RHEUMATIC FEVER/RHEUMATIC HEART DISEASE

PROF S S Danbauchi

OBJECTIVES

• For the student to learn about,

ⱶ Definition

ⱶ Clinical presentation

ⱶ Diagnosis

ⱶTreatment of rheumatic fever

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• For the student learn about complication of rheumatic fever, called rheumatic heart disease

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• The clinical presentation, diagnosis, investigations and management of Rheumatic heart disease (RHD)

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DEFINITION OF RHEUMATIC FEVER

• Acute rheumatic fever (ARF) is an autoimmune inflammatory process that develops as a sequela of streptococcal infection.
• ARF has extremely variable manifestations, and remains a clinical syndrome for which no specific diagnostic test exists.
• Persons who have experienced an episode of ARF are predisposed to recurrence following subsequent group A streptococcal infections.
• The most significant complication of ARF is rheumatic heart disease, which usually occurs after one or repeated bouts of acute illness.

INTRODUCTION - RHEUMATIC FEVER (RF)

• Common in the developing world 100/100,000 population

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• Uncommon in US approximately 2/100,000
• Peak incidence ages 5-15

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• Uncommon in the age below 3- 4 years

and above 40 years

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Diagnosis using Duckett Jones criteria and confirmation of Streptococcal infection

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RHEUMATIC HEART DISEASE

INTRODUCTION

• Acute rheumatic fever (ARF) is a sequela of streptococcal infection—typically following 2 to 3 weeks after group A streptococcal pharyngitis—that occurs most commonly in children and has rheumatologic, cardiac, and neurologic manifestations. 
•  The incidence of ARF has declined in most developed countries, and many physicians have little or no practical experience with the diagnosis and management of this condition.
• Occasional outbreaks in the United States make complacency a threat to public health.

INTRODUCTION

• Most common valves involved is mitral and aortic and others

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• Disease of poor hygienic environment

EPIDEMIOLOGY

GENERAL CONSIDERATION�

• Rheumatic fever is a systemic immune process disorder
• Sequela of beta hemolytic Streptococcal infection of the pharynx, rarely the skin
• Signs of rheumatic fever commences 2-3 weeks after throat infection but may appear 1 week or 5 weeks
• Peak incidence 5-15 years
• Rheumatic carditis and valvatids might be self limiting

PATHOPHYSIOLOGY

PATHOPHYSIOLOGY

PATHOPHYSIOLOGY

PATHOLOGY

PATHOLOGY�

• Pan carditis- endocardium, myocardium and pericardium

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• Slowly progressive valvatids with deformity

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• Perivascular granulomatous reaction with vasculitis

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• Mitral valve attacked in 70-90 %

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• Aortic in combination with mitral valve is below 30 %

PATHOLOGY

• Aortic valve alone is 3 - 5%

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• Tricuspid and pulmonary are rarely affected

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EVOLUTION OF RF

MAJOR CRITERIA

• Carditis- pericarditis, cardiomegaly, CHF, mitral or aortic regurgitations, ECG changes of change P wave contour, T wave inversion, changing quality of heart sounds, sinus tachycardia, arrhythmias
• Erythema marginatum and subcut nodules
• Sydenham's chorea
• Polyarthritis (migratory or fleeting)
• Polyarthralgia*

CLINICAL MANIFESTATIONS OF RF

ERYTHEMA MARGINATUM, THE CHARACTERISTIC RASH OF ACUTE RHEUMATIC FEVER

MINOR CRITERIA

• Fever
• Polyarthralgia
• Prolonged PR interval and PR interval ratio to > 1.0
• Raised ESR, CRP
• Positive throat culture for Streptococcus
• Rapid ASO titer rise

DIAGNOSTIC CRITERIA

MODIFIED CRITERIA RF 2015

REVISED CRITERIA FOR RF -2018

CRITERIA FOR RF DIAGNOSIS -2018

LABORATORY FINDINGS

• High titer of ASO

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• Anti DNase

MNEMONIC IN RF DIAGNOSIS

DIFFERENTIAL DIAGNOSIS

• Malaria
• Osteomyelitis
• Rheumatoid arthritis
• Endocarditis
• Chronic meningococcemia
• Systemic Lupus Erythematosus
• Sickle cell disease
• Surgical abdomen

COMPLICATIONS

• CHF/RHD

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• Arrhythmias

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• Pericarditis with pericardial effusion

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• Rheumatic pneumonitis

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• Nephritis

TREATMENT

• General- Strict bed rest, monitor pulse rate and ESR

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• Medical measures- Salicylates (aspirin), Penicillin and Corticosteroids

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MANAGEMENT ALGORITHM

MANAGEMENT OF RF

MANAGEMENT OF RF

PREVENTION OF RF

PREVENTION

• Early treatment of pharynx streptococcal infection

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• Screening for carriers of Streptococcus and treating it

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• Good hygiene (individual and environmental)

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• Prevention of recurrence - prophylaxis

RHEUMATIC HEART DISEASE

• Progress of disease from fever to RHD

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• Valves and myocardium are involved

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OVERVIEW OF VALVULAR HEART DIS

• Aortic Stenosis

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• Mitral Stenosis

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• Aortic Regurgitation
• Acute and Chronic

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• Mitral Regurgitation
• Acute and Chronic

AORTIC STENOSIS

AORTIC STENOSIS OVERVIEW:

• Normal Aortic Valve Area: 3-4 cm²

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• Symptoms: Occur when valve area is 1/4^th of normal area.

Types:

• Valvular (RHD)

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• Degenerative

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• Supravalvular (congenital- Bicuspid)

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• Subvalvular (HCM)

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ETIOLOGY OF AORTIC STENOSIS

• Rheumatic (following fever)

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Differential causes

• Congenital
• Degenerative/Calcific
• HCM - Dynamic

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Patients under 70: >50% have a acquired and congenital cause

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Patients over 70: 50% due to degenerative

PATHOPHYSIOLOGY OF AORTIC STENOSIS

• A pressure gradient develops between the left ventricle and the aorta. (increased afterload)

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• LV function initially maintained by compensatory pressure hypertrophy

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• When compensatory mechanisms exhausted, LV function declines, LV dilatation sets in.

PRESENTATION OF AORTIC STENOSIS

• ▹Syncope: (exertional)

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• ▹Angina: (increased myocardial oxygen demand; demand/supply mismatch)

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• ▹Dyspnea: on exertion due to heart failure (diastolic and systolic)

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• ▹Sudden death

PHYSICAL FINDINGS IN AORTIC STENOSIS

• Slow rising carotid pulse (pulsus tardus) & decreased pulse amplitude (pulsus parvus)

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• Heart sounds- soft and split second heart sound, S4 gallop due to LVH.

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• Systolic ejection murmur- crescendo-decrescendo character. This peaks later as the severity of the stenosis increases.
• Loudness does NOT tell you anything about severity

EVALUATION OF AS

Cardiac catheterization: Should only be done for a direct measurement if symptom severity and echo severity don’t match OR prior to replacement when replacement is planned.

MANAGEMENT OF RHD AS

• General- IE prophylaxis in dental procedures with a prosthetic AV or history of endocarditis.

• Medical - limited role since AS is a mechanical problem. Vasodilators are relatively contraindicated in severe AS

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• Aortic Balloon Valvotomy- shows little benefit.

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• Surgical Replacement: Definitive treatment

SIMPLIFIED INDICATIONS FOR SURGERY IN AORTIC STENOSIS

• Any SYMPTOMATIC patient with severe AS (includes symptoms with exercise)

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• Any patient with decreasing EF

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• Any patient undergoing CABG with moderate or severe AS

ECHO SURVEILLANCE OR FOLLOW UP

• Mild: Every 5 years

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• Moderate: Every 2 years

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• Severe: Every 6 months to 1 year

MITRAL STENOSIS OVERVIEW

• Definition: Obstruction of LV inflow that prevents proper filling during diastole

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• Normal MV Area: 4-6 cm²

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• Trans mitral gradients increases and symptoms begin at areas less than 2 cm²

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• Rheumatic carditis is the predominant cause

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• Prevalence and incidence: decreasing due to a reduction of rheumatic heart disease.

ETIOLOGY OF RHEUMATIC MITRAL STENOSIS

• Rheumatic heart disease: 77-99% of all cases

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Differential causes

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• Infective endocarditis: 3.3%

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• Mitral annular calcification (age related): 2.7%

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MITRAL STENOSIS

MS PATHOPHYSIOLOGY

• Progressive Dyspnea (70%): LA dilation 🡪 pulmonary congestion (reduced emptying)
• worse with exercise, fever, tachycardia, and pregnancy
• Increased Transmittal Pressures: Leads to left atrial enlargement and atrial fibrillation.
• Right heart failure symptoms: due to Pulmonary venous HTN
• Hemoptysis: due to rupture of bronchial vessels due to elevated pulmonary pressure

NATURAL HISTORY OF MS

• Disease of plateaus:
• Mild MS: 20 or more years after initial RHD insult
• Moderate: 10 years later
• Severe: < 10 years

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• Mortality: Due to progressive pulmonary congestion, infection, and thromboembolism.

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• In Africans the manifestation is much early averagely 5 – 10 years

PHYSICAL EXAM FINDINGS OF MS

• prominent "a" wave in jugular venous pulsations: Due to pulmonary hypertension and right ventricular hypertrophy

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• Signs of right-sided heart failure: in advanced disease

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• Mitral facies: When MS is severe and the cardiac output is diminished, there is vasoconstriction, resulting in pinkish-purple patches on the cheeks

HEART SOUNDS IN MS

• Diastolic murmur:
• Low-pitched diastolic rumble most prominent at the apex (early, mid or late diastole).

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• Heard best with the patient lying on the left side in held expiration

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• Intensity of the diastolic murmur does not correlate with the severity of the stenosis

HEART SOUNDS IN MS

• Loud Opening S₁ snap: heard at the apex when leaflets are still mobile 

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• Due to the abrupt halt in leaflet motion in early diastole, after rapid initial rapid opening, due to fusion at the leaflet tips.

• A shorter duration S2 to opening snap interval indicates more severe disease.

MANAGEMENT OF RHD MS

Serial echocardiography:

• Mild: 3-5 years
• Moderate:1-2 years
• Severe: yearly

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• Medications: MS like AS is a mechanical problem and medical therapy does not prevent progression

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• β-blockers, CCBs, Digoxin which control heart rate and hence prolong diastole for improved diastolic filling

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• Diuretics for fluid overload

MANAGEMENT OF RHD MS

• Identify patient early who might benefit from percutaneous mitral balloon valvotomy.

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• IE prophylaxis: Patients with prosthetic valves or a History for dental procedures.

AORTIC REGURGITATION

AORTIC REGURGITATION OVERVIEW

• Definition: Leakage of blood into LV during diastole due to ineffective coaptation of the aortic cusps

ETIOLOGY OF ACUTE AR

• Rheumatic fever

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Differential causes

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• Endocarditis
• Aortic Dissection

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• Physical Findings:
• Bounding and or collapsing pulse
• Wide pulse pressure
• Diastolic murmur (decrescendo)
• Florid pulmonary edema

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TREATMENT OF ACUTE AR

• True Surgical Emergency:

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• Positive inotrope: (e.g., dopamine, dobutamine)

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• Vasodilators: (e.g., nitroprusside)

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• Avoid beta-blockers

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ETIOLOGY OF CHRONIC AR

• Rheumatic heart disease

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Other causes AR

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• Bicuspid aortic valve
• Infective endocarditis

PATHOPHYSIOLOGY OF AR

• Combined pressure AND volume overload

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• Compensatory Mechanisms: LV dilation, LV Hypertrophy. Progressive dilation leads to heart failure

NATURAL HISTORY OF AR

• Asymptomatic until 4^th or 5^th decade
• Rate of Progression: 4-6% per year
• Progressive Symptoms include:

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- Dyspnea: exertional, orthopnea, and paroxysmal nocturnal dyspnea

• Nocturnal angina: due to slowing of heart rate and reduction of diastolic blood pressure
• Palpitations: due to increased force of contraction

PHYSICAL EXAM FINDINGS OF AR

• Wide pulse pressure: most sensitive

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• Hyperdynamic and displaced apical impulse

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• Auscultation-
• Diastolic blowing murmur at the left sternal border

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• Austin flint murmur (apex): Regurgitant jet impinges on anterior MVL causing it to vibrate

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• Systolic ejection murmur: due to increased flow across the aortic valve

THE EVALUATION OF AR

• CXR: enlarged cardiac silhouette and aortic root enlargement

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• ECHO: Evaluation of the AV and aortic root with measurements of LV dimensions and function (cornerstone for decision making and follow up evaluation)

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• Aortography: Used to confirm the severity of disease

MANAGEMENT OF AR

• General: IE prophylaxis in dental procedures with a prosthetic AV or history of endocarditis.

• Medical: Vasodilators (ACEI’s), Nifedipine improve stroke volume and reduce regurgitation only if pt symptomatic or HTN.

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• Serial Echocardiograms: to monitor progression.

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• Surgical Treatment: Definitive Treatment

MITRAL REGURGITATION

CHRONIC MITRAL REGURGITATION OVERVIEW

• Definition: Backflow of blood from the LV to the LA during systole

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• Mild (physiological) MR is seen in 80% of normal individuals.

ACUTE MR

• Rheumatic fever

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Other causes

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• Endocarditis

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• Acute MI (Myocardial infarction)

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• Malfunction or disruption of prosthetic valve

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• Complication of PTMC- percutaneous transluminal mitral valvuloplasty or commissurotomy

MANAGEMENT OF ACUTE MR

• Myocardial infarction: Cardiac Cath - PCI or thrombolytics

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• Most other cases of mitral regurgitation is afterload reduction:

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• Diuretics and nitrates
• nitroprusside, even in the setting of a normal blood pressure.

MANAGEMENT OF ACUTE MR

• Do not attempt to alleviate tachycardia with beta-blockers.
• Mild-to-moderate tachycardia is beneficial in these patients because it allows less time for the heart to have backfill, which lowers regurgitant volume.

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TREATMENT OF ACUTE MR

• Balloon Pump

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• Nitroprusside even if hypotensive

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• Emergent Surgery

ETIOLOGIES OF CHRONIC MITRAL REGURGITATION

• Rheumatic heart disease

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Other causes of Chronic MR

• Myxomatous degeneration (MVP)

• Ischemic MR

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• Infective Endocarditis

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• HHF/Dilated cardiomyopathy

PHYSICAL EXAM FINDINGS IN MR

• Auscultation: soft S1 and a holosystolic murmur at the apex radiating to the axilla
• S3 (CHF/LA overload)

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• In chronic MR, the intensity of the murmur does correlate with the severity.

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• Exertion Dyspnea: ( exercise intolerance)

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• Heart Failure: May coincide with increased hemodynamic burden e.g., pregnancy, infection or atrial fibrillation

THE NATURAL HISTORY OF MR

• Compensatory phase: 10-15 years

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• Patients with asymptomatic severe MR have a 5%/year mortality rate

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• Once the patient’s EF becomes <60% and/or becomes symptomatic, mortality rises sharply

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• Mortality: From progressive dyspnea and heart failure

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MANAGEMENT OF MR

• Medications
1. Vasodilator such as hydralazine

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• Rate control for atrial fibrillation with β-blockers, CCB, digoxin

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• Anticoagulation in atrial fibrillation and flutter

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• Diuretics for fluid overload

MANAGEMENT OF MR

• Serial Echocardiography:
• Mild: 2-3 years
• Moderate: 1-2 years
• Severe: 6-12 months

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• IE prophylaxis: Patients with prosthetic valves or a Hx of IE for dental procedures.

TREATMENT OF RHD

Final common pathway for rheumatic valvular heart disease is Cardiac failure

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Nonpharmacological management including life style modification

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Drugs ; generally cardiac failure drugs (diuretics, digoxin, vasodilators, anticoagulants, antiplatelets

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Surgical; repair or replacement of valves (mechanical problems)

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Prevention of Endocarditis; in the setting of surgical manipulations

Prevention of rheumatic fever recurrence

SUMMARY

• Rheumatic fever is systemic immune response to Beta hemolytic Strep infection
• The complication includes rheumatic Valvular heart disease
• RHD can present acutely or chronic
• Mitral and Aortic valves are the commonly involved
• Treatment is either medical or surgical or both

END

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