ana 2 Flashcards
(16 cards)
What is Hypertrophic Cardiomyopathy (HCM) also known as?
Idiopathic hypertrophic subaortic stenosis and Hypertrophic obstructive cardiomyopathy
HCM is characterized by myocardial hypertrophy, abnormal diastolic filling, and intermittent left ventricular outflow obstruction.
What are the key morphological characteristics of Hypertrophic Cardiomyopathy?
- Heavy, muscular, hypercontracting heart
- Primary diastolic disorder
- Disproportionate thickening of ventricular septum
- Obstruction to subaortic ventricular outflow tract
- Endocardial thickening and mural plaque formation
Extensive myocyte hypertrophy, haphazard disarray of myocytes, and interstitial & replacement fibrosis are also common.
What is the most common form of cardiomyopathy?
Dilated cardiomyopathy
It accounts for 90% of cases.
What are the causes of Dilated Cardiomyopathy?
- Idiopathic
- Myocarditis (viral)
- Alcohol abuse
- Doxorubicin
- Peripartum
- Genetic (30-40% - dystrophin gene)
It is characterized by progressive cardiac hypertrophy, dilation, and contractile dysfunction.
What are the clinical features of Hypertrophic Cardiomyopathy?
- Atrial fibrillation
- Mural thrombosis
- Embolization
- Infective endocarditis
- Intractable cardiac failure
- Ventricular arrhythmias
- Sudden death
These features pose major challenges in patient management.
What defines Restrictive Cardiomyopathy?
Primary decrease in ventricular compliance resulting in impaired filling during diastole
The contractile function of the left ventricle is usually unaffected.
What are some associated diseases with Restrictive Cardiomyopathy?
- Radiation fibrosis
- Amyloidosis
- Candidiasis
- Metastatic tumor
- Metabolic disorders
The morphology typically shows normal or slightly enlarged ventricles.
What are the major manifestations of Rheumatic Fever?
- Migratory polyarthritis of large joints
- Carditis
- Subcutaneous nodules
- Erythema marginatum of the skin
- Sydenham chorea
Minor manifestations include fever, arthralgia, or elevated acute phase reactants.
What is the pathogenesis of acute rheumatic fever believed to involve?
Hypersensitivity reaction with autoimmune mechanisms proposed
Antibodies against M proteins of streptococci cross-react with tissue glycoproteins in the heart.
What is the Jones Criteria for diagnosing Rheumatic Fever?
Either two major manifestations or one major and two minor manifestations
Major manifestations include migratory polyarthritis, carditis, subcutaneous nodules, erythema marginatum, and Sydenham chorea.
What are the complications of Rheumatic Heart Disease?
- Heart failure
- Arrhythmias (particularly atrial fibrillation in mitral stenosis)
- Thromboembolic complications
- Infective endocarditis
Chronic rheumatic heart disease is characterized by deforming fibrosis and neo-vascularization.
What is the morphology of acute rheumatic heart disease?
- Pancarditis
- Diffuse inflammation
- Aschoff bodies
- Fibrinoid necrosis in valvular cusps
- MacCallum’s plaques in the left atrium
Aschoff bodies are pathognomonic for rheumatic fever.
What is the typical age group affected by acute rheumatic fever?
Children between the ages of 5 and 15 years
About 20% of first attacks occur in middle to later life.
What is the most frequent gene affected in familial Hypertrophic Cardiomyopathy?
B-myosin heavy chain
Other affected genes include troponin T, tropomyosin, and myosin binding protein C.
What is the definition of cardiomyopathy?
Heart disease resulting from a primary abnormality in the myocardium
The three recognized types are Dilated CM, Hypertrophic CM, and Restrictive CM.
True or False: Chronic rheumatic heart disease is characterized by organization of acute inflammation.
True
It leads to deforming fibrosis and neo-vascularization.
