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CARDIOVASCULAR PATHOLOGY 3 - Coggle Diagram
CARDIOVASCULAR PATHOLOGY 3
HYPERTROPHY
HYPERTENSIVE HEART DISEASE
common cause of secondary ventricular hypertrophy
other causes are:
diabetes
obesity
congenital heart disease
drug consumption
a long-term condition that develops over many years in people who have high blood pressure
hypertension leads to remodelling of the heart shape, in particular of the left ventricle
types of LV hypertropy
concentric
due to pressure overload
hyperfunction of isometric tyoe due to an increase in downstream resistance
new sarcomeres are formed in parallel
eccentric
due to volume overload
hyperfunction of isotonic type with increased residual volume
new sarcomeres are formed in series
factors influencing LV geometry
severity, duration
volume load
age
race/ethnicity
sex
comorbidities
genetic factors
complications
infarction (starting from the endocardium)
blood clots forming in the lumen and fibrillations
heart failure
also the RV can be involved
due to increased pulmonary circulation resistance or obstacles to the outflow
caused by
diseases of pulmonary parenchyma
diseases of pulmonary vessels
disorders affecting chest movement
disorders inducing pulmonary arterial constriction
complications
chronic pulmonary heart, e.g. cystic fibrosis, extensive emphysema --> pulmonary hypertension
acute pulmonary heart, due to thromboembolic events in the pulmonary arteries
triangular infarction is observed in the case small emboli manage to pass these vessels
MYOCARDIAL NON-ISCHEMIC DISEASES
cardiomyopathies
classification
MOGES classification
Multi-functional phenotype
Organ involved
Genetic inheritance
Etiological derivation
functional Status
pathological classification
primary
only involve the myocardium
acquired, genetic or mixed
secondary
other organs can be involved
autoimmune or secondary to endocrine pathology, e.g. diabetes mellitus, infiltrative diseases, neuromuscular storage disorders, nutritional deficiencies and toxic alcohol CT
MYOCARDITIS
one of the main causes of sudden cardiac death
pathological pattern: inflammation + necrosis
quite frequent and indolent
likely to cause DILATED CARDIOMYOPATHY
gold standard for diagnosis: biopsy of myocardium
Dallas criteria on myocardial biopsy:
inflammation
necrosis of myocytes
in a biopsy, remember to look for:
weight (generally, the heart is heavier)
flabby heart (viral infections)
abscesses (bacterial infection)
sclerosis (sign of infection healing)
when evaluating the histology
look for signs of necrosis, phlogosis
and inflammatory cells
viral infections --> lymphocytes (roundish nucleus)
bacterial infections --> granulocytes (lobulated nucleus)
hypersensitivity --> eosinophils, perivascular interstitial inflammatory infiltrates, sometimes heterogeneous
sarcoidosis --> non-necrotizing granulomas with giant cells, epithelioid cells, fibroblasts
parasites --> eosinophils
very heterogeneous in clinical presentation
on the basis of the first cause of myocarditis
VIRAL MYOCARDITIS
at autopsy, FLABBY HEART
one of the most common causes of unexpected sudden cardiac death (developing into dilated cardiomyopaty if the patient survives)
no specific histologic alterations, thus we should rely on IHC, PCR molecular analysis and immunophenotyping of lymphocytes by using CD3 for T cells, CD20 for B cells and CD68 for macrophages
acute myocarditis
if it evolves towards healing, there may be a scar causing myocardial sclerosis and some CD3+ lymphocytes may be present in the scar
commonly associated with COVID19 infection
BACTERIAL MYOCARDITIS
autopsies show yellow and soft areas (abscesses), concentric hypertrophy of the left side of myocardium
at microscopy, abscesses are observed and they are full of granulocytes, myocardial cells are fragmented and lots of macrophages try to clean the area
TRYPANOSOMA MYOCARDITIS
uncommon
associated with acute or chronic forms
in chronic form, there is the presence of branch blocks, death from arrhythmias and decompensation due to dilated cardiomyopathy
trypanosoma can be identified at the microscopic level
GIANT CELL MYOCARDITIS
unknown etiology
affecting cells with several nuclei
associated with tumors like thymoma, AID, ventricular tachycardia
not benign, as it may progress to fibrosis
TAKOSUBO STRESS-INDUCED MYOCARDITIS
it simulates octopus fishing trap
aka broken heart syndrome
defined by temporary and reversible systolic abnormality of LV in the apical area resembling MI
coronary arteries are not affected
MRI can be used to diagnose dilated CMP
in women, emotional and physical stress can cause it
primary cardiomyopathies
ARRHYTHMOGENIC RV DYSPLASIA
adult prevalence 1:2000-5000
one of the most important cause of sudden cardiac death in young people
genetic form (adhesion molecules alteration)
deletion and thinning of the RV since muscle is substituted by adipose tissue
at biopsy: yellow areas and thinning of the remaining myocardium and papillary muscles
symptoms
fainting
heart palpitations
dizziness
shortness of breath
fatigue
legs swelling
persistent cough
sudden death
major criteria of diagnosis (biopsy)
extensive fibrofatty tissue replacement
with myocardial atrophy (residual myocytes, 60% by morphometric analysis or 50% if estimated)
3 samples must be taken from different regions of the RV free wall
HYPERTROPHIC
genetic, autosomal alteration
prevalence 1:5000
symptoms: shortness of breath, chest pain, changes in heart electrical system, arrhythmia and sudden cardiac death
hypercontractility associated with altered systolic and diastolic activity
diagnosis: echocardiography showing a small heart cavity with hypertrophy of the walls
at biopsy: hypertrophy of muscle which is not regular as cells acquire a Y shape (+fibrosis)
heart weight is >500 g, interventricular septum is >4 cm, asymmetrical hypertrophy involving the anteroseptal part
the mitral valve may get thicker, continuously protruding towards the septum; fibrosis of the subendocardium affected by continuous contact with hypertrophic valve
mitral valve lesion may cause reflux, causing an increase in the LA size, eventually leading to atrial fibrillation (--> flogosis of the auricles)
three different types defined on the basis of the area interventricular septum more severely affected by hypertrophy
basal type
mid-ventricular type
apical type
a LV hypertrophy which is not secondary to other physiological causes like hypertension
RV maybe compressed, looking like a banana
consequences
atrial fibrillation
ischemia, fibrosis
secondary dilation
fainting
sudden cardiac death
in the case od end-stage failur, heart transplantation is an option
LV COMPACTION
spongy form involving the apex of the LV
arrest of the normal embryological development
association with systolic dysfunction and sudden death
typical and non typical
DILATED
no signs or symptoms in the early stage of the disease
prevalence 1:2500
linked to peripartum pregnancy and association with a genetic background occurring after pregnancy
systolic dysfunction, reduced LV ejection fraction, enlarged LV
only therapy: hear transplantation
primary dilated CMP
associated with genetic forms (specific mutations of proteins involved in proper functionality of myocardium and also in musculo-degenerative disorders)
secondary dilated CMP
associated with infective, autoimmune causes, toxicity, drug-induced (CT. e.g. anthracycline)
more than 50 genes encoding for cytoskeletal myocardial proteins can be involved
gross findings
heart can be 2-3 times heavier
main hallmarks: LV dilation and flabby heart
concomitant RV dilation of the heart, resulting in a more roundish shape
mitral insufficiency, resulting in papillary
muscle dysfunction
tricuspid regurgitation associated
with annular dilation
damaged endocardium by blood flow
endothelial cell disruption associated with clots
eventually, ventricular endocardial fibrosis
microscopic
appearance
hypertrophic nuclei
myocytes may not be hypertrophic
staining alterations
eosinophilic areas indicate skeletal muscle cells suffering
RESTRICTIVE
unknown prevalence
either genetic or acquired
likely to be associated with infiltrative diseases, like amyloidosis, or with abnormal storage diseases
endocardium and myocardium are generally involved
the heart is not heavy
LV is rigid and noncompliant
impaired diastolic filling, causing pressure increase
diastolic hypertension can involve pulmonary circulation causing pulmonary hypertension and involvement of the RV with right heart failure
usually there is jugular dilation
complete disarray of the structure
amyloidosis is the most common cause and it can be associated with myeloma; also neuroendocrine tumors seem to be involved with amyloid deposition
infiltration of the ventricle wall
produces a pseudohypertrophy
variants
tropical endomyocardial fibrosis
initiated by fever and gradually leading to heart failure
typical of tropical and subtropical regions
macro: fibrosis of endocardium, appearing wide and opaque; LV fibrosis extends from apex to mitral valve, sometimes involving papillary muscles
micro: trichrome staining with red colour representing muscle and blue colour representing fibrosis
non-tropical endomyocardial fibrosis
children <3 y
incompatible with survival of the newborn
secondary forms are associated with congenital heart abnormalities
micro: endocardial fibroelastic thickening
hypereosinophilic syndrome
aka Loeffler endocardial fibrosis
lots of eosinophilic cells and granulocytes in sub-endocardium
frequently associated with thrombosis
ENDOCARDITIS
non-rheumatic
endocarditis
abacterial thrombotic
aka "marantic" or "terminal"
non-infected vegetations
frequent in cachetic patients
due to deposition of clots on axial surface of mitral, aortic and tricuspid valve
clots are friable and soluble, leading to CNS, lung and coronary embolism
SLE-related
ex-verrucosa atypical of Libman-Sacks
more likely to involve th tricuspid valve
macro: located on the edge of the valve, on tendon cords and in surrounding parietal endocardium
micro: edema, lymphocytes, plasma cells, monocytes, core debris (hematoxynophilic bodies)
infected bacterial vegetation
in acute forms
involvement of healthy valves, due to virulent agents, e.g. Staph., Strep. beta, HACEK
in subacute forms
involvement of alrady altered/prolapsed valves (more commonly aortic and mitral valves), less virulent, caused by Strep. alpha hemolytic
prolonged course, death of the patient due to embolism
septic emboli are the most common complication; in cases of CHD, right-to-left shunt may be present and may lead to paradoxical embolism (treatment leads to restitutio ad integrum, sometimes fibrosis)
in advanced lesions
permanent damage of the valve, associated with valcular insufficiency and heart failure
carcinoid heart disease
likely to produce hormones, e.g. tryptophan, affecting the right endocardium
more frequent in patients with neuroendocrine tumor of the intestine and liver metastases
sometimes, also the lung is involved (thus, the left endocardium)
skin redness, cramps, nausea, vomiting
macro: intimal fibrous thickening of internal surfaces of heart chambers and right valves
micro: smooth muscle cells, collagen and mucopolysaccharide matrix
rheumatic
endocarditis
etiology
group A beta Streptococcus
onset: tonsillitis and angina, fever
3% of pts have damage due to cross immunity protein M Streptococcus and heart glycoproteins
sites: endocardium, myocardium and pericardium
(more commonly, mitral and aortic valves)
acute phase
at the beginning, serous valvulitis
fibroinoid material deposition around the vessels or on the valve surface
micro: Anitschkow-type cells (aka "caterpillar" cells), characterized by an ovoid nucleus; they may coalesce to form multinucleated Aschoff Giant cells; also, granulomas (PATHOGNOMONIC) may be observed
chronic phase
associated with fibrosis
calcifications and pericarditis, fibrinous material deposits
there may be adhesions between 2 layers of pericardium, with the involvement of also the endocardium around the vessels and of the valve
DEGENERATIVE VALVE DISEASE
associated with vegetations formation
it is a degenerative process of valves due to repeated mechanical stress
more likely to involve mitral and aortic valves
the main alteration is fibrosis, associated with calcification and it canbe completely asymptomatic
three types
calcification / stenosis of the aorta:
valsava sinuses are completely filled by carcinoid material --> dystrophic process with progressive accumulation of salts and the valve is stiff; commissures are not fused (as in rheumatoid/infiltrative endocarditis)
--> myocardial hypertrophy, LV hypertrophy and dilation, calcium deposits
--> angina of coronal artery, syncope for cerebral oxygenation
calcification of mitral valve:
due to degenerative lesion, calcification of the annulus may take place, sometimes in a setting of Marfan syndrome; prolapse is seen at microscopy
mitral valve prolapse:
due to mucopolysaccharides within the valve; at microscopy, the valve is thickened and transparent due to the presence of this material
evolution
completely asymptomatic or complicated by heart failure