Rheumatic Heart Disease

Melvin Al Joershon R. Esporsado

Clinical Clerk

What is RHD?
ARF and CHD are diseases of
poverty.
Up to 60% of patients with ARF
progress to RHD.
Valvular damage is the hallmark of
rheumatic carditis.

Pathogenesis
Organism factors
M-serotypes (particularly types 1, 3, 5, 6, 14, 18, 19,
24, 27, and 29)
Any strain of group A streptococcus

Host factors
Approximately 36% of any population may be
susceptible to ARF

Immune Response
concept of molecular mimicry

Evaluation of the Patient With

Suspected VHD

All patients with known or suspected VHD

should undergo an initial meticulous
history and physical examination.
because decisions about treatment are based
on the presence or absence of symptoms
patients may not recognize symptoms
because they may have gradually limited their
daily activity levels

Evaluation of the Patient With

Suspected VHD

Electrocardiogram (ECG)
to confirm heart rhythm

Chest X-ray
to assess the presence or absence of
pulmonary congestion and other lung
pathology

Evaluation of the Patient With

Suspected VHD

Transthoracic echocardiogram
recommended in the initial evaluation of patients
with known or suspected VHD to confirm the
diagnosis, establish etiology, determine severity,
assess hemodynamic consequences, determine
prognosis, and evaluate for timing of intervention
recommended in patients with known VHD with
any change in symptoms or physical examination
findings

Evaluation of the Patient With

Suspected VHD
Transthoracic echocardiogram
Periodic monitoring is recommended in asymptomatic
patients with known VHD at intervals depending on valve
lesion, severity, ventricular size, and ventricular function

Exercise testing
reasonable in selected patients with asymptomatic severe
VHD to
1) confirm the absence of symptoms
2) assess the hemodynamic response to exercise
3) determine prognosis

Evaluation of the Patient With

Suspected VHD

Cardiac catheterization
for hemodynamic assessment is
recommended in symptomatic patients
when noninvasive tests are inconclusive or
when there is a discrepancy between the
findings on noninvasive testing and physical
examination regarding severity of the valve
lesion

Indication for Intervention

1. The presence or absence of symptoms
2. The severity of VHD
3. The response of the left and/or right ventricle to
the volume or pressure overload caused by VHD
4. The effect on the pulmonary or systemic
circulation
5. A change in heart rhythm

Definitions of Severity of Valve Disease

Stage

Definition

Description

At risk

Patients with risk factors for the development of VHD

Progressive

Asymptomatic
severe

Patients with progressive VHD (mild-to-moderate

severity and asymptomatic)
Asymptomatic patients who have reached the criteria
for severe VHD
C1: Asymptomatic patients with severe VHD in
whom the left or right ventricle remains
compensated
C2: Asymptomatic patients who have severe VHD,
with decompensation of the left or right ventricle

Symptomatic
severe

Patients who have developed symptoms as a result of

VHD

Basic Principles in Medical Therapy

All patients being evaluated for VHD should
also undergo GDMT for other risk factors
associated with cardiac disease
hypertension, diabetes mellitus, and
hyperlipidemia

Most patients with LV systolic dysfunction

and severe VHD should undergo intervention
for the valve itself

Secondary Prevention of Rheumatic

Fever: Recommendation
Recurrent rheumatic fever is associated
with a worsening of rheumatic heart
disease
However, infection with group A
Streptococcus does not have to be
symptomatic to trigger a recurrence, and
rheumatic fever can recur even when the
symptomatic infection is treated

Secondary Prevention of Rheumatic

Fever: Recommendation
Prevention of recurrent rheumatic fever
requires longterm antimicrobial prophylaxis
In patients with documented VHD, the
duration of rheumatic fever prophylaxis
should be at least 10 years or until the
patient is 40 years of age (whichever is
longer)

Secondary Prevention of Rheumatic Fever

Duration of Secondary Prophylaxis for

Rheumatic Fever

Frequency of Echocardiograms in
Asymptomatic Patients With VHD and
Normal Left Ventricular Function
Stage
Stage
Progressive
(stage B)

Valve Lesion
Aortic Stenosis
Every 35 y
(mild severity
Vmax 2.02.9 m/s)

Every 12 y
(moderate severity
Vmax 3.03.9 m/s)

Severe
(stage C)

Every 1 y
(Vmax 4 m/s)

Aortic
Regurgitation
Every 3-5 y (mild

Every 35 y

severity)

(MVA >1.5 cm )

Mitral Stenosis

Mitral Regurgitation
Every 35 y

(mild severity)

Every 1-2 y

Every 12 y

(moderate severity)

(moderate severity)

Every 1 y
Dilating LVmore
frequent

Every 12 y
(MVA 1.01.5 cm2)

Every 1 y
(MVA <1 cm2)

Every 6 months to
1y
Dilating LVmore
frequent

AORTIC STENOSIS

Stages of Valvular AS
Each stage is defined by:
valve anatomy
valve hemodynamics
consequences of valve obstruction on
the left ventricle and vasculature
patient symptoms

Stages of Valvular AS
A At risk of AS
B With progressive hemodynamic
obstruction
C Severe asymptomatic
D Symptomatic AS

Stages of Valvular AS
Stag Definition
Valve Anatomy
Symptoms
e
None
A
At risk of AS Bicuspid aortic valve (or
other congenital valve
anomaly)
Aortic valve sclerosis
None
B
Progressive Mild-to-moderate leaflet
AS
calcification of a bicuspid or
trileaflet valve with some
reduction in systolic motion
or
Rheumatic valve changes
with commissural fusion

Stages of Valvular AS
Stag Definition
Valve Anatomy
e
C Asymptomatic severe AS
C1
Asymptoma Severe leaflet calcification
tic severe
or congenital stenosis with
AS
severely reduced leaflet
opening

C2

Asymptoma
tic severe
AS with LV
dysfunction

Severe leaflet calcification

or congenital stenosis with
severely reduced leaflet
opening

Symptoms

Noneexercise
testing is
reasonable to
confirm
symptom
status
None

Stages of Valvular AS
Stage Definition
D Symptomatic severe AS
D1
Symptomatic
severe highgradient AS

Severe

D2

Severe

D3

Symptomatic
severe lowflow/low-gradient
AS with reduced
LVEF
Symptomatic
severe lowgradient AS with
normal LVEF or
paradoxical lowflow severe AS

Valve Anatomy
leaflet calcification or
congenital stenosis with severely
reduced leaflet opening

leaflet calcification with

severely reduced leaflet motion

Severe

leaflet calcification with

severely reduced leaflet motion

Symptoms
Exertional

dyspnea
or decreased
exercise tolerance
Exertional angina
Exertional syncope or
presyncope
HF,
Angina,
Syncope or
presyncope
HF,
Angina,
Syncope

or
presyncope

Aortic Stenosis: Medical

Therapy

Hypertension in patients at risk for developing AS (stage A)

and in patients with asymptomatic AS (stages B and C)
should be treated according to standard GDMT, started at a
low dose, and gradually titrated upward as needed with
frequent clinical monitoring
Vasodilator therapy may be reasonable if used with invasive
hemodynamic monitoring in the acute management of
patients with severe decompensated AS (stage D) with New
York Heart Association (NYHA) class IV heart failure (HF)
symptoms
Statin therapy is not indicated for prevention of
hemodynamic progression of AS in patients with mild-to-

AORTIC REGURGITATION

Acute Aortic Regurgitation

may result from:
abnormalities of the valve (primarily IE), or
abnormalities of the aorta (primarily aortic dissection)
iatrogenic complications (percutaneous aortic balloon
dilation or TAVR or following blunt chest trauma)

usually results in severe pulmonary congestion

as well as a low forward cardiac output.

Intervention in Acute AR
If resulting from IE or aortic dissection, surgery
should not be delayed
especially if there is hypotension, pulmonary edema,
or evidence of low flow

Beta blockers are often used in treating aortic

dissection.
use cautiously for other causes block the
compensatory tachycardia and could precipitate a
marked reduction in BP.

Chronic Aortic Regurgitation

Caused by:
bicuspid aortic valve and calcific valve
disease
arises from primary diseases causing dilation
of the ascending aorta or the sinuses of
Valsalva
rheumatic heart disease (the leading cause in
many developing countries)

Stages of Chronic AR
Each stage is defined by:
valve anatomy
valve hemodynamics
severity of LV dilation
LV systolic function
patient symptoms

Stages of Chronic AR

A At risk of AR
B Mild-to-moderate AR
C Severe asymptomatic
D Symptomatic AR

Stages of Chronic AR
Stag
e
A

Definition
At risk of
AR

Valve Anatomy

Progressive
AR

Bicuspid aortic valve (or other

congenital valve anomaly)
Aortic valve sclerosis
Diseases of the aortic sinuses or
ascending aorta
History of rheumatic fever or known
rheumatic heart disease
IE
Mild-to-moderate calcification of a
trileaflet valve bicuspid aortic valve (or
other congenital valve anomaly)
Dilated aortic sinuses
Rheumatic valve changes
Previous IE

Symptoms

None

None

Stages of Chronic AR
Stag Definition
Valve Anatomy
e
C
Asymptomati Calcific aortic valve disease
c severe AR Bicuspid valve (or other congenital
abnormality)
Dilated aortic sinuses or ascending
aorta
Rheumatic valve changes
IE with abnormal leaflet closure or
perforation
D
Symptomatic Calcific valve disease
Bicuspid valve (or other congenital
severe AR
abnormality)
Dilated aortic sinuses or ascending
aorta
Rheumatic valve changes
Previous IE with abnormal leaflet

Symptoms

None; exercise
testing is
reasonable to
confirm
symptom
status

Exertional
dyspnea or
angina, or
more severe
HF symptoms

BICUSPID AORTIC VALVE &

AORTOPATHY

Bicuspid Aortic Valve

Most patients with a bicuspid aortic valve will
develop AS or AR over their lifetime
TTE provides good quality images of the aortic
sinuses with accurate diameter measurements.
In 20% to 30% of patients with bicuspid valves,
other family members also have bicuspid valve
disease and/or an associated aortopathy.

Medical Management of BAV

No proven drug therapies that have been shown to
reduce the rate of progression of aortic dilation in
patients with aortopathy associated with bicuspid
aortic valve.
In hypertensive patients, control of BP with any
effective antihypertensive medication is warranted.
Beta blockers and ARBs have conceptual
advantages to reduce rate of progression but have
not been shown to be beneficial in clinical studies.

MITRAL STENOSIS

Stages of Mitral Stenosis

Each stage is defined by:
valve anatomy
valve hemodynamics,
the consequences of valve obstruction on
the left atrium (LA) and pulmonary
circulation
patient symptoms

Stages of Mitral Stenosis

A At risk of AR
B With progressive hemodynamic
obstruction
C Severe asymptomatic
D Symptomatic MS

Stages of Mitral Stenosis

Stag Definitio Valve Anatomy
e
n
A
At risk of Mild valve doming during
MS
diastole
B

Progress Rheumatic valve changes with

ive MS
commissural fusion and
diastolic doming of the mitral
valve leaflets
Planimetered MVA >1.5 cm2

Symptom
s
None

None

Stages of Mitral Stenosis

Sta
ge
C

Definition

Valve Anatomy

Asymptom Rheumatic valve changes

atic
with commissural fusion and
severe MS diastolic doming of the mitral
valve leaflets
Planimetered MVA 1.5 cm2
(MVA 1 cm2 with very severe
MS)
Symptoma Rheumatic valve changes
tic severe
with commissural fusion and

Symptom
s
None

None

Rheumatic Mitral Stenosis

Suspicion for Rheumatic MS:
a childhood history of rheumatic fever
a characteristic auscultatory finding of an
opening snap after the second heart sound
and subsequent apical diastolic murmur
(with nonspecific complaints of exertional
dyspnea with an unrevealing physical
examination)

Rheumatic MS
Recommendations

Transthoracic echocardiography is indicated in

patients with signs or symptoms of MS to
establish the diagnosis, quantify hemodynamic
severity (mean pressure gradient, mitral valve
area, and pulmonary artery pressure), assess
concomitant valvular lesions, and demonstrate
valve morphology (to determine suitability for
mitral commissurotomy)

Rheumatic MS
Recommendations

Concomitant MR should be sought and quantified

as recommended, along with other valve lesions.
Repeat TTE if with change in symptoms
progressive narrowing of the mitral valve
worsening of concomitant MR or other valve lesions
a change in hemodynamic state due to such factors as
AF, fever, anemia, hyperthyroidism, or postoperative
state.

Medical Therapy
Anticoagulation with Vitamin K antagonists (or
heparin) has long been recommended for
patients with MS with AF or prior embolism
Long-term anticoagulation: controversial (in
patients with normal sinus rhythm on the basis of
left atrial enlargement or spontaneous contrast
on TEE)
Novel oral anticoagulant agents: efficacy has not
been studied in patients with MS

MITRAL REGURGITATION

Acute MR
Acute MR may be due to disruption of
different parts of the mitral valve
apparatus
Infective endocarditis leaflet perforation or
chordal rupture
Spontaneous chordal rupture degenerative
mitral valve disease
Rupture of the papillary muscle acute STsegment elevation MI usually associated with

Acute MR
Diagnosis of the presence and etiology of
acute MR and urgent intervention may be
lifesaving.
TTE is useful in patients with severe acute
primary MR for evaluation:

LV function
RV function
Pulmonary artery pressure
Mechanism of MR

Treatment in Acute MR
Vasodilator therapy can be useful to improve
hemodynamic compensation in acute MR
reduction of impedance of aortic flow decreasing MR
while simultaneously increasing forward output

Intra-aortic balloon counterpulsation

temporizing measure for achieving hemodynamic
stability until definitive mitral surgery can be performed

Prompt mitral valve surgery

recommended for symptomatic acute severe primary MR

Chronic MR
Chronic primary (degenerative) MR vs. chronic
secondary (functional) MR
In chronic primary MR
the pathology of 1 of the components of the valve
(leaflets, chordae tendineae, papillary muscles, annulus)
causes valve incompetence

In chronic secondary MR
the mitral valve is usually normal. Instead, severe LV
dysfunction is caused either by CAD, related MI
(ischemic chronic secondary MR), or idiopathic
myocardial disease (nonischemic chronic secondary MR)

Intervention for Chronic MR

In chronic primary MR
Intervention for patients with primary MR
consists of either surgical mitral valve repair or
mitral valve replacement

In chronic secondary MR
Standard GDMT for HF forms the mainstay of
therapy
Diuretics, beta blockers, ACE inhibition or
ARBs, and aldosterone antagonists

TRICUSPID VALVE DISEASE

Tricuspid Valve Disease

Primary disorders include:
rheumatic disease, prolapse, congenital disease
(Ebsteins), IE, radiation, carcinoid, blunt chest wall
trauma, RV endomyocardial biopsyrelated trauma, and
intra-annular RV pacemaker or implantable cardioverterdefibrillator leads

Approximately 80% of cases of significant TR are

functional in nature
related to tricuspid annular dilation and leaflet tethering
in the setting of RV remodeling due to pressure and/or
volume overload

Stages of Tricuspid
Regurgitation

Stage
A

Definition
At risk of TR

Progressive
TR

Valve Anatomy
Symptoms

Primary
None or in
relation to
Mild rheumatic change
other left
Mild prolapse
heart or
Other (e.g., IE with vegetation, early
carcinoid deposition, radiation)
pulmonary/
pulmonary
Intra-annular RV pacemaker or ICD lead
vascular
Postcardiac transplant (biopsy-related)
Functional
disease
Normal
Early annular dilation

Primary
None or in
relation to
Progressive leaflet deterioration/ destruction
other left
Moderate-to-severe prolapse, limited
chordal rupture
heart or
Functional
pulmonary/
pulmonary
Early annular dilation
vascular
Moderate leaflet tethering
disease

Stag
e
C

Stages of Tricuspid
Regurgitation

Definition

Valve Anatomy

Symptoms

Asymptoma Primary
tic, severe Flail or grossly distorted leaflets
TR
Functional
Severe annular dilation (>40 mm or 21
mm/m2)
Marked leaflet tethering

Symptomat Primary
ic severe
Flail or grossly distorted leaflets
TR
Functional
Severe annular dilation (>40 mm or
>21 mm/m2)
Marked leaflet tethering

None, or in
relation to
other left
heart or
pulmonary/
pulmonary
vascular
disease
None or in
relation to
other left
heart or
pulmonary/
pulmonary
vascular

Tricuspid Regurgitation: Medical Therapy

Recommendations
Diuretics can be useful for patients with severe
TR and signs of right-sided HF (stage D)
Medical therapies to reduce elevated pulmonary
artery pressures and/or pulmonary vascular
resistance might be considered in patients with
severe functional TR (stages C and D)

COR

LOE

IIa

IIb

Tricuspid Regurgitation: Intervention

Recommendations
Tricuspid valve surgery is recommended for
patients with severe TR (stages C and D)
undergoing left-sided valve surgery
Tricuspid valve repair can be beneficial for
patients with mild, moderate, or greater
functional TR (stage B) at the time of left-sided
valve surgery with either 1) tricuspid annular
dilation or 2) prior evidence of right HF
Tricuspid valve surgery can be beneficial for
patients with symptoms due to severe primary
TR that are unresponsive to medical therapy
(stage D)

COR

LOE

IIa

IIa

Stages of Tricuspid Stenosis

Stag
e
C, D

Definitio
Valve Anatomy
n
Severe TS Thickened, distorted, calcified
leaflets

Symptoms

None or
variable
and
dependent
on severity
of
associated
valve
disease
and degree

Tricuspid Stenosis: Diagnosis and

Follow-Up
Recommendations
TTE is indicated in patients with TS to assess the
anatomy of the valve complex, evaluate severity
of stenosis, and characterize any associated
regurgitation and/or left-sided valve disease
Invasive hemodynamic assessment of severity of
TS may be considered in symptomatic patients
when clinical and noninvasive data are discordant

COR

LOE

IIb

Tricuspid Stenosis: Intervention

Recommendations
Tricuspid valve surgery is recommended for
patients with severe TS at the time of operation
for left-sided valve disease
Tricuspid valve surgery is recommended for
patients with isolated, symptomatic severe TS
Percutaneous balloon tricuspid commissurotomy
might be considered in patients with isolated,
symptomatic severe TS without accompanying TR

COR

LOE

IIb

PULMONIC VALVE DISEASE

Pulmonic Valve Disease

The pulmonic valve is rarely involved by IE or
rheumatic disease but is susceptible to
carcinoid accretion
Surgery is considered when symptoms or
signs of RV dysfunction have intervened and
PR is severe.
Treatment should focus on the cause(s) of
elevated pulmonary artery pressures.

Stages of Pulmonic
Regurgitation
Stag
e

Definitio
n

Valve Anatomy

Symptoms

C, D

Severe
PR

Distorted or absent leaflets,

annular dilation

None or
variable
and
dependen
t on
cause of
PR and RV
function

Stages of Pulmonic Stenosis

Stag
e
C, D

Definitio
Valve Anatomy
n
Severe PS Thickened, distorted, possibly
calcified leaflets with systolic
doming and/or reduced excursion
Other anatomic abnormalities
may be present, such as
narrowed RVOT

Symptoms

None or
variable
and
dependent
on severity
of
obstruction

THANK YOU!