1. VALVULAR HEART DISEASE Arthur C. Aufderheide, M.D. Med 6655. Cardiovascular System

2. RHEUMATIC HEART DISEASE (RHD): ACUTE (1) Acute rheumatic fever Group A beta-hemolytic strep (pharyngitis). In only 3% of these, rheumatic fever develops 2-6 weeks later Strep and endocardium = u shared antigen (M protein: M 18 ?) responsible for virulence (molecular mimicry) u capsular glycoprotein

3. RHEUMATIC HEART DISEASE (RHD): ACUTE (2) Clinical: u brain (Sydenham’s chorea [St. Vitus’ dance]) – purposeless, erratic motor movements u joints, skin, heart, pancarditis Incidence: declining u 1946 = 343 / 100,000 u 1986 = 0.3 / 100,000 u But still in  Third World U.S.A. Why?

4. RHEUMATIC HEART DISEASE: ACUTE Pancarditis: u Myocarditis (Aschoff bodies) w collagen focal necrosis with granuloma (histiocytic cells — multinucleate and Anitschkow) u Endocarditis u Pericarditis (fibrinoid)

5. Myocardial Aschoff body

6. Ashoff body

7. Lymphs,macs,multinucleates

8. Anitschkow cells

9. ACUTE RHD: ENDOCARDITIS Valves: u Mitral and aortic (tricuspid rare; pulmonary, "never") u Collagen necrosis: w  surface thrombus ("verrucae") w  murmur Active inflammation may continue for years after other lesions heal

10. Endocardial Aschoff body

11. pericarditis fibrinous

12. ACUTE RHD: LAB TESTS ASO (Anti-Streptolysin-O Titer — Todd test) u Others: anti-DNase B, antistreptokinase CRP (C-Reactive Protein) ECG (  P-R interval) None of above are specific for rheumatic fever

13. Minithroimbus on mitral cusp (Aschoff )

14. RHEUMATIC VEGETATIONS

16. CHRONIC RHD: MITRAL STENOSIS (1) Most common. Mostly females. Post-rheumatic fever. Thick, fibrous, calcified valve Functional defect: stenosis > regurgitation.  Lt. atrium  fibrillation (big problem)  stasis  thrombi  emboli: u  lung u  systemic

17. MITRAL STENOSIS

19. Huge left atrium

21. Short chordae

22. CHRONIC RHD: MITRAL STENOSIS (2) Lt. atrium fails  chronic passive congestion lung: u  lung hemosiderosis + fibrosis u  pulmonary hypertension u  acute pulmonary arteritis (>60 mm) Rx: u medical; balloon valvuloplasty; surgical repair preferred; prosthesis, antibiotics

23. Fishmouth mitral

24. Mitral stenosis

25. Normal LV hypertrophy Mitral stenosis

27. Atrial thrombus

28. Embolus infarct,kidney

29. Embolus infarct,spleen

30. Embolus infarct,brain

31. Calcified thrombi on mitral valve

32. Calcified embolus to coronary artery

33. CHRONIC RHD: MITRAL STENOSIS (2) Lt. atrium fails  chronic passive congestion lung: u  lung hemosiderosis + fibrosis u  pulmonary hypertension u  acute pulmonary arteritis (>60 mm) Rx: u medical; balloon valvuloplasty; surgical repair preferred; prosthesis, antibiotics

34. Mitral stenosis RVH

35. PULMONARY CONGESTION

36. HEMOSIDERIN-FILLED MACROPHAGES

37. PULMONARY FIBROSIS

39. Pulmonary arteritis

40. CHRONIC RHD: MITRAL STENOSIS (2) Lt. atrium fails  chronic passive congestion lung: u  lung hemosiderosis + fibrosis u  pulmonary hypertension u  acute pulmonary arteritis (>60 mm) Rx: u medical; balloon valvuloplasty; surgical repair preferred; prosthesis, antibiotics

41. Chordae fused, gone

43. CHRONIC RHD: MITRAL REGURGITATION Rarely “pure” if due to rheumatic fever Left heart failure occurs later than in mitral stenosis Other causes: u ruptured papillary muscle after infarction u mitral valve prolapse

44. AORTIC STENOSIS: CAUSES Congenital: u Stenotic at birth u Bicuspid; later calcification Rheumatic heart disease Atherosclerosis. Rare. Idiopathic u “degenerative” calcific aortic stenosis: most common today in USA

45. Normal aortic valve

47. Bicuspid aortic valve stenosis

48. AORTIC STENOSIS: CAUSES Congenital: u Stenotic at birth u Bicuspid; later calcification Rheumatic heart disease Atherosclerosis. Idiopathic “degenerative” calcific aortic

49. Normal aortic valve

51. rheumatic aortic stenosis;fused cusps

53. AORTIC STENOSIS: CAUSES Congenital: u Stenotic at birth u Bicuspid; later calcification Rheumatic heart disease Atherosclerosis. Rare. Idiopathic u “degenerative” calcific aortic stenosis: most common today in USA

54. Calcific aortic stenosis,etiology?

55. CHRONIC RHD: AORTIC STENOSIS (1) Given amount commissural fusion has more functional impairment of aortic than mitral Left Ventricle  (pressure and hypertrophy) Angina: u Systolic coronary artery compression slows or stops flow in myocardial capillaries. u  BP in Valsalva sinus (Venturi effect) u Valve calcification extends to narrow coronary artery orifice u Coronary atherosclerosis

56. CHRONIC RHD: AORTIC STENOSIS (2) Sudden death Stokes-Adams syncope (arrhythmia? exercise  systemic resistance  but output fixed by stenosis Left failure late but resists Rx. Hence, surgical repair before failure occurs. Failure: diastolic filling defect plus systolic contractility are decreased because afterload is increased u symptoms correlate with degree of stenosis

57. Calcific aortic stenosis, x-ray

58. AORTIC STENOSIS: Rx Medical Surgical: u Congenital, infant: valvulotomy u Acquired: w can’t repair native valve surgically, so use prosthesis w perform before symptoms severe w Quantitative CT scan good to assess status

63. Plastic ball cracked

64. ball escaped cage aorta

65. CLOT

66. CHRONIC RHD: AORTIC REGURGITATION RHD infrequent cause (some due to bacterial endocarditis or aortic dissection) Syphilis: u Commissure separation (stretched ring) u Affects ascending aorta most u Mesoaortitis weakens aorta wall  aneurysm u Symptoms do not correlate well with degree of insufficiency

67. MITRAL VALVE PROLAPSE (1) Mitral leaflet bulging into atrium in systole Valve leaflets deformed by focal myxomatous degeneration of valve connective tissue: u Marfan syndrome (fibrillin gene defect) u Chemical change: myxomatous degeneration u  in Down syndrome

68. MITRAL VALVE PROLAPSE

73. MITRAL VALVE PROLAPSE (2) Sx: systolic click. May be asymptomatic. Dx: Cardiac imaging Complications: u Left failure due to mitral regurgitation u Infectious endocarditis u Arrhythmias with sudden death u Embolism from valve thrombi Rx: Surgical repair, prosthesis

74. CHEMICAL CAUSES OF VALVULAR DISEASE Fenfluramine, phentermine (fen-phen): u Fibrous thickening of all valves u Produces pulmonary hypertension Carcinoid tumor (serotonin) Ergot alkaloids (ergotamine; methysergide) u Also pergolide mesylate (used to Rx Parkinson disease) Mechanism of all of above: deposit of fibrous tissue plaque on valve with normal architecture