ACQUIRED HEART ACQUIRED HEART DISEASESDISEASES

Ma. Rhodora R. Garcia-de Leon,M.D.Ma. Rhodora R. Garcia-de Leon,M.D.FPPS, FPCCFPPS, FPCC

Acquired Heart DiseasesAcquired Heart Diseases

Acute Rheumatic FeverAcute Rheumatic Fever

Valvular Heart diseaseValvular Heart disease

Infective EndocarditisInfective Endocarditis

MyocarditisMyocarditis

PericarditisPericarditis

Kawasaki DiseaseKawasaki Disease

ACUTE RHEUMATIC FEVERACUTE RHEUMATIC FEVER

EpidemiologyEpidemiology: Most common cause of : Most common cause of acquired heart disease in all age groups acquired heart disease in all age groups In some developing countries, incidence is In some developing countries, incidence is as high as 286/100,00 populationas high as 286/100,00 populationIn the Phil. incidence is 0.9/1,000 popIn the Phil. incidence is 0.9/1,000 popIn the USA 0.5 /100,000 popIn the USA 0.5 /100,000 popAge predilection: 5-15 yrsAge predilection: 5-15 yrsPredisposing Factors: Family History, Predisposing Factors: Family History, poverty, poor hygiene, medical deprivation poverty, poor hygiene, medical deprivation

Rheumatic Fever: EtiologyRheumatic Fever: Etiology

Believed to be an immunologic lesion that Believed to be an immunologic lesion that occurs as a delayed sequela to GROUP A occurs as a delayed sequela to GROUP A STREPTOCOCCAL INFECTION of the STREPTOCOCCAL INFECTION of the pharynx, and not of the skin.pharynx, and not of the skin.

Certain serotypes of grp A strep are more Certain serotypes of grp A strep are more frequently isolated: M types 1,3, 5, 6, 18 frequently isolated: M types 1,3, 5, 6, 18

& 24& 24

Rheumatic Fever : PathologyRheumatic Fever : Pathology

The inflammatory lesion is found in many The inflammatory lesion is found in many parts of the body, notably in the HEART, parts of the body, notably in the HEART, JOINTS, BRAIN and SKINJOINTS, BRAIN and SKIN

Valvular damage most frequently involves Valvular damage most frequently involves the MITRAL VALVE, less commonly the the MITRAL VALVE, less commonly the AORTIC, rarely the TRICUSPID & AORTIC, rarely the TRICUSPID & PULMONARY VLAVESPULMONARY VLAVES

Clinical ManifestationsClinical Manifestations

Acute rheumatic fever is diagnosed by the Acute rheumatic fever is diagnosed by the use of the use of the Revised/ Updated JONE’S CRITERIA Revised/ Updated JONE’S CRITERIA5 Major criteria, 4 Minor criteria & 5 Major criteria, 4 Minor criteria & Supporting Evidence of antecedent grp A Supporting Evidence of antecedent grp A infectioninfectionHistory of strep pharyngitis 1-5 wks (ave. 3 History of strep pharyngitis 1-5 wks (ave. 3 wks) prior to onset of symptoms; latent wks) prior to onset of symptoms; latent period of 2-6 mos in isolated choreaperiod of 2-6 mos in isolated chorea

JONES CRITERIA: JONES CRITERIA: Major ManifestationsMajor Manifestations

MIGRATORY POLYARTHRITIS MIGRATORY POLYARTHRITIS * most common: seen in 75% of * most common: seen in 75% of patients * typically involves large patients * typically involves large joints: knees, joints: knees, ankles, wrists, elbows ankles, wrists, elbows * joints are generally * joints are generally swollen, hot, red, swollen, hot, red, & & exquisitely exquisitely tender * not tender * not deforming * deforming * dramatic response to salicylatesdramatic response to salicylates

Jones Criteria: Jones Criteria: Major ManifestationsMajor Manifestations

CARDITISCARDITIS * occurs in 50% - 60% of cases * occurs in 50% - 60% of cases * usually presents as tachycardia, * usually presents as tachycardia, cardiac cardiac murmurs , + / - myo or murmurs , + / - myo or pericardial pericardial involvement involvement * cardiomegaly * cardiomegaly * signs of CHF * signs of CHF * echo findings of valvular * echo findings of valvular regurg does not regurg does not satisfy criteria if no satisfy criteria if no auscultatory auscultatory evidence evidence

Jones Criteria: Major Jones Criteria: Major ManifestationsManifestations

CHOREACHOREA (Sydenham chorea) (Sydenham chorea) * occurs in 10% - 15% of cases * occurs in 10% - 15% of cases * usually presents as an isolated, subtle, * usually presents as an isolated, subtle,

neurologic behavior disorder neurologic behavior disorder * uncontrollable movements, emotional * uncontrollable movements, emotional

lability, incoordination, facial grimacing lability, incoordination, facial grimacing disappears w/ sleep disappears w/ sleep * long latent period bet. infection & chorea * long latent period bet. infection & chorea * rarely leads to neurologic sequelae * rarely leads to neurologic sequelae

Major ManifestationsMajor Manifestations

ERYTHEMA MARGINATUMERYTHEMA MARGINATUM * rare, seen in less than 3% of cases * rare, seen in less than 3% of cases * characteristic rash is * characteristic rash is erythematous, erythematous, serpiginous, macular serpiginous, macular lesions w/ pale lesions w/ pale center, non-pruritic center, non-pruritic * usually seen in the * usually seen in the trunk, not the face * accentuated trunk, not the face * accentuated by warming the skin by warming the skin

Major ManifestationsMajor Manifestations

SUBCUTANEOUS NODULESSUBCUTANEOUS NODULES

* rare, seen in less than 1 % of cases * rare, seen in less than 1 % of cases * firm nodules, 1 cm in diameter, * firm nodules, 1 cm in diameter, along along extensor surfaces, near bony extensor surfaces, near bony prominences prominences

MINOR MANIFESTATIONSMINOR MANIFESTATIONS

ARTHRALGIAARTHRALGIA in the absence of arthritis in the absence of arthritis as a major criterionas a major criterion

FEVERFEVER

ELEVATED ACUTE PHASE REACTANTSELEVATED ACUTE PHASE REACTANTS (ESR, C-reactive protein) (ESR, C-reactive protein)

PROLONGED PRPROLONGED PR interval on ECG interval on ECG

SUPPORTING EVIDENCE OF SUPPORTING EVIDENCE OF GRP A STREP INFECTIONGRP A STREP INFECTION

an ABSOLUTE REQUIREMENT for the an ABSOLUTE REQUIREMENT for the diagnosis of RFdiagnosis of RF

Streptococcal antibody tests most reliableStreptococcal antibody tests most reliable

Elevated or increasing ASO antibody titersElevated or increasing ASO antibody titers

* Titers at least 333 TU in children; * Titers at least 333 TU in children; elevated in 80% of pxs elevated in 80% of pxs

*Others: antideoxyribonuclease B, *Others: antideoxyribonuclease B, antistreptokinase, antihyaluronidaseantistreptokinase, antihyaluronidase

Clinical CourseClinical Course

Only carditis can cause permanent cardiac Only carditis can cause permanent cardiac damage. Signs of mild carditis disappear damage. Signs of mild carditis disappear rapidly in wks; severe carditis longer, 2-6 rapidly in wks; severe carditis longer, 2-6 mos.mos.Arthritis subsides in a few days to several Arthritis subsides in a few days to several wks; no permanent damagewks; no permanent damageChorea gradually subsides in 6-7 mos or Chorea gradually subsides in 6-7 mos or longer; does not cause neurologic longer; does not cause neurologic sequelae sequelae

TreatmentTreatment

Approaches to treatment: Approaches to treatment: * 1. treat the grp A streptococcal infection * 1. treat the grp A streptococcal infection

single dose benzathine PCN;single dose benzathine PCN; 10 days of appropriate oral antibiotics 10 days of appropriate oral antibiotics 2. use of anti-inflammatory agents to 2. use of anti-inflammatory agents to

control clinical manifestations:control clinical manifestations: aspirin or steroids aspirin or steroids 3. other support therapy: including Tx for 3. other support therapy: including Tx for

CHF, chorea CHF, chorea

PreventionPrevention

Primary Prophylaxis – to prevent an initial attack Primary Prophylaxis – to prevent an initial attack of RF, a 10-day course of oral PCNof RF, a 10-day course of oral PCN

Secondary Prophylaxis – to prevent Secondary Prophylaxis – to prevent colonization / infection with grp A strep every 21 colonization / infection with grp A strep every 21 or 28 days in pxs who already have RHD or had or 28 days in pxs who already have RHD or had previous RF previous RF

given for 10 yrs or longer:given for 10 yrs or longer:

benzathine PCN, 1.2 MU, IM benzathine PCN, 1.2 MU, IM oral PCN V , 250mg BID; oral erythromycin oral PCN V , 250mg BID; oral erythromycin

250mg BID; sulpha 1 gm OD250mg BID; sulpha 1 gm OD