Pathology Cardiovascular

Cardiovascular

Pathology

inflammatory

heart diseases

Semmelweis University

2nd Department of Pathology

_______ _______

2014/2015 Autumn Semester

Tibor Glasz MD PhD

_______ _______

Inflammatory cardiac diseases ____________________ _____________________

Inflammatory heart diseases

- Endocarditides - parietal

- valvular

- Myocarditis

- Pericarditis

Pancarditis

Endocarditides

The infective endocarditides

- Risk groupes: ~ rheumatic or degenerative valvular deformities ~ congenital valvular vitia

~ valvular prostheses

~ arterial long-term catheter

~ intravenous drug abusers (15% of cases,

here: localisation typically tricuspidal!)

- Infective agents: ~ almost always bacteria (Staphylococcus auerus, Streptococcus viridans, Gonococci,

Enterobacteria; in immunodeficiency: so-called

opportunistic bacteria)

~ seldom fungi (in immunodeficiency /AIDS/ and

iv. drug abusers)

- Clinical forms:

~ acute: sudden beginning with high fever and septic crisis > despite antibiotics mortality very

high

~ subacute (endocarditis subacuta infectiva/lenta):

begins inconspicuously with uncharacteristic

systemic symptoms (weakness, fever, weight loss)


- Morphology: the same in both forms: ~ valvular vegetations along the closing lines of the

valves: small, finely granular to gross polypoid, stenosing

~ the material of the vegetations may harbour large

amounts of infective agents and is highly friable > danger

of embolism > formation of metastatic abscesses

~ valve destruction (endocarditis ulcerosa) through

necrosis, ulceration, thrombotic deposits > sacculation

(so-called valvular aneurysm) and rupture > sudden valve

insufficiency

~ extension of tissue destruction to neighbouring parts of

the aorta or myocardium > so-called paravalvular abscess,

paravalvular leak > cardiac/circulatory catastrophy!

~ in severe cases involvement of the cordae and parietal

endocardium is also possible


Photoarchive of post-mortem documentation;

2nd Department of Pathology,


Destructive infective subacute endocarditis of the aortic valves with

paravalvular abscess and septation within abscess. Risk factor to this

had apparently been a degenerative calcifying valvular deformity.




Destructive infective subacute endocarditis of the aortic valves

with paravalvular abscess. Risk factor to this had apparently

been a degenerative calcifying valvular deformity.




Ulcerative

endocarditis

(formerly known as

endocarditis

ulcerosa maligna)

with massive

vegetations,

complete tissue

demolition,

paravalvular leak

and involvement of

the parietal

endocardium. The

severe valvular

insufficiency

resulted in a

rounded dilatation

of the left ventricle.

Collection of the Museum of Pathology;



Ulcerative endocarditis of the aortic valves with massive valve destruction,

valvular insufficiency and excentric myocardial hypertrophy




Thrombopolypoid subacute endocarditis of the mitral valves

with antecedent degenerative calcification of the valve base

Ulcerative and perforating endocarditis of the mitral valves




Thrombopolypoid

subacute

endocarditis of the

mitral valves. The

cordae are

severely

thickened,

referring to

recurrence of

valvular disease.

Photoarchive of post-mortem

documentation;



- Clinical presentation: ~ Schottmllers triad (endocarditis ulcerosa;

splenic infarctions; embolic focal nephritis)

~ Oslers nodule: lividity, swelling and tenderness of the

periungual finger areas / distal phalanges through

(micro)emboli

~ sepsis

- Endocarditis of valvular prostheses: ~ vegetations along the sutures of the prostheses:

paravalvular abscess and leak


- Non-bacterial thrombotic endocarditis (formerly known as Endo-carditis marantica)

~ sterile, small vegetations of fibrin and thrombocytes

in the closing line of valves

~ in the background there is often enhanced blood-

clotting in severely diseased patients with bad general

status (terminal tumour disease, chronic renal failure,

chronic sepsis)

- Liebman-Sacks endocarditis (SLE-endocarditis) ~ 1-4 mm large, verrucous vegetations in the closing line

and on the undersurface of valves

~ fibrinoid necrosis, fibrosis, valvular deformity and

vitia are possible

~ histologically demonstration of so-called hematoxyphil-

bodies

Special forms of endocarditides /

non-infectious endocarditides

- Carcinoid-endocarditis ~ tumour site in the GI (appendix, duodenum)

~ primarily alterations of the tricuspid valves

~ vitrous-firm thickening of the valve cusps

~ similar alterations of the pulmonary semilunar valves, the

endocardium of the right ventricle and in the pulmonary

trunk possible

~ cause: high blood titer of tumour products: Serotonin,

Kallikrein, Bradikinin, Histamine, Prostaglandins,

Tachikinins)

- Accompanying endocarditis ~ as with acute myocardial infarction

~ typically a parietal localisation



Vitrous-firm thickening of the tricuspid valves

with carcinoid-endocarditis




Accompanying endocarditis with

chronic myocardial infarction




- Endocarditis syphilitica/luetica ~ extension of a luetic aortitis onto the aortic valves

~ valves thickened and firm, with insufficiency in the later

phase

- Endocarditis in rheumatoid arthritis (primary chronic polyarthritis PCP)

~ alterations similar to those seen with luetic valvulitis



Myocarditides

Myocarditides

- Clinical presentation: ~ presentation with slight symptoms or even without

any symptoms whatsoever possible

~ sometimes causes sudden progression into heart failure

or arrhythmias > sudden death syndrome

~ all age groups can be affected, most frequently in young

adults

- Classification: ~ referring to pathogenesis: infectious; non-infectious;

idiopathic

Infectious myocarditides

- Viral myocarditides:

~ Coxsackie A, B; Influenza; Echovirus; EBV; HIV; CMV

- Bacterial myocarditides:

~ Diphteria; Leptospira; Meningococci; Borrelia (Lyme-

disease)

- Protozonal myocarditides:

~ Trypanosoma (Morbus Chagas); Toxoplasmosis

- So-called specific myocarditides:

~ rheumatic fever; tuberculosis; syphilis

Non-infectious myocarditides

- Physical myocarditides:

~ irradiation therapy (ionising radiations); electric shock

- Chemical myocarditides:

~ heavy metals; drugs (cytostatics, Sulfonamides,

Penicillin)

- Post-streptococcal myocarditides:

~ as a component of rheumatic fever

- Transplantational myocarditis:

~ during rejection reaction

Idiopathic myocarditides

- Giant cell myocarditis

- Fiedlers myocarditis

- Sarcoidosis

Morphology

- Macroscopy: ~ loose and flabby dilation of the ventricles

~ patchy cut surface of the myocardium with sporadic

small foci of hemorrhages

~ dilation of the atrioventricular ostia > relative

insufficiency of valves

- Microscopy: ~ important: the primarily diseased structure is the

interstitium!

~ interstitial edema with lymphocytic, plasmacellular,

histiocytic, mastocytic infiltration

~ fibroblastic proliferation > interstitial fibrosis

~ cardiac muscle cell damaging (myocytolysis,

microinfarctions) are only secondary

http://images.md

Fatal myocarditis in a child

http://images.md

Interstitial lymphocytic infiltration

in viral myocarditis

Pericarditides

Acute pericarditides

- Fibrinous pericarditis (pericarditis sicca): ~ most frequent form of pericarditis: macroscopically cor

villosum (hairy heart) on auscultation: friction noise in

rhythm of heart beatings

~ accompanying pericarditis with acute myocardial

infarction (so-called pericarditis epistenocardiaca)

~ viral pericarditides Coxsackie A, B; HSV; Influenza

(symptomatically leading sign is thoracal pain, so it is a

diagnostic problem to differentiate from an acute

myocardial infarction!)

~ uremia

~ rheumatic fever (pancarditis rheumatica)

~ autoimmune diseases (PCP, SLE)

~ iatrogeneous pericarditis (after pericardiotomy)

- Serous pericarditis: ~ an infrequent form: in polyserositis, pericardial

carcinosis (pericarditis carcinomatosa)

- Purulent pericarditis: ~ bacterial or fungal infection

~ extension from neighbouring structures: pleural

empyema, lobar pneumonia, infectious endocarditis,

myocardial abscess

~ extension from distant infection through blood stream

(sepsis)

~ iatrogeneous: after cardiosurgery (rare)

~ severe form: pericardial sack is filled with pus

(empyema pericardii)

- Chylous pericarditis: ~ with metastatic tumour disease

Acute pericarditides

Fibrinous pericarditis




Subacute purulent

pericarditis: the

pericardial space

is filled with

massive

exsudation of

fibrinous-purulent

character. Partial

septation of the

pericardial mass is

already evident.




Fibrinous pericarditis: aspect of the

so-called cor villosum (hairy heart)




Chronic pericarditides

- Constrictive pericarditis (concretio pericardii): ~ organisation of unresolvable exsudates as result of an

acute pericarditis

~ stricture of the orifices of the large (primarily venous)

vascular trunks through pericardial scarring > severe

circulatory failure

- Adhesive mediastino-pericarditis (accretio pericardii): ~ adhesive fibrosis of the parietal myocardium to/with

mediastinal structures

- Posttuberculotic pericarditis: ~ organisation of massive caseous exsudation

(pericarditis tuberculosa caseosa) with formation of a

partly calcified pericardial fibrosis (so-called Panzerherz)

Rheumatic fever _________ ___________

The rheumatic fever

- Definition: an acute, immunopathogenic, systemic, non-purulent

inflammation, that represents disease of the complete mesenchymal

system of the body and so, belongs to the so-called collagen

diseases.

- Pathogenesis: Cross reaction against own structures (connective

tissue ground substance) a few weeks after pharyngitis caused by

Sterptococcus -haemolyticus A

- Typical symptoms: (a) high fever

(b) carditis

(c) wandering polyarthritis

(polyarthritis rheumatica/migrans)

(d) subcutaneous rheumatic nodules

(e) erythema marginatum of the skin

(f) chorea minor

The rheumatic heart disease

- valvular endocarditis

- myocarditis

- pericarditis

rheumatic pancarditis

The rheumatic endocarditis

- acute phase: edema of the heart valves rich in mucopolysaccha-

rides with friable, soft, wipable, reddish vegetations, 1-2 mm large,

along the closing line of the valves, made of masses of thrombocytes

complete regression growing of capillaries into the valve tissue

- transition into a chronic rheumatic endocarditis:

- organisation of the vegetations

- scarring, gross calcification of valves

- deforming, conflusion of valvular commissures

- formation of rheumatic vitia

Thrombopolypoid acute

rheumatic vegetations on the

mitral valve cusps




- Topography: Mitral valves 70-75% Mitral- and aortic valves 25%

Tricuspid- and pulmonary valves very rarely

- Pathophysiology: transition in a chronic rheumatic heart disease lasts long (5-30) years

probability for a chronic heart disease after an

acute rheumatic fever is quite variale: 18-65%

rheumatic fever shows a tendency to recur: the

cardial alterations will be after each recurrence more

severe: endocarditis rheumatica recidivans/recurrens

cardiac valve anomalies (e.g. congenital

bicuspidy) enhance the risk for rheumatic valve disease

The rheumatic endocarditis

Congenital bicuspidy

http://images.md

The rheumatic myocarditis

- Pathologic forms: acute Aschoffs nodules chronic fibrosis

- Aschoffs nodules:

small perivascular foci of inflammation gathering of lymphocytes, macrophages

and plasmacells

fibrinoid necrosis and degeneration of

collagen possible

+

Anitschkovs cells are histiocytes with

gathering of chromatin substance in the centre of

the nucleus: owls eye pattern

Aschoffs multinuclear giant cells result from

confluence of Anitschkovs histiocytes




Macroscopic aspect of

rheumatic myocarditis with

nodular patchiness of the

cut surface and foci of

interstitial hemorrhages

http://images.md

Aschoffs nodules http://images.md

Anitschkovs histiocyte

Aschoffs giant cell

http://images.md


http://www-medlib.med.utah.edu/WebPath

http://images.md


The rheumatic fever - Rheumatic granulomes (Aschoffs nodules):

- in the pericardium (pericarditis rheumatica)

- in joint capsules and periarticular soft tissues

(polyarthritis migrans) spontaneous regression

- Subcutaneous rheuma nodules:

multiple, 5-10 mm large nodules in the region of the

affected joints (e.g. around the olecranon, patella)

spontaneous regression

- Chorea minor: unvoluntary, jerky, excessive motion of the

extremities resulting from an encephalitis of the

extrapyramidal system, typically in childhood

and somewhat more frequently in girls spontaneous

regression

The clinical appearance of rheumatic nodules

http://images.md

http://images.md

The clinical appearance of rheumatic nodules

Chorea minor

http://images.md

Unvoluntary, jerky

movements of the

extremities

Hand writing of a patient

before... and after

spontaneous regression. (The actual status of the hand writing is applicable

for monitoring the general state of the disease.)

the rheumatic heart alterations can have however dramatic

complications:

- Complications: acute congestive cardiac insufficiency > death

valvular vegetations > systemic

embolisation

chronic valve vitia

further periods of infectious

endocarditides (acute recidivations)

The rheumatic fever

Vitia __ __

Vitium cordis

- Definition: morphologic and functional heart valve disease

- Forms: stenosis > < insufficiency

~ both forms result in a pathophysiologic overload

of the heart (stenosis pressure overload/hypertrophy;

insufficiency volume overload/hypertrophie)

~ until the heart is capable to balance pathologic

overload through use of its reserves, the vitium is

compensated

~ as soon as reserve capacities of the heart are

exhausted, the vitium will be decompensated

~ in stenosis dominates a muscular hypertrophy

~ in insufficiency dominates a ventricular dilatation

Mitral valve vitia

- Insufficiency:

~ left ventricle and atrium are dilated and slightly

hypertrophic

~ pulmonary congestion results later in a excentric

right heart hypertrophy/failure

- Stenosis:

~ the left ventricle is underloaded, so slightly atrophic

~ the left atrium is on the contrary distinctly dilated

and hypertrophic

~ in severe cases of mitral stenosis results the

pathomorphology of a fishmouth stenosis (german:

Knopflochstenose buttonhole stenosis )

Fishmouth stenosis of the

mitral valves with severe

atrial dilatation




Aortic valve vitia

- Insufficiency:

~ excentric left ventricular hypertrophie

- Stenosis:

~ primarily concentric hypertrophy of the left

ventricle, which turns excentric as decompensation

supervenes

~ backward pulmonary congestion and right heart

failure develops only later

~ a similar situation is seen in hypertonic heart

disease

Degenerative valvular base calcification and combined

aortic valve vitium (stenosis + insufficiency)




Most severe degenerative

aortic vitium (stenosis)

with a residual lumen of a

few millimeters of the

original aortic transsection

surface




Infrequent form of a valve vitium: valvula fenestrata. The hemodynamic effect of this

alteration is shown by the left ventricular hypertrophie.




Acute valvular insufficiency (vitium cordis) in infective endocarditis




Complications of the vitia

- Cor pulmonale chronicum

~ secondary overload on the right ventricle resulting from backward congestion from the diseased left heart: right

ventricular dilatation and severe muscular hypertrophy

- Relative valvular insufficiency ~ severe ventricular dilatation is followed by expansion of

the atrioventricular orifice, so the valve cusps are no more

capable of covering the enlarged lumen surface

- Ball thrombus

~ spherical, free-floating or to the wall loosely adherent thrombus in the dilated atrium or auricula

- Induratio brunea pulmonum (brown induration of the lungs) ~ in chronic left heart failure develops long-term

congestion and relative hypoxia of lung tissue

~ heart failure cells and interstitial fibrosis

Valvular surgery in case of vitia: implantation of valvular prostheses





2nd Department of Pathology

_______ _______

2014/2015 Autumn Semester

Tibor Glasz MD PhD

_______ _______

Cardiovascular

Pathology

- further aspects -

Cardiomyopathies ____________ ____________

Cardiomyopathies

Definitions:

- primary (idiopathic) cardiomyopathies: progressive myocardial diseases

of unknown origin that after variously long periods lead to a therapy

resistant circulatory insufficiency.

- secondary cardiomyopathies: progressive, diffuse myocardial diseases, that may be identical to the idiopathic forms in their clinical and pathologi-

cal presentation, yet can be derived from a detectable origin.

Therapy is possible only by heart transplantation.

Primary (idiopathic) cardiomyopathies

The following 3 groups are defined according to basic clinico-pathological

differences:

(a) dilatative (congestive) cardiomyopathy

(b) hypertrophic (obstructive) cardiomyopathy

(c) restrictive (obliterative) cardiomyopathy

Dilatative/congestive cardiomyopathy (DCM) - Morphology -

- morphologic criteria: severely enlarged heart (weight sometimes 3 times

the normal cor bovinum) with extremely dilated, ball-shaped ventricles,

rounded apex, from basis to apex progressively thinning wall and parietal

thrombi.

- further macroscopic alterations: atrial thrombosis; myocardium loose, patchy-fibrotic, pale; valves secondarily and relatively insufficient.

Coronary arteries and valves morphologically intact!

- microscopy: no diagnostic alterations, only signes of a muscular hypertrophy (enlarged muscle fibers and nuclei) and secondary signes of a

relative coronary insufficiency (myocytolysis, microinfarctions-microscars,

interstitial fibrosis, single fiber necroses)

Postmortem Photo Archive of the 2nd Dept. of Pathology;


Dilatative

cardiomyopathy.

Note the rounded

ventricle with

local endocardial

thickenings

representing

organized

remnants of

former parietal

thromboses.

- presentation in all age-groups, yet, most frequently in the young

- appearance sporadic, only seldom familiar (here genetic background possible),

sometimes molecular biologic traces of enteroviral genom detectable (viral

myocarditis in the anamnesis?)

- the clinical picture is that of a slowly developping, therapy resistant circulatory

insufficiency

- begins slowly, lingering over the years with atypical complaints, the diagnosis is

established generally in the stage of the circulatory insufficiency

- the end-diastolic volume increases progressively, the ejection fraction decreases

- leads in 5-10 years to death

Dilatative/congestive cardiomyopathy (DCM) - Clinical aspects -

- cardiac muscle contractility is secured by the strength of the sarcomeric

contraction as well as by its transmission from sarcomer to sarcolemma and further

to the extracellular matrix

- the connection between sarcomer and sarcolemma is given by the dystrophin-

sarcoglycane proteincomplex

- certain mutations of the dystrophin gene (on the X-chromosome) lead to selective

absence of the dystrophin in the myocardium (>>DCM) but not in the skeletal

musculature (e.g. no Duchenne-Beckers muscle dystrophy)

- mutation of the -sarcoglycane gene >> DCM

- mutation of the distal part of the myocardium-specific actin (contacting part

between actin and dystrophin with the help of a protein named desmin) and

mutations of desmin >> DCM

- further mutations of e.g. binding structures between neighbouring muscle cells, or

that of the energy production can lead to DCM

Dilatative/congestive cardiomyopathy (DCM) - Molecular characteristics -

Hypertrophic/obstructive cardiomyopathy (HCM) - Morphology -

- macroscopically: severely enlarged heart (weight sometimes 1000g cor

bovinum) with a disproportionate left heart hypertrophy especially at the

septum >> decreased ability to dilatation (compliance) and stenosis of the

way leading out from the ventricle with cardiac insufficiency >> hence

synonym terms: asymmetrical septal hypertrophy (ASH); idiopathic

hypertrophic subaortic stenosis (IHSS)

- microscopically: a diagnostic picture: (a) extreme hypertrophy of the

muscle fibers; (b) enlarged, bizarr nuclei with pale perinuclear rim (halo);

(c) very typically irregular-chaotic, syntitially woven fiber connections:

beside normal end-to-end connections there are end-to-side and side-to-side

fiber connections. This chaotic micromorphology explaines the clinicallly

often experienced cardiac arrhythmias.

- ethiology and pathogenesis unknown, the genetic background is

however proven (HCM is an inherited disease)

- clinical symptomes appear only around the 30th year of life

- first angina and dyspnoe on body excersize

- conduction abnormalities are often seen (arrhythmias)

- sudden cardiac death is possible

- with the disease at end stage, there is a therapy resistant cardiac

insufficiency

Hypertrophic/obstructive cardiomyopathy (HCM) - Clinical aspects -

- generally it is a familiar disease with autosomal dominant

inheritence and varying penetrance

- rarely sporadic appearance through de novo mutations is possible

- HCM is a disease of the sarcomer: as well the thick (myosin) as the

thin (actin, tropomyosin, etc.) filament genes may be affected

Hypertrophic/obstructive cardiomyopathy (HCM) - Molecular characteristics -

Restrictive/obliterative cardiomyopathy (RCM) - General comments -

- a rare disease

- important is the restricted ability of the heart ventricle to dilate (reduced

diastolic filling)

- the ventricle is capable neither of contracting nor of expanding to the

desirable degree

- the combined systolic and diastolic derangement leads to cardiac insuffi-

ciency

- the disease is generally detected very late, in the stage of cardiac insuffi-

ciency

- according to classic understanding basis of the disease lies in the parietal

endocardium, namely (a) an endocarditis parietalis fibroplastica secundum

Loeffler; or (b) an endomyocardial fibrosis

- in the background there is a severe peripheral and interstitial

eosinophilia (sometimes even an eosinophilic leukemia)

- it is a lethal disease

- the atypical, degranulated, circulating eosinophils cause endo-

myocardial necrosis by their toxic substances >> thickening and

scarring of the endocardium and the subendocardium >> formation

of parietal thrombi >> organisation of thrombi >> the very rigid

endocardium leads to myocardial motility derangements

Restrictive/obliterative cardiomyopathy (RCM) - Endocarditis parietalis fibroplastica secundum Loeffler -

- endocardial changes as with Loefflers endocarditis, yet without an

eosinophylia

- it is most frequently seen in the first 2 years of life, in adults rare

- the proliferating connective tissue that thickens the parietal

endocardium infiltrates also into the subendocardial myocardium

- prognosis depends on dimensions of the disease: focal endocardial

thickenings can remain symptomless, whereas a diffuse disease leads

quickly to cardial decompensation and death

Restrictive/obliterative cardiomyopathy (RCM) - Endomyocardial fibrosis -

Museum of Pathology;

2nd Dept. of Pathology; Semmelweis University

Restrictive

cardiomyopathy

of a new-born.

Note thickened

left-ventricular

endocardium.

Secondary cardiomyopathies

- diffuse myocardial diseases of known origin >> important, that with the therapy

of the causative circumstances also the cardiac status gets relief or will even be

cured

- (a) alcoholic cardiomyopathy the most frequent cause, that leads to a dilatative

cardiac disease. No coronary sclerosis. First symptoms are arrhythmias without

congestive signes. Beside a normal coronarogram angina pectoris is possible. At

the beginning the developping heart insufficiency can be reversed by alcohol

abstinence and specific supportive cardiotherapy. With continued alcohol abuse an

irreversible circulatory decompensation will follow. Cause of death is often

embolisation from parietal thrombi.

- (b) peripartal (pregnancy-linked) cardiomyopathy in the 3rd trimester of

pregnancy or within 6 weeks after birth. Disease characteristics as with a

dilatative cardiomyopathy. Specific therapy makes a complete recovery possible.

- (c) Hemochromatosis genetic derangement of iron uptake and -stockage. Iron

reserves appear pathologically also in parenchymal cells causing functional

alterations in many organs: liver, pancreas, heart, skin, etc. The clinical

appearance of the heart disease that of a DCM. Later the myocardium develops

progressiv rigidity through accumulating iron contents, so the clinical picture turns

into one resembling a RCM. Macroscopically the myocardium is stiffened, dark

coloured. Microscopically the muscle fibers are massively overloaded with iron

containing hemosiderin pigment (positive Prussian-blue reaction).

- (d) Amyloidosis the amyloid protein is deposited in the myocardial interstitium

and in small vessels >> thickening of myocardium all over the heart (especially in

the left ventricle). A cardiac insufficiency with lung edema and systolic functional

decrease develops typically in an unexpected, abrupt manner. Myocardium stif-

fened and rigid with a waxy-glassy hue on the cut surface >> the clinical presen-

tation is that of a RCM. Microscopically amyloid is seen as a homogenous eosino-

phylic material (congo red staining positive).


- (e) Sarcoidosis in 8% of patients with sarcoidosis also cardiac disease

develops. Sarcoidotic granulomas appear in the pericardium and

myocardium (most frequently in the upper third of the interventricular

septum and in the papillary muscles). The clinical picture is dominated by

arrhythmias. The overall picture as with RCM. Arrhythmias may lead to

sudden cardiac death.


Cardiac decompensation _________________ _________________

Cardiac decompensation - General comments -

- it is the end stage of severe heart diseases; prognosis bad

- the clinical progression is defined by the basic disease, age, overall status and

other factors (e.g. social status of the patient)

- acute cardiac decompensation: as with myocardial infarction, valve rupture in

destructive endocarditis

- chronic cardiac decompensation: as with non-treated hypertension, chronic

valvular endocarditis, cardiomyopathies

- pump failure (forward failure) disturbance of contractility

- filling failure (backward failure) disturbance of dilatation

- at the beginning cardiac decompensations are generally one sided: either left-

or right heart failure, which can later combine

- causes are:

- ischemic heart disease

- hypertension

- vitia (other than a mitral stenosis)

- diseases of the myocardium (cardiomyopathies)

- backward failure: congestion of the lungs with chronic pulmonary edema, heart

failure cells, brown stiffening of the lungs (induratio brunea pulmonum)

- clinically: dyspnoe; orthopnoe; nocturnal respiratory complaints; frequent and

blood-stained coughs; hydrothorax with compression and atelectasis of the lungs

(atelectasia e compressione); cerebral hypoxia with sleepiness (stupor) and rarely

hypoxic encephalopathy up to coma; decreased renal filtration, salt and water

retention, peripheral edemas

Cardiac decompensation - Left heart failure -

- most frequently in combination with a left heart failure congestive

cardiac decompensation

- isolated right heart failure develops in only 15% of the cases, especially

with

- mitral stenosis

- some congenital vitia

- cor pulmonale

- pulmonary fibrosis

- clinically: congestion of the superficial jugular veins; lower limb edema

(anasarca); sometimes hydrothorax; hypoxic encephalopathy as with left

heart failure; liver congestion with development of a severe nutmeg liver

and a so-called cardiac cirrhosis; hepato-splenomegaly; congestive

gastroenteritis; ascites

Cardiac decompensation - Right heart failure -



Cardiac decompensation. Cavities of both sides are tremendously dilated.

Tumors of the heart _____________ _____________

Primary cardiac tumors

- Benign

- Myxoma 25%

- Lipoma 8%

- Papillary fibroelastoma 8%

- Rhabdomyoma 7%

- Mesothelioma of the AV-Nodule 2%

- Malignant

- Angiosarcom a 7%

- Rhabdomyosarcoma 5%

- Mesothelioma 4%

- Fibrosarcoma 3%

Secondary cardiac tumors

- in 5% of all malignancy-related death cases cardiac metastases can

be found

- primary tumor locations in order of frequency are

- pulmonary carcinoma

- mammary carcinoma

- renal cell carcinoma

- malignant melanoma

- lymphoma / leukemia

Myxoma - most frequent tumor of the heart

- originates from the parietal endocardium

- macroscopy: a soft, greyish-reddish, sessile or steeled, varyingly large tumor

- microscopy: very loose, myxoid stroma with disseminated small vessels, on the surface a

covering layer endothelium

- danger of complication in approx. 50% of the cases is systemic embolisation from

fragmented tumor particles

- the lesion can unequivocally be detected radiologically

- therapy: operative resection; healing rate high; recurrences infrequent

- familiar appearance as a so-called Carney-syndrome possible: multiple cardiac myxomas,

sometimes extracardiac (e.g. cutaneous) myxomas, patchy dermal pigmentation, endocrine

hyperfunction >> in case of a myxoma, echocardiography of closer relatives is indicated

- differential diagnosis against an organized parietal thrombus is both macro- and micro-

scopically often probematic

Papillary fibroelastoma

- it is probably a residuum of an organized thrombus

- a bunch-like formation at the semilunar and cuspidal valves with hairy, repeatedly

bifurcating, thin branches and endothelial lining on the surface, usually measuring cca. 1cm

- most frequent localisation: aortic valves >> danger of complication: stenosis or occlusion

of the coronary ostia with angina pectoris or even sudden cardiac death

Rhabdomyoma

- most frequent in new-borns and small children

- possible presentation with tuberous sclerosis

- no real tumor, but a hamartoma*

- in the left ventricular myocardium multiple nodules, sometimes with elevation of the

endocardial inner surface

*Hamartoma a tumor-like lesion with tissue components, that are also present under normal conditions of the

presenting localisation, the morphologic composition and percentage relations of which being however abnormal.

Mesothelioma of the AV-nodule

- a typically cystic tumor in the location of the AV-nodule measuring

from microscopically small up to even 3 cm

- the tumor is connatal, that develops during the embryonal period,

primarily in females

- danger of complication: recurrent fits of Adams-Stokes syndrome

already in childhood; complete AV-blockage; sudden cardiac death of

unknown origin in a young person >> often makes the implan-tation

of a pacemaker inevitable

Aneurysms Vessel dissections _____________________ _____________________

Aneurysms

Definitions:

- aneurysm: a circumscribed lumen dilatation that exceeds generally 150% of the

original lumen.

- ectasy: diffuse dilatation of a vessel.

- causes:

- degeneration of mural structures by atherosclerosis (atheroma)

- infection of vascular wall structures (aneurysma mycoticum/infectivum)

- aneurysm formation in tertiary syphilis ascending aorta

- constitutional weekness of the vascular wall (so-called berry-aneurysms)

- trauma

Aneurysms - Localisations -

Aneurysms

A1 = fusiform; A2 =saccular, A3 = berry-aneurysm,

B = pseudoaneurysm (indeed: a perivascular hematoma),

C = dissecting aneurysm (indeed: dissection of the vessel wall layers)

Real an

eury

sms

An

eury

sm m

imics

Aneurysms

- morphology

- aneurysma verum (real aneurysm) vascular wall structures

are detectable in the aneurysmal wall. Forms: (a) fusiform and

(b) saccular (special form: berry-aneurysm of cranial arteries)

- aneurysma spurium (fake aneurysm) e.g. posttraumatically,

after catheterization. The perivascular hematoma looks from the

outside as if it was a real aneurysm.

- complications:

- rupture (the larger the aneurysm, the higher the risk)

- usuration (pressure-linked atrophy or erosion) of neighbouring

body parts (e.g. vertebrae)

- thrombosis

- embolisation



Berry-aneurysm of the left internal carotid artery

just before its joining the cranial basal vessels



Saccular aneurysm of

the aortic arch



Fusiform aneurysm of the

descending thoracic aorta


2nd Dept. of Pathology; Semmelweis University Huge chronic aneurysm of the

postero-apical two-third of the left

ventricular wall



Chronic aneurysm of the postero-lateral

left ventricular wall with thrombosis

Dissection

- definition: a longitudinally expanding detachment of vascular wall layers

resulting in the formation of a secondary (false) lumen that runs parallel to

the original one.

- pathogenesis: wall layers detachment is possible through - constitutional weekness (Marfans syndrome: weekness of the

elastic fibers and deposition of mucoid substances: cystic medial

degeneration of Erdheim-Gsell)

- atherosclerosis (atheromatous intimal plaques with intimal rupture

and distally from here detachment of the degenerated wall layers

under the pulsating blood stream. A second, more distal intimal

tear may lead to reunification of the two blood ways, or else, an

adventitial tear to perivascular hemorrhage.)

- hypertension

- complications:

- infarction of the supplied organ

- rupture with hematoma of the neighbouring regions (retroperito-

neal hematoma; hemascos; hematopleura; hemopericardium) >>

exsanguination

- clinical apperance of the aortic dissection:

- typical age: 40-60th years of life. With Marfans syndrome yet in

youth possible.

- on palpation: a pulsating abdominal mass

- complaint: sudden, anihilating, knife-stabbing-like thoracal/abdo-

minal pain >> sometimes followed by an acute abdomen syndrome

- the more dangerous type of the aortal dissections is the proximal

form (Typ A, or DeBakey I. and II.)

- somewhat less dangerous is the distal type (Typ B./DeBakey III.)

- early diagnosis may offer the chance of a successful operative

intervention (vascular prosthesis)

Dissection

Photomicrograph by Glasz, T;

Semmelweis University, 2nd. Dept. of Pathology Dissection of the arterial wall layers

Vasculitides ________ ________

Vasculitides of the large vessels

- Giant cell arteritis (temporal arteritis)

- over the 50th year of life; generally in females; affected are the aorta, its large branches, the extracranial branches of the carotid

artery (like the temporal artery)

- cause unknown (immunpathogenesis against wall structures is

possible)

- morphology: a segmental granulomatous panarteritis with giant

cells and elastic fiber fragmentation; later mural fibrosis

- clinically painful thickening of the vessels; pain in the facial and

mandibular region; fever; weekness; muscular pain in the neck

and the shoulders; on involvement of the ophtalmic artery visual

complaints or even blindness may occur

- Takayashu-Arteritis - in females under 40 years;

- affected are the aorta and its elastic large branches: the classical

topography is the aortic arch

- morphology: histologically an initial sign is the inflammation of

the vasa vasorum; followed by a non-segmental, granulomatous

panarteritis with giant cells upon long segments of the affected

vessel; later mural fibrosis; wall thickening at the branchings-off of

the side vessels of the aortic arch causing vascular stenosis and

weekness of the pulse in the arm (pulseless disease)

- clinically in the early stage general chronic inflammatory symp-

tomes (fever, weekness, weight loss); in later stage symptomes of

the vessel stenoses (ophtalmologic, neurologic alterations)

Vasculitides of the large vessels

- Polyarteritis nodosa - affected are the visceral main vessels and their primary side branches

(the mesenteric, lienal, renal, hepatic, coronary, etc. arteries)

- all organs may be affected (other than the lungs)

- alterations of varying ages appear synchronously in several arteries: a

segmental, nodular, necrotizing panarteritis

- initially fibrinoid necrosis of wall structures, later fibrosis; the necrotic

damaged segments dilate aneurysmatically: macroscopically a pearl chain-

like aspect; later vascular thrombosis

- peak of prevalence in young adults

- clinically fever, abdominal and muscular pains (often with melena); later

occlusive symptomes: infarctions of various organs (a very colourful

clinical picture) >> on affection of the kidneys: hypertension

- immunsuppressive therapy (corticosteroids) gives remission up to 90%

of the cases

Vasculitides of the middle sized vessels

- Kawasaki-Arteritis - develops in children younger than 5 years of age in the asiatic continent

- affected are the visceral, most frequently the coronary arteries: a seg-

mental, necrotizing panarteritis >> myocardial infarction

- exact cause unknown (suspected is an immunpathogenesis with malfunc-

tion of T-cells and macrophages)

- in case cutaneous purpures, mucosal inflammations, enlargement of

lymphatic nodules also occur: muco-cutaneous lymphnode syndrome

- Morbus Buerger (thrombangitis obliterans) - affects severely smoking males under 40 years in the small to middle

sized muscular arteries of the (primarily lower) extremities

- smoking plays a probable role: hypersensitivity against tobacco

- a segmental panarteritis with thrombosis; later intimal fibrosis, organiza-

tion of thrombi, recanalisation

- pain in resting position refers to affection of the neighbouring nerves

- smoking abstinence brings spectacular amelioration

Vasculitides of the middle sized vessels

- Schnlein-Henochs Purpure - IgA-deposition in small vessels

- begins with infection of the upper respiratory tract around the 5th year of life

- clinically: fever, joint pains, cutaneous purpures, melena, hematuria, IgA-nephro-

pathy

- generally a spontanous healing follows

- Wegeners Granulomatosis - necrotizing, granulomatous inflammation of the upper & lower respiratory organs

- furthermore, all over the body, focal, necrotizing vasculitides of small vessels +

glomerulonephritis possible

- begins around the 40th year of age; without therapy leads to death within a year

- clinically: two-sided, necrotizing pneumonitis; chronic sinusitis; nasopharyngeal

ulcerations; renal damage

- immunsuppressors may successfully be applied

- Churg-Strau Syndrom - eosinophylic, granulomatous, respiratory inflammation with necrotizing small

vessel vasculitis + asthma bronchiale

Vasculitides of the small vessels

Pathology of the veins _______________ _______________

Demonstration of the venous valves and their function (XVII. century)

Varicosity - dilation of the veins in the lower extremities

- insufficiency of venous valves >> chronic venous insufficiency

- primary varices (hormonal, working, etc. conditions)

- secondary varices (e.g. after thrombosis of the lower limb veins)

- phlebosclerosis (by recurring phlebitides, drog abusers)

Thrombosis of the deep veins - lower limb veins, periprostatic-periuteral plexus

- clinically: swelling, pains

- collaps of the nearby capillaries: paleness of the limb (phlegmasia alba dolens)

- affection of the collateral veins: decrease of venous drainage of the whole limb

(phlegmasia coerulea dolens)

- consequencies: propagation; rethrombosis; thrombembolism; organisation of

thrombi, postthrombotic syndrome, ulcus cruris venosum


Semmelweis University Thrombosis of the

periprostatic venous plexus



Thrombosis of the

periprostatic venous plexus



Pylethrombosis: blood clotting

in the portal vein



Paradox embolism: an embolus just in the phase of transition from the

right to the left circulation through the opening of a patent foramen ovale



Esophageal varices.

Note rough inner surface over

meandering submucosal veins

with a mucosal rupture.



Esophageal varices

on cut surface

Documents

Pathology Cardiovascular