The Heart

DR KANWAL ZAHRA 16th March 2013

OVERVIEW
The right side receives deoxygenated blood from the body and tissues and then pumps it to the lungs for oxygenenation Its left side receives oxygenated blood returning from the lungs and pumps this blood throughout the body to supply oxygen and nutrients to the body tissues

simplified
Cone shaped muscle Four chambers
Two atria, two ventricles

Two circulations
Systemic circuit: blood vessels that transport blood to and from all the body tissues Pulmonary circuit: blood vessels that carry blood to and from the lungs

simplified
Heart weight: 250 300 g in females 300 350 g in males 0.4 0.5 % of body wt. Right ventricular wall thickness is 0.3 0.5 cm 1.3 1.5 cm for left ventricle Cardiomegaley above 500 g is associated with ischemic changes and termed as cor bovium.

Coverings of the heart: pericardium

Fibrous pericardium Serous pericardium
(a) Parietal layer (b) Visceral layer = epicardium: part of heart wall (Between the layers is pericardial cavity)

Layers of pericardium and heart wall

Myocardium

Cardiac Valves
Tricuspid valve
RA to RV

Pulmonary valve
RV to pulmonary trunk

Mitral (bicuspid) valve

LA to LV

Aortic valve
LV to aorta

 AV (Mitral and tricuspid) valves are composed of: Annulus, leaflets, chordae tendineae and papillary muscles

 Semilunar valves (aortic and pulmonic) are composed of: 3 cusps each with a sinus 3 commissures (corpora arantii)

 Valves are relatively avascular. ATRIAL SIDE: Lined by endothelial cells on a thin layer of collagen and elastin. VENTRICULAR SIDE: A thicker layer of dense collagen. Loose myxoid C.T (zona spongiosa) in b/w. Fibrous and spongiotic regions are of equal thickness.

Function of AV valves

Function of semilunar valves (Aortic and pulmonary valves)

Conduction system
SA node (sinoatrial) In wall of RA Sets basic rate: 70-80 Impulse from SA to atria Impulse also to AV node via internodal pathway AV node (In interatrial septum) AV node through AV bundle (bundle of His) Into interventricular septum Divide R and L bundle branches subendocardial branches become (Purkinje fibers) Contraction begins at apex Composed of specialized myocytes with fewer interclated discs snd higher glycogen content

Blood supply to the heart

(theres a lot of variation)
Coronary arteries: Epicardial coronary arteries Intramural arteries Capillary network Major epicardial coronary arteries are: Left ant. Descending(LAD) Left circumflex(LCX) Both arises from left (main) coronary artery Right coronary artery

GROSS EXAMINATION AND TISSUE HANDLING

ENDOMYOCARDIAL BIOPSIES: taken via Rt. Sided cardiac catheter Indications: To moniter heart transplant rejection Tissue fragments are measured and counted during gross exam. Minimum of three preferably four samples of myocardium are recommended. Examination of at least three levels are recommended. An adequate biopsy must contain at least 50% myocardium excluding previous biopsy sites.

 CARDIAC VALVES: Removed because of Calcific degeneration or perforation as sequel of bacterial endocarditis. Mostly received in fragments, if present description of vegetations and presence and absence of non-surgery related leaflet destruction should included. In calcific degeneration, acid decalcification is required, sections from free edge to annulus.

 For mechanical heart valves, only vegetations are submitted if present. For bioprosthetic valves, the valve cusps are also submitted. MYOMECTOMY SPECIMENS: From ventricular aneurysm repair or septal myomectomy procedures. Measured, weighed and sectioned at 3mm intervals perpendicular to endocardial surface. All layers of heart should be described.

 HEART EXPLANT SPECIMENS: Should be weighed Valves and walls (circumference and diameter) should be measured Septal and ventricular configuration (ventricular hypertrophy etc) should be described. Fragments of donor tissue (inflow tract) to match with recipients anatomy are usually present in same container.

CONDUCTING AN AUTOPSY
Examination of the heart includes removing the chest plate then taking the heart out of the pericardial sac The outside surfaces are examined first and the blood vessels (coronary arteries) are dissected. The heart is opened to reveal the internal surfaces and structures, including the valves and heart muscle (myocardium). The muscle is then cut to reveal the surface color, textures and other features. Finally, tissue sections are prepared to examine under the microscope.

Tricuspid and Mitral valves

Aortic and Pulmonary valves

PATHOPHYSIOLOGY OF HEART DISEASES

Failure of pump Obstruction to flow Regurgitant flow Shunted flow Disorders of cardiac conduction Rupture of heart or a major blood vessel

HEART FAILURE
Often called CHF, when heart unable to pump blood at a rate sufficient to meet metabolic demands. Compensatory mechanisms which maintain arterial pressure and perfusion of vital organs include 1- Frank- Starling mechanism: inc filling vol. dilates the heart and inc functional cross bridges formation, enhancing contractility 2- Myocardial adaptations, hypertrophy 3- Activation of neurohumoral systems: Release of norepinephrine, activation of renin- angiotensinaldosterone system , atrial natriuretic peptide, adjust filling vol. and pressure.

LEFT-SIDED HEART FAILURE

Etiology: IHD, HTN, aortic and mitral valvular disease, myocardial disease Morphology: findings depend upon disease process. Gross: gross structural abnormalities e.g myocardial infarcts, deformed, stenotic or regurgitant valves may be present. Lt. ventricle is usually hypertrophied often dilated. M/E: non sp. Mainly myocyte hypertrophy and varying degree of interstitial fibrosis.

RIGHT-SIDED HEART FAILURE

Etiology: Lt. sided heart failure, pure Rt. Heart failure is infrequent, associated with lung disease so referred as Cor pulmonale (Latin cor, heart; pulmnle, of the lungs) Morphology: varies with cause. Rarely structural defects(valvular abn, endocardial fibrosis) may be seen. In contrast to Lt. sided failure pulm. Congestion is minimal but portal and systemic venous systems may be engorged. The only finding may be Rt. Atrial or ventricular dilatation.

CONGENITAL HEART DISEASE

Cardiac development

CONGENITAL HEART DISEASE

The structural anomalies in CHD fall into three major categories: 1- Left-to-right shunt 2- Right-to-left shunt 3- Obstruction LEFT-TO-RIGHT SHUNT: Most commonly encountered shunts include ASDs, VSDs, Patent ductus arteriosus and atrioventricular septal defects.

 1- Atrial septal defects (ASDs): classified according to their location as: 1- Secundum ASDs: 90% of all, may be single, multiple or fenestrated, near centre of atrial septum. 2- Primum ASDs: 5% of all, adjacent to AV valve. 3- Sinus venosus: 5%, near entrance of sup. vena cava, may be associated with pulm. Venous return to Rt. atrium.

2-Ventricular septal defects (VSDs): 1- Membranous VSDs: 90% of all, involves the region of membranous interventricular septum 2- Infundibular VSDs: Lie below the pulm. Valve

 3- Within muscular septum Most VSDs are single but those in muscular septum may be multiple so called Swiss cheese septum.

 3- Patent (persistent) ductus arteriosus (PDA): Occur if ductus arteriosus remain open after birth, in fetal circulation it shunts the blood from pulm. artery to aorta so bypass the lungs. Produce characteristic harsh murmer described as machinery like murmer

4- Atrioventricular septal defect (AVSD): results from failure of sup. and inf. endocardial cushions of AV canal to fuse adequately. Partial AVSD: consisting of primum ASD and cleft of ant. Mitral leaflet, causing mitral insufficiency

 Complete AVSD: large combined AV septal defect and large common AV valve (hole in centre of heart). All chambers freely communicate inducing vol. hypertrophy.

RIGHT-TO-LEFT SHUNTS
Cyanosis early in postnatal life (cyanotic heart diseases) includes: Tetralogy of Fallot Transposition of Great Arteries Persistent Truncus Arteriosus Tricuspid Atresia Total Anomalous Pulmonary venous connection

Tetrology of Fallot (TOF)

Four cardinal features include: 1- VSD 2- Obstruction to right ventricular outflow tract (subpulmonary stenosis) 3- An aorta that overrides the VSD 4- Right ventricular hypertrophy

 Morphology: Heart is enlarged and may be boot-shaped as a result of marked ventricular hypertrophy,in apical region VSD is usually large, aortic valve usually form the sup. Border so override the defect and both ventricular chambers. Obstruction to Rt. Ventricular outflow is due to subpulmonic stenosis which can be accompanied by pulm. Valvular stenosis Sometimes an aortic valve insufficiency or an ASD may also be present.

Transposition of great arteries (TGA)

Produces ventriculoarterial discordance, aorta arises from Rt. Ventricle and lies ant. To and to the Rt. of pulm. Artery which originates from Lt. ventricle

 Condition is incompatible with postnatal life unless a shunt exists for blood mixing. The infants out look depends on degree of blood mixing. Pts with TGA and VSD may have stable shunts(35%) while those with patent foramen ovale or PDA have unstable shunts.

Persistant Truncus Arteriosus

Failure of seperation of embryologic TA into aorta and pulm. Artery. Results in single great artery that receive blood from both ventricles, give rise to systemic, pulm and coronary circurlation

 Complete occlusion of tricuspid valve orifice Mitral valve is larger than normal and hypoplasia of Rt. Ventricle, Circulation is maintained by RT. to LT. shunt thru ASD, PFO and a VSD. Inc mortality rate in 1st few wks or months of life

Tricuspid Atresia

 Pulmonary veins fails to join the Lt. atrium Results when common pulmonary vein fails to develop or become atretic Fetal development is made possible by primitive venous channels that drain from lungs into Lt. innominate vein or coronary sinus PFO or ASD is always present, allowing venous blood to enter Lt. atrium. Vol. and pressure hypt of Rt. side of heart, dilation of pulm. trunck. Lt. atrium is hypoplastic but Lt. ventricle is normal in size

Total anomalous pulmonary venous connection

Obstructive congenital anomalies

Obstruction occur at the level of heart valves or within a great vessel includes: Stenosis or atresia of aortic or pulmonary valves Coarctation of the Aorta Obstruction can also occur within a chamber as with subpulmonary stenosis in TOF. Coarctation of the Aorta 2 classic forms are 1- Infantile form 2- Adult form In infantile form there is tubular hypoplasia of aortic arch proximal to PDA often symptomatic in early childhood

 In adult form there is a ridge like infolding of aorta just opposite to closed ligamentum arteriosum

It may occur solitary but in 50% of cases accompanied by bicuspid aortic valve and also be associated with aortic stenosis, ASD, VSD, mitral regurgitation, or berry aneurysms of the circle of Willis in the brain. There is cardiomegaly due to left ventricular pressureoverload hypertrophy.

Pulmonary Stenosis and Atresia

obstruction at the pulmonary valve, which may be mild to severe. Can be isolated or part of a more complex anomaly-either TOF or TGA. Right ventricular hypertrophy, poststenotic dilation of the pulmonary artery due to injury of the wall by "jetting" blood. Subpulmonary stenosis (TOF), the high ventricular pressure is not transmitted to the valve, and the pulmonary trunk is not dilated and may in fact be hypoplastic. When the valve is entirely atretic, there is no communication between the right ventricle and lungs. In such cases the anomaly is associated with a hypoplastic right ventricle and an ASD; blood reaches the lungs through a PDA.

Aortic Stenosis and Atresia

Congenital narrowing and obstruction of the aortic valve can occur at three locations: valvular, subvalvular, and supravalvular. valvular aortic stenosis: cusps may be hypoplastic , dysplastic, or abnormal in number. underdevelopment (hypoplasia) of the left ventricle and ascending aorta, sometimes accompanied by dense, porcelain-like left ventricular endocardial fibroelastosis. This is called the hypoplastic left heart syndrome, is nearly always fatal in the first week of life, when the ductus closes

 Subaortic stenosis : caused by a thickened ring (discrete type) or collar (tunnel type) of dense endocardial fibrous tissue below the level of the cusps Supravalvular aortic stenosis: inherited form of aortic dysplasia. In some cases it results from deletions on chromosome 7 that include the gene for elastin. Ch. delation cause disruption of elastin-smooth muscle cell interactions. Other features of the syndrome include hypercalcemia, cognitive abnormalities, and hallmark facial anomalies (Williams-Beuren syndrome).

Ischemic Heart Disease (IHD)

leading cause of death worldwide for both men and women (7 million total per year) results from myocardial ischemia. In 90% of cases, the cause of myocardial ischemia is reduced blood flow due to obstructive atheroscleotic lesions in the coronary arteries. so termed as coronary artery disease (CAD) or coronary heart disease. IHD usually presents as one or more of the following clinical syndromes:

Myocardial infarction Angina pectoris Chronic IHD with heart failure. Sudden cardiac death. In addition to atherosclerosis, MI may be caused by coronary emboli, blockage of small myocardial blood vessels, and lowered systemic blood pressure. Pathogenesis of IHD

MYOCARDIAL INFARCTION (MI)

MI, also known as "heart attack," is the death of cardiac muscle due to prolonged severe ischemia.
FEATURES
Onset of ATP depletion Loss of contractility ATP reduced

TIME
Seconds <2 min

to 50% of normal
to 10% of normal Irreversible cell injury Microvascular injury

10 min
40 min 20-40 min >1 hr

Evolution of Morphologic Changes in Myocardial Infarction

Time
REVERSIBLE INJURY

Gross Features

Light Microscope

Electron Microscope

0- hr

None

None

Ralaxation of myofibrils; glycogen loss; mitochondrial swelling

IRREVERSIBL E INJURY

-4 hr

None

Usually none; variable waviness of fibers at border

Sarcolemmal disruption; mitochondrial amorphous densities

4-12 hr

Dark mottling (occasional)

Early coagulation necrosis; edema; hemorrhage

Time 12-24 hr

Gross Features Dark mottling

Light Microscope Ongoing coagulation necrosis; pyknosis of nuclei; myocyte hypereosinophilia; marginal contraction band necrosis; early neutrophilic infiltrate

1-3 days

Mottling with yellow-tan infarct Coagulation necrosis, with loss of nuclei and center striations; brisk interstitial infiltrate of neutrophils Hyperemic border; central yellow-tan softening Maximally yellow-tan and soft, with depressed red-tan margins Red-gray depressed infarct borders Gray-white scar, progressive from border toward core of infarct Scarring complete Beginning disintegration of dead myofibers, with dying neutrophils; early phagocytosis of dead cells by macrophages at infarct border Well-developed phagocytosis of dead cells; early formation of fibrovascular granulation tissue at margins Well-established granulation tissue with new blood vessels and collagen deposition Increased collagen deposition, with decreased cellularity Dense collagenous scar

3-7 days

7-10 days

10-14 days 2-8 wk

>2 mo

complications following acute MI

Hypertensive Heart Disease (HHD)

SYSTEMIC (LEFT-SIDED) HYPERTENSIVE HEART DISEASE: HTN induces Lt. ventricular hypertrophy, initially without ventricular dilation. The thickness of the left ventricular wall may exceed 2.0 cm, and the heart weight may exceed 500 gm. M/E the earliest change of systemic HHD is an increase in the transverse diameter of myocytes. Enlargement often accompanied by interstitial fibrosis. PULMONARY (RIGHT-SIDED) HYPERTENSIVE HEART DISEASE (COR PULMONALE): In acute cor pulmonale there is marked dilation of the right ventricle without hypertrophy. In chronic cor pulmonale the right ventricular wall thickens, sometimes up to 1.0 cm or more.

 More subtle right ventricular hypertrophy may take the form of thickening of the muscle bundles in the outflow tract, immediately below the pulmonary valve.

 Valvular abnormalities may be congenital or acquired. Acquired stenoses of the aortic and mitral valves account for approximately two thirds of all cases. most frequent abnormalities are: Aortic stenosis: associated with calcification Aortic insufficiency: usually related to hypertension and aging. Mitral stenosis: rheumatic heart disease Mitral insufficiency: myxomatous degeneration

Valvular Heart Disease

 Calcific Aortic Stenosis: most common of all, usually the consequence of age-associated "wear and tear" Morphology: hallmark of nonrheumatic, calcific aortic stenosis is heaped-up calcified masses within the aortic cusps, free edges of the cusps are usually not involved. M/E: the layered architecture of the valve is largely preserved In contrast to rheumatic aortic stenosis commissural fusion is not usually seen. The mitral valve is generally normal.

VALVULAR DEGENERATION ASSOCIATED WITH CALCIFICATION

Mitral Annular Calcification

Degenerative calcific deposits can develop in the peripheral fibrous ring (annulus) of the mitral valve Grossly, appear as irregular, stony hard, occasionally ulcerated nodules (2-5 mm in thickness) that lie behind the leaflets. may lead to (1) regurgitation(2) stenosis (3) arrhythmias occasionally sudden death by penetration of calcium deposits to a depth sufficient to impinge on the atrioventricular conduction system. calcific nodules may also provide a site for thrombi that can embolize, can also be the nidus for infective endocarditis.

MITRAL VALVE PROLAPSE (MYXOMATOUS DEGENERATION) In mitral valve prolapse (MVP), one or both mitral valve leaflets are "floppy" and prolapse, into the left atrium during systole. Characteristic change in MVP is interchordal ballooning (hooding) of the mitral leaflets. The affected leaflets are often enlarged, redundant, thick, and rubbery. associated tendinous cords may be elongated, thinned, or even ruptured, and the annulus may be dilated. M/E : attenuation of the collagenous-fibrosa layer of the valve, accompanied by marked thickening of the spongiosa layer with deposition of mucoid (myxomatous) material

RHEUMATIC HEART DISEASE

Rheumatic fever (RF) is an acute, immunologically mediated, multisystem inflammatory disease that occurs a few weeks after an episode of group A streptococcal pharyngitis. It may manifest as acute rheumatic carditis which may progress over time to chronic (RHD). Morphology: During acute RF main lesions occur in the heart, called Aschoff bodies, consist of foci of lymphocytes occasional plasma cells, and plump activated macrophages called Anitschkow cells. These macrophages have abundant cytoplasm and central round-to-ovoid nuclei in which the chromatin is disposed in a central, slender, wavy ribbon ("caterpillar cells"), and may become multinucleated.

 Inflammation results in fibrinoid necrosis within the cusps or along the tendinous cords. Overlying these necrotic foci are small (1- to 2-mm) vegetations, called verrucae, along the lines of closure. Subendocardial lesions, exacerbated by regurgitant jets, may induce irregular thickenings called MacCallum plaques, usually in the left atrium.

 In chronic RHD cardinal features are leaflet thickening, commissural fusion and shortening, and thickening and fusion of the tendinous cords, creates a "fish mouth" or "buttonhole" stenoses. Mitral valve is affected alone in 65% to 70% of cases, aortic valve in another 25% of cases. Tricuspid and the pulmonary valve involvement is infrequent

With tight mitral stenosis, the left atrium progressively dilates and may harbor mural thrombi in the appendage or along the wall, either of which can embolize. M/E there is organization of the acute inflammation and subsequent diffuse fibrosis and neovascularization in the mitral leaflets that obliterate the avascular leaflet architecture.

 Infective endocarditis (IE) is characterized by colonization or invasion of the heart valves or the endocardium by a microbe, leads to the formation of vegetations. Morphology: hallmark of IE is the presence of friable, bulky, vegetations containing fibrin, inflammatory cells, and bacteria or other organisms on the heart valves The vegetations may be single or multiple and may involve more than one valve. sometimes erode into the underlying myocardium and produce an abscess (ring abscess). Emboli may be shed from them at any time; because the embolic fragments may contain large numbers of virulent organisms, abscesses often develop at the sites where the emboli lodge.

INFECTIVE ENDOCARDITIS

 M/E: vegetations of typical subacute IE often have granulation tissue indicative of healing. With time, fibrosis, calcification, and a chronic inflammatory infiltrate can develop.

PATHOLOGIC CRITERIA

Microorganisms, demonstrated by culture or histologic examination, in a vegetation, embolus from a vegetation, or intracardiac abscess Histologic confirmation of active endocarditis in vegetation or intracardiac abscess
CLINICAL CRITERIA

Major

Blood culture(s) positive for a characteristic organism or persistently positive for an unusual organism Echocardiographic identification of a valve-related or implant-related mass or abscess, or partial separation of artificial valve New valvular regurgitaion Minor
Predisposing heart lesion or intravenous drug use Fever Vascular lesions, including arterial petechiae, subungual/splinter hemorrhages, emboli, septic infarcts, mycotic aneurysm, intracranial hemorrhage, Janeway lesions Immunological phenomena, including glomerulonephritis, Osler nodes, Roth spots, rheumatoid factor Microbiologic evidence, including a single culture positive for an unusual organism Echocardiographic findings consistent with but not diagnostic of endocarditis, including worsening or changing of a preexistent murmur

NONINFECTED VEGETATIONS
Nonbacterial Thrombotic Endocarditis (NBTE) encountered in debilitated patients, striking association with mucinous adenocarcinomas, procoagulant effects of tumor-derived mucin or tissue factor. Endocardial trauma, well-recognized predisposing condition Deposition of small sterile thrombi on the leaflets of the cardiac valves. 1 mm to 5 mm in size, and occur singly or multiply along the line of closure of the leaflets. M/E : composed of bland thrombi that are loosely attached to the underlying valve, do not elicit any inflammatory reaction. May be the source of systemic emboli that produce infarcts in the brain, heart.

Endocarditis of SLE (Libman-Sacks Disease)

small, sterile vegetations (1-4 mm) have a warty (verrucous) appearance, located on the undersurfaces of the AV valves, valvular endocardium, chords, or on the mural endocardium of atria or ventricles. M/E: Veg consist of finely granular, fibrinous eosinophilic material that may contain hematoxylin bodies, remnants of nuclei damaged by anti-nuclear antigen bodies.

Cardiomyopathies
cardiomyopathy (literally, heart muscle disease) produce abnormalities in cardiac wall thickness and chamber size, and mechanical and/or electrical dysfunction. Primary cardiomyopathies: predominantly confined to the heart muscle. Secondary cardiomyopathies: have myocardial involvement as a component of a systemic or multiorgan disorder.

Functional Patterns and Causes

Functio Ejectio Mechanisms of nal n Heart Failure Pattern Fractio n Dilated <40% Impairment of contractility (systolic dysfunction) Causes of Phenotype Indirect Myocardial Dysfunction (Mimicking Cardiomyopathy) Ischemic heart disease; valvular heart disease; hypertensive heart disease; congenital heart disease

Genetic; alcohol; peripartum; myocarditis; hemochromatosis; chronic anemia; doxorubicin (Adriamycin); sarcoidosis; idiopathic

Hypertro 50% to Impairment of phic 80% compliance (diastolic dysfunction) Restrict 45% to Impairment of ive 90% compliance (diastolic dysfunction)

Genetic; Friedreich Hypertensive heart ataxia; storage diseases; disease; aortic stenosis infants of diabetic mother Amyloidosis; radiationinduced fibrosis; idiopathic Pericardial constriction

DILATED CARDIOMYOPATHY(DCM)
Morphology: Heart is usually enlarged, heavy (often weighing two to three times normal), and flabby, due to dilation of all chambers. Mural thrombi are common and may be a source of thromboemboli. M/E: Non specific

HYPERTROPHIC CARDIOMYOPATHY (HCM)

MORPHOLOGY: The essential feature of HCM is massive myocardial hypertrophy, usually without ventricular dilation. The classic pattern is asymmetric septal hypertrophy. On cross-section, the ventricular cavity loses its usual round-to-ovoid shape and compressed into a "banana-like" configuration by bulging of the ventricular septum into the lumen. M/E : (1) extensive myocyte hypertrophy, with transverse myocyte diameters frequently greater than 40 m (normal, 15 m); (2) haphazard disarray of bundles of myocytes (termed myofiber disarray); and (3) interstitial fibrosis.

 characterized by a primary decrease in ventricular compliance, resulting in impaired ventricular filling during diastole. may be idiopathic or associated with, radiation fibrosis, amyloidosis, sarcoidosis, metastatic tumors, or the deposition of metabolites that accumulate due to inborn errors of metabolism. Morphology. The ventricles are of approximately normal size or slightly enlarged, the cavities are not dilated, and the myocardium is firm and noncompliant. Biatrial dilation is commonly observed. M/E, there may be only patchy or diffuse interstitial fibrosis, which can vary from minimal to extensive.

RESTRICTIVE CARDIOMYOPATHY (RCM)

MYOCARDITIS
Infectious microorganisms and/or an inflammatory process cause myocardial injury
INFECTIONS Viruses (e.g., coxsackievirus, ECHO, influenza, HIV, cytomegalovirus) Chlamydiae (e.g., C. psittaci) Rickettsiae (e.g., R. typhi, typhus fever) Bacteria (e.g., Corynebacterium diphtheriae, Neisseria meningococcus, Borrelia (Lyme disease) Fungi (e.g., Candida) Protozoa (e.g., Trypanosoma cruzi [Chagas disease], toxoplasmosis) Helminths (e.g. trichinosis)

IMMUNE- MEDIATED REACTIONS

Postviral Poststreptococcal (rheumatic fever) Systemic lupus erythematosus Drug hypersensitivity (e.g., methyldopa, sulfonamides) Transplant rejection
UNKNOWN Sarcoidosis Giant cell myocarditis

 Morphology: During active phase, heart appear normal

or dilated, advanced stage the ventricular myocardium is flabby haveing pale foci or minute hemorrhagic lesions along with mural thrombi. M/E: an interstitial mononuclear, predominantly lymphocytic infiltrate associated with focal myocyte necrosis, either resolve, leaving no residual changes, or heal by progressive fibrosis. Hypersensitivity myocarditis: perivascular infiltrate composed of lymphocytes, macrophages, and a high proportion of eosinophils. Giant-cell myocarditis, inflammatory infiltrate containing multinucleate giant cells interspersed with lymphocytes, eosinophils, plasma cells, and macrophages

 Chagas disease: parasitization of scattered myofibers by trypanosomes with an infiltrate of neutrophils, lymphocytes, macrophages, and occasional eosinophils.

PRIMARY CARDIAC TUMORS Myxoma 50% of primary tumors, occur in two settings: Sporadic and familial Sporadic tumor occurs in middle-aged women (76%), usually in the left atrium (86%), nearly always as a single tumor, and without associated conditions. familial variety: young people, slightly more frequent in men, less commonly located in the left atrium (62%), multicentric, and associated with extracardiac abnormalities.

 Grossly: soft, polypoidal, pale, lobulated masses, often attached by a stalk to the septum near the foramen ovale. A papillary configuration may be apparent, Calcification may occur, and this seems to be more common in those located in the right atrium.

 M/E, round, polygonal, or stellate cells are seen surrounded by abundant loose stroma rich in acid mucopolysaccharides. Some cells form solid cords and vascular channels, continuous with the endocardial lining. Mitoses, pleomorphism, and necrosis are absent or minimal.

 ossification (petrified myxoma), occurrence of cartilaginous tissue presence of thymic and foregut remnants, having well-developed mucin-producing glands. referred as glandular myxoma, can be confused with metastatic adenocarcinoma.

development of a thymoma, presumably arising from the thymic remnants.

Other benign tumors and tumorlike conditions

Rhabdomyoma and rhabdomyomatosis Hamartoma of mature cardiac myocytes Calcified amorphous tumor of the heart (cardiac CAT) Cystic tumor of the atrioventricular nodal region Adenomatoid tumor Papillary fibroelastoma Inflammatory myofibroblastic tumor Paraganglioma

Primary malignant tumors

Sarcomas: are exceptionally rare, highly pleomorphic and unclassifiable. Of those that can be placed into a specific category, angiosarcoma is the most common. Gross: located in the atrium, presents as a large mass

 second most common category myosarcoma, either leiomyosarcoma or rhabdomyosarcoma. Other types include myxofibrosarcoma (malignant fibrous histiocytoma) osteosarcoma, fibrosarcoma, liposarcoma, syno vial sarcoma, Ewing sarcoma/ PNET, and MPNST. A non specific feature seen in high-grade sarcomas of the heart and large vessel, is the presence of plump epithelioid cells in a perivascular location

Metastatic tumors
Malignant lymphoma is a more common even than primary Any portion of the heart can be involved, conducting system results in heart block. majority of carcinomas, the primary tumor is in the thoracic cavity or contiguous areas They reaches the heart by mediastinal lymph nodes and from there extending in a retrograde fashion to the cardiac lymph vessels. Tumors spread to the heart by the hematogenous route are malignant melanoma; carcinomas of kidney, lung, and breast; choriocarcinomas; and childhood rhabdomyosarcoma.