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5: ENDOCARDITIS_RHEUMATIC FEVER T. Bacoat – Jones, DO, MPH Assistant Professor Fundamental Sciences NSU-KPCOM OBJECTIVES 1. Discuss the etiology, morphology, and secondary effects of rheumatic carditis 2. Discuss the etiology, pathogenesis, morphology, and complications of infective endocarditis 3....

5: ENDOCARDITIS_RHEUMATIC FEVER T. Bacoat – Jones, DO, MPH Assistant Professor Fundamental Sciences NSU-KPCOM OBJECTIVES 1. Discuss the etiology, morphology, and secondary effects of rheumatic carditis 2. Discuss the etiology, pathogenesis, morphology, and complications of infective endocarditis 3. Describe the major pathologic features of nonbacterial thrombotic endocarditis, Libman-Sacks endocarditis, and rheumatoid heart disease 4. Describe the major features of prosthetic valve complications DEFINITION Rheumatic fever (RF) An acute immune-mediated, multisystem disease that occurs a few weeks after an episode of a streptococcal infection (group A) Usually following pharyngitis Rarely other streptococcal infections of other sites such as skin infections Affects: multisystem involvement Rheumatic Heart Disease- chronic stage the heart valves have been permanently damaged by rheumatic feverhttps://www.hopkinsmedicine.org/health/conditions-and-diseases/rheumatic-heart-disease A sore throat about onemonth prior with fever, lasted about one week then resolved Was fine afterwards Developed left knee pain and swelling 2 weeks after onset of sore throat and fever Also has developed a non-itchy rash After a latent period of 1 - 3 weeks, Ivyzen.net Can A First Episode of Acute Rheumatic Fever (ARF) occur at any age but occurs most often between 5 years and 15 years of age, which are the peak years of age for streptococcal pharyngitis Acute Rheumatic Fever is rare before 3 years and after 21 years However, preceding symptomatic pharyngitis is recognized in only about two thirds of patients with Acute Rheumatic Fever Watkins DA, Johnson CO, Colquhoun SM, et al https://www.merckmanuals.com/professional/infectious-diseases/gram-positive-cocci/streptococcal-infections Delayed immune response to infection with  Acute rheumatic fever develops in 0.3 to 3% of untreated cases of acute Group A Beta - hemolytic strep (GAS) pharyngitis Rheumatic Fever Girls > boys Common in 3rd world countries Incidence: - related to frequency and severity of Streptococcal pharyngeal infection - more during winter & early spring - environmental factors- poor sanitation, poverty, - overcrowding : greater spread of infection, following epidemics of Strep pharyngitis https://nova.lecturio.com/#/lecture/s/8876/30006 (Hyperpyrexia) Diagnosis Jones Criteria for Acute Rheumatic Fever Diagnosis Jones Criteria for Acute Rheumatic Fever Major Criteria Minor Criteria 1. Fever (hyperpyrexia) 2. Polyarthritis 2. Arthralgia (joint pain with no signs of inflammation) 3. Chorea 4. Erythema marginatum 5. Subcutaneous nodules 3. Elevated Acute Phase reactants erythrocyte sedimentation rate or Creactive protein level (ESR or CRP) 4. Prolonged PR interval on electrocardiogram (ECG) ESR – erythrocyte sedimentation rate; CRP – C-reactive protein https://www.medscape.com/answers/228936-15667/what-are-the-jones-criteria-for-diagnosis-of-acute-rheumatic-fever-arf 2021 1. Carditis Major Criteria Low Risk vs High Risk Populations Low Risk Population Carditis (clinical or subclinical) Arthritis – only polyarthritis Chorea Erythema marginatum Subcutaneous nodules High Risk Population Carditis (clinical or subclinical) Arthritis – monoarthritis or polyarthritis Polyarthralgia Chorea Erythema marginatum Subcutaneous nodules Minor Criteria Low Risk Population Polyarthralgia Hyperpyrexia (≥ 38.5ºC) ESR ≥ 60 mm/h and/or CRP ≥ 3.0 mg/dl Prolonged PR interval (after taking into account the differences related to age; if there is no carditis as a major criterion) High Risk Population Monoarthralgia Hyperpyrexia (≥ 38.0ºC) ESR ≥ 30 mm/h and/or CRP ≥ 3.0 mg/dl Prolonged PR interval (after taking into account the differences related to age; if there is no carditis as a major criterion) ESR – erythrocyte sedimentation rate; CRP – C-reactive protein https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5911656/table/t0002/?report=objectonly Clinical Features Rheumatic Fever (RF) Major Criteria 1. Carditis 80% of patient with Rheumatic Fever (Rheumatic Carditis) Manifests as pancarditis (endocarditis, myocarditis and pericarditis), occurs in 40-50% of cases Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ Valvulitis occur in acute phase Chronic phase- fibrosis, calcification & stenosis of heart valves (fish mouth valves) Major Criteria 1. Rheumatic Fever: Carditis(Rheumatic Carditis) Acute RF: valves are thick, loss of transparency 1-3 mm small warty (verrucous) vegetations along the line of closure Most common: Tricuspid valve IV drug abusers Free margins of cuff appears rough, irregular Attach firmly; do not embolize Mitral & aortic valves commonly involved- greater mechanical stress https://www.sciencedirect.com/topics/medicine-and-dentistry/rheumatic-heart-disease Major Criteria Chronic Rheumatic Heart Disease Organization of inflammation → heals by fibrosis Permanent deformity of valves: - leaf thickening, commissural fusion - shortening, thickening and fusion of chordae tendineae Most frequency cause of Mitral stenosis (99%)Fish mouth or button hole stenosis of Mitral valve in Rheumatic Heart Disease Mitral Stenosis in 65-70% cases, mitral + aortic in 25% cases https://medchrome.com/basic-science/pathology/rheumatic-fever-rheumatic-heart-disease/ 1. Rheumatic Fever: Carditis(Rheumatic Carditis) Major Criteria 1. Rheumatic Fever: Carditis(Rheumatic Carditis) Cardiac Lesions Focal interstitial involvement of all the three layers of heart : Pancarditis Pathognomonic feature is Aschoff nodule/ body 1-2 mm tiny structures found in the vicinity of small blood vs in the endo and myocardium https://casereports.bmj.com/content/13/2/e231888 Major Criteria 2. Arthritis: earliest manifestation Occurs in 80% Begins in large joint of lower extremities Commonly - knee, ankle, elbow & wrist Painful, swollen, erythematous In children below 5 years arthritis usually mild but carditis more prominent Arthritis does not progress to chronic disease https://line.17qq.com/articles/qqnffwsmqy_p3.html https://www.sciencedirect.com/topics/neuroscience/rheumatic-fever Flitting (from joint to joint over a period of days) Fleeting (transient) migratory polyarthritis, involving major joints Major Criteria 3. Sydenham Chorea (St. Vitus Dance) Sydenham chorea (SC) is a neurological disorder of childhood resulting from infection via Group A beta-hemolytic streptococcus (GABHS), the bacterium that causes rheumatic fever. Occurs in 5-10% of cases Mainly in girls of 3-15 years age Late manifestation: 6 months after the attack of Rheumatic Fever SC is characterized by rapid, irregular, and aimless involuntary movements of the arms and legs, trunk, and facial muscles. Clumsiness, deterioration of handwriting, emotional lability or grimacing of face https://www.youtube.com/watch?v=wTCnbga3sqg Major Criteria 4. Erythema Marginatum Occurs in females INFECTIVE ENDOCARDITIS Predisposing conditions Valvular heart disease Congenital Degenerative disease – mitral and/or aortic regurgitation ccom/#/lecture/s/8836/30932 Mitral valve prolapse with regurgitation -3-to-8-fold increase of infective endocarditis Mitral valve prolapse without regurgitation –Risk = general population, small risk Rheumatic heart disease (3%) Presence of a prosthetic valve (20%) Endocardial devices- pacemaker, implantable cardiac defibrillator, increase life expectancy Non-cardiac underlying disease – Intravenous drug users (accounts for recent increase cases), chronic IV access Decrease use of prophylactic antibiotic use for routine dental Disease characterized by the presence of vegetations on cardiac valves consist of fibrin and platelet aggregates and devoid of inflammation or bacteria – sterile (no organisms or inflammation) Non-bacterial thrombotic endocarditis (NBTE) is not a common entity it is frequently underestimated Over the years Non-bacterial thrombotic endocarditis (NBTE) Has been been recognized as a condition associated with the probability of underlying diseases (cancer, autoimmune disorders, HIV) a potentially life-threatening source of thromboembolism to the brain, heart, spleen, kidney and other important organs Factors implicated in the initiation are (a)immune complexes (b)Hypoxia (c)hypercoagulability A type of sterile nonbacterial thrombotic endocarditis (NBTE) secondary to inflammation The most characteristic cardiac manifestation of the autoimmune disease systemic lupus erythematosus (SLE; lupus) The following signs of ventricular enlargement and cardiac failure may be noted in Libman-Sacks endocarditis: Malignancy and hypercoagulable states tachypnea and cyanosis are also associated with the formation of verrucous endocarditis. pulse - Plateau pulse, low-volume pulse, Most commonly involves the surface of the mitral and aortic valves All four cardiac valves and endocardial surfaces such as the chordae tendineae and endocardium surface can be involved pulsus alternans (arterial pulse alternates between strong and weak beats) jugular venous distention displaced apex beat third and/or fourth heart sounds pulmonary rales congestive hepatomegaly https://emedicine.medscape.com/article/155230-overview The presence of rheumatoid arthritis (RA) may be associated with an increased risk of major adverse cardiovascular events, also known as MACE. People with osteoarthritis may also face a 24 percent higher risk for cardiovascular disease than the general population. Pathophysiology: Elevation of inflammatory markers Circulating acute phase reactants C-reactive protein (CRP) Erythrocyte sedimentation rate (ESR) rheumatoid factor (RF) anti-citrullinated protein antibodies https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3890244/ https://www.healthline.com/health-news/rheumatoid-arthritis-may-increase-risk-for-major-cardiovascular-events Non-Infective Endocarditis https://www.grepmed.com/images/11688/marantic-nbte-noninfective-endocarditis-pathophysiology * *Carcinomatosis = A condition in which cancer is spread widely throughout the body, or, in some cases, to a relatively large region of the body https://www.grepmed.com/images/11688/marantic-nbte-noninfective-endocarditis-pathophysiology Vegetation are not caused by infection Less common than infective endocarditis prosthetic valve obstruction (including thrombosis and pannus formation), patient-prosthesis mismatch embolic events bleeding (hemorrhaging) Structural deterioration particularly bioprosthetic valves constructed from porcine or bovine pericardium (animal tissue) prosthetic heart valve regurgitation (valvular and paravalvular) infective endocarditis ** One of the deadliest complications of prosthetic valves (highest mortality rate) prosthetic valve-related hemolysis (less common in mechanical heart valves) hemolytic anemia https://www.uptodate.com https://mypages.unh.edu/ethics-legal-telehealth-braswell-miller-nelson/resources DeGaetano, H. M. (2019). Endocarditis Rheumatic Fever. FL, US. Pity, I. S. (2019). Rheumatic Fever and Rheumatic Hear Disease. Retrieved from https://www.slideshare.net/AhmedAhmed683/rheumaticfever-rhd-and-infective-endocarditis Global, regional, and national burden of rheumatic heart disease, 1990– 2015. N Engl J Med 377:713–722, 2017. doi: 10.1056/NEJMoa1603693 https://u.osu.edu/rheumaticfever08184269/quiz-questions/ https://www.doctorsintraining.com/mkt/qa/ https://study.com/academy/exam/topic/rheumatic-fever-heart-disease.html https://emedicine.medscape.com/article/236582-questions-and-answers https://www.studyblue.com/notes/note/n/valvular-heart-disease-guo/deck/12368289 https://www.chairish.com/

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