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Pathology of the Heart By: K.Mozaffari, MD, AP, CP.

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Presentation on theme: "Pathology of the Heart By: K.Mozaffari, MD, AP, CP."— Presentation transcript:

1 Pathology of the Heart By: K.Mozaffari, MD, AP, CP

2 Topics Congestive heart failure Ischemic heart disease Hypertensive heart disease Cor pulmonale Valvular heart disease Primary myocardial disease Congenital heart disease Pericardial disease Cardiac tumors

3 CONGESTIVE HEART FAILURE CONGESTIVE HEART FAILURE ( CHF) Inadequate output, forward failure Venous congestion, backward failure Left,right or all chambers involved Adaptive changes: 1-Catecholamines 2-Hypertrophy & dilation ischemic injury 3-2ndary hyperaldosteronism

4 Consequences Decompensation Venous congestion Pulmonary edema peripheral edema

5

6 MORPHOLOGY Heart :dilated chambers Lungs :boggy & congested with frothy fluid septal widening pale pink fluid hemorrhages & heart failure cells fibrosis, hemosiderin,brown induration

7 MORPHOLOGY Edema of soft tissues Fluid in body cavities Liver:nutmeg appearance centrilobular hemorrhagic necrosis cirrhosis

8 Clinical features Dyspnea Orthopnea,PND Venous congestion,edema Embolism Effusions Cyanosis,acidosis Ventricular arrhythmias

9 Ischemic heart disease Angina pectoris Acute MI Sudden cardiac death Chronic IHD with CHF

10 Epidemiology Any age, peak (60 in men,70 in women) Factors: HTN, DM, smoking, high LDL, genetics Regular exercise

11 Pathogenesis Critical stenosis:75% Acute plaque change Coronary artery thrombosis Coronary artery vasospasm

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13 Acute plaque change Fissuring, hemorrhage, rupture Disrupted plaque: Necrotic core & lipid Thin fibrous cap Rich in T cells & macrophages

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15 Coronary artery thrombosis Plaque rupture Platelet aggregation Thrombus formation Embolization

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17 Coronary artery vasospasm Tx-A2 Endothelial dysfunction Increased adrenergic activity Smoking

18 Angina pectoris Typical (stable): episodic pain to left arm 75% narrowing Relieved by rest or TNG Unstable : preinfarction increased frequency of pain Prinzmetal: at rest or sleep spasm

19 Myocardial Infarction 1.5 million / yr in the U.S deaths Men 4-5 times compared to women Risk factors the same as atherosclerosis

20 Pathogenesis Necrosis begins min after occlusion Subendocardial area more vulnerable Full size in 3-6 hrs Location of MI: site & anatomy of vessels involved (LAD,RCA) Size of MI: proximal lesions,larger infarcts collateral vessels limit the size

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22 MORPHOLOGY LAD 40-50%:anteroapical RCA 30-40%:posterior LV wall & septum LCX15-20% :lateral LV wall Transmural MI Subendocardial MI, inner third Isolated RV or atrial infarction,rare

23 No gross changes before 12 hrs hrs:slight pallor,mottling hrs:coagulation necrosis Wavy fiber change at periphery hrs:PMNs,peak on 3 rd day Contraction band at periphery with hemorrhage due to reperfusion Myocytolysis,subendocardial cells with influx of water(vacuolated)

24 4 th -7 th day: pale center, hyperemic border Macrophages, fibroblasts,capillaries migrate to center 10 th day: yellow,soft,sunken necrosis Red-purple periphery Granulation tissue & phagocytosis continue for weeks 4 th wk: resorbed necrosis less vascularity, more collagen 8 th wk: Dense scar thin,firm gray healed infarct

25 Recent MI

26 1 day old MI Necrosis Wavy fiber PMNs

27 3-4 day old Dense PMNs

28 Necrosis Hemorrhage Contraction band

29 7-10 days Complete phagocytosis

30 Granulation tissue

31 Healed MI

32 Scar tissue

33 Complications of MI Papillary muscle dysfunction & rupture External rupture of infarct Rupture of septum Mural thrombi Acute pericarditis Ventricular aneurysms

34 Papillary muscle dysfunction & rupture About 3 days after MI LV failure

35 External rupture of infarct Tamponade Between day 4-7

36 Rupture of septum Left-to-right shunt CHF

37 Mural thrombi Emboli to brain

38 Acute pericarditis Within 2-4 days May cause effusion

39 Ventricular aneurysms Thin-walled,fibrous outpouching Emboli CHF Papillary muscle dysfunction arrhythmias

40 Clinical features Chest pain:neck,jaw,epigastrium,left arm Rapid pulse,Diaphoresis & Dyspnea Pulmonary congestion & edema Cardiogenic shock,if 40% of LV involved “Silent MI” in DM, HTN, elderly pts ECG:Q waves,ST abnormality,T wave inversion

41 Lab markers 1-Total CK : sensitive, but not specific CK-MB: 2-4 hrs rise, 18 hrs peak MI excluded if no CK rise in first 2 days 2-cTnI: more specific than CK-MB Troponin remains elevated for 4-7 days 3-LD: used in the past

42 Chronic Ischemic Heart Disease Ischemic cardiomyopathy Progressive heart failure episodes of angina or MI Clinically similar to dilated CMP Severe coronary atherosclerosis Dilated chambers, fibrosis, hypertrophy Wall thickness may be normal Myocytolysis (vacuolated sarcoplasm)

43 Sudden cardiac death Death within 24 hrs The most common cause is IHD VF is the most common cause Acute plaque rupture, thrombosis,vasospasm

44 Causes of sudden cardiac death Coronary artery diseases Myocardial diseases Valvular diseases Conduction system abnormalities

45 Hypertensive heart disease LVH(AS or IHSS must be excluded) Pressure overload Increased metabolic needs Predisposed atherosclerosis IHD,CHF,MI,arrhythmias

46 Morphology Concentric hypertrophy Free wall >2cm Heart >450 g Enlarged hyperchromatic rectangular “box-car” nuclei Fibrosis Infarcts

47

48 Cor pulmonale Pulmonary heart disease Acute (emboli) >50% of vascular bed Chronic COPD RVH

49 Valvular heart disease Rheumatic fever & heart disease Calcific aortic stenosis.. Mitral valve prolapse Nonbacterial thrombotic Endocarditis Libman-sacks Endocarditis Infective Endocarditis Prosthetic cardiac valves

50 Rheumatic fever & heart disease

51 Clinical features 10 d-6 wk after pharyngitis Genetic susceptibilitiy Peak 5-15 yrs Streptolysin O, DNAse B Streptozyme test Migratory polyarthritis Pericardial effusion, tachycardia, CHF AF in MS Emboli endocarditis

52 Morphology Acute rheumatic fever: synovium,joints,skin,heart Fibrinoid necrosis Mixed inflammation Granuloma Fibrosis

53 Acute rheumatic carditis Pancarditis Aschoff bodies Anitschkow cells Diffuse interstitial infiltrates Fibrinous pericarditis Serous or serosanguineous effusion Verrucous endocarditis

54 Aschoff body

55 Verrucous endocarditis

56 Chronic rheumatic heart disease Mitral valve 95% Aortic & mitral valves 25% Right-sided valvular disease,uncommon Stenosis and/or regurgitation Heart failure Infective endocarditis

57 Chronic rheumatic mitral valvulitis Fish mouth Fused cords More in Females LA thrombi Passive lung congestion Regurgitation less frequent

58

59 Chronic aortic valvulitis More in males Always with mitral valvulitis AS lesds to LVH,CHF Fibrosis may cause AR

60 Other organs in rheumatic disease Arthritis: large joints, self-limited Pulmonary: chronic inflammation Subcutaneous nodules (Aschoff body) Erythema marginatum (maculopapular)

61 Time for a quiz !

62 1 What is a heart failure cell?

63 Alveolar macrophage filled with hemosiderin

64 2 Nutmeg –like appearance is seen on the cut surface of which organ?

65 Centrilobular hemorrhagic necrosis of hepatocytes in CHF

66 3 Silent MI is seen in which of the following? 1-diabetes mellitus 2-hypertensive patients 3-elderly patients 4-all of the above

67 4 Which enzyme is more specific for myocardial injury? Total CK CK-MB Troponin I LD

68 5 Anitschkow cells are seen in……….

69 Acute rheumatic carditis

70 6 Fish mouth deformity is seen in …….

71 Mitral stenosis

72 7 Erythema marginatum is seen in………

73 It is a maculopapular rash in acute rheumatic fever

74 8 Which coronary artery is more likely to sustain an infarction? Left anterior descending coronary artery Right coronary artery Left circumflex coronary artery

75 9 Which morphological finding appears later in the course of an MI? Coagulation necrosis Wavy fiber change PMN infiltration Granulation tissue

76 10 What is your diagnosis?

77 Cor pulmonale

78 Have a good day !

79 Calcific aortic stenosis Degeneration due to aging Sclerosis & calcification Angina,syncope,CHF Symptoms occur yrs earlier in bicuspid valves

80 Mitral valve prolapse The most common cause of isolated MR 3-5% of general population More in females, yrs of age Loose ground substance, floppy valve Also in marfan syndrome No symptoms or palpitation, fatigue, atypical chest pain Valve rupture, endocarditis,sudden death, LA thrombi

81

82 Nonbacterial thrombotic Endocarditis (NBTE) Small, sterile, friable (fibrin & platelets) Aortic > Mitral valve Hypercoagulable state ( DVT) Adenocarcinoma in 50 % Marantic endocarditis Free from inflammation or fibrosis After healing: lambl excrescences Emboli to brain or IE

83

84 Libman-sacks Endocarditis Sterile In SLE patients On mitral or tricuspid valves No predilection for closure lines

85 Infective Endocarditis Vegetations on Valve or mural endocardium Acute: little host response Subacute: abnormal valves local response granulation tissue Vegetations on Valve or mural endocardium Acute: little host response Subacute: abnormal valves local response granulation tissue

86 Etiology & Pathogenesis Bacteremia: procedures, urinary or intravascular catheters Tooth brushing NBTE IVDA (right-sided valves) Prosthetic valves (10-20% of cases) Cardiac abnormality: ( calcific AS, VSD, RHD, MVP )

87 Clinical features High fevers, chills, septicemia Low-grade fever, malaise,weight loss Changing murmurs Splenomegaly, clubbing of digits Emboli: infarcts, mycotic aneurysms, petechiae GN (Immune complex mediated )

88 Microorganisms in IE Native valve: S.Viridans (50-60%) S.Aureus (10-20%) Enterococci & HACEK (oral commensal) Prosthetic valve: Staph epidermidis G¯ bacilliFungi IVDA:S.aureusG¯ bacilliFungi & Strep

89 Acute Endocarditis Valve destruction Ring abscess Emboli Abscess at sites of emboli

90 Organisms Fibrin Blood cells

91 Subacute Endocarditis Firmer vegetations Less valve destruction Granulation tissue at base of vegetation Fibrosis,calcification Systemic emboli,less likely to undergo suppuration

92 Prosthetic cardiac valves Bioprosthetic (porcine,bovine,human) mechanical

93 complications Stiffening, calcification, perforation Thrombi Infective endocarditis Paravalvular leaks Hemolysis

94 Primary myocardial disease Myocarditis Cardiomyopathies

95 Myocarditis InfectionsImmune- mediated unknown Viral,chlamydial, Rickettsial, bacterial, Fungi,protozoal, helminthic Postviral, Rheumatic, SLE, Drug-induced, Transplant rejection Sarcoidosis Giant cell myocarditis

96 Clinical features Coxackieviruses,the most common cause Asymptomatic to severe CHF Sudden death Dilated CMP

97 Morphology Flabby,pale myocardium Mottled by hemorrhage Abscess in bacterial cases CMV inclusions Lymphocytic infiltrate & necrosis Later fibrosis is seen

98 Viral myocarditis

99 Chagas disease

100 Microabscess

101 Cardiomyopathies

102 Dilated CMP Hypertrophy, dilation, contractile dysfunction late stage of viral myocarditis Alcohol abuse Cobalt,doxorubicin Peripartum Cytoskeletal proteins mutations (dystrophin gene) Sarcomere protein genes (myosin,troponin)

103 Clinical features The most common CMP (90% of cases) The most common Dx in transplant candidates At any age (peak yrs) Sporadic or familial More in men EF <25%, progressive CHF In peripartum cases 50% recover

104 Morphology Large,flabby heart >900 g Hypertrophy & dilation in all chambers Fibrosis scant inflammation Fragile mural thrombi Emboli

105 Hypertrophic CMP IHSS (LVOT obstruction) Abnormal diastolic filling Systolic anterior motion of mitral leaflet β myosin heavy chain gene mutation Dyspnea, ischemia,angina,sudden death Risk of IE Later fibrosis & CHF

106 Morphology Hypertrophy of LV septum >800 g Haphazard hypertrophy & branching myocytes

107 Restrictive CMP Endomyocardial fibrosis: idiopathic,tropical Eosinophilic endomyocardial fibrosis: (loffler syndrome) Endocardial fibroelastosis: <2 y/o children,valvular abnormality Cardiac amyloidosis Hemochromatosis Radiation injury to heart

108 Clinical features Impaired diastolic filling due to inelastic ventricle Fatigue,dyspnea,chest pain,CHF Mural thrombi in loffler syndrome Conduction system involvement by fibrosis DDx;constrictive pericarditis

109 Morphology Thick & opaque fibrotic endocardium Eosinophilic infiltration(loffler syndrome) Endocardial fibroelastosis: porcelain-like appearance,may be local Amyloidosis:green birefringence,congo red Hemochromatosis:iron stained blue,perl stain

110 Congenital heart disease L-R shunts : 1-ASD 2-VSD 3-PDA R-L shunts : 1-Tetralogy of Fallot 2-TGA Coarctation of aorta

111 Etiology 8/1000 live births Genetic factors(trisomies) Environmental factors(rubella) Idiopathic (multifactorial):90% L-R shunt,no cyanosis until reversal of flow due to PH R-L shunt,cyanosis from birth

112 Atrial septal defect (ASD) Foramen ovale closes at birth Ostium secundum ASD:75% Ostium primum ASD :15% Sinus venosus ASD:10% The most common congenital cardiac disease first diagnosed in adults

113 ASD

114 Ventricular septal defect (VSD) The most common congenital heart defect at birth Many close spontaneously in childhood Risk of IE

115 VSD

116 Patent ductus arteriosus(PDA) Functional closure: 1-2 days after birth Ligamentum arteriosum: after a few months In RDS :delayed closure Machinary murmur Risk of IE

117 Tetralogy of Fallot The most common cyanotic congenital heart disease VSD Dextraposed overriding aorta RVH RVOT obstruction

118 Morphology Boot-shaped heart Shunt extent determined by RVOT obstruction Cyanosis PH does not develop Erythrocytosis, clubbing digits IE risk Emboli:brain abscess

119 Transposition of great arteries Complete form incompatible with life Those who survive have ASD,VSD or PDA RVH cyanosis

120 Coarctation of aorta Isolated lesion in 50% Also common in turner syndrome Preductal (infantile type): CHF, lower limbs cyanosis weak femoral pulses Postductal (more common): Hypertension of upper limbs Weak pulses in legs

121 Coarctation of aorta

122 Pericardial disease Pericarditis Pericardial effusions

123 Pericarditis Primary, uncommon: Viral (most cases) Bacteria, fungi, mycobacteria Secondary, more often: Following MI or cardiac surgery Radiation Uremia (the most common systemic disorder) RF & SLE Metastases (bloody)

124 Course Immediate hemodynamic complications (significant effusion) Resolution without sequelae Progression to chronic fibrosing process

125 Clinical features Atypical chest pain & friction rub Tamponade in acute forms: distant heart sounds, distended neck veins reduced cardiac output, shock Chronic constrictive form: Fibrotic scar tissue venous distention & low output DDx: restrictive CMP

126 Morphology Fibrinous exudation Shaggy Bread & butter

127

128 Pericardial effusions Serous: CHF, Albumin Serosanguineous: blunt chest trauma, malignancy Chylous: lymphatic obstruction Hemopericardium (pure blood): Aortic or myocardial rupture, penetrating trauma

129 Cardiac tumors Metastases: More common than primary tumors Most often involve pericardium Lung, breast, melanoma & hematopoietic are frequent primaries

130 Primary tumors (rare): Myxoma Lipoma Papillary elastofibroma Rhabdomyoma Angiosarcoma Rhabdomyosarcoma

131 Myxoma Most in LA Any age Sessile or pedunculated Stellate cells Mucopolysaccharide-rich stroma Smooth muscle cells Emboli Ball-valve obstruction Syncope & death

132 Myxoma

133 Rhabdomyoma Most common primary cardiac tumors in childhood Seen with tuberous sclerosis Mass projecting into lumen Solitary or multifocal

134 Rhabdomyoma Spider cells contain glycogen

135 Wake up! we have a quiz now

136 1 A 20 y/o girl with atypical chest pain & fatigue has mitral regurgitation on echocardiography. which statement is false: 1-fish mouth deformity of mitral valve 2-loose edematous valve tissue 3-association with marfan syndrome 4- risk of endocarditis or death

137 2 A 50 y/o man with advanced gastric cancer has small vegetations on mitral valve.After an embolic episode,leading to brain lesions he died.This lesion is called : 1-libman-sacks endocarditis 2-nonbacterial thrombotic endocarditis 3-subacute infective endocarditis 4- rheumatic endocarditis

138 3 A young boy fell unconscious & died immediately,while playing basketball.Autopsy showed a large heart with disarray of myocytes.what is your diagnosis? 1-myocarditis 2-hypertrophic cardiomyopathy 3-restrictive cardiomyopathy 4-dilated cardiomyopathy

139 4 Alcohol is likely to cause…….. 1-dilated CMP 2-ischemic CMP 3-hypertrophic CMP 4-restrictive CMP

140 5 Amyloidosis & hemochromatosis are examples of…………….. 1-dilated CMP 2-ischemic CMP 3-restrictive CMP 4-hypertrophic CMP

141 6 Loffler syndrome is associated with all of the following,except…. 1-thrombi & emboli 2-hypereosinophilia 3-constrictive pericarditis 4-restrictive cardiomyopathy

142 7 Which lesion is associated with early cyanosis? 1-ASD 2-VSD 3-PDA 4-tetralogy of fallot

143 8 What is the most common primary cardiac tumor in adults? 1-lipoma 2-rhabdomyoma 3-angiosarcoma 4-myxoma

144 9 Stellate cells in a loose stroma with smooth muscle cells are seen in…… 1-rhabdomyoma 2-lipoma 3-myxoma 4-angiosarcoma

145 10 Vegetations of subacute infective endocarditis are distinguished from those of the acute form by…….. 1-presence of fibrin & blood cells 2-size of the vegetations 3-location of vegetations 4-granulation tissue formation

146 11 All lesions cause hemopericardium, except? 1-uremia 2-ruptured aortic aneurysm 3-penetrating trauma to heart 4-ruptured MI

147 12 What is this type of endocarditis called?

148 13 What is this lesion called?

149 14 What do you see in the photographs?

150 15 A 22 y/o IV drug abuser is likely to present with which of the following? 1- Eosinophilic infiltration of myocardium 2-Tricuspid valve endocarditis 3-constrictive pericarditis 4- Hypertrophic cardiomyopathy

151 16 A 50 y/o man with a history of malignant melanoma presents with dyspnea & muffled heart sounds.what is your diagnosis? 1-Nonbacterial thrombotic endocarditis 2-Restrictive cardiomyopathy 3-Bloody pericardial effusion 4-Infective endocarditis

152 17 A 10 y/o child has a cardiac mass,the cells of which contain glycogen. what is your diagnosis? 1-lipoma 2-myxoma 3-rhabomyoma 4- angiosarcoma

153 18 A girl with turner syndrome has high blood pressure in her upper limbs & weak pulses in her legs. what is your diagnosis? 1-tetralogy of fallot 2-ASD 3-VSD 4-coarctation of aorta

154 19 A 55 y/o man with bicuspid aortic valve is likely to have 1-floppy valve 2-calcification 3-fish mouth deformity 4-all of the above

155 20 Most cases of primary pericarditis are due to…. 1-Viruses 2-Bacteria 3-Fungi or mycobacteria 4-MI or cardiac surgery

156 Good bye & Good luck


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