Rheumatic Heart Disease, by Dr. Emmanuel Rusingiza.
My name is Emmanuel Rusingiza. I'm a Pediatric
Cardiologist at Kigali University Teaching Hospital,
Rwanda. I'm going to talk about the diagnosis
and management of rheumatic heart disease.
Types of Valvular Diseases.
Rheumatic heart disease is the result of damage
to the heart valve which appear after repeated
episodes of acute rheumatic fever. Although
initial attacks can lead directly to rheumatic
heart disease, early diagnosis of rheumatic
heart disease is very important. So that secondary
prophylaxis can be started as soon as possible
to help prevent the progression of the valve
disease.
Echocardiography is an essential tool to confirm
the diagnosis and to detect any progression
of the valvular disease.
So now what are the types of rheumatic heart
disease? The types of valvular rheumatic heart
disease are the following. The mitral valve
is the most affected in over 90% of cases
of rheumatic heart disease. The next common
affected valve is the aortic valve. And usually
disease of the aortic valve is associated
with the disease of the mitral valve.
The tricuspid and pulmonary valves are rarely
directly affected. But the tricuspid regurgitation
may appear in advanced mitral valve disease
especially when there is severe pulmonary
hypertension.
So the valvular lesions include the mitral
regurgitation, which is found most commonly
in children and young adults. The mitral stenosis,
which represents longer term chronic changes
to the mitral valve more commonly seen in
adults. All the children can present both
mitral stenosis and mitral regurgitation.
Aortic regurgitation is not uncommon. And
aortic stenosis is almost never seen as an
isolated lesion.
Symptoms. The symptoms of rheumatic heart
disease depend on the valve lesion and it's
severity. Symptoms may not show for many years
until the valve disease becomes severe. Initial
symptoms of rheumatic heart disease are the
symptoms of early heart failure, which are
the breathlessness on exertion, general weakness,
cough, and sometimes hemoptysis based on the
level of the pulmonary edema, orthopnea, paroxysmal
nocturnal dyspnea, peripheral edema, which
can be generalized after awhile if there is
no intervention. Signs of chronic malnutrition
may occur as complication due to long-term
heart condition.
Specific symptoms include palpitations, in
case of atrial fibrillation, particularly
with mitral stenosis. And in this case, there
is an associated risk of embolic events. Stroke,
when there is cerebral embolism in case of
atrial fibrillation and/or effective endocarditis
of the mitral or aortic valve, but also sometimes
severe mitral stenosis can cause stroke. People
with aortic valve disease may experience syncope
in addition to heart failure symptoms.
Physical Examination.
Physical examination is a very important step.
And the clinical examination should include
assessment of severity and complications,
including signs of heart failure, which are
tachypnea, tachycardia, edema, raised jugular
venous pressure, hepatomegaly, lung crackles,
and so on. The presence of atrial fibrillation
and the presence of stroke must be assessed
as well.
The auscultation for the mitral regurgitation
reveals a pansystolic murmur heard loudest
at the apex and radiating laterally to the
axilla. [MITRAL REGURGITATION MURMUR]
Mitral stenosis reveals a low-pitched diastolic
rumble heard the best at the apex. [MITRAL
STENOSIS MURMUR]
In case of aortic regurgitation, there is
a diastolic blowing decrescendo murmur best
heard at the left sternal border with the
person sitting up. [AORTIC REGURGITATION MURMUR]
In case of aortic stenosis, the murmur is
a loud, low-pitched, midsystolic ejection
murmur best heard in the aortic area and radiating
to the neck. [AORTIC STENOSIS MURMUR]
Investigations. The investigations include
electrocardiography, which is essential to
determine the cardiac rhythm and other abnormal
patterns. Chest x-ray helps to assess the
size of the heart chambers and to detect pulmonary
congestion. Echocardiography is here the gold
standard exam. It confirms the diagnosis and
detects any rheumatic valve damage, helps
determine its severity, and assesses left
ventricular function.
Additional investigations include FBC, electrolytes,
especially sodium and potassium, renal function
tests, and liver function tests. Let us insist
a little bit on the echocardiography. In 2011,
the World Heart Federation has developed the
criteria to make echocardiography reporting
simple, reproducible, and consistent worldwide.
The echocardiographic criteria helps in screening
of rheumatic heart disease in schoolchildren.
And it is based on morphologic features and
pathological regurgitation for the mitral
and aortic valves. There are three diagnostic
possibilities based on the echocardiographic
criteria, which are definite rheumatic heart
disease, borderline rheumatic heart disease,
and normal valves.
These are some few examples of echocardiography
images. This slide shows a parasternal access
view, and you can see very well the left ventricle,
the left atrium, the aorta, the mitral valve.
Here the mitral valve looks thickened, and
the anterior leaflet, especially on the tip,
looks thickened. The posterior one looks retracted.
This is a four-chamber view, or apex view,
which shows the thickness of the mitral leaflet
and chordae. But mostly, you can see a significant
mitral regurgitation, which is posteriorly
directed jet and typical for rheumatic valvular
disease. This slide shows the long axis view
of the heart, which shows the thickness of
the mitral valve leaflets and the aortic leaflets.
This patient presents severe mitral stenosis,
aortic regurgitation, and aortic stenosis.
This is another image of long axis view, which
shows the Doppler on the aortic valve. And
there you can see very well the jet of aortic
regurgitation in red.
Management. The goal of the management of
rheumatic heart disease is to prevent disease
progression and to avoid, or at least delay,
valve surgery. Secondary prophylaxis for prevention
of recurrent acute rheumatic fever is the
main strategy.
The key elements of the effective management
of rheumatic heart disease are initial assessment,
education, and a referral to a medical or heart
specialist, if applies. In case of heart failure,
it is recommended to admit the patient for
bed rest and treatment of heart failure with
diuretics and ACE inhibitors.
Management of atrial fibrillation with the
digoxin and anticoagulation. Regular medical
and echocardiographic review. Secondary prophylaxis
is very important to prevent the recurrency
of acute rheumatic fever, which come to damage
more the heart valves. The infective endocarditis
prophylaxis is very important before dental
and surgical procedures. It is also needed
to plan for regular dental care and also contraception,
especially for female adolescents.
The complications of rheumatic heart disease
depend upon the affected valve. They are heart
failure with severe pulmonary hypertension,
infective endocarditis, stroke, atrial fibrillation,
chronic malnutrition, and ventricular dysfunction.
The treatment of congestive heart failure
depends upon the nature of the valvular lesion.
When it is due to severe mitral and aortic
regurgitation, it is important to admit the
patient for bed rest, give heart failure medications,
but, also consider the nutritional support.
When the heart failure is due to severe mitral
stenosis, the treatment is done by diuretics,
beta blockers, depending upon the score, aspiring
to prevent intra-atrial thrombus and stroke.
And the warfarin is absolutely indicated in
the case of atrial fibrillation.
The indication for heart surgery is determined
by the severity of the symptoms, the evidence
that the heart valves are significantly damaged,
the left ventricle chamber size, and the function.
The types of valvular surgery include valvular
repair or valve replacement by either a prosthetic
valve or a mechanical valve.
Factors to absolute contraindication to valve
surgery are poor left ventricle function with
valve regurgitation. Severe pulmonary hypertension
may pose an unacceptable risk for cardiac
surgery. Good nutritional status improves
post-operative outcomes. Anticoagulation is
indicated for patients who undergo valvular
replacement.
The commonly used drug is Coumadin, or warfarin.
The treatment with warfarin needs to be monitored
following replacement with mechanical valves.
Good anticoagulation management requires standardized
anticoagulation measurement using the International
Normalized Ratio called INR.
This table summarizes the indications, the
goal of INR, and the duration of anticoagulation
therapy. As you can see, most of the goal
for INR is between 2.5 and 3.5. And when there
is a mechanical valve, the direction of therapy
is lifelong. After cardiac surgery, it's very
important to plan regular long-term follow-up.
The conservative valve procedures, like valve
repair, require close observation to detect
the re-stenosis or a recurrence of valvular
regurgitation.
Ensure a secondary prophylaxis is administered
regularly using benzathine penicillin G IM
every three to four weeks to prevent recurrent
attacks by acute rheumatic fever. It is also
important to monitor the left ventricle and
prosthetic function during the follow-up.
Now, endocarditis prevention is another important
point, because infective endocarditis is a
serious complication of rheumatic heart disease
and may also occur after heart valve surgery.
This uncommonly occurs during dental and surgical
procedures. But often, the source of the infection
is not clear. Infective endocarditis most
commonly occurs in the mitral or aortic valve,
since these are the most commonly damaged
heart valves. Thank you for watching.
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