PAEDIATRIC

RHEUMATIC HEART
DISEASE

EU, DEPT OF PAEDIATRICS

OUTLINE

Introduction
Epidemiology
Valvular stenosis
Valvular insufficiency/ regurgitation
Mitral Stenosis and Mitral Regurgitation
Aortic Stenosis and Aortic Regurgitation
Tricuspid Stenosis and Tricuspid Regurgitation
Pulmonary Stenosis and Pulmonary Regurgitation
Valve repair procedures
Summary

INTRODUCTION
Anatomy: The Valves

Mitral Valve

INTRODUCTION
RHD is the permanent heart valve
damage resulting from one or more
attacks of acute rheumatic fever (ARF)
It is thought that 40-60% of patients with
ARF will go on to developing RHD.
The commonest valves affected are the
mitral and aortic, in that order. However
all four valves can be affected

INTRODUCTION
Acute rheumatic heart disease often produces
a pancarditis (39% of pts) characterized by
endocarditis, myocarditis, and pericarditis.
Endocarditis is manifested as valve
insufficiency.
The mitral valve is most commonly and
severely affected (65-70% of patients), and the
aortic valve is second in frequency (25%). [MV
+AV = 50%, AV alone = 25%, MV+AV+TV
= 15%]

INTRODUCTION
The tricuspid valve is deformed in
only 10% of patients and is almost
always associated with mitral and
aortic lesions.
The pulmonary valve is very rarely
affected.
Pericarditis, when present, rarely
affects cardiac function or results in
constrictive pericarditis

INTRODUCTION
Chronic manifestations due to residual
and progressive valve deformity occur in 939% of adults with previous rheumatic
heart disease.
Fusion of the valve apparatus resulting in
stenosis or a combination of stenosis and
insufficiency develops 2-10 years after an
episode of ARF.
Atrial dilatation, arrhythmias and ventricular
dysfunction are other chronic sequelae.

INTRODUCTION
Recurrent episodes may cause
progressive damage to the valves.
Fusion occurs at the level of the valve
commissures, cusps, chordal attachments,
or any combination of these.
Rheumatic heart disease is
responsible for 99% of mitral valve
stenosis in adults in the USA (possibly
more so in developing countries)

EPIDEMIOLOGY
Chronic RHD is estimated to occur in 5-30
million children and young adults. (Av. 15
million) M=Fs
282,000 new cases worldwide each year
233,000 individuals die from RHD each year
Mortality rate from RHD is 1-10%
RHD is more severe in females compared to
males

Valve insufficiency from acute rheumatic valve

disease resolves in 60-80% of pts who adhere
to antibiotic prophylaxis

VALVULAR
INSUFFICIENCY/REGURGITATION

Scarring and retraction of valve leaflets or

weakening of supporting structures incomplete
closure of the valve leads to leakage or
backflow of blood from the previous chamber
EFFECTS: causes the heart to pump the same
blood twice (as the blood comes back into the
chamber)
The heart dilates to accommodate more blood
(the usual blood it needs to pump +
regurgitated blood)
Ventricular dilation and hypertrophy
eventually leads to heart failure

VALVULAR STENOSIS
The valve opening narrows
The valve leaflets may become fused or
thickened so that the valve cannot open freely
obstructs the normal flow of blood
EFFECTS: the chamber behind the stenotic
valve is subject to greater stress must
generate more pressure or work hard to force
blood through the narrowed opening
Initially, the heart compensates for the
additional workload by gradual hypertrophy

and dilation of the myocardium

Heart failure

MITRAL
STENOSIS
(MS)
Most common
valvular
disorder in
rheumatic fever
May also be caused by
bacterial infection,
thrombus formation, or
calcification
Obstruct blood flow
from LA

MITRAL
STENOSIS

PATHOPHYSIOLOGY OF MS
Narrowing of mitral
valve
left atrial
pressure

Hypertrophy
left atrium

blood flow
to left
ventricle

pulmonary
pressure
pulmonary
congestion

O2/CO2 exchange
(fatigue, dyspnea,
orthopnea)

CO
Left
ventricular
atrophy
Right-sided
Heart failure

Fatigue

CLINICAL FEATURES MS

Exertional dyspnoea and fatigue (most common)

Orthopnea, paroxysmal nocturnal dyspnea, cough,
hemoptysis, cyanosis
Right-sided heart failure distended neck veins,
peripheral edema, hepatomegaly, abdominal
discomfort
Auscultation: S1 followed by an opening snap--created
by forceful opening of mitral valve and a rumbling
diastolic murmur loudest at apex

Vitals: Pulses becomes irregular & faint, BP.

INVESTIGATIONS FOR MS
CXR- left atrial enlargement
ECG atrial fibrillation may develop
(50-80%
of pts.)
Echocardiogram (2D Echo) most
sensitive in
diagnosis

MANAGEMENT OF MS
Sodium restriction, diuretics to relieve pulmonary
congestion, bed rest, sitting position
Digitalis improve cardiac contraction, HR, treat
atrial fibrillation
Anticoagulants (blood thinners) eg warfarin,
aspirin,
Surgical interventions:
Mitral commissurotomy separation or incision of the
stenosed valve leaflets at their borders or commissures
Balloon mitral valvuloplasty
Mitral valve replacement when stenosis is severe

Balloon
mitral
valvulopla
sty

MITRAL REGURGITATION
(MR)
Incomplete closure of the mitral
valve
RHD is the predominant cause
May also be due to congenital
anomaly, infective endocarditis,
rupture of papillary muscle following
Myocardial Infection (in adults)

 a leaking mitral valve - Stroke volume, CO

- Left atrial hypertrophy
- Pulmonary congestion

MITRAL
REGURGITATION

PATHOPHYSIOLOGY
OF MRIncomplete closure of
mitral valve
Backflow of blood to
the left atrium
vol. of blood
ejected by left
ventricle

Left atrial pressure

CO

Left atrial
hypertrophy
Pulmonary
pressure

Right-sided heart
failure

Right ventricular
pressure

CLINICAL MANIFESTATIONS
OF MR

Fatigue and weakness due to Cardiac

Output predominant complaint

Exertional dyspnea & cough pulmonary
congestion
Palpitations (older children) due to atrial
fibrillation (occur in 75% of pts.)
Right-sided heart failure distended neck veins,
edema, ascites, hepatomegaly
Auscultation: blowing, high-pitched systolic
murmur
(apex) S1 is diminished S3 severe
regurgitation

INTERVENTIONS for MR
Restrict physical activity to prevent
fatigue & dyspnea
Na+ intake, diuretics relieve
congestion
Digitalis, vasodilators promote adequate
ventricular emptying and prevent or
decrease regurgitation
ACE inhibitors arterial dilation,
afterload
Surgery: Valvuloplasty (repair or
reconstruction) Valve replacement

AORTIC
STENOSIS
(AS)
Narrowing of the aortic valve
flow of blood from the left ventricle
to the aorta
blood volume and pressure in the
LV.
LV hypertrophy develops as a
compensatory
mechanism to continue pumping
blood through
the narrowed valve opening

AORTIC STENOSIS

AORTIC
STENOSIS

PATHOPHYSIOLOGY
OF AS
Stiffening/Narrowi
ng of Aortic Valve

Left ventricular hypertrophy

Compression of
coronary arteries

O2 supply

CO
Myocardial
O2 needs

Myocardial
ischemia
(chest pain)

Incomplete emptying of
left atrium
Pulmonary congestion

Right-sided
heart failure

CLINICAL FEATURES OF AS
Fatigue & exertional dyspnoea 1st
symptoms
due to CO and pulmonary congestion
Chest pain (angina) most common
symptom
occurs during exercise due to inability of the

heart to increase coronary blood flow to cardiac

muscle

Exertional syncope, vertigo, periods of

confusion due to CO

CLINICAL FEATURES OF AS
Weakness, orthopnea, Paroxysmal nocturnal
dyspnoea, pulmonary edema (severe cases)
Signs of right-sided heart failure end-stage
symptoms : - if untreated, survival rate: 1.5-

years
Auscultation: harsh, rough, mid-systolic
murmur

INTERVENTIONS IN AS

Restrict activity
Digitalis
Na+ restriction, diuretics
Nitroglycerin for chest pain
Surgical:
Balloon aortic valvuloplasty
Aortic valve replacement if not done - poor
prognosis

AORTIC
REGURGITATION (AR)

Most common

cause
of AR is
Rheumatic
fever
Other causes:
connective
tissue
disease
(Marfans
syndrome),
severe

AORTIC
REGURGITATION

Incomplete closure of
the
PATHOPHYSIOLOGY
OF
AR
aortic valve

Backflow of blood to
Left ventricle
Left ventricular
hypertrophy &
dilation

Left atrial pressure

Left atrium
hypertrophy

Left-sided heart
failure
(late stage)

Pulmonary
pressure

CO
Right-sided heart
failure

Right
ventricular
pressure

CLINICAL FEATURES OF AR
Patient may remain asymptomatic
for years --- heart compensates by
hypertrophy & dilation
First signs and symptoms:
heightened awareness of the heart
beat & palpitations esp. when pt. lies
on left lateral position
Tachycardia, associated with left
ventricular dilatation

CLINICAL FEATURES OF AR
Bounding pulse, marked carotid artery
pulsation, apical pulse force and
volume of contraction of the hypertrophied
LV
Decompensation occurs (cardiac muscle
fatigue)

exertional dyspnea
chest pain myocardial ischemia
left-heart failure fatigue, orthopnea, PND
right-heart failure peripheral edema

Auscultation: soft, blowing diastolic

murmur

MANAGEMENT OF AR
Antibiotic prophylaxis before any
invasive or dental procedures
Avoid physical exertion, competitive
sports
Vasodilators, calcium channel
blockers, ACE inhibitors
Aortic valvuloplasty or valve
replacement

TRICUSPID STENOSIS
Usually occurs together with aortic or mitral
stenosis
May be due to rheumatic heart disease
blood flow from right atrium to right
ventricle leads to right ventricular output
which leads to left ventricular filling
leads to
Cardiac output
blood accumulates in systemic circulation
causing systemic pressure

CLINICAL FEATURES OF TS
Signs and symptoms: symptoms of
right-sided
heart failure

hepatomegaly
peripheral edema
neck vein engorgement
CO fatigue, hypotension

TRICUSPID REGURGITATION
TR is uncommon, may be caused by
Rheumatic
fever, bacterial endocarditis
May also be caused by enlargement of right
ventricle
An insufficient tricuspid valve allows blood to
flow back into the right atrium venous
congestion & right ventricular output

blood flow towards the lungs

TRICUSPID REGURGITATION

TRICUSPID REGURGITATION
May not produce any symptoms
If moderate-to-severe TR exists, the following
may result:

Active pulsing in the neck veins

Swelling of the abdomen
Pedal oedema
Fatigue, tiredness, weakness
Decreased urine output

On palpation, there may be a lift (beating of

enlarged RV)
Murmur on auscultation, systolic murmur

PULMONARY VALVE
STENOSIS

Is a rare condition, usually congenital in origin

flow of blood to the pulmonary artery due to
narrowing of PV blood flows back to right
ventricle
and right atrium RV hypertrophy to
compensate for
blood volume and force blood to the pulmonary
artery
Signs and symptoms:

harsh systolic murmur

fatigue, dyspnea on exertion, cyanosis
poor weight gain or failure to thrive in infants
hepatomegaly, ascites, edema

PULMONARY
REGURGITATION
PR is a rare condition, usually caused
by
infective endocarditis, tumors or RF
blood flows back into Right ventricle
Right
ventricle and atrium hypertrophy
symptoms
of Right-sided heart failure

VALVE REPAIR PROCEDURES

Valvuloplasty: Repair of cardiac valve
Commisurotomy: Separation of fused
leaflets
Baloon valvuloplasty: Using a baloon to
separate fused/stenosed valve leaflets
Annuloplasty: Repair of valve annulus
(junction of the valve leaflets and the
muscular heart wall)
Chordoplasty: is repair of chordae
tendineae (Usually done for MR)

SUMMARY
RHD is a sequelae to acute rheumatic fever
that is improperly managed
Progressive valvular destruction due to
inflammatory processes
Heart failure is usually an inevitable end
result of valvular dysfunction with risk of
long term morbidity or death.
Valvular regurgitation/insufficiency and or
stenosis can occur, singularly or together
Surgical repair is usually warranted in
severe cases. Long term follow up is
necessary

QUESTION
S