Rheumatic valvular heart disease pediatrics AG

RHEUMATIC

VALVULAR HEART

DISEASE

LEARNING OBJECTIVES :-

I. To know the incidence of RVHD.

II. The pathophysiology, clinical features, diagnostic

modalities & treatment options of commonly

occurring valvular lesions.

III. Why to Emphasis prevention.

INTRODUCTION

Rheumatic Heart Disease causes the permanent

heart valve damage resulting from one or more

attacks of Acute Rheumatic Fever.

It is thought that 40-60% of patients with ARF will

go on to developing RHD without proper secondary

prophylaxis*.

The commonest valves affecting are the mitral and

aortic, in that order. However all four valves can be

affected*.

*AHA guidelines 2006

-: MITRAL REGURGITATION :-

MITRAL REGURGITATION :-

Pathophysiology :-

Structural changes include loss

of valvular substance with

shortening & thickening of

cordae tendineae.

With increasing severity of MR,

dilation of Left atrium &

ventricle result in dilation of

mitral valve ring.

Pulmonary hypertension may

eventually develops.

Small

vegetations

are

formed at

injured parts

MITRAL REGURGITATION

Clinical Manifestations :-

History –

i. Usually asymptomatic with mild MR

ii. Rarely, fatigue & palpitations

Physical Examination –

i. JVP normal.

ii. Heaving, hyperdynamic apex beat in severe MR

MITRAL

REGURGITATION


S1 normal or diminished.

S2 widely split.

S3 commonly present.

Regurgitant systolic murmur

starting with s1, Grade 2 to

4/6 at apex with good

transmission to left axilla.


Electrocardiography :-

Normal in mild cases.

Left ventricular hypertrophy Or LV dominance with or

without Left atrial hypertrophy is usually present.

Atrial fibrillation is rare in pediatric age.

X ray studies :-

LA & LV enlarged.

Pulmonary venous congestion if CHF.

Echocardiography :-

2D echo shows dilated LA & LV.

Color flow mapping & Doppler studies


Management :-

Medical-

i. Preventive measures against SBE & prophylaxis

against recurrence of rheumatic fever.

ii. No activity restriction for mild cases.

iii. Afterload-reducing agents

iv. Anticongestive therapy if CHF.

v. For atrial fibrillation digoxin indicated.


Management :-

Surgical* –

i. Severe MR

ii. Progressive cardiomegaly with symptoms.

iii. Pulmonary hypertension.

iv. Valve repair preferred over valve replacement.

v. Valve function checked by 2D echo every 6 to 12

months.

vi. Valve replacement warrants anticoagulation for

life time.


-: MITRAL STENOSIS :-

MITRAL STENOSIS :-

Pathophysiology :-

Fibrosis of mitral ring, commissural adhesions,

contractures of valve leaflets, cordae & papillary

muscles

Takes 10 years or more to fully established lesion.

Usually recognized in adult life.

Significant MS causes left atrial enlargement,

pulmonary venous hypertension, leading to right

ventricular & atrial dilatation.

MITRAL STENOSIS


History –

i. Usually asymptomatic with mild MS.

ii. Breathlessness with or without exertion is commonest

symptom, orthopnea, nocturnal dyspnea, palpitations

in more severe cases.


i. Increased right ventricular impulse.

ii. Distended Neck veins.

MITRAL STENOSIS

Clinical Manifestations

:-

Loud S1 at apex.

Narrowly split S2

with accentuated

P2

An opening snap

Low frequency mid

diastolic rumble at

apex

Occasionally,

crescendo

presystolic murmur.

MITRAL STENOSIS


Right axis deviation, Left atrial hypertrophy & Right

ventricular hypertrophy

Atrial fibrillation is rare.

X ray studies :-

LA & RV enlarged & main PA segment prominent.

Pulmonary venous congestion, interstitial edema

shown as Kerley’s B lines

Redistribution of pulmonary blood flow with increased

pulmonary vascularity to upper lobes.

Echocardiography :-

2D echo shows dilated LA & LV.

Color flow mapping & Doppler studies

MITRAL STENOSIS

Echocardiography :-

Most accurate noninvasive tool.

2D echo defines structural abnormality.

Doppler estimates pressure gradient.

o Less than 5 mmHg mild

o 6 to 12 mm Hg moderate

o More than 12 severe

Natural History :-

Asymptomatic mild MS become symptomatic with

exertion.

Recurrence of rheumatic fever worsens the stenosis.

SABE is rare.

Hemoptysis common.

MITRAL STENOSIS

Management :-

Medical-

i. Mild to moderate MS managed with

anticongestive measures.

ii. Good dental hygiene & antibiotics prophylaxis

against SBE

iii. Varying degrees of restriction of activity.

iv. Recurrence of rheumatic fever should be

prevented

MITRAL STENOSIS

Management :-

Surgical* –

i. MS with symptoms

ii. Significant MS with failure to thrive warrants

balloon or surgical intervention.

iii. Failed balloon dilatation.

iv. Mitral commissurotomy for pliable valve without

calcification.


-: AORTIC REGURGITATION :-

AORTIC REGURGITATION

Pathophysiology :-

Almost always associated with mitral valve disease.*

In chronic aortic rheumatic aortic regurgitation,

sclerosis of aortic valves results in distortion &

retraction of the cups.

Regurgitation of blood leads to volume overload with

dilatation & hypertrophy of left ventricle.

*M.Park pediatric cardiology 5th edition



History –

i. Usually asymptomatic with mild AR.

ii. Exercise tolerance is reduced with moderate to

severe AR.


i. Hyperdynamic precordium with moderate or severe

AR with laterally displaced apical impulse.

ii. Diastolic thrill at left third intercostals space

iii. Wide pulse pressure with bounding water hammer

pulse with severe AR



S1 is decreased in intensity.

S2 may be normal or single.

High pitched diastolic decrescendo murmur is the

hallmark of AR.

Longer the murmur more severe the regurgitation.

Systolic murmur at right second intercostal space may

be present.

Severe AR presents with to & fro murmur*

A mid diastolic mitral rumble (Austin flint murmur) at

apex. *M.Park pediatric cardiology 5th edition



Left ventricular hypertrophy in severe AR.

X ray studies :-

Cardiomegaly involving Left ventricle.

Dilated ascending aorta & prominent aortic knob.

Echocardiography :-

Left ventricular dimensions are increased.

Left ventricular dimensions are proportional to severity

of AR.


Natural History :-

Mild to moderate AR remains Asymptomatic for long

time but when symptoms begin to appear patient

deteriorate rapidly.

Anginal pain, CHF, Multiple premature ventricular

contractions are unfourable signs occurring with severe

AR.

Infective endocarditis is rare


Management :-

Medical-

i. If CHF develop digoxin, diuretics may be

beneficial but the benefits are rarely maintained.

ii. When used on long term basis ACE inhibitors

reduce the dilatation & hypertrophy of LV

iii. Activity restriction not needed in mild AR.


Management :-

Surgical* –

i. AR with symptoms

ii. Asymptomatic AR with Left ventricular systolic

dysfunction

iii. Asymptomatic AR with progressive Left

ventricular enlargement

iv. Repair is favored over replacement.


EFFECT OF ENVIRONMENTAL

FACTORS ON RHEUMATIC HEART

DISEASE

WHY SECONDARY PREVENTION ?

Sadly, RHD can go undetected with the result

that patients present with debilitating heart

failure.

At this stage surgery is the only possible

treatment option.

Patients living in poor countries have limited or

no access to expensive heart surgery.

THIS IS TOO

LATE

Antibiotic Administration Dose

Benzathine

penicillin

Single IM injection

every 21 days

1.2 MU > 27 kg

600 000 U < 27 kg

Penicillin V BD PO daily 250 mg bd

Erythromycin

ethylsuccinate

BD po daily Use same dose as above.

SECONDARY PREVENTION

STOPS SORE THROAT, PREVENTS RECURRENCES OF ARF AND

AIDS IN REGRESSION OF RHD*

Oral penicillin has been shown to be less effective than Penicillin IMI*


WHO SHOULD RECEIVE* ?

Patients with documented history of rheumatic fever.

Isolated rheumatic chorea


Awareness ♦ Surveillance ♦ Advocacy ♦ Prevention

Secondary prevention: Duration*

CATEGORY DURATION OF PROPHYLAXIS

All persons with ARF

without carditisMINIMUM 5 years after most recent episode or

age 21, whichever is longer


and carditis but

without residual heart

disease (no valvular

disease)

MINIMUM 10 years after most recent episode

or age 21, whichever is longer


and carditis with

residual heart disease

(persistent valvular

disease)

MINIMUM 10 years after most recent episode

or age 35 and then specialist review for need to

continue. Post surgical cases definitely lifelong.


Secondary prevention: specifics

PENCILLIN

Secondary prophylaxis also reduces the severity of

RHD.

It is associated with regression of heart disease in

approximately 50-70% of those with good

adherence over a decade and reduces mortality.

Route:

BPG is most effective when given as a deep

intramuscular injection.

Secondary prevention: Adherence

• Use a 23-gauge needle- deeper is better

• Local pressure to area for 10 seconds

• Warm syringe to room temperature

• First allow alcohol to dry.

How can we reduce the pain associated with

IM Penicillin?

• Deliver injection very slowly(over 2-3mins)

• Distraction techniques

• Good rapport with the case, is a significant aid to

injection comfort, compliance and understanding.

• Can Use 0.5-1ml of 1% lignocaine. Reduces

pain significantly and excellent for younger

patients.

Secondary prevention: Adherence

Ensuring that patients understand their disease, are informed regarding their future and receive secondary prophylaxis

EDUCATIONHealth education is critical at all levels

Lack of parental awareness of the causes and consequences of

ARF/RHD is a key contributor to poor adherence amongst children on

long-term prophylaxis.

!!! THANK YOU !!!

Healthcare

Rheumatic valvular heart disease pediatrics AG