Pathology of Myocardial Disease Flashcards
Restrictive Cardiomyopathy Etiology
- Infiltrative causes: amyloid, sarcoid
- Non-infiltrative causes: idiopathic scleroderma
- Other causes: hemochromatosis, diffuse interstitial fibrosis, sarcoidosis, ect.
Radiation Fibrosis
6 months to 2 years after completion of RT
extent and severity of fibrosis remains stable 2 years after completion of RT
No previous evidence of clinical acute radiation pneumonitis
shortness of breath and possibly leading to pulmonary hypertension and congestive heart failure
Restrictive Cardiomyopathy Clinical Presentation:
Symptoms:
Signs:
Management:
Restricted:
Symptoms: Dyspnea and fatigue
Signs: Rales, peripheral edema, JVD, gallops, tachycardia, and regurgitation murmurs.
Kussmaul Sign: paradoxical increase in JVD that occurs in inspiration
Management: treatment of underlying cause, digoxin, diuretics,
Restricted: Na diet
Restrictive Cardiomyopathy
ventricular muscle is firm and not relaxed well to in filtration
diastolic failure
ventricular filling is restrictive
Gross Pathology of Restrictive Cardiomyopathy
Ventricles are of normal size, stiff myocardium, and cavities are not dilated.
microscopic exam show patchy interstitial fibrosis, and myocyte hypertrophy
Endomyocardial Fibrosis (EMF) :
Is idiopathic progressive disorder of the tropical regions of the world mainly Africa.
What are the characteristics of Endomyocardial fibrosis?
restrictive cardiomyopathy.
EMF is considered part of a spectrum of a single disease process that includes loeffler’s endocarditis
should be distinguished from endocardial fibroelastosis which is characterized by fibrotic thickening of mural endocardium.
Prognosis is poor, disease is progressive
treatment is endomyocardial resection
Infectious Causes of Endomyocardial fibrosis:
Infectious causes, inflammatory causes,
nutritional,
What is the tool of choice for endomyocardial fibrosis?
Cardinal feature is a pathcy fibrosis consisting of collagen deposition fibrblast proliferation of the endocardial surface–> restrictive disease
mild eosinophilia
increased levels of cytokines, TGF-B1
myocardial fibrosis with eosinophilia
Loeffler’s Restrictive Cardiomyopathy Features
Peripheral eosiophilia and eosinophilic infiltration of multiple organs is the cardinal feature
impaired relaxation and ventricular filling
systolic function and wall thickness usually normal
What is Loeffler’s Lung?
clonal eosinophilia caused by mutations in genes that are susceptible to tyrosine kinase inhibitors alone or in combo with bone marrow transplantaion
poor prognosis
Endocardial Fibrosis increased incidence is
Following coxsackie B myocarditis
