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Home , Aschoff body, Infective endocarditis

Pathology of Rheumatic disease, Infective and

Objectives:

At the end of this lecture, the students should be able to: Editing file (1) Understands the clinicopathological features of rheumatic heart disease which is a major cause of acquired mitral and diseases in the Kingdom of Saudi Arabia. (2) Know the pathological causes and pathophysiological consequences of stenosis and incompetence of all the cardiac valves but particularly the mitral and aortic valves. Editing file (3) Understands the pathology of infective endocarditis so as to be able to identify patients at risk and when appropriate ensure prophylactic treatment is given.

Black: original content. Green: Boy‘s doctor notes . Red: Important. Dark orange: Girl’s Doctor notes. Light Purple:From Robbin’s. Grey: Explanation. Blue:only found in boys slides. Pink: Only found in girls slides. Rheumatic heart disease (RHD)

Helpful video

Definition It is a heart disease caused by rheumatic (RF) which is a condition that can result from inadequately treated streptococcal throat . It has two types: A- Acute B- Chronic

Rheumatic fever Acute Chronic an acute, immune mediated, Types of multi-system inflammatory Rheumatic An acute, immune mediated, multi-system, non-suppurative disease that occurs a few weeks inflammatory disease that occurs a few weeks(1-5 weeks) after, heart disease Definition after, group A-beta hemolytic group A-beta hemolytic streptococcal infection with cardiac streptococcal infection. and extracardiac manifestations an acute, immune mediated, multi-system inflammatory disease(mainly the heart) Site Mainly in the heart, joints, central nervous system and skin. that occurs a few weeks(1 to 5) after, group A-beta hemolytic streptococcal infection with ● Occurs in only 3% of patients with group A streptococcal

pharyngitis. ● It is seen mainly in children, 5 to 15 years of age. ● is a major health problem in 3rd world ACUTE countries and in crowded, low socioeconomic urban Rheumatic Fever Epidemiology areas. ● The and mortality of rheumatic fever has declined over the past 30 years (due to improved socioeconomic condition and rapid diagnosis and Etiopathogenesis treatment of strep. pharyngitis). of rheumatic fever

Etiopathogenesis: (The pathogenesis of RF remains unclear and is not yet completely understood) antibodies the causative cross react the antigen the causative these antibodies cross react organisms the antigen with certain antibody organisms with certain antigens present (streptococci) antibody reaction antigens reaction leads (streptococci) in the heart and joints stimulates in leads to present in the to stimulates the (because these antigens are the formation . heart and inflammation. formation of similar to streptococcal of antibodies antibodies antigen) joints Repeated attacks or a single severe attack can lead to chronic rheumatic heart disease leading to cardiac failure. Acute rheumatic heart disease (RHD)

Pericarditis is the Cardiac manifestations of Rheumatic FEVER Cardiac manifestations of Rheumatic FEVER inflammation of ● Also called as acute rheumatic heart disease or acute rheumatic / Subendocardial ● Also called acute rheumatic heart disease or acute rheumatic carditis/ pancarditis. pancarditis. lesions can be ● What is pancarditis? It’s the inflammation of all heart layers (Endocarditis, , ● What is pancarditis? It’s the inflammation of all heart layers (Endocarditis, which lead seen commonly ) myocarditis, pericarditis) secretions to in the left atrium collect between Pericarditis Endocarditis Cardiac called as Pericarditis the visceral and MacCallum ● Inflammation of , the valves inflammation of pericardium leading parietal manifestations plaques. ● Inflammation of pericardium (valvulitis) & chordae tendineae to accumulation of secretions pericardium (It’s ● Lead to accumulation of ● Results in fibrin deposition on valve leaflets (Fibrinous or serofibrinous) between of rheumatic forming tiny thrombi along lines of closure Text Cardiac manifestations of Rheumatic FEVER called bread and secretions (fibrinous or the visceral and parietal pericardium called rheumatic vegetations ● Also called acute rheumatic heart disease or acute rheumatic carditis/ pancarditis. butter) FEVER serofibrinous) between the visceral (It’s called bread and butter). ● Mitral & aortic valves are mainly involved ● What is pancarditis? It’s the inflammation of all heart layers (Endocarditis, myocarditis, & parietal layers pericarditis) Endocarditis: is ● Aschoff bodies/nodules are uncommon in ● Like butter between two slices of the valves inflammation of the bread therefore also called Pericarditis: Myocarditis: endocardium ,the ● May either resolve completely or progress “bread & butter” pericarditis to scarring with development of chronic Endocarditis: ● inflammation of pericardium leading to ● inflammation of the Myocarditis: valves (surface of the fibrotic deformities of the heart valves and is inflammation of the endocardium ,the accumulation of secretions (Fibrinous or myocardium valves have ) inflammation of chordae tendineae leading to CHD many valves (surface of the valves have necrosis) serofibrinous) between the visceral and parietal ● many Aschoff bodies and chordae the myocardium years later. and chordae tendineae this will results in Text pericardium like butter between two slices of can be seen. and many tendineae this will fibrin deposition on the valve forming tiny bread and therefore also called "bread and ● It might cause sudden results in fibrin Aschoff bodies Myocarditis Subendocardial nodules thrombi along lines of closure called butter” pericarditis. death. deposition on the rheumatic vegetations. Mitral and aortic can be seen. It valve forming tiny ● Inflammation of myocardium ● Commonly seen in left atrium valve are mainly involved might cause thrombi along lines Endocarditis: Subendocardial lesions: ● Many aschoff bodies seen ● Called as MacCallum plaques of closure called ● inflammation of the endocardium including ● can be seen sudden death. ● Might cause sudden death the heart valves (valvulitis)(surface of the commonly in the rheumatic valves have necrosis) and chordae tendineae vegetations. Mitral left atrium called and aortic valve What are Rheumatic vegetations? resulting in fibrin deposition on valve leaflets as MacCallum are mainly involved ● A mass of platelets, fibrin, microcolonies of , and scant are multiple tiny granulomatous forming tiny thrombi along lines of closure plaques. inflammatory cells. lesions of the heart. They are situated called rheumatic vegetations. ● Tiny(size of pin’s head), sessile arranged in a row and firmly with the underlying next to small . ● Mitral and aortic valve are mainly involved. tissue. ● This acute inflammation may either resolve ● These are situated in the valve cusp, a few millimeters away from the free completely or progress to scarring with margin(this is the traumatized area) what is it? development of chronic fibrotic deformities of the heart valves and chordae tendineae leading to chronic rheumatic heart disease a focus of eosinophilic many years later. what do necrosis,plump activated / they consist histiocytes called Anitschkow Some of of? the macrophages become Sterile multinucleated to form Aschoff giant vegetations cells. location myocardium and pericardium. Uncommon in the endocardium and heart valves.

Extra cardiac (outside the heart) manifestations of Rheumatic FEVER Extra cardiac manifestations of Rheumatic FEVER

Arthralgia Migratory polyarthritis skin nodules and erythema Sydenhem’s chorea Arthralgia Migratory polyarthritis marginatum. chronic interstitial inflammation Rheumatic nodules and fibrinous pleuritis.

Text Text

Text Text Acute rheumatic heart disease (RHD)

Aschoff bodies (characteristic lesion of rheumatic fever)

They are multiple tiny granulomatous lesions of the heart, Definition situated next to small arteries and are characteristically seen in the myocardium (rheumatic myocarditis).

An Aschoff body, consists of: ● Focus of eosinophilic collagen necrosis (representing the site of antibody-antigen reaction), ● Plump activated macrophages/ histiocytes called Anitschkow/caterpillar cells. ● Some of the macrophages become multinucleated to Components form Aschoff giant cells. ● Chronic inflammation.

Aschoff nodule ● Found mainly in the myocardium and pericardium. ● Uncommon in the endocardium and heart valves. Site ● They ultimately "heal" by fibrosis resulting in a nodule of scar tissue.

Extra cardiac (outside the heart) manifestations of Rheumatic FEVER

Migratory polyarthritis ● Which is "fleeting arthritis" in the large joints e.g. knee, ankle, elbow Arthralgia wrist etc. It is self limiting with no chronic deformities. Aschoff bodies may be present in the synovial membrane, joint capsule, ligament etc. with joint effusion.

skin nodules and Sydenhem’s chorea (St. Vitus' dance) erythema marginatum. ● Characteristized by series of rapid involuntary purposeless movements of the face and arms. This occurs late in the disease.

Rheumatic nodules: chronic interstitial inflammation and fibrinous pleuritis. ● mainly seen over the ● In . it’s uncommon. bony prominences e.g. knuckle, elbow, patella etc. Acute rheumatic heart disease (RHD)

Acute Rheumatic There is no specific Fever/Clinical features test for rheumatic fever. The diagnosis is Clinical features ● Acute symptoms usually subside within 3 months. made based on the age 5-15 ● The mortality from acute rheumatic carditis is low clinical findings when High serum antistreptolysin O (ASO either: Peak incidence: 5-15 years titer/ antibodies to group A History of sore throat streptococcal antigens) two major or History of sore throat one major and two (Symptoms start 10 days to 6 weeks after High serum Anti-DNAase B minor. they are called Serum antistreptolysin O anti-DNAase B, and group A streptococcal pharyngitis) antihyaluronidase are raised and provide evidence of a jones criteria. recent infection with group A . Throat/ pharyngeal cultures are

usually negative. High serum Antihyaluronidase Acute symptoms usually subside within 3 (When the symptoms start) months

Jones criteria ● There is no specific test for rheumatic fever. ● The diagnosis is made based on the clinical findings when either:

Major criteria // Minor criteria ● two major or one major and two minor criterias are met.

Major Minor

Carditis (murmurs, pericardial friction Carditis: Elevated acute phase reactants: rubs, weak , Previous rheumatic fever Murmurs, pericardial friction rubs, weak ● Elevated ESR (erythrocyte and heart sounds, tachycardia and sedimentation rate). ) arrhythmias cardiomegaly, pericarditis, ● Increased CRP (C-Reactive and congestive . protein). Migratory polyarthritis which is self Arthralgia ● limiting with no chronic deformities. Pain of the joint with no infection . If there’s an infection it’s called Aschoff bodies may be present in the Migratory polyarthritis of the large joints 1 arthritis Arthralgia synovial membrane, joint capsule, ligament etc. with joint effusion. Erythema marginatum of the skin Fever

Erythema marginatum of the Subcutaneous nodules ECG changes skin and Subcutaneous nodules Fever Prolonged PR interval

Sydenhem’s chorea (St. Vitus' dance) Previous rheumatic fever Sydenhem’s chorea is characteristized Lab tests indicative of inflammation by a series of rapid involuntary purposeless movements of the face (ESR,CRP,leukocytosis) and arms. This occurs late in the and 1: Pain of the joint with no infection . If there’s an infection it’s called arthritis disease. abnormal ECG Chronic rheumatic heart disease

myocarditis and Typically resolve without permanent sequelae pericarditis (without fibrosis).

● heals by fibrosis (scarring) ● result in irreversible deformity (leads to heart murmurs). ● Severe valvular scarring develops months or years (10-15 acute years) after acute RF. common valvulitis or ● Most harmful effect of rheumatic disease is due to chordae involvement of cardiac valves.The valve leaflets develop

tendinitis diffuse fibrosis, become thickened, shrunken, and less

Most common Most (Endocarditis) movable which can lead to:

○ cardiac failure Rare ○ thromboembolism

Components of rheumatic fever ○ infective endocarditis.

Valves affected Never Valves affected:

Common side Most common common Rare Never

Combined Tricuspid Pulmonary Left side of mitral/aortic valve valve Common side

Pulmonary valve Left side of heart

Type of damage to the valves Valves affected in chronic rheumatic heart disease

stenosis (reduction of diameter)→ regurgitation (improper closure)→ Most Mitral valve Common Less Fibrosis of valve leaflets Fibrosis of chordae tendineae common Rare Never side common valve Therefore patient can have mitral stenosis (most common), mitral Left side of Combined regurgitation, and aortic regurgitation. Tricuspid Pulmonary heart Mitral valve mitral/ aortic (So mitral and valve valve aortic valves) valves

Combined mitral/aortic valve

Tricuspid valve Chronic rheumatic heart disease

Manifestations can continue for years or decades after the initial episode of rheumatic fever

infective dilation hypertrophy endocarditis

Signs and thrombo-e symptoms (Depending on valve(s) mbolism involved)

congestive Treatment: may cardiac require valve 1. Valves from caws & pigs murmurs heart failure Chronic rheumatic heart disease surgery 2. Artificial valves

Congestive heart failure Adhesive pericarditis

fibrosis in the pericardium is Atrial . causing adhesion between the 5 parietal and Fibrous pericardium

Bacterial infective 1 4 endocarditis Complications Mural thrombi

scarred valves of rheumatic form in cardiac chambers, heart disease provide an give rise to thromboemboli, attractive environment for 2 3 which can produce infarcts bacteria to grow. in various organs

Summary:

Immunological multi-system Pancarditis Strep infection (Many systems gets (Inflammation of all heart reactions involved) layers)

Pericardium Myocardium Valvular

Bread and Aschoff Mitral Aortic butter bodies Fish Fibrinous mouth/butto nhole Infective Endocarditis (IE)1 Helpful video

Definition ★ infection of the cardiac valves or mural/ inner surface of the endocardium, resulting in the formation of an adherent mass of thrombotic debris mixed with microorganisms. ★ Mitral valves are the most common sites of IE followed by aortic valve Acute IE: ★ Vegetations may be single or multiple, involve one or more valve(s), differ in appearance according to the causative agent. Subacute IE: 1- caused by highly virulent organisms (staph.aureus) 1- It is an infection in a previously 2- infects even Infective Acute IE Subacute IE abnormal/ damaged valves by normal/healthy valves Endocarditis organisms of low virulence 3- progresses rapidly is Divided Infected Previously because it’s already damaged Normal/healthy 4- Has little local host into (α-hemolytic streptococci valves abnormal/damaged reaction (due to the fast progression, viridans) the body does not get enough time to 2- progresses slowly react) Causative Highly virulent organisms Low virulence (a-hemolytic 3- It induces a local inflammatory 2 organisms ( aureus) streptococci viridans) reaction

Progress Rapidly Slowly

Induces local inflammatory Host reaction Little local host reaction3 reaction

❖ depends to some extent on the offending organism and the ❖ Prognosis: stage at which the infection is treated. About 1/3rd of cases of endocarditis are still fatal.

❖ Why it’s Infective endocarditis is a particularly difficult infection to eradicate because of the avascular nature of the heart difficult to valves. Endocardium gets most of the oxygen from the chambers of the treat? heart by the flow of blood not capillaries so won’t be effective. Cardiac murmurs fever

Positive for the after 6 weeks: , organisms (only minority of petechiae4, and clubbing of cases remain negative) Clinical the fingers features

and chills

1: The difference between IE & the endocarditis rheumatic fever: The endocarditis rheumatic fever is non infectious (sterile) and aseptic. It’s also a from a sore throat/pharyngitis. IE though is infectious (contains microorganisms) 2: because the valve is already damaged it doesn’t need to be highly virulent to cause infection :) 3: due to the fast progression, the body does not get enough time to react 4: lesions in the skin Infective Endocarditis (IE)

Risk factors Transient bacteremia from any procedure may lead to infective endocarditis e.g. dental procedures, urinary catheterization, infected indwelling vascular catheters gastrointestinal Intravenous drug abusers1: endoscopy, and obstetric procedures. In children: an underlying ● end up injecting microorganisms 1 cardiac lesion (congenital intravenously when taking IV drugs, leading to IE. In adults: More than half of adults with bacterial endocarditis have no predisposing heart disease is most ● The : most common site cardiac lesion. and congenital heart disease are the most frequent common). (half of the cases) cause for bacterial endocarditis in adults. ● S. aureus: in 50% of cases ● Elderly (due to degeneration of 2 In adults: More than half of adults with bacterial Intravenous drug abusers can end up injecting micro-organisms intravenously when taking endocarditis have no predisposing cardiac heart valves e.g. calcific aortic intravenous drugs, leading to IE. The tricuspid valve is infected in half of cases. About 50% lesion. The rest: Mitral valve prolapse and stenosis) of the IE in IV drug abusers are caused by S. aureus. congenital heart disease are the most frequent ● diabetics cause for bacterial endocarditis in adults. ● pregnant women 3 The elderly (due to degeneration of heart valves e.g. calcific aortic stenosis), diabetics and pregnant women are at increased risk. prosthetic valves: Prosthetic valve endocarditis is caused commonly by Rheumatic heart disease 4 coagulase-negative staphylococci (e.g. S. epidermidis). In children: an underlying cardiac lesion (congenital heart disease is most common).

5 Transient bacteremia from any procedure may lead to infective endocarditis People with prosthetic valves are at high risk of developing IE . Prosthetic valve e.g. dental procedures, urinary catheterization, infected indwelling vascular endocarditis is caused commonly by coagulase-negative staphylococci (e.g. S. catheters gastrointestinal endoscopy, and obstetric procedures. epidermidis). 6 Rheumatic heart disease. Complications 7 Septicemia or septic systemic embolization of infected vegetations which travel to multiple sites, causing infarcts or in many organs (e.g. neurologic deficits due to embolization to the brain or infarcts of the myocardium due to embolization to the coronary )

Pulmonary emboli is seen in tricuspid valve/ right sided endocarditis e.g. IV drug addicts. Risk factors

Arrhythmias, valvular regurgitation and congestive heart failure (due to destruction of a valve). Intravenous drug abusers1 can end up In children: an underlying injecting micro-organisms intravenously Valve ulceration & perforation, rupture of chordae tendineae. cardiac lesion (congenital when taking intravenous drugs, leading heart disease is most to IE. The tricuspid valve is infected in half common). of cases. About 50% of the IE in IV drug Mycotic/infected aneurysms of vessels & renal failure abusers are caused by S. aureus.

In adults: More than half of adults with bacterial The elderly (due to degeneration of endocarditis have no predisposing cardiac heart valves e.g. calcific aortic stenosis), 1: Intravenous blood abusers share the same needles which cause → → inferior vena cava → right side of the lesion. Mitral valve prolapse and congenital heart → pumped to the body heart disease are the most frequent cause for diabetics and pregnant women are at 2: The infected will break → descending aorta → throughout the body → blocks the area it lands in. bacterial endocarditis in adults. increased risk.

● Prosthetic valves: high risk of developing IE. Rheumatic heart disease ● Prosthetic valve endocarditis is caused commonly by coagulase-negative staphylococci (e.g. S. epidermidis).

Transient bacteremia from any procedure may lead to infective endocarditis e.g. dental procedures, urinary catheterization, infected indwelling vascular catheters gastrointestinal endoscopy, and obstetric procedures. Endocarditis

Libman-Sacks endocarditis

Less common, non-infective, verrucous endocarditis associated with: elevated levels of circulating immune complexes. Seen in patients with systemic erythematosus

Endocarditis of carcinoid syndrome

Secretory products of carcinoid syndrome, especially 5-hydroxytryptamine can cause endocarditis. The endocardial plaques are seen in the right side of heart

Nonbacterial thrombotic endocarditis (marantic endocarditis)

- Characterized by sterile (no infection) vegetations (small masses of fibrin, platelets, and other blood components) on the leaflets of the cardiac valves. There is no infective organism. It is aseptic. - Aortic valve most common site. The fibrin deposits are randomly arranged. - May embolize to different parts of the body including brain, but the emboli are sterile. - Pathogenesis/ association: a. Subtle endothelial abnormality Other types of Endocarditis b. Hypercoagulability. c. Association with malignancy (50%) and other debilitating diseases

Diagrammatic comparison of the lesions in the four major forms of vegetative endocarditis

IE NBTE LSE RHD (infective (nonbacterial thrombotic (Libman-Sacks (rheumatic heart disease) endocarditis) endocarditis) endocarditis)

characterized by typically exhibits small, The rheumatic fever phase has small or large, irregular bland vegetations, of RHD is marked by a row medium-sized masses on the valve usually attached at of warty, small vegetations vegetations on either cusps that can the line of closure. One along the lines of closure of or both sides of the extend onto the or many may be the valve leaflets. valve leaflets. cords. present. Valvular heart diseases (VHD) Helpful Videos Two basic types 1: Almost always due to primary cuspal abnormality stemming from a chronic process (e.g., calcification or valve scarring) 2: Result from either intrinsic disease of the 1 2 Stenosis of valves Regurgitation of valves valve cusps (e.g., endocarditis) or disruption of the supporting structures (e.g., the aorta, 3 mitral annulus, tendinous cords, papillary Failure to open Insufficiency or failure to close muscles, or ventricular free wall) without Both cause murmurs4 primary cuspal injury. 3: Thereby allowing regurgitation (backflow) Mitral valve prolapse of blood 4: Produced from turbulent flow through Causes: Causes: diseased valves 1 the most frequent valvular lesion in developed countries

1 Most common cause of acquired VHD: post inflammatory scarring. 3 Can occur even with 2 Seen in young women. Seen in young women. (e.g. late complication of rheumatic prosthetic valves fever or secondary to various other inflammatory processes.) 3 Patients are predisposed to infective endocarditis. Most common cause of acquired valvular heart disease is post secondary to thrombus 2 4 Most patients asymptomatic but can occasionally lead to mitral insufficiency inflammatory scarring e.g. as a late Congenital formation or infectious 4 and arrhythmias. complication of rheumatic fever or endocarditis. secondary to various other inflammatory processes. There is myxoid/mucoid degeneration of the valve which causes: Right side of heart 1. Ballooning of mitral valves (floppy cusp)→ Stretching of the mitral valve 5 2. producing a parachute deformity of the cusp with prolapse of the cusp into the atrium during systole. 3. These changes produce a characteristic systolic murmur 3 1. TRICUSPID VALVE 2. 1 ➢ Rarely involved in ➢ Can be affected in rheumatic heart disease. congenital malformations 6 Can be a component of Marfan syndrome can occur even with ➢ When involved it is along e.g.tetralogy of Fallot may be congenital prosthetic cardiac valves with the mitral and aortic ➢ Is very rarely involved in valves (not alone). rheumatic heart disease Pathogenesis unknown 7

3.Aortic valve Aortic valve 2 4 Aortic stenosis Aortic regurgitation/ insufficiency Aortic stenosis Aortic regurgitation/ insufficiency Most common cause of acquired valvular heart disease is can be secondary to - Commonly caused by calcification - Can be caused by: post - Commonly caused by - Can be caused by: thrombus formation or and is called as calcific aortic 1- Non-dissecting . inflammatory scarring e.g. as a calcification and is called as 1- Non-dissecting aortic infectious late complication of rheumatic stenosis. 2- Rheumatic heart disease. calcific aortic stenosis. aneurysm. fever or secondary to various endocarditis. - Calcific aortic stenosis affects: 3- Infective endocarditis. other inflammatory processes. 1- Normal aortic valve as part of the 4- Syphilitic (luetic) aortitis(rare). - Calcific aortic stenosis affects: 2- Rheumatic heart disease. aging degenerative process in > 60 1- Normal aortic valve as part 3- Infective endocarditis. yrs old. of the aging degenerative 4- Syphilitic (luetic) 2- Congenital . process in > 60 yrs old. aortitis(rare). 3- Valves scarred by rheumatic 2- Congenital bicuspid aortic heart disease. valve. 3- Valves scarred by rheumatic heart disease.

1: No symptoms picked up when doing an investigation for something else. Mitral valve prolapse (Pathogenesis unknown) Valvular heart diseases (VHD) (MVP)

4. Mitral valve Helpful video Mitral valve prolapse (MVP)

● A condition in which the two valve flaps of the mitral valve do not close smoothly or evenly, but instead bulge (prolapse) upward into the left atrium. ● most frequent valvular lesion in developed countries. ● Seen in young women. ● Pathogenesis is unknown ● There is myxoid/mucoid degeneration1 of the valve which causes ballooning of mitral 2 valves (floppy cusp), results in stretching of the mitral valve,producing a parachute 3 deformity of the cusp with prolapse of the cusp into the atrium during systole. These changes produce a characteristic systolic murmur (mid-systolic click). Valves scarred by ● Most patients asymptomatic but can occasionally lead to mitral insufficiency and rheumatic heart disease arrhythmias.

● Can be a component of Marfan syndrome2. Uncommon for you to be asked about ● Patients are predisposed to infective endocarditis. ● No vegetations, no fibrosis. 1: On histologic examination, the essential change is thinning of the valve layer known as the fibrosa layer of the valve, on which the structural integrity of the leaflet depends, accompanied by expansion of the middle spongiosa layer owing to increased deposition of myxomatous (mucoid) material. 2: a disorder of that is caused by a defect in the gene controlling the production of fibrillin, and is Congenital characterized by abnormal elongation of the long bones and often by ocular and circulatory defects. bicuspid aortic Mitral stenosis valve Mitral stenosis

- More common than regurgitation. - Most commonly due to rheumatic heart disease.

**a disorder of connective tissue that is caused by a defect in the gene controlling the production of fibrillin, and is Mitral regurgitation characterized by abnormal elongation of the long bones and often by ocular and circulatory defects - Pathogenesis: Pericarditis Valve closed → blood can’t flow to left ventricle which will increase the pressure in the left atrium ● Inflammation of leading to hypertrophy and dilatation → Due to high pressure in left atrium the blood coming from pericardium the pulmonary won’t be able to fill in the left atrium → The blood will return to the lungs ● Lead to accumulation of which will lead to pulmonary congestion and pulmonary hypertension → Right side of the heart secretions (fibrinous or may get affected later (leading to congestive heart failure) serofibrinous) between the visceral & parietal layers

● Leaflets are thickened, fibrotic and fused leading to fish mouth ● Like butter between two buttonhole deformity (stenosed valve looks like fish's mouth or slices of bread therefore buttonhole) also called “bread & butter” ● secondary Ca++ deposition. pericarditis No symptoms picked up when doing an investigation for something else. Mitral regurgitation Tricuspid valve Pulmonary valve - Usually due to rheumatic heart disease. - Can also be due to MVP, IE, papillary muscle injury in MI. Rarely involved in rheumatic heart Very rarely involved in rheumatic - Leads to left and dilatation. disease, and when involved it is heart disease, but it can be along with the mitral and aortic affected in congenital valves (not alone). malformations e.g. tetralogy of Fallot.

➢ Rarely involved in Right side of heart rheumatic heart disease. ➢ When involved it is ➢ Can be affected in congenital 3. TRICUSPID VALVEalong with the mitral and aortic malformations valves (not alone). e.g.tetralogy of Fallot ➢ Is very rarely involved in rheumatic heart disease

4. PULMONARY VALVE Summary Rheumatic Fever

Types Acute Chronic

Severe/repeated attacks of Post group A Streptococcus infection Cause rheumatic fever

● Scarring Characteristic Aschoff bodies ● Thickened valvular cusps

Fibrinous or serofibrinous Pericarditis “Bread and butter” Left side of the heart Myocarditis Aschoff bodies Site Endocarditis Rheumatic vegetations ● Mitral valve alone ● or combination of Subendocardial MacCallum plaques mitral/aortic valve lesions

● Elevated Antistreptolysin O ● Cardiac murmurs ● Jones criteria: ● Thromboembolism Clinical features ○ Two major ○ One major and two minor ● Infective endocarditis

Infective Endocarditis

Site of infection Mitral valve followed by aortic valve, Tricuspid valve is seen in IV drug users

Types Acute Subacute

Cause Streptococcus Aureus 훼 hemolytic Streptococcus Viridans

Affect Normal valves Damaged valves

Progress Rapid and ⅓ of cases are fatal Slow

● Fever ● Clubbing of the fingers Clinical features ● Cardiac murmur ● +ve blood culture for the organisms ● Petechiae ● Splenomegaly

● Septicemia ● Valve ulceration and perforation Complications ● Renal failure Valvular Heart Disease

Cause Post inflammatory scarring as a late complication of Rheumatic Fever

Stenosis Regurgitation Types Mitral Aortic Mitral Aortic

Cause RHD Calcification RHD RHD Dr.AlRakabi notes

Rheumatic fever Definition A multi-system immune mediated disease, it’s also considered to be the most common cause for valvular diseases.

Pathogenesis Pharynx infection by Group A streptococcus → Immune system recognize the M protein on Group A strept (has similar molecular structure to certain parts in the body, e.g. Heart) → produces M protein antibodies(will attack all sites that have similar structure to M protein → Antigen-antibody complex deposition → Complement cascade (C3a, C5a) which will recruit inflammatory cells → inflammation and CD4+ cells activation. Diagnosis 1- Throat swab culture. 2- Serological tests: Anti-streptolysin O , Anti-hyaluronidase. 3- Jones criteria (2 major or 1 major and 2 minor).

Valvular diseases Valvular diseases are either stenosis or regurgitation, both causes murmurs of the heart, Mitral valve is the most common valve involved followed by Aortic > tricuspid > pulmonary.

Myxomatous Mitral stenosis Calcific aortic stenosis degenerative (floppy) (Due to rheumatic fever) mitral valve

- Will cause Fish mouth - Due to old age. - Mitral regurgitation. deformity to the - Dystrophic - Systolic click is valve. calcification leading heard by - Treated with to Bicuspid aortic stethoscope. antibiotics and valve. - Chordae anticoagulants before surgery to tendineae is prevent endocarditis. relaxed. Dr.AlRakabi notes Full Notes? Click here

Infective endocarditis Definition Inflammation of the endocardium most commonly caused by bacteria.

Types

1- Acute: More severe and caused by staphylococcus aureus. 2- Subacute: Less severe and caused by Streptococcus viridans.

Risk factors 1- Congenital heart disease e.g. ASD, VSD. 2- Prosthetic (artificial) valve. 3- Rheumatic heart disease. 4- Drug addict (most common cause is S.aureus).

1- Fever. Symptoms 2- Roth spots.(Eye hemorrhage) 3- Olser’s node (Painful). 4- Peteachiae.

Other types of endocarditis

Nonbacterial thrombotic Libman-Sacks endocarditis endocarditis

- Marantic(due to wasting over - Presence of sterile vegetations in time). people with Systemic Lupus - Usually diagnosed at autopsies. Erythematosus. - Occurs in people with chronic - Vegetations will be on the surface of disease, the cusps (inner/outer), not the lines - Doesn’t show colonies of bacteria of closure. under microscope, only fibrin.

Answer key: Answers Explanation File

A 1) ; B 2) ; C 3) ; A 4) ; A 5) ; A Quiz 6)

1) A 30-year-old woman presents with a heart 2) A 16-year-old girl, who arrived in the United States from murmur and SLE There is a history of recurrent Africa, comes to the hospital with and respiratory distress. On physical examination, the patient episodes of arthritis, skin rash, and is short of breath, wheezing, and gasping for air. A . Blood cultures are prominent pansystolic and a prominent negative. Laboratory tests for antinuclear third heart sound are heard on cardiac . An X-ray study of the chest shows marked enlargement of antibodies (ANA) and anti–double-stranded the heart. The patient expires despite intense supportive DNA are positive. Which of the following is the measures. At autopsy, microscopic examination of the most likely cause of heart murmur in this myocardium discloses aggregates of mononuclear cells patient? arranged around centrally located deposits of eosinophilic collagen. What is the appropriate diagnosis?

A) Libman-Sacks B) Mitral valve prolapse A)Acute bacterial B) Rheumatic heart endocarditis endocarditis disease

D) pectoris C) Subacute bacterial D) Systemic lupus C) Myocardial infarct endocarditis erythematosus

E) Rheumatic fever E) Viral myocarditis

3) A 40-year-old woman with a history of rheumatic 4) A 53-year-old woman presents with a 6-week history of fever presents with shortness of breath, weight loss, fever, fatigue, and weight loss. Her temperature is 38.7°C fatigue, and abdominal distension. Physical (103°F), rate 110 per minute, and blood pressure 140/80 mm Hg.Physical examination reveals petechiae examination shows rales in the lungs, and clubbing of the fingers. The patient develops mental hepatosplenomegaly, and 2+ pitting of the status changes, suffers a massive , and expires. The legs. A chest X-ray reveals only left atrial mitral valve is examined at autopsy (shown in the image). enlargement and pulmonary edema. What is the Which of the following is the appropriate pathologic most likely cause of pulmonary edema in this diagnosis? patient?

A) . B) Aortic stenosis. A) Bacterial endocarditis. B) Carcinoid heart disease

C) Mitral stenosis. D) Pulmonic stenosis. C) Libman-Sacks D) Marantic endocarditis endocarditis

E) Tricuspid insufficiency. E) Marantic endocarditis

5) A 10-year-old boy with a 2-week history of an upper 6) For the patient described in Question 5, respiratory infection was admitted to the hospital with which of the following is the most common , fever, joint swelling, and diffuse rash. The patient is treated and discharged. However, the patient suffers life-threatening complication of his valvular from recurrent pharyngitis and, a few years later, heart disease? develops a heart murmur. This patient’s heart murmur is most likely caused by exposure to which of the following pathogens?

A) Beta-hemolytic B) . A) Congestive heart failure B)Dissecting aneurysm. streptococcus

C) Epstein-Barr virus D) Staphylococcus C) Hemolytic D) Myocardial aureus

E) Streptococcus viridans E) Pulmonary thromboembolism Team leaders ● Raghad AlKhashan ● Mashal Abaalkhail

Team Members

● Alhanouf Alhaluli ● Abdulaziz Alghamdi ● Amirah Alzahrani ● Alwaleed Alarabi ● Danah Alhalees ● Alwaleed Alsaleh ● Deana Awartani ● Faisal Almuhid ● Elaf AlMusahel ● Jehad Alorainy ● Lama Alassiri ● Khalid Alkhani ● Lama Alzamil ● Mohammad Alhamoud ● Leena Alnassar ● Mohammad Aljumah ● Leen Almazroa ● Mohanad makkawi ● Njoud Alali ● Muath Aljehani ● Noura Alturki ● Nawaf AlBhijan ● Reema Alserhani ● Suhail Basuhail ● Rema Almutawa ● Abdulla Alhawamdeh ● Taibah Alzaid ● Hani Alhudhaif ● Tariq Aloqail