Download Rheumatic heart disease (RHD)

Rheumatic heart disease (RHD)
By : Dr. Sanjeev
Rheumatic heart disease
 The sequelae of rheumatic fever consist of
mitral, aortic and tricuspid valve disease
 The mitral valve involvement manifests
predominantly as mitral regurgitation and
less common as mitral stenosis
 The aortic and tricuspid valve involvement
presents exclusively as aortic and tricuspid
regurgitation
 Rheumatic aortic stenosis has never been
described below the age of 15 years.
Terms
 Regurgitation : results from failure of a
valve to close completely, thereby allowing
reversed flow
 Stenosis : failure of a valve to open
completely, thereby impeding forward flow
 Pure : only stenosis or regurgitation is
present
 Mixed : both stenosis and regurgitation
coexist in the same valve, but one of these
defects usually predominates
Heart sounds
 First heart sound : when AV valve
closed (mitral and tricuspid)
 Second heart sound : pulmonary
and aortic valve closed
 Third heart sound: increase volume
of blood within the ventricle
 Fourth heart sound : just after
atrial contraction at the end of
diastole and immediately before S1.
Mitral regurgitation
 Is the commonest manifestation of
acute as well as previous rheumatic
carditis
Hemodynamics
 When mitral regurgitation is present -----
blood leaks backwards through the mitral
valve and into the left atrium when the heart
contracts (systolic phase) --- regurgitant
volume of blood reaches the left atrium
during ventricular systole, however, during
diastole it can pass freely across the mitral
valve ---- thus, mean atrial pressure =
normal or is only slightly increased (because
left atrial pressure increases during systole, it
drops during diastole) ---- there is thus no
increase in pulmonary venous pressure and
no pulmonary congestion --------
Cont…
 ---- the increased volume of blood handled by the
left atrium and left ventricle results in an increase in
the size of both these chambers -- Mitral
regurgitation provides two exits for the left
ventricular blood flow -- the forward flow through
the aortic valve into the systemic circulation and the
backward leak into the left atrium -- the forward
output becomes insufficient during exertion --
this decrease in the systemic output results in
fatigue, the commonest symptom of significant MR - absence of pulmonary congestion prevents
occurrence of dyspnea unless the MR is severe or
the left ventricular myocardium is failing ----
Cont…
 With failing left ventricle, the left ventricular diastolic
pressure increases, the left atrial and pulmonary
venous pressure increases and pulmonary congestion
appears -- there is an increase in pulmonary
arterial pressure and features of pulmonary arterial
hypertension appear.
 Presence of features of pulmonary arterial
hypertension in a patient having pure MR
suggests :
 1. severe MR or
 2. failing left ventricular myocardium, or
 3. acute MR
Cont…
 MR developing during acute RF is of sudden onset. In
addition there is active myocarditis resulting in poorly
functioning left ventricular myocardium. Thus the left
ventricular failure can occur even with relatively
moderate leaks during the acute illness.
 The size of the left atrium also plays significant role in
MR
 With acute MR the left atrial size is normal and the
increased volume reaching the left atrium increases
the left atrial and the pulmonary venous pressure,
resulting in pulmonary congestion and feature of left
ventricular failure
Cont….
 In long standing MR the left atrium increases in
size to accommodate the regurgitant volume
without increasing the left atrial pressure and
features of LVF are absent.
 Another adjustment consists of decrease in the
systemic vascular resistance to help increase
the forward flow.
R = P/Q
 where R is the vascular resistance (fluid
resistance), P is the pressure difference, and Q
is the rate of blood flow through it.
Cont…
 The maximum ejection of blood into
the aorta takes place during early
systole. The combination of these two
factors results in an increased systolic
and decreased diastolic pressure in
the systemic circuit . The pulse
pressure is, therefore, increased
resulting in the small water hammer
pulse of MR
Aetiology
 Dilatation of valve ring (Acute rheumatic fever,
Cardiomyopathy)
 Damage to the valve cusp and chordae (Rheumatic heart
disease, Infective Endocarditis)
 Damage to the papillary muscle (myocardial ischaemia ,
infarction)
 Mitral valve prolapse (congenital, degenerative,
connective tissue disease such as Marfan’s syndrome).
 Trauma — Chest trauma can rarely cause breakage of the
chords that hold the mitral leaflets in their normal
position. Untethered leaflets swing widely, allowing valve
leakage.
Clinical features
 1. Fatigue : when cardiac output starts to fall
 2. Dysponea : when pulmonary venous
hypertension occurs, dysponea on exertion,
orthopnea and paroxysmal nocturnal dysponea
(PND) may ocuur
 3. Pulse rate increased to maintain an adequate
cardiac output
 4. Features of left ventricular failure are absent
and appear late unless the mitral regurgitation is
acute, severe or left ventricular myocardium is
failing
Cont..
 5. Heart size is dependent on the severity of MR as
well as the status of the left ventricular myocardium.
 6. Apex beat is shifted down and out, farther than the
normal position, due to ventricular dilatation
 7. Systolic thrill (<10 %) due to the direction of the
regurgitant stream which is backwards into the left
atrium
 8. Systolic murmur is heard over the cardiac apex
(mitral area) with following characteristic :  1. Pansystolic murmur extending from s1 to s2
 2.High frequency murmur (diaphragm)
 3. murmur radiates towards the left axilla and to the
back below the scapula
Cont…
 9. First heart sound may be normal or
diminished in intensity
 10. Severe MR, when a large amount of
blood flows downs suddenly from the left
atrium to the left ventricle during diastole,
a third sound (s3) or ventricular gallop is
produced. Immediately after such a third
sound, a short mid diastolic murmur may
also be heard.
Investigations
Chest X-Ray:
ECG
Echocardiography
Doppler
Cardiac catheterization
Chest X-Ray
Backflow of blood due to incompetent mitral valve
 Heart is enlarged transversely
 The pulmonary vascular markings are typically normal,
since pulmonary venous pressures are usually not
significantly elevated.
 Pulmonary vascular markings prominent (marked pul.
HTN)
Cont…
 ECG: Atrial fibrillation, left atrial
enlargement (if patient is in sinus rhythm).
left ventricular hypertrophy can be seen
 Echocardiography: Images mitral valve,
left ventricular function and left atrial size.
LA and LV will be dilated.
 Doppler will quantify regurgitation
 Cardiac catheterization can be done for
pressure measurements
Differential diagnosis : Atrial septal defect
 Coarctation of aorta with MR
(congenital)
 Left ventricular fibroelastosis
 Myocarditis
Management : Medical Management :
 Low sodium diet
 Diuretics (patient with orthopnoea and
PND)
 Vasodilator: Sodium Nitroprusside or
Nitroglycerine may be used in acute and/or
severe MR.
 ACE inhibitors are used for treatment of
chronic MR (decreased the after load).
Cont…
 Digoxin is used for patients with atrial
fibrillation or associated left ventricular failure.
 Anticoagulant for patients with atrial fibrillation,
for prevention of thromboembolism and who
already developed features of systemic
embolization to prevent further embolization.
 Infective Endocarditis prophylaxis.
 Prophylaxis for Rheumatic fever if MR is of
rheumatic origin.
Cont…
Surgical Management :
 Symptomatic patients despite optimal
medical therapy
 Asymptomatic or mildly symptomatic
patient in presence of progressive LV
dysfunction.
 Mitral valve repair (Annuloplasty with valve
Reconstruction) can be done if valvular
cusps and basic architecture is preserved.
 Otherwise markedly deformed, with
shrunken, calcified leaflets requires mitral
valve replacement with a prosthesis.
Complications :
 Atrial fibrillation (in case of severe MR
and chronic long standing MR)
 Systemic embolization
 Infective endocarditis
 Congestive heart failure
 Pulmonary hypertension
Mitral stenosis
Normal size: 5 sq. cm
Cardiac symptoms due to mitral stenosis start to
be appear only when the valve is reduced to 2
sq.cm
Severe stenosis < 1 cm2
Aetiology :
• Acute RF with rheumatic endocarditis (99%)
• Some due to calcification of senile mitral valve
apparatus
•
Congenital (very rare)
Pathophysiology :  Blood cannot flow freely from the left atrium to the left
ventricle during diastole -- left atrial pressure as well as
volume increases --- increase in pressure and volume
occurs in the pulmonary veins and capillaries --- when
the pulmonary venous pressure exceeds the plasma oncotic
pressure, fluid from the vessels flow out into the interstitial
space and alveoli of the lungs --- leads to pulmonary
arterial hypertension --- right ventricle has to work more
during systole to push the blood into the pulmonary artery -- leads to right ventricular hypertrophy and later on to
right ventricular dilatation -- if pulmonary HTN becomes
severe, the amount of blood going to the left atrium from
the right ventricle and pulmonary congestion tends to
become less.
Clinical features :  Symptoms :
 1. Dyspnoea (commonest symptom) : due to pulmonary
venous congestion.



Mild stenosis -dyspnoea occurs on exertion or when the
heart rate increases due to any reason.
Severe stenosis -dyspnoea at rest
May develop orthopnoea and PND
 2. Cold extremities, with or without peripheral cyanosis
and a smaller volume pulse -- decreased cadiac output
in severe MS (recognized on the bed side)
Cont..
•
•
•
•
•
•
3. Fatigue (due to low cardiac output)
4. Palpitation (Atrial fibrillation, Sinus
tachycardia)
5. Haemoptysis (Pulmonary congestion,
Pulmonary embolism)
6. Cough, chest pain
7. Symptoms of Thromboembolism
8. Oedema, ascites (right heart failure)
Signs
•
Irregularly irregular pulse (atrial fibrillation)
–
–
–
–
–
–
Mitral facies (bluish pink hue over the malar
prominences)
Auscultation: Loud S1 , opening snap, mid
diastolic murmur
Signs of raised pulmonary capillary pressure:
Basal crepitation, pulmonary oedema, and pleural
effusion
Signs of pulmonary hypertension: RV heave,
loud P2
Signs of right heart failure : E.g. Raised JVP,
Hepatomegaly
Signs of systemic Thromboembolism : E.g.
Stroke, Acute limb ischaemia
Mitral Facies
Investigations :
ECG : Atrial fibrillation,Left Atrial
abnormality, Right ventricular
enlargement
• Echocardiogram : Structural
imaging of mitral valve, valve area,
left atrial dimension, presence of
thrombus in LA, pulmonary arterial
pressure, RV dilatation.
Chest X-Ray






:
Straightening of the left border with fullness &
outwards bulging of the pulmonary conus
There is double border on the right side
Pulmonary vasculature increases
Normally, Heart is normal in transverse
diameter
Cardiomegaly (rt. Ventricular enlargement)
Kerley B line
Dr S Chakradhar
31
Management
1
•
•
•
•
. Medical management :
Penicillin prophylaxis for rheumatic fever.
Prophylaxis for infective endocarditis.
Low sodium intake, diuretics.
If patient is in Atrial fibrillation ---- use
digoxin  low dose B-blocker.
• Anticoagulation for at least 1 year for
patients who suffered Thromboembolism
and permanently to those with AF.
2. Surgical management :
a. Mitral valvotomy :
Symptomatic patients whose valve area is less
than 1.0 cm2/m2 body surface area.
 Two methods :
1. Percutaneous ballon mitral valvotomy and
2. Surgical valvotomy : Indicated in Re-stenosis ,
unsuccessful balloon valvotomy,.
 Restenosis is frequent. This procedure cannot be
done if there is significant regurgitation,
calcification of the mitral valve or thrombus in
left atrium.

b. Mitral valve replacement :
 This is procedure of choice in :
 Critical mitral stenosis i.e. < 0.6 cm2/m2
body surface area
 Significant mitral regurgitation
 Calcified mitral valve leaflets
Complications




Atrial fibrillation
Systemic emboli
Pulmonary hypertension and
Heart failure
AORTIC STENOSIS
Aortic valve area : 3 square cm
Aetiology :
Infants ,children, adolescents
 Congenital
1. Valvular aortic stenosis
2. Subvalvular aortic stenosis
3. Supravalvular aortic stenosis
Cont…
Young adults to middle aged
 Calcification and fibrosis of bicuspid
aortic valve
 Acute rheumatic fever with
endocarditis
Pathophysiology :  When it gets narrowed, left ventricle has to pump harder to
send blood across the narrowed aortic valve into the aorta  increased work load -left ventricular hypertrophy -
hypertrophied ventricle manages to maintain the cardiac
output inspite of stenosis - during atrial systole, plenty of
blood comes to the left ventricle (atrial kick) - left
ventricle becomes more stretched due to such atrial kicks
and as per Frank Starling`s law, it now contracts more
vigorously and thus more blood goes out of the ventricle
into the aorta - gradually , the oxygen demand of the left
ventricle increases - cause angina and sudden death - if
left ventricle is overworked for prolonged period - LVF -
aorta blood will be less -left ventricular end diastolic
pressure and diastolic volume start to rise -left arterial
and pulmonary venous pressure increases and the patient
starts to feel dyspnoeic ( pulmonary congestion and
hypertension)
Clinical features :  Mild or moderate ----- Asymptomatic
 Cardinal symptoms like (1, 2, and 3)
1. Exertional dyspnoea (signs of LVF):- initially
exertional dyspnoea later PND.
2. Angina
3. Syncope : due to inadequate blood flow through the
stenosed aortic valve and arrhythmia.
4. Fatigue and palpitation
5. Apex beat : heaving or forceful and sustained type
(finger lifted up during systole, remains up for
sometime and then falls down
Cont…
6. Auscultation : three main signs :
Aortic ejection sound or click : heard over the
cardiac apex by the diaphragm, in early systole,
immediately after the first sound.
Aortic ejection murmur : mid systolic murmur,
heard over the right 2nd intercostal space by the
side of the sternum, radiates to the neck
towards both the carotids, and also called
diamond shaped ejection systolic murmur.
Aortic component of the second sound is either
late or soft
Cont…
 7. Fourth heart sound : due to increased
stiffness of the left ventricle, the atrium
contracts vigorously during atrial systole
and pushes the a large amount of blood
into the left ventricle, due to such strong `
atrial kick`, S4 becomes audible. It is a soft
and low pitched sound and is heard just
before S1. best heard over the cardiac apex
by using the bell of the stethoscope.
Investigations :  ECG: may show LV hypertrophy and ST
depression and T wave inversion; left
bundle branch block is common;
 Chest X-Ray : may show LV enlargement
in PA view and calcification of aortic valve
in lateral view.
 Echocardiography : will show abnormal
aortic valve with left ventricular
hypertrophy or dilatation.
 Doppler echocardiography : will estimate
the pressure gradient
enlargement of the ascending aorta(white arrow).
left ventricle is enlarged (red arrow) and the heart is mildly
enlarged overall.
The lateral view on the right demonstrates calcifications in the
region of the aortic valve leaflets (circle). generally, the aortic
valve lies above a line drawn from the carina to the junction of
the diaphragm with the anterior chest wall. The mitral valve lies
below the line.
Management
 Strenuous physical activity should be
avoided
 Sodium restriction, digitalis and diuretics
are used if there is heart failure.
 Vasodilators should be avoided or used with
extreme caution.
 Asymptomatic stenosis in elderly
conservative management is appropriate
Valve replacement in :
1. Patients with calcified AS with critical
obstruction (valve area <0.5 cm2/m2
BSA).
2. Patients with symptomatic aortic
stenosis (moderate to severe
stenosis) even with normal cardiac
output at rest.
3. Patients who exhibit LV dysfunction
even they are asymptomatic.
Cont…
• Penicillin prophylaxis for rheumatic
fever.
• Prophylaxis for infective endocarditis.
Complications
 Endocarditis
 Cardiac arrhythmias : atrial
fibrillation, ventricular arrhythmias,
complete heart block
 Left ventricular failure
Differential diagnosis
 Hypertrophic cardiomyopathy
 Innocent systolic murmur eg. In
anemia, thyrotoxicosis
 Hypertension
AORTIC REGURGITATION
Definition
 When the aortic valve is damaged
and cannot close completely during
diastole, blood from the aorta
regurgitates into the left ventricle,
such a state is called AR.
 Clinically pure aortic regurgitation –
without associated mitral valve
disease – is rare and occurs in 5 – 8
% patients
Pathophysiology :
 Blood regurgitates from the aorta into the
left ventricle during diastole -amount of
blood regurgitating into the left ventricle
depends upon :
 1. size of the regurgitant hole in the aortic
valve *
 2. pressure gradiant between the aorta and
the left ventricle during diastole
 3. duration of the diastole
Cont…
 When blood regurgitates from the aorta into the left
ventricle during diastole it starts to dilate - hypertrophy
of left ventricle - with progessive increase in the amount
of regurgitant blood, the left ventriclar muscle fibre gets
stretched further and as per Frank starling`s law, these
fibres contract more vigorously, thereby increasing the
stroke volume - but when the left ventricle is dilated too
much and for a long period, its capacity to contract starts
to decreased - stroke volume also decreases and the
volume overload in the left ventricle increases further
during diastole - peripheral vasodilation - hands and
feet are warm and diastolic pressure is very low (not clear
why there is peripheral vasodilation) -
Cont…
 later, when ventricular failure occurs, neuro
– hormonal activation leading to an
increase in sympathetic vasoconstriction
tone and increased intrinsic vascular
stiffness and fall in cardiac output ----
when AR develops suddenly -- the left
ventricular myocardium is failing and the
left ventricular end diastolic pressure goes
up - increase in left atrial pressure and
pulmonary congestion.
Clinical features :  1. Palpitation (main symptom) : due to
increased force of contraction of the left
ventricle
 2. Dyspnoea, orthopnoea and PND
 3. Sweating a lot when congestive failure
develops
 4. Anigna pectoris due to :
 1. low aortic diastolic pressure, due to which
coronary blood flow is reduced
 2. increase in oxygen demand of the left
ventricle as a result of left ventricular dilatation
and hypertrophy
Cont….
 5. Peripheral physical signs of aortic insufficiency are
related to the high pulse pressure and the rapid decrease
in blood pressure during diastole due to blood returning to
the heart from the aorta through the incompetent aortic
valve : 1. large-volume, 'collapsing' pulse also known as:
Watson's water hammer pulse or Corrigan's pulse
(rapid upstroke and collapse of the carotid artery pulse)
 2. De Musset's sign (head nodding in time with the heart
beat)
 3. Quincke's sign (pulsation of the capillary bed in the
nail)
 4. Hill's sign (a ≥ 20 mmHg difference in popliteal and
brachial systolic cuff pressures)
Cont….
 5. Müller's sign (pulsations of uvula)
 6. Traube's sign - two sound heard over femoral
arteries
 7. Duroziez sign - Systolic-diastolic murmur produced
by compression of femoral artery with a stethoscope
 8. Pistol shot - Loud systolic sound over femoral
arteries
 9. Gerhardt's sign (enlarged spleen and pulsation felt
over the spleen)
 10. Rosenbach's sign (pulsatile liver)
 11. Landolfi's sign (alternating constriction &
dilatation of pupil)
Cont..
 6. cardiomegaly (inspection and palpation) : apex
beat shifted further down and out and is forceful and
ill sustained (or hyperdynamic) in character.
 Auscultation :
 1. First and second heart sound both are normal
 2. Third heart sound or ventricle gallop (severe AR)
due to large amount of blood suddenly coming to the
left ventricle from the mitral and aortic valves during
early diastole. Important sign in AR is early diastolic
murmur : high frequency murmur, an early diastolic
murmur, decrescendo murmur and best heard over
the left side of the mid sternum over the second
aortic area
Cont…
 Many patients of AR have a mid
diastolic rumbling murmur at the
apex, as in MS, such murmur is called
Austin Flint murmur -- when the
blood regurgitates from the aorta into
the left ventricle in AR, the
regurgitant flow strikes the anterior
leaflet of the mitral valve and gives
rise to the Austin Flint murmur .
Investigations
 ECG: may show LV hypertrophy and ST
depression and T wave inversion
 Chest X-ray: may show cardiac and aortic
dilatation. There may be features of left
heart failure.
 Echocardiogram: dilated LV with vigorous
contraction. Vegetation may be visible if
cause is infective endocarditis. There may
be fluttering of AML (Anterior mitral leaflet)
Management
 Remove the treatable cause like
infective endocarditis, Rheumatic fever
 Medical therapy: low sodium diet,
diuretics, and ACE inhibitors.
 Surgery is advised if there is
progressive LV dysfunction even when
patient is asymptomatic or with mild
symptoms.