Path: Cardiomyopathies Flashcards
Most common form of cardiomyopathy.
Dilated
Pathology of dilated cardiomyopathy.
Progression of dilation of heart chambers with mild hypertrophy of myocardium.
Systolic Dysfunction
Most common cause of dilated cardiomyopathy.
Toxin-Induced (EtOH)
-chemotherapy agents (doxorubicin) can also cause it but are not as common
Cause of dilated cardiomyopathy in children.
Mitochondrial Disease
Most common X-linked cause of cardiomyopathy.
Dystrophin Gene Mutation
Main two culprits that cause myocarditis that can lead to dilated cardiomyopathy.
- Coxsackie B
2. Enterovirus
Describe Arrhythmogenic RV Cardiomyopathy for real.
Inherited disease disease of the cardiac muscle that affects the RV leading to V-tach or fib and possibly sudden cardiac death.
Autosomal Dominant and very thin RV wall that accumulates fat tissue.
Pathology of Hypertrophic Cardiomyopathy.
Genetic mutation of genes encoding sarcomere proteins. Leads to a loss of compliance.
-diastolic dysfunction
Gross characteristic seen on pathological exam of a Hypertrophic Cardiomyopathy heart and region of the heart where most hypertrophy occurs.
Banana-like configuration. Ventricular septum hypertrophies the most and leads to a smaller left ventricular chamber size.
How do cardiac myocytes appear on biopsy in a heart with Hypertrophic Cardiomyopathy?
completely disorganized thick muscle fibers
Major cause of Restrictive Cardiomyopathy.
Amyloidosis: a variety of conditions where normally soluble proteins become insoluble and are deposited in the extracellular space of various organs or tissues, disrupting normal function, in this case the heart. Often associated with Diabetics.
Leads to a loss of compliance.
-diastolic dysfunction
Method to diagnose amyloidosis on biopsy.
- Congo Red dye stains the amyloid.
2. shine UV light to reveal apple green birefringence
Name two conditions that lead to cardiomyopathy due to catecholamine overload.
- Pheochromocytoma
2. Broken Heart Syndrome (Takotsubo)
Why are sickle cell patients susceptible to cardiomyopathy?
They receive many blood transfusions leading hemosiderosis (high iron in the blood). The iron disrupts metal-dependent enzyme systems leading to dilated systolic dysfunction and oxygen free-radical damage.
How can Cardiomyopathy caused by iron overload be diagnosed?
Prussian blue stain on biopsy
Main two microbes that cause general myocarditis.
- Coxsackie B virus
2. Adenovirus
When looking at a biopsy of heart with myocarditis, what is the diagnosis if the biopsy shows:
- many lymphocytes
- Eosinophils
- fused neutrophils
- Protozoans
- Viral
- Hypersensitivity rxn
- Giant Cell myocarditis
- Chagas caused by Trypanosomi cruzi
What is the diagnosis for a myocarditis that shows fused neutrophils within granulomas on biopsy?
Sarcoid Myocarditis
-Sarcoidosis is a disease with the formation of many non-caseating granulomas (granulomas with no necrosis in the center) in multiple organs. The cause is unknown but thought to be related to immune reactions to environmental, infectious or medical interaction.
What is the treatment for any dilated cardiomyopathy?
Transplant
Most common tumors in the heart.
Metastatic (secondary) Tumors
Most common primary tumor in adult hearts.
Atrial Myxoma: may cause ball valve obstruction or embolism
What is Carney Syndrome?
Autosomal Dominant inherited Myxoma disorder. Includes:
- Multiple Myxomas
- Skin Lesions - blue nevi, lentigines (small pigmented spot on skin)
- Endocrine disorders - cushing, acromegaly
Most common complication of Lipomas that form in the heart.
Ball Valve obstruction, must be removed or patient will die.
arrhythmias can also occur
Site of Papillary Fibroelastomas and major complication.
Valve tissue (mainly papillary muscle) Can embolize
Most common primary tumor in children.
Rhabdomyoma
Condition associated with rhabdomyoma formation and characteristic cells seen on biopsy.
Tuberous Sclerosis
-mutation in genes that code proteins that act as tumor growth suppressors. Tumors may form in many different organs.
Spider Cell seen on biopsy
Two most common malignant neoplasms that are associated with the heart.
Angiosarcoma - forms in blood vessels around the heart.
Mesothelioma - forms in the membrane surrounding the heart and lungs
Describe Carcinoid Heart Disease.
Paraneoplastic Syndrome involving a serotonin-secreting tumor. Mostly affects valves or involves plaque formation in the RIGHT SIDE OF THE HEART.
Rarely affects the left side.
Why does Carcinoid Heart disease rarely affects the left side.
The carcinoid tumors are located elsewhere in the body, so the vasoactive amines released by the tumor arrive at the heart through the blood. They enter the right side first where the damage occurs and rarely make it past the lungs to affect the left side.
