1. ACQUIRED HEART DISEASES
Ma. Rhodora R. Garcia-de Leon,M.D.
FPPS, FPCC

2. Acquired Heart Diseases
Acute Rheumatic Fever
Valvular Heart disease
Infective Endocarditis
Myocarditis
Pericarditis
Kawasaki Disease

3. 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: yrs
Predisposing Factors: Family History, poverty, poor hygiene, medical deprivation

4. 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

5. 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

6. 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

7. 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

8. 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

11. 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

12. 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

14. Major Manifestations SUBCUTANEOUS NODULES
* rare, seen in less than 1 % of cases * firm nodules, 1 cm in diameter, along extensor surfaces, near bony prominences

16. 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

17. 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

18. 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

20. 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

21. 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