Valvular Heart

Disease
Allan B. Ruales, M.D., FPCP,
FPCC

Aortic Stenosis

Occurs in 25% of all pxs with

chronic VHD
80% of adult pxs with valvular AS
are male
Etiology : congenital (bicuspid),
rheumatic, degenerative

Aortic Stenosis

Rheumatic almost always associated

with rheumatic involvement of mitral
valve. Favored if associated with severe
AR
Degenerative calcific known as senile or
sclerocalcific AS, most common cause in
North America and Western Europe. 30%
of persons >65 years old exhibit aortic
valve sclerosis with systolic murmur but
without obstruction, while 2% exhibit
frank stenosis

Other forms of Left

ventricular outflow tract
obstruction

Hypertrophic cardiomyopathy
subaortic obstruction
Discrete congenital subvalvular
Supravalvular above the valve

Pathophysiology

Systolic pressure gradient between LV &

aorta
If experimental severe obstruction is
produce suddenly LV will dilate with
reduction of stroke volume.
Usually obstruction is gradual, with
maintenance of normal CO by concentric
hypertrophy, a compensatory mechanism
to reduce the systolic stress developed by
the myocardium

Pathophysiology

Large transaortic valvular gradient may

exist for many years without reduction of
CO or LV dilatation until decompensation
occurs
Critical obstruction : gradient >50
mmHg or aortic orifice area <0.5
sqcm/sqm BSA
CO maybe normal at rest even with
severe AS but fail to rise during exercise

Pathophysiology

Late in the course CO & gradient

declines with elevation of LA, PA, and
RV pressure
Hypertrophied LV muscle elevates
oxygen requirement to cause ischemia
with or without coronary arterial
narrowing
AS intensifies severity of
accompanying MR

Symptoms

Rarely symptomatic until valve orifice

is <0.5 sqcm/sqm BSA in adults.
Symptoms usually in 6th to 8th decade
Cardinal symptoms : Exertional
dyspnea, angina pectoris, and syncope
Dyspnea : elevation of pulmonary
capillary pressure caused by LA and
LV diastolic pressure as a result of
decrease LV compliance and dilatation

Symptoms

Angina develops later and reflects

an imbalance between increased
oxygen requirement & reduce
availability
Exertional syncope fixed CO will
cause a decline in arterial pressure
as a result of vasodilation of
exercising muscles

Symptoms

Heart failure symptoms in late

stages
Coexisting MS will mask clinical
findings by AS because the reduced
CO induced by MS will lower the
gradient across the AV.

Physical Findings

Rhythm is usually sinus until late in the

course, AF should suggest associated
mitral valve disease
Systemic BP usually normal but late in
the course, stroke volume declines and
pressure may fall with narrow pulse
pressure
Pulse rises slowly to a delayed
sustained peak, pulsus parvus et tardus

Physical Findings

a wave in JVP is accentuated due to

diminished distensibility of RV cavity
caused by bulging, hypertrophied IVS
LV impulse is hyperdynamic &
displaced laterally
Systolic thrill at base of heart, jugular
notch, and carotid arteries, its absence
suggest a mild AS (except in pxs with
emphysema, thick chest wall, thoracic
deformity, or heart failure

Auscultation

Congenital noncalcific AS (children

& adolescent) early systolic
ejection sound (OS of aortic valve)
Ejection sound disappears when
valve becomes calcified and rigid

Auscultation

S2 can be synchronous or paradoxic

depending on severity. Loud A2 pliable
valves. S4 at apex reflects LV hypertrophy
and elevated LV end-diastolic pressure. S3
occurs when LV dilates
Murmur ejection systolic crescendodecrescendo, low-pitched, rough and
rasping in character, loudest at base, most
commonly at 2nd right intercostal space
radiating to the carotids

Laboratory Examination

ECG LV hypertrophy, its absence

does not exclude severe obstruction.
LA enlargement should suggest
associated mitral valve disease

Laboratory Examination

Chest Xray maybe normal for many

years because the initial response is
concentric LVH. Some rounding of
cardiac apex, critical AS is associated
with post stenotic dilatation of ascending
aorta. Absence of valvular calcification in
an adult suggests that severe valvular AS
is not present. Late stages shows LV
enlargement, pulmonary congestion,
enlargement of LA, PA and RV.

Echocardiography

LV hypertrophy with multiple, bright

echoes within aortic root
(calcification)
LV dilatation & reduced systolic
shortening reflects LV dysfunction
Doppler will estimate transvalvular
gradient
Identify other lesions

Catheterization

Pxs with clinical signs of AS and

symptoms of myocardial ischemia
(suspected with associated CAD)
Pxs with multivalvular disease
Young, asymptomatic pxs with
noncalcific congenital AS to define
severity of obstruction to LV outflow
Pxs with suspected LV outflow in
sub- or supravalvular regions

Natural History

Pxs with severe AS death most

commonly in 7th and 8th decade
Average time to death : angina 3
years, syncope 3 years, dyspnea
2 years, congestive heart failure
1.5 to 2 years

Natural History

>80% who died had symptoms <4

years
CHF cause of death 1/2 to 2/3 of
pxs
Sudden death (presumed
arrhythmia) 10 to 20% at average
age 60 years previously symptomatic

Treatment

Moderate or severe AS periodic

follow-up
Asymptomatic severe AS avoid
strenous activity
Heart failure digitalis glycosides,
sodium restriction, and cautious use
of diuretics (avoid volume depletion
since this may cause reduction of CO)

Surgery

AVR recommended in symptomatic pxs

with critical AS, in asymptomatic pxs with
severe AS exhibiting LV dysfunction
Operative risk for pxs without heart failure :
4%
Long-term postoperative survival
correlates inversely with preoperative LV
dysfunction
Pxs with LV dysfunction has poor prognosis
when treated medically
CABG with AVR for AS with CAD pxs

Surgery

10-year survival 60%

Bioprosthetic valves 30% will fail
in 10 years requiring re-replacement
30% with mechanical valves
develop significant bleeding
complications secondary to use of
anticoagulants

Percutaneous Balloon
Aortic Valvuloplasty

Prefered procedure in children and

young adults with congenital &
noncalcific AS
High restenosis rate in calcific valves
In pxs too ill or frail to undergo surgery
In pxs with life-threatening AS with
advanced extracardiac disease
Bridge to operation pxs with severe
LV dysfunction

Aortic Regurgitation

Etiology primary valve disease or

primary aortic root disease
Primary valve disease
males : with pure or
predominant
valvular AR

Aortic Regurgitation
females : pxs with AR and
associated mitral valve disease
2/3 of pxs : rheumatic in
origin
Other causes : IE,
Prolapse of aortic valve, congenital
fenestration, blunt chest trauma

Etiology

Primary Aortic Root Disease

marked aortic dilatation
Cystic medial necrosis of
ascending aorta in Marfan syndrome
Idiopathic dilatation of aorta
Osteogenesis imperfecta

Etiology
Severe Hypertension
Retrograde dissection of aortic
annulus
Syphilis
Ankylosing rheumatoid
spondylitis

Pathophysiology

Total stroke volume (effective

forward stroke volume + regurgitant
volume) increased
Entire LV stroke volume is ejected
into a high-pressure zone (aorta)
Increased LV end diastolic volume
(increased preload) major
hemodynamic compensation for AR

Pathophysiology

Dilatation of LV eject a larger

stroke volume without increasing
the relative shortening of each
myofibril

Pathophysiology

LV dilatation will increase LV systolic

tension required to develop a level of
systolic pressure Laplaces law
(myocardial wall tension is the
product of intracavitary pressure
and LV radius)

Pathophysiology

As LV function deteriorates enddiastolic volume rises & forward

stroke volume & EF decline
Thickening of LV wall also occurs in
chronic AR autopsy will show large
heart >1 kg
Effective forward CO is normal or
slightly reduced at rest and fails to
rise normally on exertion

Pathophysiology

Signs of LV dysfunction reduction of EF

Advanced stages elevation of LA, PA
wedge, PA, and RV pressures and
lowering of forward CO at rest
Myocardial ischemia elevation of
oxygen requirements by both LV
dilatation & elevated LV systolic tension,
and reduced supply when arterial
pressure is subnormal reducing coronary
perfusion pressure

History

Family history Marfan syndrome

Compatible with IE, or Ankylosing
spondylitis
Asymptomatic for 10 15 years
Early complaint of awareness of heartbeat
on supine position, sinus tachycardia or
PVCs with palpitations and head pounding
during exertion or emotion
Exertional dyspnea first symptom of
diminished cardiac reserve

Other Symptoms

Orthopnea, PND, and excessive

diaphoresis
Chest pain not necessary with CAD
Congestive hepatomegaly and ankle
edema late
Acute, severe AR Pulmonary
edema and/or cardiogenic shock

Physical Findings

Jarring of entire body & bobbing

motion of head with each systole
Abrupt distention & collapse of large
arteries
Detect conditions predisposing to AR
Marfan syndrome, rheumatoid
spondylitis, & VSD

Arterial Pulse

Corrigans pulse water-hammer

pulse, rapidly rising and suddenly
collapsing arterial pulse during late
systole & diastole
Quinckes pulse capillary
pulsations, alternate flushing &
paling of skin at root of nail while
pressure is applied to tip of nail

Arterial Pulse

Traubes sign booming, pistolshot sound heard on auscultation

over femoral arteries
Duroziezs sign to-and-fro murmur
audible if femoral artery is lightly
compressed with stethoscope

Arterial Pulse

Widened pulse pressure elevation

of systolic and depression of
diastolic pressure
Severity of AR is not always directly
correlated with pulse pressure

Palpation

LV impulse heaving & laterally

displaced inferiorly, with systolic
expansion and diastolic retraction of
apex.
Diastolic thrill palpable along left
sternal border

Palpation

Systolic thrill palpable in jugular

notch & transmitted along carotid
arteries : not necessary coexisting
AS but markedly increased blood
flow across aortic orifice
Carotid arterial pulse bisferiens :
two systolic waves

Auscultation

Absent A2, audible S3 with systolic

ejection sound, occasional S4
Murmur high-pitched, blowing,
decrescendo diastolic heard best 3rd
ICS along left sternal border. Brief in
mild AR, becoming louder & longer
as severity increases

Auscultation

Primary valvular AR murmur is

louder at left than right sternal
border
Aneurysmal dilatation of aortic root
louder at right sternal border

Auscultation

Midsystolic ejection murmur heard

best at base & transmitted to carotid
arteries. (Not to be confused with
AS) Higher pitched, shorter, & less
rasping than the murmur of AS

Auscultation

Austin Flint murmur soft, lowpitched, rumbling middiastolic bruit

heard best at apex : produced by
displacement of anterior mitral
leaflet by the AR stream
Murmur of AR intensified by
isometric exercise and reduced by
inhalation of amyl nitrite

Laboratory Examination

ECG normal for mild AR, for severe

chronic AR LV hypertrophy with
strain
Chest X-ray Apex displaced
downward & to the left. Moderately
dilated ascending aorta & knob for
primary valve disease, aneurysmal
dilated aorta for AR cause by primary
disease of aortic wall

Echocardiography

Useful in determining the cause of AR

detect dilatation of aortic annulus,
thickening & failure of leaflet
coaptation
Doppler is very sensitive in detecting
AR and helpful in assessing the severity
Serial study is valuable in evaluating LV
performance and to detect progressive
myocardial dysfunction

Cardiac Catheterization and

Angiography

Accurate confirmation of magnitude

of regurgitation and status of LV
function
Condition of coronary arteries may
be evaluated preoperatively

Treatment

Surgery principal treatment of AR

carried out before developing heart
failure
Timing of operation pxs with
chronic AR usually do not become
symptomatic until after the
development of myocardial
dysfunction, and surgery often does
not restore normal LV function

Surgery

Pxs with severe AR careful clinical

follow-up and noninvasive testing
with echocardiography every 6month intervals to determine the
optimal time of surgery
After the onset of LV dysfunction but
prior to the development of severe
symptoms

Surgery

In asymptomatic pxs with progrssive

LV dysfunction (LVEF<55% or LV
end-systolic volume>55ml/sq m,
55/55 rule)
Repair or AVR with or without aortic
graft mechanical or bioprosthesis
Overall operative mortality for
isolated AVR 4.3%

Acute Aortic
Regurgitation

Most common causes IE, aortic

dissection, trauma
LV has no time to dilate stroke volume
declines and ventricular diastolic
pressure rises markedly, pulse pressure
is often not markedly widened, physical
signs of severe chronic AR is absent.
Mitral valve will close prematurely so
that S1 is soft or absent. Diastolic
murmur of AR is brief.

Acute Aortic
Regurgitation

Pxs will present with pulmonary

congestion & edema with
hypotension
Prompt surgical treatment is
required

Tricuspid Stenosis

Generally rheumatic in origin and

more common in women
Does not occur as an isolated lesion
Usually associated with MS
Significant TS occurs in 5 to 10% of
pxs with severe MS
Commonly associated with some
degree of TR

Pathophysiology

Diastolic pressure gradient between

RA & RV which is augmented when
transvalvular blood flow increases
during inspiration
Gradient >4mmHg will elevate RA
pressure to result in systemic venous
congestion (ascites & edema)

Symptoms

Development of MS precedes TS, pxs

initially have symptoms of
pulmonary congestion (improvement
would raise the possibility that TS is
developing)
Little dyspnea for degree of
hepatomegaly, ascites, and edema
Fatigue secondary to low CO

Physical Findings

Severe TS marked hepatic congestion

resulting in cirrhosis, jaundice,
malnutrition, anasarca, and ascites
Congestive hepatomegaly & splenomegaly
Distended jugular veins with giant a
waves if sinus in rhythm with slow y
descent
OS of tricuspid valve with diastolic
rumbling murmur along left lower sternal
margin and xiphoid process (augmented
during inspiration)

Laboratory Examination

ECG RA enlargement (tall, peaked

P waves in II, and prominent,
upright P in V1
Chest X-ray prominent RA with less
evidence of congestion
Echocardiography thickening of
tricuspid valve. Doppler will
estimate the gradient

Treatment

Intensive salt restriction and diuretics

Surgical relief of TS is carried out at
the time of mitral operation
(moderate to severe TS with gradient
>4 mmHg and orifice < 1.5 to 2 sq
cm)
Repair or replacement (bioprosthetic
valve)

Tricuspid Regurgitation

Most commonly is functional and

secondary to marked dilatation of RV
& tricuspid annulus

Tricuspid Regurgitation
Etiology
RV enlargement secondary to inferior
wall infarct with RV involvement, late stages of
heart failure due to RHD or CHD with severe
pulmonary HPN, IHD, cardiomyopathy, and cor
pulmonale
Organic TR Rheumatic fever, infarction
of RV papillary muscles, tricuspid valve
prolapse, carcinoid heart disease,
endomyocardial fibrosis, IE, & trauma

Clinical Features

Systemic venous congestion & reduction of

CO
Marked hepatomegaly, ascites, pleural
effusions, edema, systolic pulsations of
liver, positive hepatojugular reflux
Prominent RV pulsation along left
parasternal region with a blowing
holosystolic murmur along lower left
sternal margin (intensified during
inspiration and reduced on strain of
Valsalva maneuver)

Laboratory Examination

ECG will show changes characteristic

of lesion responsible for the
enlargement of the RV
Chest X-ray enlargement of RA and
RV
Echocardiography will demonstrate
the RV dilatation and status of the
tricuspid leaflets. Doppler will estimate
the severity of TR & PA pressure

Treatment

Isolated TR with no pulmonary HPN

(e.g. IE or trauma) usually well
tolerated and does not require
operation

Treatment

Treatment of underlying cause of heart

failure usually reduces severity of
functional TR
In pxs with mitral valve disease & TR
secondary to pulmonary HPN & massive
RV enlargement effective surgical
correction of mitral valvular abnormality
will result in lowering of PA pressures and
gradual disappearance of TR, or repair, or
replacement

Pulmonic Valve Disease

Pulmonic valve least affected by

rheumatic fever, and uncommonly the
seat of IE
Pulmonic regurgitation most
common acquired abnormality of
pulmonic valve secondary to dilatation
of pulmonic ring secondary to severe
pulmonary HPN

Pulmonic Regurgitation

Graham Steell murmur highpitched, decrescendo, diastolic

blowing murmur along left sternal
border
Little hemodynamic significance
unless serious pulmonary HPN is
also present

Valve Replacement

Operative results dependent on :

pxs myocardial function &
general medical condition on operation
technical ability of operative
team & quality of postoperative care
durability, hemodynamic
characteristics, and thrombogenecity
of the prosthesis

Late Complications of valve

replacement

Paravalvular leakage
Thromboemboli
Bleeding due to anticoagulants
Mechanical dysfunction of the
prosthesis
IE

Bioprosthetic or Artificial
Mechanical Valve?

Similar considerations mitral or

aortic positions, stenotic,
regurgitant or mixed lesions
Mechanical on lifetime
anticoagulants (hazard for
hemorrhage)

Bioprosthetic or Artificial
Mechanical Valve?

Primary advantage of bioprosthesis

reduced thromboembolic
complication (except for pxs with
chronic AF)

Bioprosthetic or Artificial
Mechanical Valve?

Major disadvantage of bioprosthesis

mechanical deterioration (results
in need to replace in 30% of pxs by
10 years and in 50% by 15 years)

Bioprosthetic or Artificial
Mechanical Valve?

Bioprosthesis is not use in pxs <35

years but useful in pxs >65 years old, in
women who expect to become pregnant,
pxs in whom anticoagulation is
contraindicated
Mechanical prosthesis (St. Jude or other
double-disk tilting valve) preferable in
pxs without contraindications to
anticoagulants and <65 years old