L23- CVS Pathology IV Flashcards
define systemic hypertensive heart disease and briefly explain pathogenesis
-LV hypertrophy, no evidence of other causes besides HTN
Sustained pressure overload –> GFs + local mechanical effects –> upregulate actin / myosin expression => hypertrophy
HTN LV hypertrophy is considered (concentric/eccentric) and the heart will weigh (2). In long-standing cases (3) is often observed. On histology, myocytes will appear (4).
1- concentric
2- 400-600g
3- RV hypertrophy and dilatation
4- enlarged with large hyperchromatic, rectangular, ‘box-car’ shaped nuclei
describe the clinical features (and progression) of systemic hypertensive heart disease
Early- asymptomatic
- ->
- angina
- signs/sxs of LHF
- ->
- CVA, renal failure (via HTN)
- SCD (sudden cardiac death)
Pulmonary hypertensive heart disease, aka (1), is defined as (2). Note- (3), (4) are excluded from (2).
1- cor pulmonale
2- R sided cardiac chamber disease secondary to pulmonary parenchymal or vascular disease
3- pulmonary HTN due to LHF
4- pulmonary HTN due to congenital heart disease
describe the appearance of acute cor pulmonale
(pulmonary hypertensive heart disease)
-Pulmonary Embolism => sudden inc of burden on RV / R heart
=> RV dilatation (no hypertrophy)
The most common cause of chronic cor pulmonale is (1), but other causes include (2). The R heart will have the following changes: (3).
(pulmonary hypertensive heart disease)
1- COPD
2- idiopathic, pulmonary fibrosis, cystic fibrosis, marked obesity
3- RV hypertrophy, RA hypertrophy and dilatation
(1) is the generalized inflammation of myocardium where (2) is the primary cause to its development and myocardial (3). It is associated with (4) of the myocardium.
1- myocarditis
2- inflammatory process
3- myocardial injury
4- necrosis / degeneration of myocytes
The most common cause of myocarditis is (1), specifically (2) in most cases. Pathogenesis involves the following three steps, (3).
1- viral
2- coxsackie A/B, enteroviruses
3- direct viral cytotoxicity –> cell-mediated immune rxns against infected myocytes –> CKs released aggrevate myocardial dysfunction
describe the gross and histological appearance of viral myocarditis
Gross:
- dilated, flabby heart (or normal)
- cut surface that has patchy pallor and mottling
- possible mural thrombi
Histo:
- interstitial inflammation, mainly lymphocytes, few plasma cells
- focal necrosis of myocytes – adjacent to inflammatory cells
describe clinical presentation of myocarditis and the investigation used in diagnosis
- Varies: asymptomatic – sudden acute HF / arrhythmias
- Nonspecific (mostly): flu-like sxs, fatigue, dyspnea, palpitations, chest pain, fever (may mimic acute MI sxs)
Investigations: PCR for viral nucleic acids, serological studies
list the complications of myocarditis
- acute HF
- dilated cardiopathy –> chronic HF (viral type)
- arrhythmias: ventricular is most dangerous, leading to possible SCD
describe the outcomes of myocarditis
- mostly a self limiting disease (spontaneous resolution)
- if Pt survives acute phase –> inflammation resolves –> healing with total resolution or progressive fibrosis
(majority recover completely)
list the 3 parasites (and theie associated disease) that cause myocarditis
- trichinella spiralis; trichinosis: infects muscle
- trypanosoma cruzi; Chaga’s disease
- toxoplasmosis, toxoplasma gondii
Parasitic Myocarditis, indicate the microorganism:
(1) is most common cause
(2) common in immunocompromised
(3) related to cats
(4) endemic in South America
(5) parasitization of scattered myofibers by it with accompanied mixed inflammatory infiltrate
(6) shows eosinophilic predominance on histology
Trichella Spiralis (trichinosis): 1, 6
Trypanosoma Cruzi (Chaga’s disease): 4, 5
Toxoplasmosis (toxoplasma gondii): 2, 3
(bacterial/fungal/parasitic/fungal) myocarditis is occurs in immuno-compromised individuals, where it is characterized by the presence of (2) in the myocardium
1- fungal (Candida spp.)
2- mixed inflammatory infiltrate: neutrophils, lymphocytes, plasma cells
list the 2 types on noninfectious myocarditis
- immunologically medicated
- giant cell myocarditis
Immunological medicated (noninfectious) is related to (1) diseases and (2) drugs. It is described as (3) in nature and severity and the inflammatory response is composed of (4).
1- SLE, polymyositis
2- (drug hypersensitivies) methyldopa, sulfonamides
3- mild, self-limiting (resolves spontaneously)
4- lymphocytes, macrophages, high in eosinophils
Giant cell myocarditis has a (1) cause and can be associated with (2) diseases. It is dangerous in (3) people as (4) can cause death. Its histological appearance is described as (5).
1- idiopathic (maybe immune origin) 2- SLE, thyrotoxicosis 3- 20-40s people 4- CHF, arrhythmias 5- granulomatous inflammation: giant cells, lymphocytes, eosinophils plasma cells + focal extensive necrosis
define Cardiomyopathy and list the three main categories
-primary myocardial disease excluding ischemia, HTN, valvular lesions, congenital anomalies, inflammatory disorders as causes
1) dilated CM (includes arrhythmogenic RV CM)
2) hypertrophic CM
3) restrictive CM
The most common type of CM is (1), aka (2). It is characterized by progressive cardiac (dilatation/hypertrophy), (concentric/eccentric) hypertrophy, and (diastolic/systolic) dysfunction. It will eventually lead to (6).
1- DCM (dilated) 2- congestive CM 3- dilatation 4- eccentric hypertrophy 5- systolic, contractile dysfunction 6- progressive HF
list the common caused of DCM (hint- 8)
1) idiopathic (most cases)
2) genetic mutations
3) EtOH: direct cytotoxicity
4) Viral Myocarditis (coxackie B virus)
5) Nutritional disturbances: thiamine/vitB1 deficiency, chronic anemia
6) chemotherapy: doxorubicin
7) pregnancy associated / peripartum DCM
8) stress provoked
describe the genetic causes of DCM
(25-35% of cases)
Most common: AD mutations affecting cytoskeleton
Less common: X-linked mutations to dystrophin
Uncommon: mutations of mitochondrial proteins in oxidative phosphorylation
Peripartum CM (DCM) occurs during (1) and is associated with (2). (3)% of cases will recover spontaneously.
1- late gestation to few weeks after delivery
2- volume overload, HTN, nutritional disturbances
3- 50%
DCM will be exhibited in (1) heart chambers. In total the heart will appear (2) with (3) sized walls. There will (4) dysfunction, which can lead to (5), which can lead to (6).
1- all four chambers 2- enlarged, flabby, heavy (2-3x) 3- normal, thin, or thick walls 4- contractile dysfunction 5- mural thrombosis 6- embolization
DCM Histology:
(1) myocyte appearance
(2) interstitial appearance
(3) is also possibly seen due to (4) dysfunction leading to (5)
1- hypertrophied, enlarged nuclei // some thin and stretched out and irregular
2- fibrosis, mainly in endocardium
3- necrosis (myocytes)
4- contractile dysfunction
5- poor perfusion / ischemia
DCM:
- mostly affects (1) individuals
- fundamental defect is (2)
- EF of (3)
- progresses to (4)
- Tx: (5)
1- 20-50 age group 2- contractile dysfunction 3- <25% (50-65% is normal) 4- CHF 5- cardiac transplantation
ARVC, aka (1), is a (2) type disorder in nature / cause. It is characterized by (3) due to (4). (3) can lead to the following progression: (5).
It is associated with a (6) defect and (7) disease.
1- arrhythmogenic right ventricular cardiomyopathy
2- AD inherited
3- thin RV
4- myocyte replacement by fatty infiltration
5- RHF, arrhythmia –> SCD
6- desmosomal adhesion protein
7- Naxos Syndrome
HCM is characterized by (1- describe effect) and (2), and in 30% of cases (3). HCM will present with (4).
1- myocardial hypertrophy –> small LV chamber
2- abnormal diastolic filling
3- intermittent LV outflow obstruction (ant. leaflet of MV)
4- exertional dyspnea or SCD in young adults
HCM is inherited in a (1) fashion and is the result of a (2) mutation in one of the following protein: (3). (2) will result in a (gain/loss) of function leading to (5) progression.
1- AD
2- point mutation
3- [sarcomeric protein] β-myosin heavy chain (most common), myosin binding protein C, cardiac troponin T
4- gain of function
5- myocyte hypercontractility –> inc energy use –> intense compensatory hypertrophy (myofiber disarray) + fibroblast proliferation
HCM will have a (1) appearance on the walls and a (2) appearance of the cavity. (3) is the hallmark feature and (4) is another possible critical feature.
1- thick, massive hypertrophy w/o dilatation
2- banana-like configuration (longitudinal section)
3- Asymmetric Septal hypertrophy (most prominent in subaortic region)
4- endocardial plaque/sclerosis in L outflow tract
HCM histology:
(1) myocyte appearance
(2) myocyte organization
(3) interstitial appearance
1- hypertrophy, diameter >40µm (15µm is normal)
2- myofiber disarray: haphazard arrangement of hypertrophied, abnormally branching myocytes
3- fibrosis
list the clinical features and symptoms of HCM and include its treatment
- exertional dypnea
- angina / MI
- harsh ejection systolic murmur
- SCD via arrhythmia
- CHF
Tx:
i) therapy to relax ventricles
ii) partial septal muscle excision (reduce outflow obstruction)
(1) is the least common type of CM. It is characterized by (2) leading to (3) progression and (diastolic/systolic) dysfunction.
1- Restrictive CM (RCM)
2- stiff walls
3- loss of ventricular compliance –> impaired ventricular filling during diastole
4- diastolic dysfunction OR systolic if systole is not forceful
list the common causes of RCM
- endomyocardial fibrosis (most common)
- Loffler’s syndrome
- radiation fibrosis
- amyloidosis
- hemochromatosis
- metastatic tumors
list the clinical features and complications due to RCM
**indicate Dx requirement
Sxs: exertional dyspnea, fatigue, chest pain
Complications: arrhythmias, CHF
Dx: endomyocardial biopsy
RCM Morphology:
(1) ventricle appearance
(2) cavity appearance
(3) myocardium appearance
(4) atrium appearance
(5) interstitial appearance
1- normal or slightly enlarged 2- not dilated 3- firm 4- both atria dilated 5- patchy variable fibrosis
EMF is a (1) type of CM commonly found in (2) age group and (3) regions. It is characterized by (4) leading to (5) in the affected chambers.
(endomyocardial fibrosis) 1- restrictive CM 2- children, young adults 3- Africa, tropics 4- dense fibrosis of endocardium and subendocardium (from apex to AV valves) 5- reduced volume and compliance
Loefflers Endomyocarditis is a (1) type of CM characterized by (2). It is caused by abundance of (3) in the periphery releasing (4) to damage (5).
1- restrictive CM 2- endocardial fibrosis, with large mural thrombi 3- eosinophils + abnormal degranulation 4- MBP (major basic protein) 5- endocardium --> necrosis, fibrosis