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Rheumatic valvular
Heart Disease
By
Prof. Magdy Abou Al-Khair
Pediatric Cardiology Unit
Mansoura University Children’s Hospital
Rheumatic valvular Heart Disease
• Mitral valve involvement is the commenst
(75%).
• Aortic valve involvement in (25%).
• Combined stenosis and regurgitation of the
same valve may occur.
• Involvement of tricuspid valve is very rare.
• Involvement of pulmonic valve almost never
occurs.
Rheumatic valvular Heart
Disease
 MITRAL STENOSIS.
 MITRAL REGURGITATION.
 AORTIC REGURGITATION.
Rheumatic valvular Heart Disease
Mitral stenosis
Prevalence
It is the most common valvular involvement in adult
rheumatic patients.
In countries where rheumatic fever is more
prevalent on in Egypt, severe MS occurs in children under
age of 15 years.
Rheumatic valvular Heart Disease
Mitral stenosis
Pathology
- Thickening of the leaflets and fusion of the commissures
dominate the pathologic findings. Calcification with
immobility of the valve results over time.
- The left atrium (LA) and right-sided heart chambers
become dilated and hypertrophied.
- In patients with severe pulmonary venous hypertension,
pulmonary congestion and edema, and fibrosis of the
alveolar walls, hypertrophy of the pulmonary arterioles
and loss of lung compliance result.
Rheumatic valvular Heart Disease
Mitral stenosis
Clinical manifestations
History
- Patients with mild MS are asymptomatic.
- Dyspnea with or without exertion is the most common
symptom.
- Orthopnea, nocturnal dyspnea, or palpitation is
present in more severe cases.
Rheumatic valvular Heart Disease
Mitral stenosis
Clinical manifestations
Physical examination
- An increased RV impulse is palpable along the LSB.
- Peripheral pulses may be weak with narrow pulse pressure.
- Neck veins are distended if right-sided heart failure supervenes.
- A loud SI at the apex and a narrowly split S2 with accentuated
P2 are audible if pulmonary hyptertension is present.
- An opening snap (a short snapping sound accompanying the
opening of the mitral valve) is followed by a low-frequency mitral
diastolic rumble at the apex.
- A crescendo presystolic murmur may be audible at the apex.
- A high-frequency diastolic murmur of PR (Graham Steel’s
murmur) is present at the ULSB.
Rheumatic valvular Heart Disease
Mitral stenosis
Rheumatic valvular Heart Disease
Mitral stenosis
Laboratory Examination
Electrocardiography
RAD,LAH, and RVH (caused by pulmonary
hypertension) are common. Atrial fibrillation is rare in children.
X-ray study
-The LA and RV usually are enlarged and the MPA segment
usually is prominent.
-Lung fields show pulmonary venous congestion, interstitial
edema shown as Kerley’s B lines (dense, short, horizontal lines
most commonly seen in the costopherenic angles), and
redistribution of PBF (with increased pulmonoary vascularity) to
the upper lobes.
Rheumatic valvular Heart Disease
Mitral stenosis
Laboratory Examination
Echocardiography
- Echo is the most accurate noninvasive tool for the
detection of MS.
-Two-dimensional echo shows doming of thick mitral
leaflets, a small mitral valve orifice inscribed by the
thickened valve, and a dilated LA.
-Doppler studies can estimate the pressure gradient across
the mitral valve and the level of PA pressure.
Rheumatic valvular Heart Disease
Mitral stenosis
Complications
- Recurrence of rheumatic fever.
- Atrial flutter or fibrillation.
-Thromboembolism (related to the chronic atrial
arrhythmias) are rare in children.
- SBE can occur, but it is rare.
- Hemoptysis can develop from the rupture small vessels
in the bronchi as a result of long-standing pulmonary
venous hypertension.
Rheumatic valvular Heart Disease
Mitral stenosis
Management
A- Medical:
-Good dental hygiene and antibiotic prophylaxis against
SBE.
-Recurrence of rheumatic fever is prevented with penicillin
or sulfonamid.
-Varying degrees of restriction of activity may be
indicated.
-If atrial fibrillation develops (rare in children), digoxin
should be used to control ventricular response.
-Ballon valvuloplasty is an alternative to surgical closed
commissurotomy and may delay surgical intervention.
Rheumatic valvular Heart Disease
Mitral stenosis
Management
B- Surgical
Indications:
-Symptomatic patients (dyspnea on exertion, pulmonary
edema, paroxysmal dyspnea).
-Recurrent
atrial
fibrillation,
phenomenon, and hemoptysis.
thromboembolic
Rheumatic valvular Heart Disease
Mitral stenosis
Management
Procedures
-Closed mitral commissurotomy remains the procedure
of choice for those with a pliable mitral valve without
calcification or MR.
-Valve replacement may be indicated in patients with
calcified valves and those with MR.
Rheumatic valvular Heart Disease
Mitral Regurgitation
Prevalence
- MR the most common valvular involvement in
children with rheumatic heart disease.
Pathology
- Mitral valve leaflets are shortened because of fibrosis.
- When the degree of MR increases, dilatation of the
LA, LV and the mitral valve ring may occur.
Rheumatic valvular Heart Disease
Mitral Regurgitation
Clinical Manifestations
History
- Patients usually are asymptomatic during childhood
(because MR does not produce pulmonary congestion in
the early phase)..
- Fatigue (caused by reduced forward cardiac output).
- Palpitation (caused by atrial fibrillation) develop.
Rheumatic valvular Heart Disease
Mitral Regurgitation
Clinical Manifestations
Physical examination
-The jugular venous pulse is normal in the absence of CHF.
- A heaving, hyperdynamic apical impulse is palpable in severe
MR.
-The SI is normal or diminished. The S2 may split widely as a
result of shortening of the LV ejection and early aortic closure.
- The S3 commonly is present and loud.
- The hallmark of MR is a regurgitant systolic murmur starting
with S1, grade II-IV/VI, at the apex, with good transmission to
the left axilla (best demonstrated on left decubitus position).
- A short, low-frequency diastolic rumble may be present at the
apex.
Rheumatic valvular Heart Disease
Mitral Regurgitation
Rheumatic valvular Heart Disease
Mitral Regurgitation
Laboratory Examination
Electrocardiograph
-ECG is normal in mild cases.
-LVH or LV dominance, with or without LAH, is usually
present.
-Atrial fibrillation is rare in children but often develops in
the adult.
X-ray study
-The LA and LV are enlarged in varying degrees.
-Pulmonary vascularity usually is within normal limits, but
a pulmonary venous congestion pattern may develop if
CHF supervenes.
Rheumatic valvular Heart Disease
Mitral Regurgitation
Laboratory Examination
Echocardiography:
-Two-dimensional echo shows dilated LA and LV, and the
degree of dilatation is related to the severity of MR.
-Colour-flow mapping of the regurgitant jet into the LA
and Doppler studies can assess the severity of the
regurgitation.
Rheumatic valvular Heart Disease
Mitral Regurgitation
Management
Medical:
-Preventive measures against SBE and prophylaxis against
recurrence of rheumatic fever are important.
-Activity need not be restricted in most mild cases.
-Afterload-reducing agents are useful in maintaining the
forward stroke volume.
-Anticongestive measures (with diuretics and digoxin) are
provided if CHF develops.
-If atrial fibrillation develops (rare in children), digoxin is
indicated to slow the ventricular response.
Rheumatic valvular Heart Disease
Mitral Regurgitation
Management
Surgical:
Indications.
Intractable CHF, progressive cardiomegaly with
symptoms, and pulmonary hypertension.
Procedures.
Mitral valve repair or valve replacement is
performed under cardiopulmonary bypass.Valve repair
surgery is preferred over valve replacement in children as
long as the valve is pliable. Valve replacement is necessary
if the valve is thick, scarred, and grossly deformed.
Rheumatic valvular Heart Disease
Aortic Regurgitation
Prevalence
- AR is less common than MR . Most patients with AR have
associated mitral valve disease.
Pathology
- Semilunar cusps are deformed and shortened.
- The valve ring is dilated so that the cusps fail to oppose
tightly.
- The commissures usually are fused to a varying degree.
Rheumatic valvular Heart Disease
Aortic Regurgitation
Clinical Manifestations
History
- Patients with mild regurgitation are asymptomatic.
- Exercise tolerance is reduced with more severe AR or
CHF.
Rheumatic valvular Heart Disease
Aortic Regurgitation
Clinical Manifestations
Physical examination
- The precordium may be hyperdynamic with a laterally
displaced apical impulse.
- A diastolic thrill occasionally is present at the 3rd or 4th left
intercostol space.
- A wide pulse pressure and a bounding water-hammer
pulse may be present with severe AR.
- The SI is decreased in intensity. The S2 may be normal or
single.
Rheumatic valvular Heart Disease
Aortic Regurgitation
Clinical Manifestations
Physical examination
- A high –pitched diastolic decrescendo murmur, best
audible at the 3LICS or 4LICS, is the auscultatory
hallmark. This murmur is more easily audible with the
patient sitting and leaning forward.
- A systolic murmur of varying intensity may be present at
the 2RICS because of relative AS caused by an increased
stroke volume.
- The combination of the diastolic and systolic murmurs
gives rise to a to- and- fro murmur.
- A mid-diastolic mitral rumble(Austin Flint murmur)
occasionally is present at the apex when the AR is severe.
Rheumatic valvular Heart Disease
Aortic Regurgitation
Rheumatic valvular Heart Disease
Aortic Regurgitation
Laboratory Examination
Electrocardiography
- The ECG may be normal in mild cases.
- In severe cases,LVH usually is present.
- LAH may be present in long- standing cases.
Rheumatic valvular Heart Disease
Aortic Regurgitation
Laboratory Examination
X-ray study
- Cardiomegaly involving the LV is present.
- A dilated ascending aorta and/or a prominent aortic knob
frequently are present.
- Pulmonary venous congestion develops if LV failure
supervenes.
Rheumatic valvular Heart Disease
Aortic Regurgitation
Laboratory Examination
Echocardiography.
- The LV dimension is increased, but the LA remains
normal in size.
- Color-flow and Doppler examination can be used to
estimate the severity of the regurgitation.
Rheumatic valvular Heart Disease
Aortic Regurgitation
Clinical Manifestations
Natural History
- The patient remains asymptomatic for a long time, but if
symptoms begin to develop, many patients deteriorate
rapidly.
- Anginal pain and CHF are unfavorable symptoms.
- Infective endocardits is a rare complication.
Rheumatic valvular Heart Disease
Aortic Regurgitation
Management
Medical.
- Good oral hygiene and antibiotic prophylaxis against SBE
are important.
- Prophylaxis should be continued against the recurrence of
rheumatic fever with penicillin or sulfonamides.
- Activity need not be restricted in mild cases, but varying
degrees of restriction are indicated in more severe cases.
- If CHF develops, digoxin, diuretics, and after loadreducing agents may be beneficial.
Rheumatic valvular Heart Disease
Aortic Regurgitation
Management
Surgical
Indications.
A major clinical decision in AR is the timing of aortic valve
replacement .Ideally,it should be performed before
irreversible dilatation of the LV develops.
- Symptomatic patients with anginal pain or dyspnea on
exertion.
- In asymptomatic patients
* significant cardiomegaly (cardiothoracic ratio greater than
55% on chest x-ray films).
* Ejection fraction less than 40%.
* Stress-test- induced symptoms
Procedure. Aortic valve replacement is performed under
cardiopulmonary bypass.