RHEUMATIC HEART DISEASE

Dr Romila Chimoriya
Lecturer
Department of Pediatrics
INTRODUCTION
 Most common form of acquired heart disease in all age groups

 Rheumatic involvement of the valves is the most important

sequelae of acute rheumatic fever

 A single episode of acute rheumatic fever usually results in

complete healing of valvular lesions while repeated episode,
especially on previously affected valves results in RHD

 Mitral valve involvement occurs in about three fourths

 Aortic valve involvement in about one fourth of the cases

 The mitral valve > aortic valve >right sided heart valves
PATHOGENESIS FOR RHD
 The valvular lesions begin as small verrucae composed of
fibrin and blood cells along the borders of one or more of the
heart valves

 As the inflammation subsides, verrucae disappears and leave

scar tissue

 With repeated attacks , new verrucae form near the previous

ones, and the mural endocardium and chordae tendineae
become involved

 Repeated episodes, on previously affected valves result in

rheumatic heart disease
MITRAL REGURGITATION
 Most common valvular involvement in children with RHD(80-
85%)

 Loss of valvular substance, and shortening and thickening of

the chordae tendineae

 Systolic leak of blood into the left atrium- left atrial pressure
increases during systole and decreases during diastole-mean
left atrial pressure stays normal- no increase in pulmonary
venous pressure and no pulmonary congestion in mild MR

 If pulmonary congestion is present, it suggests acute MR or

severe MR
 Increase in left sided heart
 MR-2 exits for LV blood-forward flow-aorta

-back flow-LA
-Forward flow-insufficient during exertion-fatigue(most
common symptom)
-Dyspnea-severe MR,LVF
-Pulmonary artery hypertension in MR-Severe MR,LVF
CLINICAL FEATURES
i. Mild MR:
 Mild cases are asymptomatic

 Pansystolic murmur

 Apical impulse-normal

 No pulmonary hypertension

 Regurgitant volume<25%

 No cardiomegaly
CLINICAL FEATURES
ii. Moderate MR
 Fatigue and exertional dyspnoea due to reduced systemic
output
 Apical impulse-hyperdynamic

 Pansystolic murmur

 Pulmonary hypertension will occur later

 Cardiomegaly
CLINICAL FEATURES
iii. Severe MR:
 Severe dyspnoea

 Cardiomegaly-left atrial enlargement and LVH

 Severe pulmonary hypertension

 Parasternal heave

 Presence of a thrill

 Holosystolic murmer

 Hyperdynamic apical impulse

 Wide split of the second heart sound S2 (except in severe pulmonary

hypertension)
 S3 gallop

 Third heart sound S3 louder

INVESTIGATIONS
Electrocardiogram :
 Prominent bifid P waves

 Signs of left ventricular hypertrophy

 Associated right ventricular hypertrophy if pulmonary hypertension is

present
X-ray chest:
 Cardiomegaly due to enlargement of the left atrium and ventricle

 Congestion of perihilar vessels, a sign of pulmonary venous hypertension

 Calcification of the mitral valve is rare in children

Echocardiography :
 Enlargement of the left atrium and ventricle, an abnormally thickened
mitral valve
 Doppler studies demonstrate the severity of the mitral regurgitation.
DIFFERENTIAL DIAGNOSIS
 Ostium Primum ASD
 COA with MR(Congenital)

 Left ventricular fibroelastosis

 Congenital corrected TGA

 Papillary muscle dysfunction indilatation of left ventricle

 Ostium secundum ASD with floppy mitral valve

 Marfan and hurler syndrome

 Anomalous origin of left coronary artery from

pulmonary artery
TREATMENT
Management
• Prophylaxis against recurrences of rheumatic fever(for
10 years or 40 years, which ever is longer, sometimes
lifelong prophylaxis)
• Treatment of heart failure
• Treatment of Arrythmia
• Treatment of infective endocarditis

Surgical
• Despite adequate medical therapy have persistent heart
failure, dyspnea with moderate activity, and progressive
cardiomegaly, often with pulmonary hypertension.
MITRAL STENOSIS
 Fibrosis of the mitral ring, commissural adhesions, and
contracture of the valve leaflets, chordae, and papillary
muscles over time

 Not usually recognized until adult life

 Increased pressure , enlargement and hypertrophy of the

left atrium pulmonary venous hypertension
increased pulmonary vascular resistance Right
ventricular hypertrophy Tricuspid regurgitation
right-sided heart failure.
CLINICAL MANIFESTATIONS
 Mild lesions are asymptomatic
 Severe degrees of obstruction are associated with
exercise intolerance and dyspnea
 Critical lesions can result in orthopnea, paroxysmal
nocturnal dyspnea, and overt pulmonary edema, as well
as atrial arrhythmias
 Signs of right heart failure: hepatomegaly, ascites, and
edema.
 Hemoptysis

 Raised jugular venous pressure

CLINICAL MANIFESTATIONS
 A loud S1 at the apex
 A narrowly split S2 with
accentuated P2 are audible if
pulmonary hypertension is present
 Mid diastolic murmur is low-
pitched, mid diastolic rumbling
murmur, with presystolic
accentuation of grade III intensity.
It is best heard in the mitral area
and is localised therein. It starts
after the Opening snap
 A high-frequency diastolic
murmur of PR (Graham Steell's
murmur) is present at the upper
left sternal border
ASSESSMENT OF SEVERITY
 Accentuated first heart sound
 Mitral opening snap

 Delayed diastolic murmur with late diastolic

accentuation
 Closer opening snap to second sound more severe mitral
obstruction
INVESTIGATIONS
ECG:
 Features of left atrial dilation : Prominent and notched P waves (p-
mitrale)
 Features of RVH: right axis deviation

 Features of AF: absence of P wave

X-ray chest:
Left atrial enlargement
 Elevation of the left upper lobe bronchus.

 Double shadow, that is, a shadow within the shadow. The dilated left
atrium causes a shadow in the upper and outer border while that of
the right ventricle causes a shadow in the lower and inner border.
 Straightening of the left border of the heart is due to the dilated left
atrial appendage and the pulmonary artery. Also, the aortic knuckle
will be small. This is also called mitralisation of the heart.
INVESTIGATIONS
INVESTIGATIONS
X-ray chest:
 Cardiomegaly-it is due to right ventricular enlargement

 Features of pulmonary hypertension

 Features of pulmonary venous congestion

 Calcification of the mitral valve

ECHO:
 Thickening of the mitral valve, distinct narrowing of the mitral
orifice during diastole and left atrial enlargement.
 Doppler can estimate the trans-mitral pressure gradient
DIFFERENTIAL DIAGNOSIS
 Congenital MS
 Cor triatriatum

 Obstruction ofpulmonary veins

 Left atrial myxoma

TREATMENT
• Medical treatment: Mild and moderate MS is managed with
anticongestive measures (e.g., digoxin, diuretics).

• Surgical valvotomy

• Balloon valvuloplasty is indicated for symptomatic, stenotic,

pliable, noncalcified valves of patients without atrial
arrhythmias or thrombi
AORTIC REGURGITATION
 Pure AR without associated mitralvalve disease is rare
 5-8%

 In chronic rheumatic aortic insufficiency, sclerosis of the

aortic valve results in distortion and retraction of the
cusps

 Regurgitation of blood leads to volume overload with

dilation and hypertrophy of the left ventricle
CLINICAL MANIFESTATIONS
 Palpitations
 Sweating and heat
intolerance
 Dyspnea on exertion ,
 Wide pulse pressure with
bounding peripheral
pulses
 Systolic blood pressure is

progress to orthopnea and elevated, and diastolic

pulmonary edema pressure is lowered
 Angina precipitated by  Left ventricular apical

heavy exercise heave

 Nocturnal attacks with  A diastolic thrill may be

sweating, tachycardia, present

chest pain, and
hypertension .
CLINICAL MANIFESTATION
 High-pitched diastolic
decrescendo murmur, best audible
at the third or fourth left
intercostal space
 This murmur is more easily
audible with the patient sitting
and leaning forward.
 A systolic murmur of varying
intensity may be present at the
second right intercostal space
because of relative AS caused by
an increased stroke volume
 A mid-diastolic mitral rumble
(Austin Flint murmur) may be
present at the apex when the AR
is severe
INVESTIGATIONS
X-ray chest:
show enlargement of the left ventricle and aorta

ECG:
 left ventricular hypertrophy and strain with prominent P
waves

ECHO:
 large left ventricle and diastolic mitral valve flutter or
oscillation caused by regurgitant flow hitting the valve
leaflets
DIFFERNTIAL DIAGNOSIS
 PDA
 AV fistulae

 VSD with AR

 Ruptured sinus of valsalva

 Anemia

 Thyrotoxicosis

 Marfan syndrome

 Hurler syndrome

 Takayasu aortoarteritis
TREATMENT
 Aortic insufficiency does not regress

 Afterload reducers (ACE inhibitors or angiotensin

receptor blockers) and prophylaxis against recurrence of
acute rheumatic fever

 Surgical intervention (valve replacement): Surgery is

considered when early symptoms are present, ST-T wave
changes are seen on the electrocardiogram, or evidence
of decreasing left ventricular ejection fraction is noted
TRICUSPID REGURGITATION
 Features of TR -20-50%
 TR-organic or functional

 Systolic backflow of blood from RV-RA

 Volume overload of RA/RV

 RHD-TR is associated with MS or MR

 TR associated with MS-organic or functional due to PAH

 TR associated with pure MR-organic

CLINICAL FEATURES
 Pain in the right hypochondrium due to congested liver
 Fatigue-decrease in systemic output

 Apex beat-displaced outward and downward

 Pansystolic murmur
MANAGEMENT
 Decongestive measures
 Tricuspid annuloplasty or repair