Cardiac Pathology 2

Definition of Cor Pulmonale
AKA Pulmonary Heart Disease

Cor Pulmonale refers to conditions in which there is dysfunction of the right side of the heart as a result of pulmonary disease, where pulmonary hypertension develops, leading the the failure of the right side of the heart

NOTE: Right sided failure that is the result of left sided failure is NOT Cor Pulmonlae

Pathogenesis of Cor Pulmonale
1. Pulmonary Disease results in the development of pulmonary hypertension

2. Pulmonary hypertension causes an increased workload on the right ventricle

3. Hypertrophy of the right ventricle as a result in increased work load and the increased pressure that needs to be generated to overcome the increased pulmonary pressures

4. Failure of the right ventricle

Disorders that are associated with the development of Cor Pulmonale
Cor Pulmonale is associated with disorders of the lung that will result in increase resistance in the pulmonary vasculature. These disorders may include

1. Alveolar hypoxia/acedemia that will lead to vasoconstriciton

2. Emphysema, intersitital lung disease, and pulmonary thromboembolism

3 . Increased blood viscocity that is associated with sickle cell disease and polycythemia vera

Chronic vs. Acute Cor Pulmonale
Chronic Cor Pulmonale is mainly associated with right ventricular hypertrophy

Actue Cor Pulmonale is mainly associated with right ventricular dilation

For of Cor Pulmonale that is associated with right ventricular hypertrophy
Chronic

Form of Cor Pulmonale that is associated with right ventricular dilation
Acute

REMEMBER: Acute Cor Pulmonale is mainly associated with a pulmonary embolism

Physical Findings of a Cor Pulmonale Patient
Patients that are suffering from Right Sided Heart Failure as a reuslt of Cor Pulmonale will present with:

1. Chronic Cough
2. Shortness of breathe and easy fatigueability
3. Pitting edema of the ankles and legs

Presents with a Chronic Cough, Shortness of Breathe, Easy Faitugeability, and pitting edema of the ankles and legs
Cor Pulmonale

Pitting vs Non Pitting Edema
Pitting edema is edema that can be indented after pressure is applied to it by a finger or another object. It is commonly associated with right sided heart failure and is not responsive to treatmet with diuretics

Non pitting edema will not hold an indentation after pressure is applied to it, and non pitting edema is mainly associated with disorders of the lymphatics and it responsive to diuretic therapy

This valvular insufficiency may be the result of Cor Pulmonale
Tricuspid Regurgitation

Other conditons, besides cor pulmonale, that can result in the hypertrophy of the right ventricle
1. Left ventricular failure

2. Mitirial valve disease

3. Congential Heart Disease

A “Flea Bitten Kidney” is associated with what disease
Malignant hypertension

The kindeys in a person that suffers from malignant hypertension will develop petichiae, giving them the appearance that they have been bitten numerous times by fleas

REMEMBER: Malignant hypertension is defined as a diastolic pressure that remains above 110 mmHg

Classifications of Cardiomyopathies
1. Dilated Cardiomyopathies
2. Hypertrophic Cardopmyopathies
3. Restrictive Cardiomyopathies

REMEMBER: NONE of these conditons will present with chest pain

Dilated, Hypertrophic, and Restictive are all classificaitons for what type of heart pathology
All are forms of a cardiomyopathy

This disease is associated with Alcoholism, Beriberi, Coxsakievirus as well as cocaine and doxarubicin use. Studies will reveal that the patient has a decreased ejection fraction, pulmonary edema, and a balloon like heart on X-ray. Imaging will also show diltion of all 4 ventricles
Dilated Cardiomyopathy

REMEMBER: NO CHEST PAIN!

Common Causes od Dilated Crdiomyopathy
1. Alcoholism
2. Beriberi
3. Coxsacheivirus Infection
4. Cocaine Use
5. Doxarubincin Use

REMEMBER: Dilated cardiomyopathies will present with a decreased ejection fraction due to the dilation of all 4 cardiac chambers. It IS NOT associated with chest pain.

This cardiac disease has a genetic basis, it is caused by a defect in beta myosin heavy chain leading to decrease contractility of the heart muscle, and will ultimately result in decreased ejection fraction.
Hypertrophic Cardiomyopathy

REMEMBER: NO CHEST PAIN

The typical patient is a young athlete that collapses suddenly while playing a sport
Hypertophic Cardiomyopathy

REMEMBER: This disease is the result of defective beta myosin heavy chain, leasing to hypertophy of the heart because it will need to work harder to pump blood through the body.

Disease that presents with a baloon shaped heart
Dilated Cardiomyopathy

Disease that presents with a banana shaped left ventricle
Hypertrophic Cardiomyopathy

Hypertrophic Cardiomyopathy
A form of cardiomyopathy that result from a defect in the beta myosin heavy chain, leading to decreased contractility of the heart

The heart will hypertophy and will eventually fail if this condition is not treated.

Result in a decreased ejection fractions

Classic patient is a young adult who suddenly collapses while playing a sport

Present with a banana shaped heart

REMEMBER: This will cause S4

This cardiac disease presents with complete loss of heart compliace and the stiffening of the heart muscle
Restrictive Cardiomyopathy

REMEMBER: This disease does NOT present with chest pain. RESTricitive indicates that there is a problem at REST (Diastole), AKA the heart does not fill appropriately due to the loss of compliance.

Restrictive Cardiomyopathy
A disease of the heart that is caused by the loss of heart compliance and the inability of the heart to fill during diastole. It has been linked to diseases such as amyloidosis, sarcoidoisis, and hemachromatosis.

Disease that is associated with Amyloidoisis, Hemochromatosis, and sarcpoidoisis
Restrictive Cardiomyopathy

Stain that Identifies Amyloid Depositis
Congo Red

Stain that identifies hemosiderin deposits
Prussian Blue

Loeffler’s Endocarditis
A disease of the heart that is found in tropical regions of the world. It will result in the fibrotic thickening of the heart, especially at the apex of the left and right ventricles, causing a restrictive cardiomyopathy.

Pathogenesis of Rheumatic Heart Disease
Rheumatic Heart disease follows a strep pyogenes infection of the throat.

Antibodies will be formed to the M protein, the virulence factor of the strep pyogenes bacteria.

The antibodies will clear the infeciton from the throat, but then will attack the myocardial cells of the heart, causing granolomatous inflammation and the formaton of Aschoff bodies

REMEMBER: Because this disease is the result of soluable antibody complexes, it is classified as a type 2 hypersensitivity reaction

Tyoe of hypersensitivity reaciton that is involved in Rheumatic Fever
Type 2

REMEMBER: The soluable antibodies that were developed for the M protein of strep pyogenes will attack the myocardial cells of the heart. This will result in the formaiton to Aschoff bodies and damage to the heart tissues

Aschoff Bodie
Granulomatous Inflammation that is associated with Rheumatic Fever

REMEMBER: These are formed via a type 2 hypersensitivity reaction

Rheumatic Fever vs. Rheumatic Heart Disease
Rheumatic Fever occurs when there is attack of the myocardium by the soluable antibodies that are formed during a strep pyogenes infections (a type 2 hypersensitivity)

Rheumatic heart disease occurs not as a result of a strep pyogenes infection, but as a result of rheumatic fever, when the immune system will attack the myocardium AND the valves.

JONES criteria of Rheumatic Fever
Joint (migratory arthritis)
<3 Disease (myocarditis) Nodule formation Erythema Marginatum Sydenham Chorea/Sore Throat/Strep infection REMEMBER: Females are more likely to present with Chorea than males

This disease will present with migratory arthritis, myocarditis, subcutnaeous nodules, erythema marginatium and sydenham chorea. It always follows a strep pyogenes infection of the throat, and is a result of a type 2 hypersensitivity reaciton in which there is attack on the myocardium
Rheumatic Fever

REMEMBER: Strep Pyogenes is a gram positive, bacitracin sensitive (differentiating factor from GBS), beta hemolytic catalase negative organism.

Strep Pyogenes and the Heart
Strep pyogenes is a gram positive, bacitracin sensitive (differentiating factor from GBS), beta hemolytic, catalase negative organism.

It is associated with the formation of Rheumatic Fever via a type 2 hypersensitivity reaction.

Structural Lesions associated with Rheumatic Fever
Commisural Fusion of the valves
Aschoff Bodies
Antisckow Cells
Thickening of the Chordae tendenae

CAT

The most commonly affected valed in Rheumatic Fever
Mitrial

REMEMBERL The aortic valve is the secondly most afffected valve followed by the tricuspid valve. The aortic valve is almost never affected in this disease

Pathologically, this disease will present with aschoff bodies in the myoccardium, increase c reactive protein, increased sedementation rate, thickening of the chordae tendenae, and commisural fusion of the valves
Rheumatic Fever

Significance of an increased SED rate
Indicated Inflammation

Significance of an increased C reactive protein
Indicates inflammation

REMEMBER: C reactive protein is made in the liver. It is most likely associated with the induction of IL6, which will then increase the C reactive protein

Mechanism of C reactive protein increase
C reactive protein is likely caused by an increase in IL6

REMEMBER: C reactive protein is made in the liver

REMEMBER: disease that are associated with increased IL6 and therefore increased C reactive protein are Arthritis, Acute Inflammation, Atherosclerosis, Alzheimers, Aging, Diabetes, and Cancer

Diseases associated with increased C reactive protein
Arthritis
Acute inflammation
Atherosclerosis
Alzheimers
Aging
Diabetes
Cancer
Rhematic Fever

What are the chances that a person suffering from impetigo contagiosum will get rheumatic fever
NONE

While Impetigo Contagiosum is caused by strep pyogenes, it is more likely to cause post strep glomerulonephritis (hematuria, htn, and oliguria) and not rhematic fever

A common valvular lesion that commonly affect women, that will present with a mid systolic click. As a result of this condition, blood will flow backwards into the left atrium from the left ventricle during systole
Mitrial Valve Prolapse

Mitrial Valve Prolpase
the most common valvular disease in which the mitrial valve baloons back into the atrium during systole, causing the back flow of blood from the left ventricle into the left atrium

Presents as a midsytolic click

More common in females

REMEMBER: Mitirial Valve prolapse is also a common condition in people that suffer from Marfans syndrome

Symptoms of MVP
Usually this condition is asymptomatic, but if it does present with symptoms, they typically are fatigue, arrythmias, and periodic syncope.

Usually an asymptomatic disease of a heart valve, but may present with fatigue, arrythmias, and periodic syncope
Mitrial Valve Prolapse

Presents as a midsytolic click
Mitrial Valve Prolapse

Murmur is heard in the 5th intercostal space on the midclavicular line

Strep Viridans and Mitrial valve Prolapse
patients that suffer from mitrial valve prolapse (or any damage to the heart) are at increased risk of infection by strep viridans following a dental procedure. All patients therefore should be given prophylaxis treatment

REMEMBER: Strep Viridians is a gram postive, alpha hemolytic, non-optochin sensitive (differentiation from strep pneumo) organism that like to attack damaged hearts

Genetics and Mitrial Valve Prolapse
Mitrial Valve prolapse is a common condition in those people that suffer from marfans syndrome

REMEMBER: Marfans syndrome is a autosomal dominant conditon that commonly affects males. It is a result of a defect in fibrillin, which causes problems with the formaiton of connective tissues. These individuals are also at a high rish of disseciton of the ascending aorta.

Cardiovascular Findings associated with Marfans syndrome
MVP
Aortic Regurgitaiton
Aortic Dissection
Medial Necrosis

This is an autosomal dominant condtion that commonly presents with MVP, aortic regurgitaiton, and aortic dissection. It is more common in males and is due to a defect in fibrillin
Marfan’s Syndrome

The second most common congential defect of the heart that will present with present with clinical symptoms in the 30’s
Atrial Septal Defect

REMEMBER: This defect occurs in the septum that separates the right and the left atrium. Originally, it is a left to right shunt, meaning that blood will flow from the left atrium to the right atrium. Because the blood in the left atrium is oxygenated, there are typically no clinical symptoms of this disease until the shunt becomes a Right to Left shunt as a result of right sided hypertrophy and therefore an increase in the right sided pressure. Once this occurs, patients will present with right sided failure symptoms, pulmonary edema, and arrythmias

Atrial Septal Defect
The second most common congenital anomoly of the heart

A hole opens between then right and the left atrium, allowing blood to be shunted from the left atrium to the right atrium. Originally, this is classified as a left to right shunt, because the pressure gradients force the blood to move from the high pressure left atrium to the low pressure right atrium. As the right atrium is continuously overloaded with fluid, it will begin to hypertrophy, and the pressure will rise, causing the shunt to convert to a right to left shunt. This will allow blood to bypass the pulmonary circulation, and will also cause an overload of the left side of the heart, resulting in pulmonary edema, cyanosis, and arrythimas to develop.

Definition of pulmonary hypetension
The pressure in the pulmonary vessels will be more than 30 mmHg

Ausculationt of an ASD
Fixed S2 Splitting

This congential defect will present with a fixed splitting of S2
ASD

Paradoxical Embolism
An Embolism that is able to pass through a congential defect and enter the systemic circulation.

REMEMBER: Normally, emboli will be filtered out in the lungs, but a defect will allow the emboli to bypass the lung and therefore not be filtered from the circulation.

Common patients that are afected by tetrology of fallot, transposition of the great vessels, and pulmonary atresia
These are the three major congenital heart diseases that will present in childhood

The most common congential defect of the heart that may result in congestive heart failure, bacterial endocarditis, arrythmias, and pulmonary hypertension
VSD

REMEMBER: VSD is the formation of a hole in the septum that separates the left and the right ventricles. Orginally, because the ventricles are almost at equal pressures, there is a slight left to right shunt, but this does not cause clinical significance. Over time, if the pressure on the right side increases, the shunt may change into a right to left shunt, and therefore cyanosis may develop in the patient

Eisenmenger’s Sydrome
Occurs when a septal defect in the heart swithces from a left to righ shunt to a right to left shunt

Presents with a loud harsh pansystolic murmur at the left sternal boarder that radiates over the tricuspid area
VSD

Presents as a systolic murmur best heard at the pulmonic area
ASD

REMEMBER: You will also hear fixed splitting of S2

Valve that is commonly affected in VSD
the aortic valve

This congential anomoly presents with a communicating shunt from the pumonary arteries to the aorta. It is the third most common congenital anomoly of the heart and is asociated with maternal rubella infections
Patent Ductus Arteriosus

REMEMBER: In utero, the ductus arteriosus allows blood to bypass the lungs, by having blood pass into the aorta from the pulmonary arteries. When the baby is born, the pressures will change and the blood will shunt from the aorta into the pulmonary arteries. As time goes on though, the pulmonary resistance will increase and obstructive pulmonary disease will cause the blood to reverse flow and travel from the pulmonary arteries into the aorta

PDA
AKA Patent Ductus Arteriosus

A comminicating branch between then aorta and the pulmonry arteries that fails to close after birth.

Congenital Heart Defect associated with a Rubella Infection
PDA

Murmur Associated with PDA
Machine Gun Murmuer

Medications that can be used to close the PDA
Asprin
Indomethacin

Medications that may cause a PDA
PGE2 inhibitors

Ligamentum Arteriosum
The name that is given to the remnant of the fetal ductus arteriosus

A syndrome that occurs as the result of incorrected childhood spetal defects in which there is reversal of of the blood flow from left to right to right to left due to an increase in pressure on the right side of the system (ir. right sided hypertrophy, pulmonary hypertension).
Eisernmenger’s Syndrome

REMEMBER: Once Eisenmengers develops, it is irreversible, as the pulmonary hyperpension is too severe.

The congential defect of the heart that will present with (HOPS) Hypertrophy of the right ventricle, Overriding Aorta, Pulmonary Stenosis, and vSd
Tetrology of Fallot

REMEMBER: This is classically and right to left shunt, while the other septal defects are left to right shunts at firts.

Tetralogy of Fallot
A right to left shunt that presents with (HOPS)

Hypertrophy of the Right Ventricle
Overriding Aorta
Pulmonary Stenosis
vSd

REMEMBER: The degree of stenosis and the degree the aorta overrides the circulation is the factor that determines the severity of the disease.

Presents with a boot shaped heart
Tetralogy of Fallot

REMEMBER: This shape is caused by the hypertrophy of the right ventricle

Clinical Presentation of Tetrology of Fallot
Cyanotic spells that will present with blue skin, rapid breathing, and episodes of syncope

Congential defect that presents with spells of blue skin, rapid breathing, and episodes of syncope. It is the only spetal defect that is commonly a right to left shunt and causes the heart to appear like a boot
Tetralogy of Fallot

Treatment of Tetraology of Fallot
Treatment involved widening of the stenotic pulmonary vessels and closure of the VSD as EARLY AS POSSIBLE

What congntial heart disease has a higher incidence in patients living in high altitudes
PDA

This is primarily due to the lack of oxygen at high levels. It has been postulated that the ocygen concentration in the first few breathes of air inhibti PGE2 and thefore promote the closure of the ductus arteriosus. Low oxygen in the air however will not inhibit PGE2 and therefore will not promote the closure.

Adult vs Child Congential Heart Disease
This classification is based entirely on when Eisenmenger’s disease presents. The defect is classified as childhood when Eisermenergers presents in childhood and is adult when it presents in adults

VSD, PDA, ASD, Coarciton are all diseases that are associated with late onset Eisenmengers

Tetrology of Fallot, Transposition of the Great Vessels, total Anomalous Pulmonary Return, and Tricupid Atresia are all associteed with childhood onset

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