1. Rheumatism Infective endocarditis Chronic valvular vitium of the heart
Pathology Department
Zhejiang University School of Medicine
Zhu keqing 竺可青

5. RHEUMATISM An immunologically mediated disease,
related to an episode of group A (β-hemolytic)
streptococcal pharyngitis.
Multisystem inflammatory disease, heart and joints
are favored sites.
Rheumatic fever, acute rheumatic carditis
Repetitive attacks lead to chronic valvular deformities
The pathologic feature is Aschoff bodies. 5

6. The pathogenetic sequence and key morphologic features of acute rheumatic heart disease

7. (1) alteration and exudation
ESSENTIAL MORPHOLOGY
(1) alteration and exudation
Serous, fibrinous exudate, and leukocytes infiltration, mucoid degeneration and fibrinoid necrosis.
Outcomes:
Complete resolution
Fibrosis
Granulomas 7

8. (2) Proliferation or granulomas Hallmark: Aschoff bodies
Fibrinoid necrosis, Anitschkow cells
Aschoff giant cells
Lymphocytes
Plasma cells 8

9. Aschoff body
During acute RF, focal inflammatory lesions are found in various tissues. They are most distinctive within the heart, where they are called Aschoff bodies.
They consist of foci of swollen eosinophilic collagen surrounded by lymphocytes (primarily T cells), occasional plasma cells, and plump macrophages called Anitschkow cells (pathognomonic for RF).
These distinctive cells have abundant cytoplasm and central round-to-ovoid nuclei in which the chromatin is disposed in a central, slender, wavy ribbon (hence the designation “caterpillar cells”).形态
Some of the larger macrophages become multinucleated to form Aschoff giant cells. 巨细胞

10. Aschoff body 10

11. Aschoff bodies 11

12. Aschoff bodies spindle scar
(3) Fibrosis
Aschoff bodies spindle scar 12

13. Rheumatic endocarditis Sites: mitral and aortic valves
Fibrinoid necrosis
Vegetations: small, warty, along the line of closure 13

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15. 15

16. 16

17. Precipitation of fibrin Organization, fibrosis
Thickening, shortening, commissural fusion of leaflets and tendinous cord 17

19. Rheumatic myocarditis
Perivascular
Aschoff body 19

20. Rheumatic pericarditis
Serous exudate pericardial effusion
Fibrinous exudate
Cor villosum
Constrictive pericarditis 20

21. 21

22. Large joints: migratory Local signs: arthralgia
Rheumatic arthritis
Serous inflammation
Complete resolution
Large joints: migratory
Local signs: arthralgia
Self-limited, no chronic deformity 22

23. Rheumatic arteritis Often involves small arteries, Fibrinoid necrosis
Mononuclear infiltration
Aschoff bodies
Fibrosis
Lumen narrowing 23

24. CNS changes Rheumatic arteritis, Subcortical encephalitis
Minor chorea / Sydenham chorea
(a neurologic disorder with involuntary purposeless, rapid movements) 24

25. ACUTE: CHRONIC: -Inflammation -Aschoff bodies -Anitschkow cells
-Pancarditis
-Vegetations on chordae tendinae at leaflet junction
CHRONIC:
THICKENED VALVES
COMMISURAL FUSION
THICK, SHORT, CHORDAE TENDINAE
Granuloma:Giant Cell::Aschoff Body:Anitschkow Cell 25

26. CLINICAL FEATURES Migratory Polyarthritis Myocarditis
Subcutaneous nodules
Erythema marginatum
Sydenham chorea
A GREAT classical Sydenham chorea (St. Vitus “Dance”) can be seen at 26

27. 风心小结
（肉牙肿性炎）3 stages
变质渗出期---黏液样变/纤维素样坏死---1月
增生期/肉牙肿期---风湿细胞/Aschoff cell/ Aschoff body---2/3月
纤维化期/愈合期---4/6月

28. Rheumatic heart disease (小结）
1 Rheumatic endocarditis
---valvulitis---vegetations白色血栓
---verrucous endocarditis疣状心内膜炎
---McCallum斑
2 Rheumatic myocarditis ---Aschoff body
3 Rheumatic pericarditis---绒毛心Cor villosum ---constrictive pericarditis
4 Rheumatic pancarditis

29. Rheumatic arthritis –游/大/无-舔关节咬心脏
Erythema annullare 环形红斑-非特异性渗出性炎
Subcutaneous nodules 皮下结节-风湿小体
Rheumatic arteritis-风湿小体
Chorea minor 小舞蹈症
皮质下脑炎

30. 感染性心内膜炎 Infective endocarditis
病原微生物直接侵袭心内膜特别是心瓣膜而引起的心内膜炎
急性感染性心内膜炎
亚急性感染性心内膜炎 30

31. 急性感染性心内膜炎 病原：致病力强的化脓菌 金黄色葡萄球菌、溶血性链球菌、肺炎球菌 心瓣膜：发生在原来无病变的正常心内膜
主要累及二尖瓣、主动脉瓣
临床上：起病急，发展快，病程短，死亡率高 31

32. 亚急性感染性心内膜炎 病原：致病力较小病原微生物 草绿色链球菌，肠球菌，真菌等。 心瓣膜：发生在原来有病变的心内膜 主要累及二尖瓣、主动脉瓣
菜花状或息肉状疣状赘生物：
纤维素、血小板、NC、坏死物
细菌团
血管：动脉栓塞和血管炎；
肾： 微栓塞致灶性肾小球肾炎。
临床上：病程长，数月或1年 32

33. 33

34. 34

35. ABE/SBE区别
Acute endocarditis describes a destructive, tumultuous infection, frequently of a previously normal heart valve, with a highly virulent organism, that leads to death within days to weeks of more than 50% of patients despite antibiotics and surgery.
In contrast, organisms of low virulence can cause infection in a previously abnormal heart, particularly on deformed valves. In such cases, the disease may appear insidiously and, even untreated, pursue a protracted course of weeks to months (subacute endocarditis). Most patients with subacute IE recover after appropriate antibiotic therapy.

37. Morphology.
In both the subacute and acute forms of the disease, friable, bulky, and potentially destructive vegetations containing fibrin, inflammatory cells, and bacteria or other organisms are present on the heart valves.
Osler小结-皮下小动脉炎
Systemic emboli may occur at any time because of the friable nature of the vegetations, and they may cause infarcts in the brain, kidneys, myocardium, and other tissues.
Because the embolic fragments contain large numbers of virulent organisms, abscesses often develop at the sites of such infarcts (septic infarcts).

38. Splinter hemorrhages, Janeway lesions (palms, soles), Osler’s “nodes” (raised), Roth’s spots (eye).
Do you think that for every skin lesion you see there may be 10 visceral lesions which you do NOT see? Ans: Yes, I believe so! 38

39. Chronic valvular vitium of the heart 心瓣膜病
Vavular stenosis
Valvular insufficiency

40. Stenosis is the failure of a valve to open completely, thereby impeding forward flow.
Insufficiency, in contrast, results from failure of a valve to close completely, thereby allowing reversed flow.

41. Aortic stenosis: calcification of anatomically normal and congenitally bicuspid aortic valves.
Aortic insufficiency: dilation of the ascending aorta, related to hypertension and aging.
Mitral stenosis: rheumatic heart disease.
Mitral insufficiency: myxomatous degeneration (mitral valve prolapse).

42. AS MS 70% of all VHD Rheumatic Heart Disease
Calcification of a deformed valve
“Senile” calcific AS
Rheum, Heart Dis. MS
Rheumatic Heart Disease
If you have 4 valves and 2 possibilities of each valve (stenosis or regurgitation), then you have 8 possibilities, but these 2/8 cover 70% of all.
So that mean, practically, clinically, R>L and Stenosis>Regurgitation 42

43. 二尖瓣狭窄X线检查
左心房增大 胸骨左缘第3肋间心浊音界增大，使心腰消失，如梨形，称二尖瓣型心（三大一小—梨型心）
风心病二狭，瓣口面积1.2cm2

44. 主动脉瓣关闭不全X线检查 左心室增大 向左下增大，心腰加深，似靴形：见于主动脉瓣关闭不全 男，36岁，风心病，主动脉瓣关闭不全
左心室80mm

45. LVH (but no hypertension), ischemia
AORTIC STENOSIS
2X gradient pressure
LVH (but no hypertension), ischemia
Cardiac decompensation, angina, CHF
50% die in 5 years if angina present
50% die in 2 years if CHF present
LVH is almost a reflex knee jerk conclusion to AS, but in this case there may be NO systemic hypertension. 45

46. MITRAL ANNULAR CALCIFICATION
Calcification of the mitral “skeleton”
Usually NO dysfunction
Regurgitation usually, but Stenosis possible
F>>M 46

47. REGURGITATIONS MVP AR MR Rheumatic Infectious Aortic dilatations
Syphilis
Rheumatoid Arthritis
Marfan MR
MVP
Fen-Phen
Papillary muscles, chordae tendinae
Calcification of mitral ring (annulus)
Define:
Stenosis?
Regurgitation?
Insufficiency?
Incompetence?
Prolapse? 47

48. Mitral Valve Prolapse (MVP)
MYXOMATOUS degeneration of the mitral valve
Associated with connective tissue disorders
“Floppy” valve
3% incidence, F>>M
Easily seen on echocardiogram 48

49. MVP: CLINICAL FEATURES
Usually asymptomatic
Mid-systolic “click”
Holosystolic murmur if regurg. present
Occasional chest pain, dyspnea
97% NO untoward effects
3% Infective endocarditis, mitral insufficiency, arrythmias, sudden death 49

50. 50

51. VEGETATIONS INFECTIVE >5mm NON-Infective <5mm
Vegetations: 1) rheumatoid = small, at chordae tendinae junction, 2) infectious = big (>5 mm), 3) lupus (Libman-Saks) = BOTH sides
4) NBTE = non-bacterial thrombotic endocarditis (<5 mm) 51

52. ARTIFICIAL VALVES Mechanical Xenografts (porcine)
60% have complications within 10 years 52

53. 人工瓣膜置换术后并发症 1. PVE 2. Thrombosis and thromboembolism 3. Hemorrhage
4. Perivalvular leakage
5. AVB
6. Prosthetic valve functional disturbance