Term
| Best diagnosis method for all cardiomyopathies ? |
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Definition
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Term
| Most powerful risk stratifier for all cardiomyopathies ? |
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Definition
LVEF
<= 30% improved survival with ICD placement |
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Term
| Histo of hypertensive heart disease is ? |
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Definition
| Enlarged myocytes containing hyperchromatic, box-car shaped nuclei |
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Term
| Diff. b/w acute and chronic cor pulmonale ? |
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Definition
Acute cor pulmonale - Due to pulmonary embolism usually. Right ventricle dilated but no hypertrophy
Chronic cor pulmonale - COPD is most common cause, other causes like IPF, cystic fibrosis, and obesity also.
Right ventricular hypertrophy |
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Term
| What disease is it when athletes die suddenly ? |
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Definition
| Hypertrophic cardiomyopathy |
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Term
| Hypertrophic cardiomyopathy is due to what ? |
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Definition
Frequently Autosomal dominant with 55% penetrance by age 30
Genetically due to cardiac sarcomere proteins:
B M C A
Beta myosin heavy chain
Myosin binding protein C
Cardiac troponin T and I (incr SCD)
Alpha-tropomyosin |
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Term
| Histo of Hypertrophic Cardiomyopathy ? |
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Definition
Myocyte hypertrophy
Myofiber dissarya, haphazard branching myocytes
Interstitial fibrosis
Small vessel disease |
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Term
| Can storage disorders (enzyme deficiencies) cause hypertrophic cardiomyopathy ? |
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Definition
Yes
Like Fabry's disease and Pompe's disease and LAMP2 (Danon's disease) and PRKAG2 |
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Term
| 90% of cardiomyopathies are of what type ? |
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Definition
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Term
| Is hypertrophic cardiomyopathy primarily a diastolic dysfunction ? |
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Definition
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Term
| Causes of dilated cardiomyopathy ? |
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Definition
Most of the time idiopathic (unknown)
Genetic mutations in Dystophin gene on X chromosome or mitochondrial genes
Alcohol
Viral myocarditis
Thiamine def. or chronic anemia
Peripartum cardiomyopathy |
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Term
| Histo of dilated cardiomyopathy ? |
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Definition
Myocytes hypertrophied with enlarged nuclei
Interstitial and endocardial fibrosis
Small subendocardial scars replace individual cells, which reflects healing |
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Term
| How does patient with Dilated Cardiomyopathy present ? |
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Definition
Presents like CHF:
Orthopnea
S3 gallop
EF less than 25%
Apex beat displaced
Lung crackles
Death due to CHF or arrhythmias |
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Term
| Causes of Restrictive Cardiomyopathy ? |
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Definition
Least common type of cardiomyopathy
Endomyocardial fibrosis is most common cause
Other causes are:
Loeffler's Syndrome
Radiation fibrosis
Amyloidosis
Hemochromatosis
Metastatic Tumors |
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Term
| Clinical presentation of restrictive cardiomyopathy ? |
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Definition
Loss of ventricular compliance leads to systole not forceful
Exertional dyspnea, fatigue, chest pain, ascites, peripheral edema
Complications are arrhythmias and CHF |
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Term
|
Definition
Arrhythmogenic Right Ventricular Cardiomyopathy
Fibrofatty replacement of RV wall with thinning
Triangle of Dysplasia
Frequent VT in young adults
Ultimately involves LV failure
If survive, progresses to RV failure
Defect in Desmosomal Adhesion proteins
Naxos Syndrome |
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Term
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Definition
Long QT Syndrome
Brugada Syndrome
Catecholaminergic VT |
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Term
| What is Commotio Cordis ? |
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Definition
Sudden death following precordial impact like in a karate match
Impact is directly over heart
Impact timing - T wave upstroke in window b/w 15 - 30 milliseconds prior to apex (K channels may play a role) |
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Term
| Defs of stenosis and regurgitation ? |
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Definition
Stenosis - Failure of valve to open completely, thereby impeding forward flow
Regurgitation, incompetence, insufficiency - Failure of valve to close completely, thereby allowing reversed flow, so blood leaks |
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Term
| Pathogenesis of Rheumatic Heart Disease ? |
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Definition
| Involves cross-reactivity b/w the immune response to Strep cell surface antigens M proteins and antigens on cardiac myocytes and with heart valve glycoproteins |
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Term
| 3 phases of Rheumatic Heart Disease ? |
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Definition
Early (to 4 weeks): Degeneration
Intermediate (4-12 weeks): Granulomatous
Anitschkow Cells - Histiocytes, enlarged, owl-eye nuclei
Aschoff Cells - Multinucleated giant cells, basophilic cytoplasm
Late: Fibrous Healing
Valvular fibrosis leading to stenosis/regurgitation |
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Term
| Most common cause of death in acute rheumatic fever ? |
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Definition
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Term
| Clinical presentation of Acute Rheumatic Fever ? |
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Definition
Pancarditis
Joints - Chronic inflammatory infiltrate and edema
Erythema Marginatum - Maculopapular rash
Skin nodules - contain local lesions that are essentially large Aschoff bodies |
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Term
| "Fish Mouth" (thickening of valve leaflets) and fusion of commissures and chordae tendineae is seen in what disease ? |
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Definition
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Term
| 2 most common valve involvements in chronic rheumatic heart disease ? |
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Definition
Mitral valve alone
Mitral and aortic valve |
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Term
| Characteristics of Mitral Valve Prolapse ? |
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Definition
Most cases b/w 20 and 40 yrs of age
More common in females
May arise as complication of Marfan Syndrome
Midsystolic click - like parachute opening
Chordae tendinae may rupture in severe cases
Increased mitral regurgitation
Most pts are asymptomatic |
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Term
| Histo of mitral valve prolapse ? |
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Definition
| Myxoid Degeneration - Excessive amounts of loose, edematous, basophilic ground substance in valve |
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Term
| What is the cardiac symptom in Carcinoid disease ? |
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Definition
| Right sided, plaque like tricuspid and pulmonary valve fibrosis |
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|
Term
|
Definition
Is for bioprosthetic valves.
Pulmonary valve to aortic valve autograft |
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Term
| What is infective endocarditis ? |
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Definition
Is infection/inflammation of inner lining of heart, usually bacterial
More commonly affects left-sided valves, except for IV drug abusers
Has a lot of predisposing lesions or nidus |
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Term
| Vegetations in infectious endocarditis is made up of what ? |
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Definition
F B I
Fibrin
Bacterial colonies
Inflammatory cells |
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Term
| Two types of infective endocarditis ? |
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Definition
Subacute and Acute (Short incubation)
Also HACEK group of organisms seen in infants and immunocompromised children |
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Term
| In infective endocarditis, organism that is most common in abnormal valves and in i.v. drug abuse ? |
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Definition
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Term
| Gross features of heart in infective endocarditis ? |
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Definition
Bulky, friable vegetations on valve orifice
Rapid destruction of valves due to rupture
** Ring abscess ** - abscesses in perivalvular myocardium |
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Term
| Clinical symptoms of infective endocarditis ? |
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Definition
Acute or subacute symptoms along with
Changing cardiac murmurs
Splenomegaly
Clubbing of fingers
Also may see:
Splinter hemorrhages
Janeway lesions
Roth spots
Osler nodes |
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Term
| Histo of non-infective endocarditis (NBTE nonbacterial thrombotic endocarditis or marantic endocarditis) ? |
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Definition
Small nodules composed of fibrin and delicate layers of aggregated platelets
Minimal inflammation and scarring |
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