Valvular Heart Disease

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

Etiology and Pathogenesis

Multiple factors contribute to the pathogenesis, including -hemolytic streptococcal pharyngitis and immunological status of the human body Cross immune response between host and streptococcal antigens Streptococcal pharyngitis Abnormal reaction-autoimmunity disease

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

Migratory polyarthritis: involves large joints

lasts 1~5 weeks, subsides without residual deformity prompt response to salicylates or nonsteroidal agents

Erythema marginatum: rare Subcutaneous nodules: uncommon Chorea: least common, most diagnostic

2Minor manifestations Clinical findings: fever, polyarthralgias Laboratory findings

Elevated acute phase reactants: ESR (erythrocyte sedimentation rate) CRP (C reactive protein)

ECG change: prolonged P-R interval A history of RF

Supporting evidence of an antecedent group A streptococcal infection:
Positive throat culture or rapid streptococcal antigen test Elevated or rising titers of antistreptococcal antibodies (anti-streptolysin O and anti-DNase B)

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

If allergic to penicillin, erythromycin be given

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

3. Treatment of symptoms and complications

If heart failure is present, digitalis preparations should be used cautiously because cardiac toxicity may occur with conventional dosages

Prevention
Primary prevention Early treatment of streptococcal pharyngitis
Penicillin or erythromycin

Secondary prevention To prevent recurrence of rheumatic activity

Long-acting penicillin (benzathine penicillin) 1.2 million units im, every 4 weeks Sulfonamides or erythromycin may be substituted

Mitral stenosis (MS)

Etiology
Most commonly rheumatic fever rheumatic heart diseaseRHD

Symptoms commence mostly in 2nd~4th decade

2/3 of all patients are female 25% of all patients with RHD have pure MS

40% have combined MS and mitral regurgitation (MR)

Other rare causes

Far less frequently, MS is congenital in etiology
observed primarily in infants and young children

Calcification of mitral annulus (when subvalvular or intravalvular extension is extensive)

observed in old patients

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

2. Effect of elevated LAP on pulmonary circulation

The elevated LAP in turn raises pulmonary venous and capillary pressures (PVP, PCP), resulting in exertional dyspnea LAP PVP PCP Dyspnea Lung compliance Pulmonary hypertension results from: 1. Passive backward transmission of the elevated LAP 2. Reactive pulmonary arteriolar constriction, which presumably is triggered by left atrial and pulmonary venous hypertension 3. Organic obliterative changes in the pulmonary vascular bed, which may be considered to be a complication of longstanding and severe MS

3. Effect of pulmonary hypertension on RV

Pulmonary hypertension

RV hypertrophy & dilation secondary TR, PR

Right ventricular failure

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:

Accentuated S1 Opening snap (OS) sharp, follows A2 along left

sternal border or at apex

Both suggest MV leaflets flexible

Marked calcification or thickening of the MV leaflets S1 becomes softer, and OS may disappear probably because of diminished motion of the leaflets

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

Cardiac signs secondary to pulmonary hypertension and RV dilation

RV pulsation is present at the left parasternal region

Accentuated or splitting of P2 may be heard in the second left intercostal space

Other signs of pulmonary hypertension:
Pulmonic ejection sound, owing to dilation of the PA Graham Steell murmur of PR: a decrescendo diastolic murmur along the left sternal border

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

Right ventricular hypertrophy Arrhythmia

Premature atrial contraction atrial fibrillation

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

Provide other important information

Cardiac chamber size (LA, RV) Left ventricular contractility Pulmonary arterial pressure Other coexisted valvular or congenital abnormalities Mural thrombi

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

Diagnosis and differential diagnosis

Diagnosis Diastolic rumbling murmur at apex
ECG or X ray reveals LA dilatation Confirmed by echocardiography

Differential diagnosis Diastolic murmur at apex

Increased flow across mitral valve
Severe MR Massive left to right shuntsVSD, PDA Hyperdynamic circulationhyperthyroid, anemia

Austin-Flint murmursevere AR Left atrial myxoma postural change of the murmur

other signs of myxoma

Graham Steell murmur

should be differentiated from aortic regurgitation

Complication
Atrial fibrillation Common
cardiac output by about 20% LA, age Incidence

Acute pulmonary edema Severe

Dyspnea and cyanosis; unable to lie on back pink, frothy sputum; both lungs filled with rales

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

Right ventricular failure

Late stage, main cause of death Dyspnea and hemoptysisprotective effect (RV CO pulmonary circulationLAP; thickening of alveolus & pulmonary capillary walls)

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

Acute pulmonary edema

Dilate venous system, reduce preload (nitrates) Avoid dilating small artery Digitalis glycosides do not benefit patients with MS and sinus rhythm, but are of great value in slowing the ventricular rate in patients with AF and in the treatment of right-sided HF

Treatment of Arrhythmias

AF with rapid ventricular rate:

Ventricular rate (70~80 bpm) Digitalis glycosidesiv cedilanid, oral digoxin -blockers

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

Prophylactic anticoagulant treatment AF Previous embolic episodes LA thrombus revealed by echocardiography

Long term anticoagulant treatment with warfarin is necessary in patients without contraindication

Right ventricular failure Restriction of sodium intake Diuretics Nitrates

Indications for relieving stenosis

Symptomatic patients with moderate to severe MS (MVA1.5 cm2), or evidence of pulmonary hypertension with RV hypertrophy Recurrent systemic emboli despite anticoagulation with moderate or severe stenosis

Percutaneous balloon mitral valvuloplasty PBMV

Procedure of choice for pure MS with pliable and noncalcific valve

Surgical techniques Open mitral commissurotomy

Indication: Patients without significant MR valvular calcification, involvement of chordae and papillary muscle, left atrial thrombus or restenosis

Mitral valve replacement

Indication: Severe distortion and extensive calcification of the valve and subvalvular apparatus; Associated with significant MR or aortic valve disease

Mitral regurgitation (MR)

Etiology
Mitral valve apparatus and/or LV structural and functional abnormality

RHD: common(1/3); + MS and/ or aortic valve disease Mitral valve prolapse (MVP)
myxomatous degeneration, floppy and redundancy

Ischemic heart disease (or CAD)

papillary muscle dysfunction

Mitral annular calcification

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

Clinical manifestations Symptoms

Chronic MR
Mild no symptom Severe left-sided heart failure
Weakness, fatigue (CO) Dyspnea (pulmonary congestion) RHD: symptoms occur late, once present, LV dysfunction is usually irreversible MVP: asymptomatic, or atypical chest pain, palpitation, fatigue; in severe MR, left HF occur at late stage

Acute Mild mild exertional dyspnea

Severe acute left HF, pulmonary edema,
or cardiac shock

Physical examination
Cardiac impulse at apex
Hyperdynamic Displaced laterally, inferiorly (Chronic)

Changes of heart sounds

S1(RHD) or normal (MVP, CAD) S3 (severe MR): prominent Mid or late systolic clickMVP Acute: P2S4

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

MVPmid- to late-systolic murmur Dysfunction of papillary muscles:

Variable (early, mid, late or holosystolic) Rupture of the chordae: musical

(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%

Estimate the severity of MR

4 cm2 Color flow jet area 4~8 cm2 8 cm2 Mild Moderate Severe

3Measure cardiac chamber sizes, evaluate LV function, pulmonary artery pressure, provide data concerning other valvular lesions

Radionuclide angiography and MRI

Evaluate LV function Estimate the severity of regurgitation
The regurgitant fraction can be estimated from the ratio of LV to RV (LV/RV) stroke volume

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

Management of AFsimilar to that in MS (slow ventricular rate, anticoagulation)

Treatment of heart failurerestriction of sodium intake, angiotensin-converting enzyme inhibitors (ACEI), diuretics, digitalis glycosides

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

Mitral valve repair Indications

MVP Chordal rupture Mitral annulus dilation

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

Aortic stenosis (AS)

Etiology
RHD
Common, + AR and mitral valve disease

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

3. Syncope: typically exertional

Arterial pressure cerebral perfusion
Mechanisms: Increase blood flow to exercising muscle without compensatory increase in cardiac output Severe arrhythmias

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

Heart sound changes

S1 normal or soft A2 weak or absent paradoxical splitting of S2 prominent S4

Aortic ejection sound

congenital AS or pliable valve AS

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

M-mode and 2D echo

Observe aortic valve opening, thickening and calcification Helpful in determining the etiology of AS Also invaluable in detecting associated mitral valve disease and in assessing LV performance, hypertrophy, and dilatation

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

Diagnosis and differential diagnosis

Diagnosis
Typical systolic murmur of AS
Associated with AR and/or mitral valve damageRHD Pure AS: Infants and young childrenunicuspid malformation Childhood ~65 yearscalcification of bicuspid AV 65 yearsdegenerative calcification

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

Commissural incision under direct vision

In children and adolescents with noncalcific severe congenital AS

Percutaneous balloon valvuloplasty Indications

Children and adolescents with congenital noncalcific AS Adults with severe calcific AS who are poor candidates for surgery or as an intermediate procedure prior to surgery:
Patients with cardiogenic shock due to critical AS Patients with critical AS who require an urgent noncardiac operation Pregnant women with critical AS Patients with critical AS who refuse surgical treatment

Aortic regurgitation (AR)

Etiology
Primary disease of the aortic valves and/ or aortic root

Aortic valve disease

RHD most common, about 2/3
+ AS and/or mitral valve disease

Infective endocarditis Congenital deformity: bicuspid valves Myxomatous degeneration of the aortic valve

Aortic root dilatation

Marfan syndrome Aortic dissection (involve annulus or leaflets) Syphilitic aortitis

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)

de Mussets sign (head bobs with each heartbeat )

Apical impulse: diffuse and forceful, displaced laterally

and inferiorly (hyperactive, enlarged LV)

Heart sound: An S3 gallop is common with LV failure

 Heart murmurs
Aortic diastolic murmur:

High-pitched, blowing, decrescendo pattern

When AR is due to primary valvular disease, the diastolic murmur is best heard along the left sternal border in the 3rd and 4th intercostal spaces

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

Chronic: LV enlargement, associated with dilatation of

the ascending aorta Severe, aneurysmal dilatation of the aorta suggests aortic root diseaseMarfan syndrome Pulmonary congestionLV heart failure

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

Diagnosis and differential diagnosis

Diagnosis
Characteristic diastolic murmur associated with peripheral arterial signs, make a diagnosis of AR Combined with other information, etiology is usually found

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

Patients with HF:

Vasodilators (ACEI), diuretics and digitalis glycosides

Surgical treatment
Valvular replacement

Indications:
Symptomatic patients Asymptomatic patients with LV dysfunction, with persistent or progressive LVESVor EFat rest

Repair or replacement of the root

Aortic root disease

Acute AR Medical treatment

Principle: reduce pulmonary venous pressure, increase cardiac output, and stabilize hemodynamics Intravenous nitroprusside Diuretics Positive inotropic agent Antibioticsactive IE

Surgical treatment
Urgently required Valvular replacement or aortic valve repair