FrequencyFrequency

Leading cause of death and common cause of Leading cause of death and common cause of structural heart damage until 1960 structural heart damage until 1960

US has experienced a resurgence of RF in the US has experienced a resurgence of RF in the last two decades. Why? Possibly more last two decades. Why? Possibly more virulent strains of Group A streptococci.virulent strains of Group A streptococci.

ARF problem in high-risk areas of the tropics, ARF problem in high-risk areas of the tropics, countries with limited resources and where countries with limited resources and where there is minority indigenous populationsthere is minority indigenous populations

Cardiac involvement = major cause of long-Cardiac involvement = major cause of long-term morbidityterm morbidity

Most cases = 5-15 years oldMost cases = 5-15 years old

2 Patterns of 2 Patterns of PresentationPresentation

Sudden onset = typically begins as polyarthritis Sudden onset = typically begins as polyarthritis 2-6 weeks after streptococcal pharyngitis 2-6 weeks after streptococcal pharyngitis FeverFever ToxicityToxicity

Insidious (subclinical) = initial abnormality is Insidious (subclinical) = initial abnormality is mild carditismild carditis

Age at onset influences complication order: Age at onset influences complication order: younger children = carditis, older = arthritisyounger children = carditis, older = arthritis

Modified Jones CriteriaModified Jones Criteria

Diagnosis requires: Diagnosis requires: High index of clinical suspicionHigh index of clinical suspicion Evidence of previous strep infectionEvidence of previous strep infection 2 major Jones criteria 2 major Jones criteria

OROR

1 major + 2 minor Jones criteria1 major + 2 minor Jones criteria

Major criteriaMajor criteria

Carditis: cardiomegaly, new murmur, CHF, Carditis: cardiomegaly, new murmur, CHF, pericarditispericarditis

Migratory polyarthritis: fleeting, large jtsMigratory polyarthritis: fleeting, large jts Subcutaneous nodules: firm and painless Subcutaneous nodules: firm and painless

along extensor surfaces of the wrist, elbow and along extensor surfaces of the wrist, elbow and kneeknee

Erythema marginatum: serpiginous rashErythema marginatum: serpiginous rash Chorea (“St Vitus dance”): rapid, purposeless Chorea (“St Vitus dance”): rapid, purposeless

movements of the face and upper extremitiesmovements of the face and upper extremities

Minor criteriaMinor criteria

Clinical: arthralgia, fever and previous Clinical: arthralgia, fever and previous history of ARFhistory of ARF

Laboratory: ^ESR/CRP, prolonged PR Laboratory: ^ESR/CRP, prolonged PR interval, supporting evidence of interval, supporting evidence of antecedent group A strep infection (+ antecedent group A strep infection (+ throat culture, + rapid strep screen, throat culture, + rapid strep screen, ^strep antibody titer)^strep antibody titer)

Lab StudiesLab Studies

None to specifically diagnoseNone to specifically diagnose Strep antibody tests discloses preceding Strep antibody tests discloses preceding

strep infectionstrep infection Isolate group A strep in throat cultureIsolate group A strep in throat culture Acute phase reactions: ESR, CRP, ^ Acute phase reactions: ESR, CRP, ^

seum complement, mucoproteins, alpha-seum complement, mucoproteins, alpha-2, and gamma globulins2, and gamma globulins

Prolonged PR intervalProlonged PR interval

NewerNewer

Rapid detection test for D8/17: This Rapid detection test for D8/17: This immunofluorescence technique for immunofluorescence technique for identifying the B cell marker D8/17 is identifying the B cell marker D8/17 is positive in 90% of patients with rheumatic positive in 90% of patients with rheumatic fever. It may be useful for identifying fever. It may be useful for identifying patients who are at risk for developing patients who are at risk for developing rheumatic fever. rheumatic fever.

Radiographic StudiesRadiographic Studies

Echocardiography: helpful in Echocardiography: helpful in establishing carditisestablishing carditis

Synovial fluid: may demonstrate ^ WBCsSynovial fluid: may demonstrate ^ WBCs

PreventionPrevention

Treat all GABHS pharyngitis with Treat all GABHS pharyngitis with antibioticsantibiotics

Confirm infection with:Confirm infection with: Rapid strep followed by culture in children Rapid strep followed by culture in children

and adolescentsand adolescents Rapid strep in adultsRapid strep in adults

TeatmentTeatment

Steroids, ASA and Naproxen for pain and Steroids, ASA and Naproxen for pain and inflammationinflammation

Digitalis for heart failureDigitalis for heart failure Haloperidol for choreaHaloperidol for chorea Consultations to cardiologist, rheumatologist Consultations to cardiologist, rheumatologist

and neurologistand neurologist Prophylaxis for ARF pts x 5 yrs to prevent Prophylaxis for ARF pts x 5 yrs to prevent

future infections (benzathine penicillin G 1.2 future infections (benzathine penicillin G 1.2 million U IM q month, pen V 250mg PO bid, million U IM q month, pen V 250mg PO bid, erythromycin 250 mg PO bid)erythromycin 250 mg PO bid)

Course & ComplicationsCourse & Complications

CarditisCarditis Mitral stenosisMitral stenosis CHFCHF Approximately 75% cases, acute attack Approximately 75% cases, acute attack

lasts 6 weekslasts 6 weeks 90% cases resolve in < 12 weeks90% cases resolve in < 12 weeks

Rheumatic Heart DiseaseRheumatic Heart Disease

Chronic rheumatic Chronic rheumatic heart disease is the heart disease is the leading cause of leading cause of mitral valve stenosis mitral valve stenosis and valve and valve replacement in the replacement in the U.S.U.S.

Produces a Produces a pancarditispancarditis

Cardiac InvolvementCardiac Involvement

Characterized by endocarditis, Characterized by endocarditis, pericarditis and myocarditispericarditis and myocarditis

Endocarditis manifested as valve Endocarditis manifested as valve insufficiency: mitral 60-75% of the timeinsufficiency: mitral 60-75% of the time

Aortic valve 25% of the timeAortic valve 25% of the time Tricuspid 10% of the timeTricuspid 10% of the time 9-39% adults with previous RF have 9-39% adults with previous RF have

residual progressive valve diseaseresidual progressive valve disease

Cardiac MorbidityCardiac Morbidity

Fusion of valve apparatus occurs 2-10 Fusion of valve apparatus occurs 2-10 years status post infectionyears status post infection

RHD responsible for 99% of mitral valve RHD responsible for 99% of mitral valve adult disease in the U.S.adult disease in the U.S.

May have associated atrial fibrillation and May have associated atrial fibrillation and or left atrial thrombus formationor left atrial thrombus formation

Prognosis worse for femalesPrognosis worse for females

Cardiac ManifestationsCardiac Manifestations

Pancarditis = most serious & second most Pancarditis = most serious & second most common complication (50%). Advanced cases common complication (50%). Advanced cases c/o dyspnea, mild-to-mod chest discomfort, c/o dyspnea, mild-to-mod chest discomfort, pleuritic chest pain, edema, cough, orthopneapleuritic chest pain, edema, cough, orthopnea PE = carditis detected by new murmur & PE = carditis detected by new murmur &

tachycardia out of proportion to fever. New/ tachycardia out of proportion to fever. New/ changing murmurs needed to dx R valvulitis. changing murmurs needed to dx R valvulitis.

CHF and pericarditis. CHF and pericarditis. Patients with acute RF should be examined Patients with acute RF should be examined

frequentlyfrequently

Valvular Insuff MM of RFValvular Insuff MM of RF

Apical pansystolic mm: high-pitched, blowing-Apical pansystolic mm: high-pitched, blowing-quality of mitral regurg radiating to left axilla. quality of mitral regurg radiating to left axilla. Unaffected by respiration or position. >Grade 2/6 Unaffected by respiration or position. >Grade 2/6 . .

Apical diastolic mm ( Carey-Coombs ) heard with Apical diastolic mm ( Carey-Coombs ) heard with active carditis and accompanies severe mitral active carditis and accompanies severe mitral insufficiency. Bell of stethoscope, with patient in left insufficiency. Bell of stethoscope, with patient in left lateral position and breath held in expiration. Low lateral position and breath held in expiration. Low pitched, rumbling, & resembles distant drum.pitched, rumbling, & resembles distant drum. Basal diastolic mm early mm of aortic regurg: Basal diastolic mm early mm of aortic regurg:

high-pitched, blowing, decrescendo, heard best high-pitched, blowing, decrescendo, heard best along right upper sternal border after deep along right upper sternal border after deep expiration with pt leaning forward.expiration with pt leaning forward.

Complications of RF MMComplications of RF MM

Congestive heart failure Congestive heart failure Heart failure may develop secondary to Heart failure may develop secondary to

severe valve insufficiency or myocarditis. severe valve insufficiency or myocarditis. The physical findings associated with The physical findings associated with

heart failure include tachypnea, heart failure include tachypnea, orthopnea, jugular venous distention, orthopnea, jugular venous distention, rales, hepatomegaly, a gallop rhythm, rales, hepatomegaly, a gallop rhythm, and peripheral swelling and edema.and peripheral swelling and edema.

More ComplicationsMore Complications

PericarditisPericarditis A pericardial friction rub A pericardial friction rub Increased cardiac dullness to percussion and Increased cardiac dullness to percussion and

muffled heart sounds consistent with pericardial muffled heart sounds consistent with pericardial effusion.effusion.

A paradoxical pulse (drop in systolic blood pressure A paradoxical pulse (drop in systolic blood pressure with inspiration) with decreased systemic pressure with inspiration) with decreased systemic pressure and perfusion and evidence of diastolic indentation and perfusion and evidence of diastolic indentation of the right ventricle on echocardiogram reflect of the right ventricle on echocardiogram reflect impending pericardial tamponade. In this clinical impending pericardial tamponade. In this clinical emergency, pericardial effusion should be treated by emergency, pericardial effusion should be treated by pericardiocentesis.pericardiocentesis.

Valve DeformitiesValve Deformities

Mitral stenosis in 25% of patients with CRHD and in association with mitral Mitral stenosis in 25% of patients with CRHD and in association with mitral insufficiency in another 40%. Progressive fibrosis results in enlargement of left insufficiency in another 40%. Progressive fibrosis results in enlargement of left atrium and formation of mural thrombi. The stenotic valve is funnel-shaped, with a atrium and formation of mural thrombi. The stenotic valve is funnel-shaped, with a "fish mouth" resemblance. On auscultation, S1 is initially accentuated but becomes "fish mouth" resemblance. On auscultation, S1 is initially accentuated but becomes reduced as the leaflets thicken. P2 becomes accentuated, and the splitting of S2 reduced as the leaflets thicken. P2 becomes accentuated, and the splitting of S2 decreases as pulmonary hypertension develops. An opening snap of the mitral decreases as pulmonary hypertension develops. An opening snap of the mitral valve often is heard at the apex, where a diastolic filling murmur also is heard. valve often is heard at the apex, where a diastolic filling murmur also is heard.

Aortic stenosis from chronic rheumatic heart disease typically is associated with Aortic stenosis from chronic rheumatic heart disease typically is associated with aortic insufficiency. The valve commissures and cusps become adherent and aortic insufficiency. The valve commissures and cusps become adherent and fused, and the valve orifice becomes small with a round or triangular shape. On fused, and the valve orifice becomes small with a round or triangular shape. On auscultation, S2 may be single because the aortic leaflets are immobile and do not auscultation, S2 may be single because the aortic leaflets are immobile and do not produce an aortic closure sound. The systolic and diastolic murmurs of aortic valve produce an aortic closure sound. The systolic and diastolic murmurs of aortic valve stenosis and insufficiency are heard best at the base of the heart. stenosis and insufficiency are heard best at the base of the heart.

Thromboembolism occurs as complication of mitral stenosis. It is more likely to Thromboembolism occurs as complication of mitral stenosis. It is more likely to occur when the left atrium is dilated, cardiac output is decreased, and the patient is occur when the left atrium is dilated, cardiac output is decreased, and the patient is in atrial fibrillation. The frequency of this complication is decreased with use of in atrial fibrillation. The frequency of this complication is decreased with use of anticoagulation and surgical repair of valve abnormalities.anticoagulation and surgical repair of valve abnormalities.

Other ComplictionsOther Complictions

Cardiac hemolytic anemia related to disruption of Cardiac hemolytic anemia related to disruption of RBCs by deformed valve. Increased destruction and RBCs by deformed valve. Increased destruction and replacement of platelets also may occur.replacement of platelets also may occur.

Atrial arrhythmias typically are related to a Atrial arrhythmias typically are related to a chronically enlarged left atrium (from mitral valve chronically enlarged left atrium (from mitral valve abnl). Successful cardioversion of A fib to sinus abnl). Successful cardioversion of A fib to sinus rhythm more likely successful if left atrium is not rhythm more likely successful if left atrium is not markedly enlarged, mitral stenosis is mild, & the pt markedly enlarged, mitral stenosis is mild, & the pt has been in A fib < 6 months. Pts should be has been in A fib < 6 months. Pts should be anticoagulated before cardioversion to decrease risk anticoagulated before cardioversion to decrease risk of systemic embolization.of systemic embolization.

Doppler-Doppler-echocardiographyechocardiography

In ARHD this identifies & quantitates valve insufficiency & ventricular In ARHD this identifies & quantitates valve insufficiency & ventricular dysfunction.dysfunction. With mild carditis, Doppler evidence of mitral regurgmay be present With mild carditis, Doppler evidence of mitral regurgmay be present

during acute phase of disease but resolves in weeks to months. In during acute phase of disease but resolves in weeks to months. In contrast, patients with moderate-to-severe carditis have persistent contrast, patients with moderate-to-severe carditis have persistent mitral and/or aortic regurgitation.mitral and/or aortic regurgitation.

The most important features of mitral regurg from acute rheumatic The most important features of mitral regurg from acute rheumatic valvulitis are annular dilatation, elongation of the chordae to the valvulitis are annular dilatation, elongation of the chordae to the anterior leaflet, and a posterolaterally directed mitral regurgitation jet.anterior leaflet, and a posterolaterally directed mitral regurgitation jet.

In chronic rheumatic heart disease, echocardiography may be used to In chronic rheumatic heart disease, echocardiography may be used to track the progression of valve stenosis and may help determine the track the progression of valve stenosis and may help determine the time for surgical intervention. The leaflets of affected valves become time for surgical intervention. The leaflets of affected valves become diffusely thickened, with fusion of the commissures and chordae diffusely thickened, with fusion of the commissures and chordae tendineae. Increased echodensity of the mitral valve may signify tendineae. Increased echodensity of the mitral valve may signify calcification.calcification.

Cardiac CatheterizationCardiac Catheterization

In ARHD this procedure is not indicated. In ARHD this procedure is not indicated. With chronic disease, heart With chronic disease, heart catheterization has been performed to catheterization has been performed to evaluate mitral and aortic valve disease evaluate mitral and aortic valve disease and to balloon stenotic mitral valves.and to balloon stenotic mitral valves.

ElectrocardiogramElectrocardiogram

Sinus tachycardia most frequently accompanies ARHD Sinus tachycardia most frequently accompanies ARHD Second-degree (intermittent) and third-degree (complete) AV Second-degree (intermittent) and third-degree (complete) AV

block with progression to ventricular standstill have been block with progression to ventricular standstill have been described. Heart block in the setting of rheumatic fever, described. Heart block in the setting of rheumatic fever, however, typically resolves with the rest of the disease however, typically resolves with the rest of the disease process.process.

When acute rheumatic fever is associated with pericarditis, ST When acute rheumatic fever is associated with pericarditis, ST segment elevation may be present and is marked most in lead segment elevation may be present and is marked most in lead II, III, aVF, and V4-V6.II, III, aVF, and V4-V6.

Patients with rheumatic heart disease also may develop atrial Patients with rheumatic heart disease also may develop atrial flutter, multifocal atrial tachycardia, or atrial fibrillation from flutter, multifocal atrial tachycardia, or atrial fibrillation from chronic mitral valve disease and atrial dilation.chronic mitral valve disease and atrial dilation.

TreatmentTreatment

Attempts are made to obtain aspirin blood Attempts are made to obtain aspirin blood levels at 20-25 mg/dL, but, due to variable GI levels at 20-25 mg/dL, but, due to variable GI absorption of the drug, stable levels may be absorption of the drug, stable levels may be difficult to achieve during the inflammatory difficult to achieve during the inflammatory phase. Aspirin is maintained at anti-phase. Aspirin is maintained at anti-inflammatory doses until the signs and inflammatory doses until the signs and symptoms of acute rheumatic fever are symptoms of acute rheumatic fever are resolved or subsiding (6-8 wk) and the acute resolved or subsiding (6-8 wk) and the acute phase reactants have returned to normal phase reactants have returned to normal levels. levels.

Treatment Con’tTreatment Con’t

moderate-to-severe carditis indicated by cardiomegaly, moderate-to-severe carditis indicated by cardiomegaly, CHF, or third-degree heart block, oral prednisone CHF, or third-degree heart block, oral prednisone should be added to salicylate therapy. Prednisone should be added to salicylate therapy. Prednisone should be continued for 2-6 weeks, tapered during the should be continued for 2-6 weeks, tapered during the last week of therapy. Adverse effects can be minimized last week of therapy. Adverse effects can be minimized by discontinuing prednisone therapy after 2-4 weeks by discontinuing prednisone therapy after 2-4 weeks and maintaining salicylates for an additional 2-4 weeks. and maintaining salicylates for an additional 2-4 weeks. Additional treatment for patients with acute rheumatic Additional treatment for patients with acute rheumatic fever and congestive heart failure should include fever and congestive heart failure should include digoxin, diuretics, supplemental oxygen, bed rest, and digoxin, diuretics, supplemental oxygen, bed rest, and sodium and fluid restriction sodium and fluid restriction

DigoxinDigoxin

Digoxin initiated only after checking electrolyte values Digoxin initiated only after checking electrolyte values and correcting abnormal findings in serum K. The total and correcting abnormal findings in serum K. The total loading dose is 20-30 mcg/kg orally with 50% of the loading dose is 20-30 mcg/kg orally with 50% of the dose given initially, followed by 25% of the dose 8 and dose given initially, followed by 25% of the dose 8 and 16 hours after the initial dose. Maintenance doses 16 hours after the initial dose. Maintenance doses typically are 8-10 mcg/kg/d orally in 2 divided doses. typically are 8-10 mcg/kg/d orally in 2 divided doses. For older children and adults, the total loading dose is For older children and adults, the total loading dose is 1.25-1.5 mg orally, and the maintenance dose is 0.25-1.25-1.5 mg orally, and the maintenance dose is 0.25-0.5 mg/d orally. Therapeutic digoxin levels are present 0.5 mg/d orally. Therapeutic digoxin levels are present at trough levels of 1.5-2 ng/mL. The diuretics most at trough levels of 1.5-2 ng/mL. The diuretics most commonly used in conjunction with digoxin for children commonly used in conjunction with digoxin for children with congestive heart failure include furosemide and with congestive heart failure include furosemide and spironolactone, both at doses of 1-2 mg/kg/dose twice spironolactone, both at doses of 1-2 mg/kg/dose twice per day per day

ProphylaxisProphylaxis

Pts with RF & carditis but no valve disease should Pts with RF & carditis but no valve disease should receive prophylactic antibiotics for 10 years or well into receive prophylactic antibiotics for 10 years or well into adulthood, whichever is longer. adulthood, whichever is longer.

Pts with RF & carditis & valve disease should receive Pts with RF & carditis & valve disease should receive antibiotics at least 10 years or until aged 40 years. antibiotics at least 10 years or until aged 40 years. Patients with RHD require antibiotic prophylaxis before Patients with RHD require antibiotic prophylaxis before certain surgical and dental procedures to prevent certain surgical and dental procedures to prevent bacterial endocarditis. Patients who have had RF bacterial endocarditis. Patients who have had RF without valve disease do not need prophylaxis. Pts with without valve disease do not need prophylaxis. Pts with endocarditis resistant to penicillin should receive endocarditis resistant to penicillin should receive erythromycin. erythromycin.

ConfusionConfusion

Preventive and prophylactic therapy is Preventive and prophylactic therapy is indicated after rheumatic fever and rheumatic indicated after rheumatic fever and rheumatic heart disease to prevent further damage to heart disease to prevent further damage to valves. The initial course of antibiotics given to valves. The initial course of antibiotics given to eradicate the streptococcal infection also eradicate the streptococcal infection also serves as the first course of prophylaxis. An serves as the first course of prophylaxis. An injection of 0.6-1.2 million units of benzathine injection of 0.6-1.2 million units of benzathine penicillin G intramuscularly every 4 weeks is penicillin G intramuscularly every 4 weeks is the recommended regimen for secondary the recommended regimen for secondary prevention for most patients in the United prevention for most patients in the United States. States.