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ACQUIRED HEART DISEASES. Ma. Rhodora R. Garcia-de Leon,M.D. FPPS, FPCC. Acquired Heart Diseases. Acute Rheumatic Fever Valvular Heart disease Infective Endocarditis Myocarditis Pericarditis Kawasaki Disease. ACUTE RHEUMATIC FEVER.
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ACQUIRED HEART DISEASES Ma. Rhodora R. Garcia-de Leon,M.D. FPPS, FPCC
Acquired Heart Diseases • Acute Rheumatic Fever • Valvular Heart disease • Infective Endocarditis • Myocarditis • Pericarditis • Kawasaki Disease
ACUTE RHEUMATIC FEVER • Epidemiology: Most common cause of acquired heart disease in all age groups • In some developing countries, incidence is as high as 286/100,00 population • In the Phil. incidence is 0.9/1,000 pop • In the USA 0.5 /100,000 pop • Age predilection: 5-15 yrs • Predisposing Factors: Family History, poverty, poor hygiene, medical deprivation
Rheumatic Fever: Etiology • Believed to be an immunologic lesion that occurs as a delayed sequela to GROUP A STREPTOCOCCAL INFECTION of the pharynx, and not of the skin. • Certain serotypes of grp A strep are more frequently isolated: M types 1,3, 5, 6, 18 & 24
Rheumatic Fever : Pathology • The inflammatory lesion is found in many parts of the body, notably in the HEART, JOINTS, BRAIN and SKIN • Valvular damage most frequently involves the MITRAL VALVE, less commonly the AORTIC, rarely the TRICUSPID & PULMONARY VLAVES
Clinical Manifestations • Acute rheumatic fever is diagnosed by the use of the Revised/ Updated JONE’S CRITERIA • 5 Major criteria, 4 Minor criteria & Supporting Evidence of antecedent grp A infection • History of strep pharyngitis 1-5 wks (ave. 3 wks) prior to onset of symptoms; latent period of 2-6 mos in isolated chorea
JONES CRITERIA: Major Manifestations • MIGRATORY POLYARTHRITIS * most common: seen in 75% of patients * typically involves large joints: knees, ankles, wrists, elbows * joints are generally swollen, hot, red, & exquisitely tender * not deforming * dramatic response to salicylates
Jones Criteria: Major Manifestations • CARDITIS * occurs in 50% - 60% of cases * usually presents as tachycardia, cardiac murmurs , + / - myo or pericardial involvement * cardiomegaly * signs of CHF * echo findings of valvular regurg does not satisfy criteria if no auscultatory evidence
Jones Criteria: Major Manifestations • CHOREA (Sydenham chorea) * occurs in 10% - 15% of cases * usually presents as an isolated, subtle, neurologic behavior disorder * uncontrollable movements, emotional lability, incoordination, facial grimacing disappears w/ sleep * long latent period bet. infection & chorea * rarely leads to neurologic sequelae
Major Manifestations • ERYTHEMA MARGINATUM * rare, seen in less than 3% of cases * characteristic rash is erythematous, serpiginous, macular lesions w/ pale center, non-pruritic * usually seen in the trunk, not the face * accentuated by warming the skin
Major Manifestations • SUBCUTANEOUS NODULES * rare, seen in less than 1 % of cases * firm nodules, 1 cm in diameter, along extensor surfaces, near bony prominences
MINOR MANIFESTATIONS • ARTHRALGIA in the absence of arthritis as a major criterion • FEVER • ELEVATED ACUTE PHASE REACTANTS (ESR, C-reactive protein) • PROLONGED PR interval on ECG
SUPPORTING EVIDENCE OF GRP A STREP INFECTION • an ABSOLUTE REQUIREMENT for the diagnosis of RF • Streptococcal antibody tests most reliable • Elevated or increasing ASO antibody titers * Titers at least 333 TU in children; elevated in 80% of pxs • *Others: antideoxyribonuclease B, antistreptokinase, antihyaluronidase
Clinical Course • Only carditis can cause permanent cardiac damage. Signs of mild carditis disappear rapidly in wks; severe carditis longer, 2-6 mos. • Arthritis subsides in a few days to several wks; no permanent damage • Chorea gradually subsides in 6-7 mos or longer; does not cause neurologic sequelae
Treatment • Approaches to treatment: * 1. treat the grp A streptococcal infection single dose benzathine PCN; 10 days of appropriate oral antibiotics 2. use of anti-inflammatory agents to control clinical manifestations: aspirin or steroids 3. other support therapy: including Tx for CHF, chorea
Prevention • Primary Prophylaxis – to prevent an initial attack of RF, a 10-day course of oral PCN • Secondary Prophylaxis – to prevent colonization / infection with grp A strep every 21 or 28 days in pxs who already have RHD or had previous RF given for 10 yrs or longer: benzathine PCN, 1.2 MU, IM oral PCN V , 250mg BID; oral erythromycin 250mg BID; sulpha 1 gm OD