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CARDIOVASCULAR PATHOLOGY 4 - Coggle Diagram
CARDIOVASCULAR PATHOLOGY 4
PERICARDIAL DISEASES
causes
infectious diseases
neoplastic
autoimmune
metabolic
traumatic
drug-related
PERICARDITIS
types
acute:
inflammation of pericardium together with two of the following criteria:
pericardial chest pain
pericardial rubs
new widespread ST elevation or PR depression
4 pathological patterns
serous
generally caused by non-infectious inflammatory etiologies, e.g. RA, which involves all 3 layers of the heart, or SLE, that may involve the valves
there is a little more liquid in the pericardial sac, appearing clear, with no corpuscular particles
fibrinous
the most common form, sometimes evolving from serous pericarditis; a rub can be heard (the 2 pericardial leaflets are in contact)
causes:
early pericarditis folllows acute infarction and it is localized in the visceral pericardium as a response to necrosis
late Dressler typeis due to the presence of autoAbs reactive to post-infarction necrosis and involves the whole pericardium
uremia, radiation
RA, SLE
trauma
severe inflammation
routine cardiac surgery
post-cardiac surgery is a cause of serous fibrinous pericarditis due to post-myocardial infarction, Dressler syndrome or post-pericardiotomy syndrome
uremic pericarditis is not so common (preventable by dialysis); it is due to toxic metabolites (nitrogen waste) and interleukins release
1 more item...
hemorrhagic
blood mixed with a fibrinous or suppurative effusion
causes:
neoplastic invasion
break of the myocardium, following an infarction leading to a tamponade
caseous
due to TBC, associated with necrotizing granulomas and caseous necrosis
similar to sarcoidosis: same epithelioid cells and giant cells (look for necrosis for DD)
purulent or suppurative
several bacteria are involved; the liquid is yellow, full of granulocytes and bacteria
sometimes it can be sub-pericardial (microabscesses in the sub-pericardial serosa due to emboli from infective endocarditis)
evolution of acute pericarditis
chronic
more than 3 months
"constrictive pericarditis"
thick white pericardium is observed
with possible visible calcifications
layers are stuck together and the heart cannot fully dilate due to constriction
similar symptoms to restrictive
CMP, but different causes
sometimes, mediastinum may be involved
suppurative / caseous pericarditis or irradiation
incessant
more than 4-6 weeks but less than 3 months without remission
recurrent
associated with recurrence of pericarditis AFTER a documented episode & symptom-free interval for 4-6 weeks or longer
PERICARDIAL EFFUSION
the pathologist must look at cells to see if it is serous (no cells), reactive (inflammatory cells, e.g. lymphocytes), neoplastic or hematic
etiology
idiopathic
infectious
connective tissue diseases (SLE, SS)
pericardial injury syndrome (trauma)
metabolic causes (uremia, hypothyroidism)
medication-induced
sarcoidosis
allergic syndromes
tumors (lung and breast cancer!!!)
in the presence of metastases, the serosal layer is generally involved; in the case of a mesothelioma, both pericardial layers can be involved
IHC can help differentiate mesothelial reactivity (induced by any injury, like in the pleura in pneumonia) from neoplastic (mesothelioma)
mesothelial cells:
WT1, D2-40, calretinin, CK5/6, HEG1
BAP1 is a tumor-suppressive gene found
in the nucleus; it disappears in mutations
associated with mesothelioma
antibodies used for DD
cytokeratins used to check for intestinal/lung sources:
CD20 - in mesothelioma, + in intestinal carcinoma,
CD7+ and CD20- in lung cancer,
calretinin- in primary tumors,
TTF1+ in lung cancer (typical antigen of the thyroid)
CEA: - in mesothelioma, + in carcinoma
in the case of colon cancer primary tumor:
CD7- in cancer cells
TTF1-
CK20+ in the cytoplasm
CDX2+ (SPECIFIC MARKER)
metastases may affect the whole serosa and, when associated with intestinal cancers, it develops into carcinoids
many small islets of cancer cells
everywhere in the serosa
CARDIAC TUMORS
primary
very rare, mainly benign
RHABDOMYOMA
more common in children
associated with tuberous sclerosis
and certain somatic gene mutations
(TSC1, Ch9, TSC2, Ch16)
localized in LV and septum
macro: roundish nodules
micro: large vacuolated cells
containing myofibrils
symptoms related to tuberous sclerosis:
epilepsy, convulsions, mental delay,
sebaceous adenomas, periungual
fibroids, hypochromic skin, spots
MYXOMA
rare
likely to give rise to emboli
in echocardiography: complete
obstruction of the atrium, because it
arises into the atrial cavity
soft and gelatine-like substance,
white and transparent
a possibility of neoplastic embolism
needs to be considered
secondary
metastases on the serous pericardium also
affects the myocardium; malignant / benign
likely to originate from melanomas (look
for melanoma IHC markers to exclude it)
other primary:
bronchial carcinoma
breast cancer
multiple myeloma
lymphoma, leukemia
CONGENITAL HEART DISEASES
definition
abnormalities of the heart and large vessels present at birth due to embryological alterations between the 3rd and 8th week of IU life
causes
genetic causes: chromosomes 13, 15, 18, 21 (Down syndrome and Turner syndrome)
mutations of transcription factors
rubella
epigenetic causes: simple/complex, isolated/associated, syndromic/nonsyndromic
findings
SHUNTS
abnormal connection allowing blood to move from one side to another without the normal efflux of the blood
L --> R: blood is oxygenated
late cyanosis and
lung hyper-afflux
atrial defect
in fetal life, there is a hole between the atria, causing R-->L shunt; at birth, placental circulation stops and lungs start to work: the pressure in the LA increases and the shunt becomes L-->R if the two holes persist
the two holes are:
foramen ovale (atrium-atrium)
Botallo duct (aorta-pulmonary vein)
patency of the Botallo arterial duct
it is between the aortic arch and the pulmonary artery and it mostly affects premature babies (F>M) between the 1st 10-15 hours of life
symptoms:
continuous murmurs with a locomotive sound
premature infants may have respiratory distress or other serious complications (e.g. necrotizing enterocolitis)
in large shunts: pulmonary artery hypertension, high pulmonary vascular resistance, Eisenmenger's syndrome
ventricular defect
not so rare
the most frequent CHD
located at specific sites of septum
(90% --> membranous septum),
showing Swiss cheese appearance
with many small holes
associated with other alterations
(e.g. Fallot's tetralogy)
isolated in 30% of cases
R --> L: blood is not oxygenated
early cyanosis
and hypo-afflux
tetralogy of Fallot
(Blue baby syndrome)
defect of the interventricular septum of myocardium
overriding of the aorta
pulmonary stenosis
RV hypertrophy: secondary effect, due to the increase in pressure of pulmonary artery
--> arrhythmogenic arrhythmia is caused by RV hypertrophy due to substitution of muscle with adipose tissue
forms
pink tetralogy: pulmonary stenosis is not complete
classic tetralogy: the baby is blue with
severe pulmonary artery stenosis
transposition of the Great Vessels
either an atrioventricular discordance (inappropriate connection of RA to LV) or ventriculoarterial discordance (transposition of great vessels)
RV hypertrophy
LV hypoplasty
the baby survives if there is an atrial / ventricular septum defect or patent ductus arteriosus
tricuspid atresia
complete disclosure of the right atrioventricular orifice
high mortality in the first weeks/month because of the obstruction of bloof flow
rarely, there may be a bidirectional shunt
OBSTRUCTIVE ANOMALIES
aortic coarctation
child form
tubular hypoplasia of t he aorta
symptoms +++
impossible to survive without cure
adult form
lots of collateral circulations develop
hypertension of the upper parts
hypotension of the lower areas
aortic stenosis and atresia
alteration of the valve cusps sometimes associated with Patent Botallo duct, allowing coronary revascularization (if closed --> fatal)
in stenosis, sub- or supra-valvular membranes could be present in the valve that partially closes the outflow of blood from the left ventricles
ascending aortic dysplasia with wall thickening due to elastin gene mutation >> lumen narrowing
pulmonary artery stenosis and atresia
isolated or associated with Tetralogy of Fallot
RV hypertrophy
if completely atretic, the flow is guaranteed via the PDA
TRANSPLANT PATHOLOGY
indications
adults:
CAD, non-ischemic CMPs
children:
CHD <1y, CMP
pathophysiology
primary graft dysfunction
the heart is transplanted but there is a rejection
acute rejection may be cellular (T cells) or humoral (antibodies), causing capillary endothelial changes
key elements
myocyte damage
lymphocyte presence
grading:
Grade 0: no rejection, absence of inflammatory infiltrates
Grade 1R, mild: interstitial and/or perivascular infiltrate with up to 1 focus of myocyte damage
Grade 2R, moderate: >2 foci of lymphocytic infiltrates wih associated myocyte damage
Grade 3R, severe: diffuse lymphocytic infiltrate with multifocal myocyte damage, +/- edema, hemorrhade or vasculitis
secondary graft dysfunction
it results from hyperacute rejection and it is either due to AB0 incopatibility or from pre-formed cytotoxic antibodies directed against MHC antigens
biopsy before transplant
(from the RV septum)
basal biopsy in the donor
in the recipient
1 biopsy every week during the 1st month
1 biopsy every 2 weeks during the 2nd month
1 biopsy every 6-7 weeks from 3rd to 7th month
cardiac allographic vasculopathy
immune- or non immune-mediated
likely to affect all the other organs
it may begin as early as 3 months after transplant
it can be found in 7-y-pts with DM or intracranial hemorrhage
no symptoms like angina (the transplanted heart is denervated)
micro: no atherosclerosis and no calcium, rather hyperplasia of the fibromuscular intima, concentrically hyperplastic
simulators of rejections
Quilty effect:
it refers to the presence of lymphocytic infiltrates in the subendocardial area, while the muscle is not generally infiltrated; consider that is may be an alteration caused by mechanical biopsy
Ischemic damage:
it may be difficult to distinguish from necrosis, due to rejections; they are more frequent in biopsies from the 6th week after transplant; C4d is the marker used to detect necrosis
Alterations from biopsy site and thrombotic material:
when the biopsy is performed always in the same spot, fibrotic and scar tissue start showing sclerosis and granulation tissue
Infections:
these patients are under cortisone!!!
Common pathogens: Cryptococcus, CMV, Toxoplasma, opportunistic fungi
Lymphomas:
they are B lymphomas, both CD20+ and EBV+; also think of lymphoma in the presence of monoclonal atypical lymphocytes