1. Rheumatic heart disease CVS 4
Hisham Alkhalidi

2. Rheumatic fever Definition Aetiology
Pathological changes (Aschoff body)
Cardiac and non-cardiac manifestations of rheumatic fever with special emphasis on valvular manifestations (mitral and aortic valves involvement)

3. group A β-hemolytic streptococcal pharyngitis
Rheumatic fever
Acute
Immunologically mediated
Multisystem inflammatory disease
Occurs few weeks after an episode of:
group A β-hemolytic streptococcal pharyngitis

4. Rheumatic fever
Usually affects children 5-15 years, but first attacks can occur in adults
In economically depressed urban areas or developing countries, RF and RHD remain important public health problems

5. hypersensitivity reaction
It appears that the M proteins of certain streptococcal strains induce host antibodies that cross-react with glycoprotein antigens in the heart, joints, and other tissues.
Pericarditis is not a big problem as it resolves

6. Rheumatic fever Acute Lymphocytes and macrophages and plasma cells
central necrosis activated macrophages (Anitschkow cells) with prominent nucleoli
activated macrophages can also fuse to form giant cells.

7. Acute rheumatic mitral valvulitis superimposed on chronic rheumatic heart disease.
Small vegetations (verrucae 1-2 mm) are visible along the line of closure of the mitral valve leaflet (arrows). Previous episodes of rheumatic valvulitis have caused fibrous thickening and fusion of the chordae tendineae
These irregular, warty projections probably arise from the precipitation of fibrin at sites of erosion caused by underlying inflammation and collagen degeneration.

8. Chronic RHD
Mitral stenosis with diffuse fibrous thickening and distortion of the valve leaflets, commissural fusion (arrows)
Thickening and shortening of the chordae tendineae
CHRONIC
leaflet thickening, commissural fusion and shortening, and thickening and fusion of the chordae tendineae
Calcification deep in leafelts

9. Valvular stenosis and regurgitation
Chronic RHD
Organization of the acute inflammation and subsequent scarring
Aschoff bodies are replaced by fibrous scar, so diagnostic forms of these lesions are rarely seen in chronic RHD
The major functional consequence of RHD is:
Valvular stenosis and regurgitation
(stenosis tends to predominate);
indeed, RHD is overwhelmingly the most frequent cause of mitral stenosis accounting for 99% of cases

10. MITRAL VALVE IS THE COMMONEST VALVE AFFECTED
MAT
70 % M
25 % M+A
With tight mitral stenosis, the left atrium progressively dilates and may harbor mural thrombi. Long-standing congestive changes in the lungs may induce pulmonary vascular and parenchymal changes and in time lead to right ventricular hypertrophy. With pure mitral stenosis, the left ventricle is generally normal.
P very rare

11. Diagnosis of acute RHD
serologic evidence of a previous streptococcal infection + two or more of the following Jones criteria:
(1) carditis, (2) migratory polyarthritis of the large joints, (3) subcutaneous nodules, (4) erythema marginatum of the skin, and (5) Sydenham chorea, a neurologic disorder with involuntary purposeless, rapid movements
One of the Jones criteria manifestations and two minor manifestations (nonspecific signs and symptoms that include fever, arthralgia, or elevated blood levels of acute-phase reactants)
Arthritis for a period of days and then subsides spontaneously, leaving no residual disability
the carditis include pericardial friction rubs and arrhythmias
Myocarditis can be so severe that resulting cardiac dilation causes functional mitral insufficiency and even CHF
Myocarditis and arthritis are temporarily

12. Chronic rheumatic heart disease
More likely to occur when the first attack:
In early childhood
Sever
Recurrence
The long-term prognosis is highly variable
Surgical repair or replacement of diseased valves has greatly improved the outlook for patients with RHD
Embolization from mural thrombi, primarily within the atria or their appendages
Infective endocarditis superimposed on deformed valves

13. Chronic RHD
The signs and symptoms of valvular disease depend on which valve(s) are involved
Mitral stenosis is the most common manifestation
Cardiac murmurs
Cardiac hypertrophy and dilation
CHF
Arrhythmias (atrial fibrillation in the setting of mitral stenosis)
Thromboembolic complications
Increased risk of subsequent infective endocarditis.
Left atrial hypertophy -> enlargement and thrombi formation
Embolization from mural thrombi, primarily within the atria or their appendages
Infective endocarditis superimposed on deformed valves
Myocarditis  cardiac dilation  functional mitral valve insufficiency or even congestive heart failure

15. Endocarditis and pericarditis CVS 5
Hisham Alkhalidi

16. Lectures 5
Pathology of endocarditis and pericarditis: definitions, classification and causes with emphasis on subacute bacterial endocarditis, marantic endocarditis and chronic constrictive pericarditis

17. (RHD) is marked by a row of small, warty verrucae along the lines of closure of the valve leaflets
IE typically shows large, irregular, and destructive masses that can extend onto the chordae.
(NBTE) typically shows small, bland vegetations, usually attached at the line of closure. One or many may be present.
Libman-Sacks endocarditis (LSE) has small or medium-sized vegetations on either or both sides of the valve leaflets, or elsewhere on the endocardial surface

18. Infective endocarditis (IE)
A serious infection, characterized by:
Microbial invasion of heart valves or mural endocardium
Often with destruction of the underlying cardiac tissues
Results in bulky, friable vegetations composed of necrotic debris, thrombus, and organisms

19. IE, Types Acute endocarditis, usually: Subacute endocarditis:
Suggests a destructive infection
Involvement of a highly virulent organism (staph. Aureus)
Attacking a previously normal valve
Death within days to weeks in more than 50% of patients despite antibiotics and surgery
Subacute endocarditis:
Low virulence organisms (strept. Viridans)
Colonizing a previously abnormal heart, especially when there are deformed valves
The disease typically appears insidiously
Follows a protracted course of weeks to months
Most patients recover after appropriate antibiotic therapy
Acute can lead to abscess
Subacute less destructuive

20. IE Clinical presentation and complications
Acute:
Fever, rigor, malaise
Large vegetation -> emboli:
Infarction
Metastatic infection
affects distant organs like spleen, brain or heart
Kidney: Ag-Ab complex -> GN-> nephrotic syndrome or Renal failure
Congestive heart failure due to valve disease
Can lead to ring abscess and perforation of the aorta and myocardium
Death up to 60%

21. IE Clinical presentation and complications
Subacute:
Insidious
Splenomegaly
Non specific fever, weight loss
Small vegetations, so less embolic
Low mortality

22. IE
Diagnosis is largely made on the basis of positive blood cultures, echocardiographic findings, and other clinical and laboratory findings
arrythmia

23. IE Pathogenesis Bacteremia is a pre-requisite
Other organs infection
IV drug abuse:
Usually Staph aureus, right heart side (Tricuspid)
Dental or surgical procedure
Trivial injury, skin, gut, urinary bladder
Contributory conditions are immunosupression and neutropenia
Staph affects both normal and sick valves and account up to 20% of all infection
"culture-negative" endocarditis : deep infection, difficult to isolate or previous Rx

24. IE Favorable conditions of infections
Congenital defects
Chronic RHD
MV Prolapse
Deg. Calcific stenosis
Bicusped aorta
Prosthetic valve (5% in 5 years)
Indwelling catheters

25. Diagnsosis
Blood C/S is a major step
Duke’s criteria

27. Nonbacterial Thrombotic Endocarditis NBTE (Marantic endocarditis)
Attributed to:
Disseminated intravascular coagulation
Hypercoagulability
Association with malignancy (specially adenocarinoma)
Valvular damage is not a prerequisite for NBTE
NBTE can be part of Trousseau
Endocardial trauma (e.g., from an indwelling catheter) is also a well-recognized predisposing condition

28. Nonbacterial Thrombotic Endocarditis NBTE (Marantic endocarditis)
Gross: loose adherent groups of small nodules on the lines of valve closure (similar to those of acute rheumatic fever), valve leaflets are normal
Micro: SMALL, sterile, fibrin and platelets aggregates, no inflammation or fibrosis.
Clinically asymptomatic, if large -> may embolize, may become infected
Typically mitral valve
NON destructive

30. Endocarditis associated with SLE
Unknown etiology
Both sides of the valve
Deformity to the valve by healing of fibrinoid necrosis and mucoid degeneration
Sterile
Mitral then tricuspid
Antiphopholipid syndrome

32. aortic and mitral valves are the most common valves affected in IE

34. Carcinoid heart disease
Right side firbosis bioactive products, Tricupsid then pulmonay unless lung carcinoid

36. Pericarditis Primary vs secondery Acute vs chronic
Uremia is the most common systemic disorder associated with pericarditis
Isolated pericardial disease is unusual, and pericardial lesions are almost always associated with disease in other portions of the heart or surrounding structures, or are secondary to a systemic disorder.
Pericarditis can be due to irradiation
Primary mostly viral

37. Pericarditis Serous Fibrinous, serofibrinous Purulent Hemorrhagic
Caseous

38. Pericarditis Serous: Exudate Non bacterial causes: Rheumatic fever SLE
Tumor
Uremia
Primary viral
Serous: CHF, hypoalbuminemia of any cause

39. Pericarditis Fibrinous, serofibrinous Purulent
Same causes of serous
Most commonly due to MI
Purulent
Bacteria (staph, strept, pneuomcocus)
Fungi
Parasite
Can lead to mediastionpericaridtis or congestive pericarditis
MI rub
Firbinous bread-and-butter pericarditis
Purulent can be up to .5 liter

40. Pericarditis Hemorrhagic: Caseous: Blood + fibrin or pus Surgery TB
Tumor
Caseous:
Fungi
Fibrocalcific constrictive pericarditis
Serosanguinous: blunt chest trauma, malignancy, ruptured MI, or aortic dissection
Chylous: mediastinal lymphatic obstruction

41. Healing Resolution Fibrosis: Epicardial plaque Thin
Thick massive adhesion
Less common secondery causes :rheumatic fever, systemic lupus erythematosus, and metastatic malignancies.
Pericarditis can (1) cause immediate hemodynamic complications if a significant effusion is present (see below), (2) resolve without significant sequelae, or (3) progress to a chronic fibrosing process.

42. Adhesive mediastinopericarditis: Constrictive pericarditis:
heart contracts against the surrounding structures
Constrictive pericarditis:
1 cm thick dense fibrous obliteration
Limit diastolic expansion and cardiac output
Pump against surrounding lead dilatation and hypertrophy

43. Chronic Pericarditis: Morphology
Ranges from delicates adhesions to dense fibrotic scars that obliterate the pericardial space.
In extreme cases the heart can’t expand during diastole : constrictive pericarditis

44. Clinical picture Atypical chest pain (worse on reclining)
High pitch friction rub.
Significant exudate cardiac tamponade  faint distant heart sounds, distended neck veins, declining cardiac output and shock
Chronic constrictive pericarditis  venous distension and low cardiac output.
The consequences of pericardial effusions depend on the ability of the parietal pericardium to stretch. This, in turn, depends on the amount of fluid and the tempo of its accumulation. Thus, slowly accumulating effusions-even as large as 1000 mL-can be tolerated without clinical manifestation. In contrast, rapidly developing collections of as little as 250 mL (e.g., ruptured MI or ruptured aortic dissection) can restrict diastolic cardiac filling to produce fatal cardiac tamponade.