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Rheumatic fever
Rheumatic fever (RF) is generally classified as a connective tissue or collagen-vascular disease
It is an inflammatory reaction that causes damage to collagen fibrils and to the ground substance of connective tissue Involves multiple organs: primarily the heart, the joints, and the central nervous system
Recurrent attacks of RF may cause fibrosis of heart valves, leading to chronic valvular heart disease
Epidemiology
Peak incidence ages 5~15 years Rare before age 4 years and after age 40 years
The incidence of RF and prevalence of rheumatic heart disease (RHD) are markedly variable in different countries:
In developed country, such as the united states, the incidence of RF 2/100,000 In many developing countries, the incidence of acute RF approaches or exceeds 100/100,000
Pathology
Pathological characters:
Exudative and proliferative inflammatory reactions involving connective or collagen tissue Affects primarily the heart, joints, brain, cutaneous and subcutaneous tissues
Pathological process
Exudative stage Proliferative stage: Aschoff nodule (pathognomonic) Fibrosis and calcification (scar formation)
Recurrent attacks of RF (rheumatic carditis, valvulitis) scar formation and deformity of heart valves chronic RHD
Valvular involvement: Mitral valve: 75%~80% Aortic valve: 30% Tricuspid & pulmonary valves: 5%
Clinical findings
1Major manifestations
Carditis: pericarditis, cardiomegaly, congestive heart
failure, and mitral or aortic regurgitation murmurs
Erythema marginatum: rare Subcutaneous nodules: uncommon Chorea: least common, most diagnostic
Diagnosis
Based on Jones criteria and confirmation of streptococcal infection
Guidelines for the diagnosis of initial attacks of RF (Jones criteria, updated 1992)
If supported by evidence of preceding group A streptococcal infection, the presence of two major manifestations or of one major and two minor manifestations establishes the diagnosis of acute RF
Treatment
General Measures Strict bed rest Medical Measures 1. Control streptococcal infection Penicillin is of choice
benzathine penicillin, 1.2 million units im once, or procaine penicillin, 600,000 units im daily, 10 days
2. Antirheumatic therapy
(1) Salicylates
Of choice in patients with little or no cardiac involvement; Particularly effective in reducing fever and relieving joint pain and swelling Aspirin 0.6~0.9 g / 4h in adults; lower doses in children
(2) Corticosteroids
Used in patients who do not respond well to adequate doses of salicylates Prednisone 40~60 mg orally daily, tapering over 2 weeks
Prevention
Primary prevention Early treatment of streptococcal pharyngitis
Penicillin or erythromycin
Very rarely, MS is a complication of carcinoid disease or connective tissue disease (systemic lupus
erythematosus, SLE; rheumatoid arthritis)
Pathology
Fibrosis, thickening, rigid and calcification of the valve apparatus
Rheumatic fever results in four forms of fusion of the mitral valve apparatus leading to stenosis: Commissural, cuspal, chordal, and combined Characteristically, mitral valve cusps fuse at their edges, and fusion of the chordae results in thickening and shortening of these structures
Commissural adherent and fusion restricted opening of mitral valve fish mouth shape of mitral valve orifice
Thickening, fusion and shortening of the chordae or papillary muscles funnel-shaped change of valve apparatus
Secondary changes
Chronic MS Dilatation of the left atrium Fibrosis of the atrial wall Development of mural thrombi Hypertrophy and dilation of RV
Hemodynamic changes
MS involves mainly LA and RV
1. Effect of MS on left atrioventricular pressure gradient and left atrial pressure (LAP)
MVA Normal 46cm2 transvalvular gradient LAP
Mild MS
Moderate Severe
1.5cm2
1.01.5cm2 1.0cm2
5-10mmHg
10-20mmHg 20mmHg
25mmHg
Clinical manifestations
Symptoms
Onset in patients with moderately severe MS MVA 1.5 cm2 Dyspnea: Principal symptom, appears at early stage
Precipitated by exertion, fever, AF or pregnancy Exertional dyspnea, orthopnea, paroxysmal nocturnal dyspnea, acute pulmonary edema
Hemoptysis
Profuse hemorrhage: rupture of bronchial submucosal varices Blood-stained sputum Pink, frothy sputum Cough occurs frequently respiratory infection, compression of left bronchus Hoarseness (Ortners syndrome), less common Compression of left recurrent laryngeal nerve
Physical examination
Cardiac signs of MS
Changes of heart sounds:
Diastolic murmur of MS A low-pitched, diastolic rumbling murmur, localized at or near apex, with pre-systolic accentuation in patients with sinus rhythm Auscultation of the murmur is facilitated by placing the patient in the left lateral position and auscultate during expiration When the patient is in the left lateral recumbent position, a mid-diastolic or presystolic thrill may be palpable at apex
When RV dilation is companied by TR, a pansystolic murmur may be audible in the 4th or 5th intercostal space in the left parasternal region
Other signs
Mitral face: malar flush Signs of right heart failure:
Systemic venous hypertension, hepatomegaly, edema, and ascites are all signs of severe MS with elevated pulmonary vascular resistance and right heart failure
Laboratory examination
Electrocardiography (ECG)
Left atrial enlargement Mitral valve P wave
P-wave duration in lead II 0.12 s Large terminal negative P force in lead V1
Radiological findings
Mitral valve heart Marked enlargement of LA Enlargement of RV Dilatation of PA Pulmonary congestion Interstitial edema (manifested as Kerley B lines)
Echocardiography
The most valuable technique for diagnosing MS, and determining its severity M-mode echo
Thickened, calcified leaflets open poorly, close slowly (EF slope) The double peaks disappear Both leaflets move anteriorly during early diastole
Two-dimensional echo:
Fusion, thickening, doming of the valve leaflets, and poor leaflet separation in diastole; mitral orifice area
Doppler echo
Most accurate noninvasive technique for quantifying the severity of MS Spectrum Doppler: measure transvalvular gradient, MVA Color Doppler: display high velocity color jet
Cardiac catheterization
Its value in assessment of patients with MS or suspected MS has been largely superseded by echocardiography If surgery is planned, coronary angiography is performed to ascertain whether or not bypass grafting is indicated in patients at risk of having coexisting coronary artery disease
Complication
Atrial fibrillation Common
cardiac output by about 20% LA, age Incidence
Thromboembolism
Develop in 20% of patients About 2/3 found in the cerebral vessels Recurrent and multiple Risk factors: AF, LA55mm, a history of recent embolism or a low cardiac output
Infective endocarditis
Occurs less frequently on rigid, thickened, calcified valves and is therefore more common in patients with mild than with severe MS
Respiratory infection
Common Induce and aggregate heart failure
Management
General treatment Patients with RHD should receive penicillin
prophylaxis to prevent recurrence of RF and prophylaxis for IE Avoid and control anemia and infections Asymptomatic patients: avoid strenuous exertion Patients with dyspnea should reduce physical activity, restrict sodium intake, and take oral diuretics
Treatment of complications
Profuse hemoptysis
Measures designed to reduce pulmonary venous pressure, including sedation, assumption of the upright posture, and aggressive diuresis, are used to treat hemoptysis
Treatment of Arrhythmias
In patients with mild MS without marked LA dilation who have been in AF less than 6~12 months, elective cardioversion (electrical or pharmacological) should be considered
RHD: common(1/3); + MS and/ or aortic valve disease Mitral valve prolapse (MVP)
myxomatous degeneration, floppy and redundancy
Severe dilatation of LV
result in dilatation of the mitral annulus and lateral movement of papillary muscle
Infective endocarditis
valve leaflets destruction, perforation, retraction; valve closure interfered by vegetation
Other causes:
Rupture of the chordae congenital abnormalities obstructive hypertrophic cardiomyopathy
Hemodynamic changes
MR involves mainly LA and LV Chronic MR
Compensation: MR LV volumeLV, LA
LVEDV SV CO, EF
Decompensation:
Left HF, LAP and LVEDP pulmonary congestion, pulmonary hypertension, right HF (hepatomegaly, edema, and ascites) CO
Acute MR
MR LA, LV volumeLVEDPLAP pulmonary congestion, pulmonary edema SV and CO
Physical examination
Cardiac impulse at apex
Hyperdynamic Displaced laterally, inferiorly (Chronic)
Systolic murmur
RHDPansystolic, blowing, high-pitched murmur maximal at the apex
Anterior valve lesion, radiate to the axilla and back Posterior leaflet abnormality, radiate to the base
(Acute MR: not pansystolic murmur, but lower-pitched, decrescendo, and softer than the murmur of chronic MR)
Laboratory examination
ECG
Chronic (severe) MR:
LA dilation, Atrial fibrillation LV enlargement and non-specific ST-T changes Acute MR: sinus tachycardia
Radiological findings
Chronic (severe) MR:
Cardiomegaly with LA, LV; pulmonary congestion, interstitial edema with Kerley B lines (left HF) C-shaped calcification of mitral annulus
Acute MR:
Normal cardiac silhouette or mild LA dilation overt pulmonary congestion, edema
Echocardiography
1Display anatomy of the mitral valve apparatus Useful in determining the etiology of MR (2D) 2Confirm the existence of MR
Doppler (color, spectrum): reveal high-velocity jet into LA during systole Sensitivity~100%
3Measure cardiac chamber sizes, evaluate LV function, pulmonary artery pressure, provide data concerning other valvular lesions
Cardiac catheterization
Confirm the diagnosis of MR and estimate its severity, evaluate cardiac function and pulmonary artery pressure Coronary angiography is performed to determine presence of CAD prior to surgery
Diagnosis
Chronic MR
Typical systolic murmur at apex associated with enlargement of LA and LV
Acute MR
Sudden onset of dyspnea Systolic murmur at apex Normal cardiac silhouette, but obvious pulmonary congestion etiology existed
Confirmed by Echocardiography
Differential diagnosis
Tricuspid regurgitation (TR) SM heard best along the left sternal border augmented during inspiration Ventricular septal defect (VSD) SM loudest at the left sternal border accompanied by a parasternal thrill Systolic ejecting murmur at left sternal border: aortic or pulmonic stenosis hypertrophic obstructive cardiomyopathy Echocardiography
Complication
Atrial fibrillation seen frequently in severe cases Infective endocarditis more common than in MS Systemic embolism less common than in MS Heart failure occur early in acute MR but late in chronic MR
Management
Chronic MR Medical treatment
Prevention: same as in MS Asymptomatic patients with normal cardiac functionfollow-up regularly
Surgical treatment
Mitral valve replacement Indications
Severe MR and in functional Class or Functional Class associated with LV dilation
(LVESD45mm on echocardiography)
Severe MR, progressive deterioration of LVEF, LVESD and LVEDD
Acute MR
Principle
Reduce pulmonary venous pressure Increase cardiac output Correct etiology
Medical treatment
Intravenous nitroprusside Intravenous diuretics ACEI and other vasodilators
Surgical treatment
Mitral valve replacement Mitral valve repair
Degenerative calcific AS
Common in the elderly, accompanied by calcification of the mitral annulus
Congenital abnormalities
Calcific stenosis of congenitally bicuspid aortic valve Congenital aortic stenosis
Hemodynamic changes
Normal aortic orifice area (AOA): 3.0~4.0 cm2 AOA 1.0cm2, LVSP, with significant transvalvular gradient Compensation ASLV pressure load Concentric LVHcomplianceLVEDPLAH Maintain systolic wall stress and CO LVEDV Decompensation LVEDVwall stress, myocardial ischemia, fibrosis left HF
Clinical manifestations
Symptoms
Cardinal symptoms: dyspnea, angina and syncope 1. Dyspnea: exertional dyspnea
orthopnea paroxysmal nocturnal dyspnea acute pulmonary edema (varying degrees of pulmonary venous hypertension)
2. Angina pectoris:
occurs frequently in patients with critical AS, >1/3 associated with coronary artery disease
Mechanisms of ischemia
Myocardial oxygen consumption: LVH, LVSP, LVET Relative decrease in myocardial capillary density Subendocardial coronary artery compression: LVDP Coronary perfusion pressure: AO pressure, LVDP Imbalance between myocardial oxygen demand and supply
Physical examination
Systolic ejection murmur
Blowing, harsh, crescendo-decrescendo Maximal at aortic area (R2 or L3, 4) Transmitted to the neck and apex May be associated with systolic thrill The more severe the AS, the longer the duration of the murmur When the LV fails and the CO falls, the murmur becomes softer or disappear
Other signs
Left ventricular heave Systolic and pulse pressures Delayed and diminished carotid pulses
Laboratory examination
ECG
Severe: LVH and secondary ST-T changes, LAarrhythmias
Radiological findings
Normal size or slightly enlarged heart Calcification of the aortic valve Poststenotic dilatation of the ascending aorta Pulmonary congestion
Echocardiography
Establish a diagnosis, and determine the severity of AS
Doppler echo
Allows calculation of the aortic valve gradient
Estimate the severity of the stenosis 30 mmHg Mild AS MPG 30~50 mmHg Moderate AS 50 mmHg Severe AS Color Doppler flow imaging is helpful in the detection and determination of the severity of any accompanying aortic regurgitation
Cardiac catheterization
Determine the severity of AS by measuring systolic LV and aortic pressure simultaneously, and calculating the valve area
An average pressure gradient of 50mmHg or peak pressure gradient of 70mmHg represent severe AS Coronary angiography is performed in most adults to assess for concomitant coronary disease
Confirmed by echocardiography
Differential diagnosis
Transmitted murmurMR, TR, VSD Other LVOT obstructive disease
Congenital supravalvular AS Congenital subvalvular AS Hypertrophic obstructive cardiomyopathy
Management
Medical treatment
Treatment of Arrhythmias: prevent AF with an antiarrhythmic agent when premature atrial contractions are frequent; when AF does occur, restore sinus rhythm Treatment of angina pectoris: nitrates Treatment of heart failure: diuretics must be used with caution; vasodilators should be avoided
Surgical treatment
Valve replacement
Indications:
Repeated occurrence of syncope, angina pectoris or significant left heart failure
Asymptomatic patients with progressive LV dysfunction and/or LV hypertrophy, and very high transvalvular gradient (80mmHg) Severe ASAOA0.7 cm2
Infective endocarditis Congenital deformity: bicuspid valves Myxomatous degeneration of the aortic valve
Hemodynamic changes
Chronic AR
Compensation ARLV volume LV, LVEDV SV(CO) Decompensation: LV systolic dysfunction LV failure (EF, LVESV)
Acute AR
AR LV volume LVDP LAP CO pulmonary congestion pulmonary edema
Clinical manifestations
Symptoms Chronic AR
Asymptomatic for many years Palpitation, precordial discomfort, head pounding (related to SV) LV failure (dyspnea, fatigue): occur at late stage Angina pectoris or chest pain: less common
Acute AR
mildno symptom severeAcute LV failure and hypotension
(pulmonary edema)
Physical examination
Chronic, severe AR Peripheral arterial signs: Owing to wide pulse pressure:
SBP, DBP
Water-hammer pulse (rapid rise and fall) Pistol shot sounds (booming systolic & diastolic sounds
heard over femoral artery) Duroziezs sign (systolic, diastolic murmur over partially compressed femoral artery) Quinckes sign (subungual capillary pulsations)
Heart murmurs
Aortic diastolic murmur:
However, when it is due mainly to dilatation of the ascending aorta, the murmur is often more readily audible along the right upper sternal border
Austin-Flint murmur: apical mid or late diastolic low-pitched murmur: common in severe AR, owing to partial closure of MV by the regurgitant jet
Ejection systolic murmur: common harsh at the base of the heart accompanied by a systolic thrill Acute AR S1 soft or absentP2S3 and S4 AR murmur: lower pitched and shorter than that of chronic AR Austin-Flint murmur: brief
Laboratory examination
ECG
Acute: sinus tachycardia; nonspecific ST-T changes Chronic: LV enlargement and hypertrophy, arrhythmias
Radiological findings
Acute AR: cardiac size normal or slightly enlarged
signs of pulmonary congestion, pulmonary edema
Echocardiography
Confirm diagnosis, estimate severity, identify the cause
2-D echo:
Structural changes of the valve leaflets and/or aortic root
M mode echo:
Diastolic fluttering of the anterior leaflet of the mitral valve is an important echocardiographic finding in AR Serial assessments of LV size and function
Doppler echo:
Sensitive, accurate noninvasive technique for detecting AR LVOT diastolic regurgitant jet, estimate the severity of AR
Cardiac catheterization
Quantify the severity of AR Evaluate the coronary and aortic root anatomy
Differential diagnosis
Graham Steell murmur (pulmonary hypertension associated with dilatation) Austin-Flint murmur: differentiated from that of MS
Management
Chronic AR Medical treatment
Asymptomatic patients with severe AR and LV dilation: Vasodilators (ACEI, et al) reduce the severity of AR, should be used to prolong the compensated period -blocker: slow the rate of aortic dilation in Marfans
Surgical treatment
Valvular replacement
Indications:
Symptomatic patients Asymptomatic patients with LV dysfunction, with persistent or progressive LVESVor EFat rest
Surgical treatment
Urgently required Valvular replacement or aortic valve repair