Manifestations

Answer 26

Hyperthyroidism:
Ischemic heart disease symptoms/CHF occurs if underlying cardiac pathology is present
Cardiac symptoms that develop due to hyperthyroidism will respond to Methimazole/Propylthiouracil (PTU)

Hypothyroidism
Angina pectoris is usually not experienced because of hypothyroidism low metabolic demands
But, angina/MI can be precipitated once thyroid hormone tx is started.
Must begin T4 (Levothyroxine) tx slowly but progressively

Question 27

What is Rheumatoid Arthritis—Cardiac Manifestations of Autoimmune Diseases

Answer 27

A chronic, systemic, inflammatory condition that affects:

peripheral joints, muscles, vessels, ligaments and tendons primarily
but, due to its systemic involvement, hematologic, neurologic, pulmonary and cardiac systems can be attacked as well.

Question 28

Diagnostic Labs for Rheumatoid Arthritis—Cardiac Manifestations of Autoimmune Diseases

Answer 28

Rheumatoid Factor + in 70%
Anticyclic citrullinated peptide (anti-CCP) antibody + in 70-80%
ESR or CRP

Question 29

Rheumatoid Arthritis—4 Cardiac Manifestations of Autoimmune Diseases

Answer 29

Cardiovascular Disease is most common cause of death
Higher rate of carotid atherosclerosis
Higher rate of CAD

Pericarditis is most common finding
10-50% will have pericardial effusion on echocardiogram
Few Pts will progress to:
 Tamponade
Constrictive pericarditis

Mitral/Aortic Valvular vegetations
Granulomatous and inflammatory processes
Can cause valvular insufficiency (regurgitation)

CHF (systolic and diastolic)
Occurs at 2x the rate in RA Pts than in the general public

Question 30

Treatment for RA–Cardiac Manifestations of Autoimmune Diseases

Answer 30

NSAIDs
DMARDs (disease modifying antirheumatic drugs)
Glucocorticoids
Anti-TNF
Immuno-modulators: Abatacept, Rituximab

Urgent pericardiocentesis
If Pt is “tamponading” (very rare for RA)

Pericardiodectomy
If there’s constrictive pericarditis (Very rare for RA)

Question 31

Systemic Lupus Erythematosus

Answer 31

An inflammatory, autoimmune disease with systemic damage of organs mediated by auto-antibodies and immune complexes.

Systems involved: skin, renal, cardiac, joints, hematologic, neurologic

Question 32

Cardiac Manifestations of Systemic Lupus Erythematosus (SLE)

Answer 32

SLE can affect all layers of the heart

Pericarditis most common complication
Rarely leads to tamponade or constrictive pericarditis

Myocarditis
Associated with HF especially in the setting of HTN

Question 33

Accelerated atherosclerosis due to

Answer 33

Endothelial damage from:
Autoimmune attack
Chronic inflammation
Oxidative damage to arteries
Chronic glucocorticoid Use

Question 34

Treatment for cardiac disease (SLE)

Answer 34

Life style modifications:
Smoking cessation
Diet and exercise

Statins (HMG-COA reductase inhibitors)

Give hydroxychloroquine (Plaquenil) (non-steroidal) vs. glucocorticoids when possible
To reduced potential for hyperlipidemia, HTN, DM

Control HTN/CHF

High dose glucocorticoids (in SLE) used for:
Heart failure
Embolic events
Arrhythmias

Question 35

Heart disease resulting from Rheumatic fever

Answer 35

Acute Rheumatic Fever is caused by Group A beta-hemolytic Streptococcus (GAS) Upper airway infection, such as in “strep throat.”

Question 36

Cardiac Manifestations of Rheumatic Heart Disease

Answer 36

Seen in 60% of (Untreated or repeated infections) Acute Rheumatic Fever

Mitral/aortic Valvular disease is the “hallmark” of rheumatic carditis
Stenosis or regurgitation can be seen
Pancarditis
Endocarditis
Arrhthymias 

Dx: 
Throat swab, rapid strep test
Serologic tests:
Anti-streptolysin O (ASO) titer
Anti-DNase B (ADB) titer

Question 37

Signs and Symptoms of Rheumatic Heart Disease (RHD)

Answer 37

Clinical symptoms:
Constitutional:
Fever, malaise
Joints:
Arthralgias
Skin: 
Scarlet fever rash w/ Strawberry tongue
Chest Pain

Clinical signs:
Mitral/Aortic murmur
Friction rub
ECG: ↑PR interval, Afib
↑ CRP, ESR, WBC

Question 38

Pathophysiology Of Acute Rheumatic Fever

Answer 38

ARF caused by →GAS → triggers autoimmune reaction → damage to cardiac structures:

Endocarditis and valvular damage w/ vegetation deposit
Mitral regurgitation and dilatation from ventricular backflow
Myocardium
With recruitment of “Ascoff” cells and myocarditis
Pericardium
Serofibrinous Pericarditis

The acute inflammatory process can subside w/o Tx, but leaves behind damaged tissue

Question 39

Cardiac Manifestations of Rheumatic Heart Disease Treatments

Answer 39

Valvular repair/replacement when heart failure (HF) cannot be treated conventionally

ASA/NSAIDs have little effect on RHD related carditis

Control HTN

Tx HF: 
Loop diuretics (furosemide) for fluid overload control (dyspnea/edema)

ACEi, Beta Blockers (bisoprolol, metoprolol XR)

ARBs are not as effective as ACEi or cardioselective BB, but they are used in pts who cannot take ACEi, BB

High dose, short course of glucocorticoids for Acute heart failure

Question 40

Rheumatic Heart Disease (RHD) Prevention

Answer 40

Primary prevention
Treat GAS infections

Secondary Prevention:
Long term strategy to prevent recurring GAS episodes
Benzathine penicillin, for 5-10 years, sometimes for life, depending on cardiac disease severity

Allergy alternatives: Erythromycin