Rheumatic valvular diseases - Dr. S. Srinivasan

Rheumatic
Valvular Heart Disease
S . Srinivasan
Professor of Paediatrics
MGMCRI, Plillayaarkuppam
Puducherry
MGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12th Feb 16

Learning Objectives
• To discuss the common etiologies of
valvular stenosis and regurgitation.
• To recognize the signs and symptoms of
valvular stenosis and regurgitation
• To clinically recognize, identify clinical
features of Rh. Mitral and Aortic Valvular
Diseases and their attendant
complications
• To offer a plan of investigative work up
and interpret a few important findings
• To offer preventive and treatment
modalities recommended in treating and
preventing complications
• To identify & refer children with RHD for
further work up, medical and surgical
management

Rheumatic fever
• Inflammatory autoimmune response
triggered by Group A beta-hem.
streptococcal pharyngitis
• Children & Adolescents
• Poor SE Status, overcrowding, poor
sanitation, developing &
underdeveloped countries

Acute rheumatic Carditis
Pancarditis
ENDOCARDITIS  MV Insufficiency (65 -100%)
 AV Regurgitation (20-30%)
 TV (10% - ass.with MR,AR or both)
MYOCARDITIS  Marked Sinus tachycardia
 S3;
 changing Murmurs ;
 Carey-Coomb’s MDM
Pericarditis Chest pain over the left chest and axilla
Pericardial Rub on auscultation
Rarely affects cardiac function
Rarely results in large effusions or constrictive
pericarditis

PREVALENCE RHD IN INDIA
( ECHO proven)
0.67/1000 to 0.12/1000 children
(Periwal et al Bikaner) 2006
 0.5 per 1000 children
(Misra et al. 2003 -2006)

Genetic studies in RF
Progression to Chronic RHD
Strong correlation to:
HLA antigen DR class II alleles
Inflammatory protein-
encoding genes MBL2 and
TNFA
Genes

Chronic Rheumatic Heart Disease
in Children & Young Adults
Worldwide occurrence of RHD
Estimated
occurrence
5-30 million Children
& Young Adults
New Cases 2.5 - 3 Lakhs of RHD
added every year
Deaths
attributed to
Chronic RHD
90 Thousands – 2.5
Lakhs / Year

Rheumatic heart disease
Valvular Stenosis
and/or Insufficiency
( Regurgitation)
( structural damage due to
fibrosis, thickening,
shortening and fusion
of valvular cusps and
apparatus )
Post Infective ( GpA-ᵦ hemolytic Strep) Autoimmune mediated
Cardiac inflammation and scarring
Pancarditis
-Myocarditis
-Endocarditis &
-Pericarditis)
Acute RF Chronic RH Disease

RHD
• More severe in females than in males
• Mitral Insufficiency of Ac.RF resolves
in 60-80% of patients who adhere to
antibiotic prophylaxis
• Aortic Regurgitation in 20-30 % of ARF
persists in spite of strict adherence to
Secondary Rheumatic Prophylaxis

Rheumatic ( Valvular )
Heart Disease
 Permanent structural alterations to
heart valve cusps and supporting
structures caused by a single or more
attacks of ARF.
 RHD.: 40-60% of children with ARF
 Valves involved :
-- Mitral > Aortic > TV>PV
-- MR(MI) > MR+MS > MS
-- MR+AR > MS+AR > AR
-- Rt. Heart Valves in RHD : Rare
-- AS of Rheumatic etiology : Uncommon
-- ( seen beyond Adolescent age group )

Etiology
Pathophysiology
History & Physical Exam
Natural History
Investigative Evaluation
Complications
Treatment
 Prevention

Mitral Regurgitation

Most commonly observed Murmurs
of ARF
Aortic
Regurgitation
High-pitched, blowing,
decrescendo, early diastolic murmur
of Aortic Regurgitation ,heard best
along the right upper and mid-left
sternal border after deep expiration
while the patient is leaning forward.
 Apical high-pitched,
blowing-quality murmur
pansystolic murmur
radiating to the left
axilla
MV insufficiency
◦ Apical diastolic murmur (also known as a Carey-
Coombs murmur) in active carditis and
accompanies severe mitral insufficiency

Mild (physiological) MR:
80% of normal individuals
Definition
Backflow of blood from the
LV to the LA during systole

Chronic Mitral Regurgitation
Age Group Etiologies
YOUNG  Rheumatic
 MVP
 Her. Connective Tissue Disorders
 Ac. Collagen Vascular Disorders
Elderly  Rheumatic heart disease
 Myxomatous degeneration
(MVP)
 Ischemic MR
 Infective Endocarditis

Pathophysiology
Volume Overload
Compensatory
Mechanisms
LA enlargement
LVH
Increased contractility
Progressively
increasing VO in
Chronic MR
 Progressive LA dilation
 Pulm.Arterial Hypertension
 RV Dysfunction
 Progressive LV volume
overload
 LV dilationMGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12th Feb 16

Physical Exam findings
Symptoms Exertion Dyspnea (exercise Intolerance)
- Worsening with severity
 Palpitation ( more marked in
combined MS &MR)
Signs Pulse : NV/HV/LV; Irregularly irregular in
AF
Precardial prominence, LV type of APEX
Auscultation
Heart Sounds soft S1; Loud S2 in PAH;
S3 (CHF/LA overload)
Murmurs Holosystolic murmur at

Rheumatic Mitral Regurgitation:
Natural History
Compensatory
phase
10-15 years
Asymptomatic
severe MR
5%/year mortality rate
Severe
Symptomatic MR
Sharp Rise in mortality
rate
MR with EF <60%
Cause of
Mortality
Progressive CHF,
Complications of MR like
Arrhythmias, EmbolismMGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12th Feb 16

Mitral Regurgitation : imaging studies
Chest
X-Ray
LA enlargement, Central
Pulmonary Artery enlargement
ECG To look for : LA enlargement,
Atrial Arrhythmias like Atrial
flutter, fibrillation and LVH
ECHO To estimate LA, LV size and
function
To assess valve structure
TEE inconclusive transthoracic ECHO

Mitral Regurgitation-
Medical Management
1. Hydralazine & other Vasodilators
2. -blockers, CCB, digoxin : To
control Heart Rate in atrial
fibrillation with MR
3. Anticoagulants in atrial fibrillation
and flutter
4. Diuretics for fluid overloadMGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12th Feb 16

Management of MR
• Serial Echocardiography:
– Mild: 2-3 years
– Moderate: 1-2 years
– Severe: 6-12 months

IE prophylaxis
 Dental procedures
 Prosthetic valves

MR: Surgical Indications

MR: Important Indications for
MV Replacement
 Symptomatic Severe MR
• Any cardiac related Symptoms at
rest or exercise with (repair if
feasible)
 Asymptomatic MR
EF <60%
New onset Atrial Fibrillation

Mitral Stenosis

Mitral Stenosis
• Recurrent episodes: Progressive Valvular
Damage
• Residual and progressive valve deformity
• 10-40% of older children 2-10 years after
previous ARF with MR
• Fusion of the valve apparatus (at the level
of the valve commissures, cusps, chordal
attachments, or any combination of these )
resulting in stenosis or a combination of
stenosis and insufficiency
99% of MS in adults:
Rheumatic etiology

Mitral Stenosis
Definition
LV inflow Obstruction:
 Impaired LV diastolic filling
Normal MV Area : 4-6 cm2
Basic Facts
Onset of
Symptoms
MV Area < 2 cm2 With Increasing
Transmitral gradients
Predominant
Cause
Rheumatic HD
Prevalence and
incidence
Decreasing due to a
reduction of RHD

Etiology of Mitral Stenosis
Rheumatic
heart disease
77-99% of all
cases
Mitral annular
calcification
2.7%
Infective
endocarditis
3.3%

Mitral Stenosis
Pathophysiology
Cause Physical Symptoms & Signs
impaired LA
emptying resulting in
Increasing PV
Pressue in capillaries
Progressive Dyspnea (70%)
due to pulmonary congestion
 Palpitations
(worsening with exercise,
fever, tachycardia, and
pregnancy)
Increasing
Transmitral
Pressures
Haempotysis, Progressive
Dyspnea , PND, Pedal Oedema,
Increased JVP, Hepatomegaly
LA enlargement; LA
Dilatation
Pulmonary venousMGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12th Feb 16

Natural History of MS
• Disease of plateaus:
– Mild MS: 1 - 10 years after initial
ARF
– Moderate: 5 -10 years later
– Severe: Beyond 10 years
Mortality Pulmonary Oedema
Infections-BE,LRI, and
Thromboembolism
Pulmonary EmbolismMGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12th Feb 16

Physical Exam Findings of MS
JVP prominent "a" wave
Signs of right-sided
heart failure
in advanced disease
Mitral facies Severe MS &
Cachexia with
GRetdn

• Loud S1
• Opening S1 snap: Apex when leaflets
are still mobile
Due to the abrupt halt in leaflet motion
in early diastole, after rapid initial
rapid opening, due to fusion at the
leaflet tips
 Shorter the S2 -OS interval, severer
the MS
Heart Sounds in MS

Low-pitched Diastolic Rumble
 Most Prominent at the Apex
 Best heard in the Left lateral
position
 Bell of the steth
Expiration
 Mild Exercise when in doubt
Heart Murmur in Mitral Stenosis
MidDiastolic Murmur

Evaluation of MS
CXR LA enlargement
Pulmonary congestion
Signs of PAH
ECG LA enlargement
Atrial Fibrillation
ECHO: GOLD STANDARD
To
Assess
MV Leaflet mobility
Gradient
MV Area
Nature of damage

Complications of Chronic RHD
(Established Valvular disease )
1. heart failure from valve
insufficiency (acute rheumatic
carditis)
2. Atrial Arrhythmias ( rare in
children)
3. Pulmonary Edema
4. Recurrent Pulmonary Emboli
5. Infective Endocarditis
6. Intracardiac Thrombus Formation
7. Systemic Emboli.

Management of MS
Serial echocardiography
-blockers, CCBs, Digoxin
which control heart rate and
hence prolong diastole for
improved diastolic filling
Duiretics for fluid overload
Mild: 3-5 Years
Moderate:1-2 Years
Severe: Yearly
Medications
Medical therapy does not prevent
progression as MS is a mechanical problem
and

Management of MS
• Identify patient early who might
benefit from percutaneous mitral
balloon valvotomy
 REMEMBER to Implement :
 IE Prophylaxis & Secondary RHD
Prophylaxis

Simplified Indications for Mitral
valve replacement
• ANY SYMPTOMATIC Patient with
NYHA Class III or IV Symptoms
• Asymptomatic moderate or
Severe MS with a pliable valve
suitable for PMBV

Aortic Regurgitation

Etiology of
Aortic Regurgitation
• Physical Findings:
Wide pulse pressure
Diastolic murmur
Florid pulmonary edema
Acute
ARF( 20-30% in children )
Endocarditis
Aortic Dissection
Chronic AR
Bicuspid aortic valve
Rheumatic
Infective endocarditis
Collagen Vascular Disorders

Pathophysiology of AR
• Combined pressure & volume
overload
• Compensatory Mechanisms
• LV dilation, LVH
• Progressive dilation
• Heart Failure

Natural History of AR
• Asymptomatic until 4th or 5th decade
• Rate of Progression: 4-6% per year
• Progressive Symptoms include:
- Dyspnea: exertional, orthopnea, and
paroxsymal nocturnal dyspnea
- Nocturnal angina: due to slowing of
heart rate and reduction of diastolic
blood pressure
- Palpitations: due to increased force of
contraction

Physical Exam findings of AR
• Wide pulse pressure: most sensitive
• Hyperdynamic and displaced apical
impulse
• Auscultation-
– Diastolic blowing murmur at the left
sternal border
– Austin flint murmur (apex): Regurgitant
jet impinges on anterior MVL causing it
to vibrate
– Systolic ejection murmur: due to
increased flow across the aortic valve

Evaluation of AR
CXR enlarged cardiac silhouette
and aortic root enlargement
ECHO Evaluation of the AV and
aortic root with
measurements of LV
dimensions and function
(cornerstone for decision
making and follow up
evaluation)
AortographyMGMCRI- 8th&9th Semesters MBBS-UG PEDIATRICS Theory Lecture 12th Feb 16

Management of AR
• General: IE prophylaxis in dental
procedures with a prosthetic AV
or history of endocarditis.
• Medical: Vasodilators (ACEI’s),
Nifedipine improve stroke volume
and reduce regurgitation only if pt
symptomatic or HTN.
• Serial Echocardiograms: to
monitor progression.
• Surgical Treatment: Definitive Tx

Simplified Indications for
Surgical Treatment of AR
• ANY Symptoms at rest or exercise
• Asymptomatic treatment if:
–EF drops below 50% or LV
becomes dilated

THANK YOU

Rheumatic valvular diseases - Dr. S. Srinivasan

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Rheumatic valvular diseases - Dr. S. Srinivasan