MCardiology

Clinical Features of Valvular

Heart Disease
P r o f . D r. Wa n A z m a n Wa n A h m a d
Department of Medicine

Mei 2020
 Goals and Objectives
• Discuss the common etiologies of
valvular stenosis and regurgitation.
• Recognize the signs and symptoms of
severe valvular stenosis and regurgitation
• How to evaluate patient with valvular
heart disease
• Principle of management of patient with
valvular heart disease
 Overview
• Aortic valve Disease
 Aortic Stenosis
 Aortic Regurgitation
• Mitral Valve Disease
 Mitral Stenosis
 Mitral Regurgitation
• Triscupid Valve Disease
 Tricuspid Stenosis
 Triscuspid Regurgitation
• Pulmonary Valve Disease
 Pulmonary Stenosis
 Pulmonary Regurgitation
 Aortic Stenosis
• Aortic stenosis is narrowing of the aortic valve
orifice

• It is a disease in which there is progressive

obstruction to LV outflow which results
 ↑ afterload
 ↓ systemic and coronary blood flow
 Progressive hypertrophy of the Left ventricle
 Aortic sclerosis
is defined as irregular valve thickening without
obstruction to LV outflow

 Present in about 25% of adults over 65 years of age

 Associated with clinical factors such as age, sex,

hypertension, smoking, serum LDL and lipoprotein (a)
levels and diabetes mellitus

 The presence of aortic sclerosis in subjects without

known coronary artery disease is associated with
adverse clinical outcome – 50% increase in Myocardial
Infarction and Cardiovascular death.
 Aortic valve (AV)
• Normal adult AV area is 3.0 to 4.0 cm2
• When AV is ↓ to ¼ its normal size,
significant hemodynamic changes occur

 Severe: AV area less than 1 cm2

 Moderate: AV area 1.0 to 1.5 cm2
 Mild: AV area 1.5 to 3 cm2
AS : Site of narrowing & Causes
• Supravalvular ( Williams’ Syndrome)
• Subvalvular ( Congenital Membrane)
• Valvular
 Congenital (1 - 30 years old)
 Biscuspid ( 40 - 60 years old)
 Rheumatic ( 40 - 60 years old)
 Senile degenerative (>70 years old)

•
 AS - Causes
• Patients presenting with signs and symptoms of AS but with a
normal AV on echo the cause of stenosis could be
supravalvular or subvalvular.

• Congenital malformation of the valve may result in stenosis

in young adult.

• Calcific degerative AS is an active disease process

characterized by lipid accumulation, inflammations and
calcification with many similarities to atherosclerosis.

• Rheumatic AS due to fusion of the commisures with scarring

and eventual calcification of the cusps, usually associated with
MV disease and is less common now
 AS : Natural history
• Prolonged latent period with very low
morbidity and mortality

• Rate of progression
  of AVA 0.12 cm2 per year
  Systolic Pressure Gradient by
10 - 15mm Hg per year
 Clinical Features of AS
• Most patients are asymptomatic in the early
stages of the disease
• Classic Symptoms
 Syncope: (exertional)
 Angina: (increased myocardial oxygen
demand; demand/supply mismatch)
 Dyspnea: on exertion due to heart failure
(systolic and diastolic)
• Sudden death
 Physical Findings in Aortic Stenosis
• Slow rising carotid pulse (pulsus tardus) &
decreased pulse amplitude (pulsus parvus)
[low volume]

• Heart sounds- soft and split second heart

sound, S4 gallop due to LVH.

• Ejection systolic murmur that is loudest in the

aortic area and radiates to the carotids –
 The murmur cresendo-decrescendo
character.
 It’s the length not the intensity that correlates
with severity
 Initial Investigation
ECG:
• Left ventricular hypertrophy and strain.
• If Atrial fibrillation is presence concomitant
MV disease must be suspected

CXR:
• LV predominance with dilatation of the
ascending aorta
• Calcification of aortic valve
 Echocardiography

• Diagnosing and assessment of AS severity

• Assessment of LV wall thickness, size and
function
• Reevaluation of patients with AS and changing
symptoms or sign
SAX in a pt. with AS due to sclerodegenerative disease of the aortic annulus
and valve. Note that in this systolic frame, the right, left, and noncoronary cusps
are almost immobilized, leaving a slit-like opening at the commissures between
the three cusps.
SAX in a pt. with AS secondary to rheumatic fever. Note the thickening
of the edges of the aortic cusps and fusion at the commissures.
LAX showing a systolic frame. Note the doming of the aortic valve
in patient with congenital AS
Biscupid valve in Systole
 Management
• Therapeutic decision for Intervention is based on
symptoms
• Indications for surgery
 Any SYMPTOMATIC patient with severe AS
(includes symptoms with exercise)
 Any patient with decreasing ejection fraction
 Any patient undergoing CABG with moderate or
severe AS
 Patient with high risk surgery can be consider
Transcatheter Aortic Valve Replacement (TAVI)
 Aortic Regurgitation Overview
• Leakage of blood into LV during diastole due
to ineffective coaptation of the aortic cusps
• It may arise from disease affecting the valve
leflets such as endocarditis or valve mobility
in degenerative calcific and rheumatic disease
• Dilatation of aortic root causes failure of the
valve leaflet coaptation and aortic dissection
causes distruption of the structural support of
the aortic valve
• It can be acute or chronic
 Acute AR
• Causes:
 Endocarditis
 Aortic Dissection

• The ventricle has a limited capacity to adapt to the

sudden volume load
• back pressure rapidly develops into pulmonary oedema

• Physical Findings:
 Wide pulse pressure
 Diastolic murmur
 Florid pulmonary edema
 Major causes of chronic AR
slow insidious LV dilatation and prolonged asymptomatic phase

Leaflet Abnormalities Ao Root or Ascending Aorta Abnormalities

•Rheumatic disease
•Infective endocarditis
•Congenital Abnormality (mostly
biscupid aortic valve)
•Myxomatus degeneration
•Calcific Degenerative
•Systemic lupus erythematosus
•Rheumatoid arthritis
•Ankylosing spondylitis
•Takayasu’s artheritis
•Whipple’s disease
•Crohn’s disease
•Drug induced valvulopathy
Age-related aortic dilatation(idiopathic)
Annuloaortic ectasia
Cystic medial necrosis of the aorta (isolated or Marfan
syndrome
Systemic hypertension
VSD with prolapse of Aortic cusp
Ankylosing spondylitis
Behcet syndrome
Psoriatic arthritis
Reiter’s syndrome
Osteogenesis imperfecta
Ehlers-Danlos syndrome
Aortitis (syphilis, giant-cell arteries)
 Pathophysiology of AR

• Combined pressure AND volume overload

• Compensatory Mechanisms: LV dilation,

LVH. Progressive dilation leads to 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
Ejection systolic murmur: due to increased flow
across the aortic valve
GOOD TO REMEMBER
 Initial Investigation of AR
• ECG: LVH and strain

• CXR: enlarged cardiac silhouette and

aortic root enlargement

• ECHO: Diagnosis and Evaluation of the

AV and aortic root with measurements of
LV dimensions and function (cornerstone
for decision making and follow up
evaluation)
 Aortic Regurgitation

Mild Moderate Severe

 Treatment of Acute AR

• Surgical Emergency:
• Positive inotrope: (eg, dopamine,
dobutamine)
• Vasodilators: (eg, nitroprusside)
• Avoid beta-blockers
• Intra aortic balloon pump is
contraindicated
 Management of Chronic AR

Determinants of survival
• Symptom
• LV systolic function
• LV end-systolic size
 Indication for AVR in severe chronic AR
(future reference)

Indication Class
AVR is indicated for symptomatic patients with severe AR I
irrespective of LV systolic function. (Level of Evidence: B)
AVR is indicated for asymptomatic patients with chronic severe AR I
and LV systolic dysfunction (ejection fraction 0.50 or less) at rest.
(Level of Evidence: B)
AVR is indicated for patients with chronic severe AR while I
undergoing CABG or surgery on the aorta or other heart valves.
(Level of Evidence: C)
AVR is reasonable for asymptomatic patients with severe AR with IIa
normal LV systolic function (ejection fraction greater than 0.50) but
with severe LV dilatation (end-diastolic dimension greater than 75
mm or end-systolic dimension greater than 55 mm).* (Level of
Evidence: B)
 Medical Therapy:Vasodilator Drug
 Chronic therapy in patients with severe AR who have
symptoms as LV dysfunction when surgery is not
recommended because of additional cardiac or non
cardiac factors. 1B

 Short term therapy to improve the haemodynamic profile

of patients with severe HF symptoms and severe LV
dysfunction before proceeding with AVR. IIaC

 Long term therapy in asymptomatic patient with severe

AR who have LV dilatation but normal systolic function
IIbB
 Mitral Stenosis Overview
• Definition: Obstruction of LV inflow that
prevents proper filling during diastole
• Normal MV Area: 4-5 cm2
• Transmitral gradients and symptoms begin
when MVA is less than 2 cm2
• Rheumatic carditis is the predominant
cause
• Prevalence and incidence: decreasing due to
a reduction of rheumatic heart disease.
 MITRAL STENOSIS - Causes

• Rheumatic
50% has no h/o Rheumatic fever.

Rheumatic process
 immobility and thickening of MV leaflet,
 fusion of commissures,
 leaflet calcification and subvalvular fusion

• Congenital
Pathophysiology & Clinical presentation
• Progressive Dyspnea (70%): LA dilation 
pulmonary congestion (reduced emptying)
– worse with exercise, fever, tachycardia,
and pregnancy
• Increased Transmitral Pressures: Leads to
left atrial enlargement and atrial fibrillation.
• Right heart failure symptoms: due to
Pulmonary venous hypertension
• Hemoptysis: due to rupture of bronchial
vessels due to elevated pulmonary pressure
 MS : Natural history
• Long latent period (20-40 years) from the
occurrence of RF to onset of symptoms.
• Once symptoms develop there is another
period of almost a decade before
symptoms become disabiling
• Mortality: Due to progressive pulmonary
congestion, infection, and
thromboembolism.
 Physical Exam Findings of MS

• prominent "a" wave in jugular venous

pulsations: Due to pulmonary hypertension and
right ventricular hypertrophy
• Signs of right-sided heart failure: in advanced
disease
• Mitral facies: When MS is severe and the
cardiac output is diminished, there is
vasoconstriction, resulting in pinkish-purple
patches on the cheeks
 Heart Sounds in MS
• Loud First Heart Sound
• Diastolic murmur:
– Low-pitched diastolic rumble most prominent
at the apex.
– Heard best with the patient lying on the left
side during expiration
– Intensity of the diastolic murmur does not
correlate with the severity of the stenosis
 Heart Sounds in MS

• Loud Opening S1 snap: heard at the apex

when leaflets are still mobile (pliable) 
– Due to the abrupt halt in leaflet motion in
early diastole, after rapid initial opening, due
to fusion at the leaflet tips.
– A shorter S2 to opening snap interval
indicates more severe disease.
 Initial Investigation
• ECG: may show atrial fibrillation and LA
enlargement
• CXR: LA enlargement and pulmonary
congestion. Occasionally calcified MV
• ECHO: The GOLD STANDARD for
diagnosis. Asses mitral valve mobility,
gradient and mitral valve area
 MS : Echo grading system
Based on 2D features
 Leaflet thickening
 Leaflet calcification
 Leaflet mobility
 Subvalvular fusion
Score
1 is the least involvement and
4 the most severe involvement
 MS : The mitral valve echo score
• Is the sum of these 4 features of mitral valve

• Score of ≤ 8 is indicative of a pliable non-

calcified valve and is suitable for percutaneous
balloon commissurotomy

• Score ≥ 10 indicates a calcified fibrotic valve

with subvalvular fusion that may be more
appropriate for mitral valve replacement
MS : Criteria for determining severity

Severity Gradient MVA cm2

mmHg

Mild < 5.0 > 1.5

Moderate 5.0 - 10.0 1.0 - 1.5
Severe > 10.0 < 1.0
Doming of the AMVL and PMVL SAX view at the level of the mitral valve

Apical four-chamber view

The leaflets are totally fused, and
there is marked increase in
echocardiographic densities from
the entire mitral apparatus. This is
caused by deformity, fibrosis, and
calcification.
 Recommendations for Percutaneous
Mitral Balloon Valvotomy
Indication Class

1. Symptomatic patients (NYHA functional Class II, III, or IV),moderate I

or severe MS and mitral valve morphology favorable for
percutaneous balloon valvotomy in the absence of left atrial
thrombus or moderate to severe MR.

2. Asymptomatic patients with moderate or severe MS (mitral valve IIa

area < 1.5cm2)* and valve morphology favorable for percutaneous
balloon valvotomy who have pulmonary hypertension (pulmonary
artery systolic pressure >50 mm Hg at rest or 60 mm Hg with
exercise) in the absence of left atrial thrombus or moderate
to severe MR.

3. Patients with NYHA functional Class III-IV symptoms, moderate or IIa

severe MS (mitral valve area < 1.5 cm2),* and a nonpliable calcified
valve who are at high risk for surgery in the absence of left atrial
thrombus or moderate to severe MR.
Double balloon Inoue balloon
 Recommendation for Mitral Valve
Replacement for Mitral Stenosis

Indication Class

 Mod-severe MS (MVA ≤ 1.5cm2) and NYHA III-IV who I

are not considered candidates for PTMC or MV repair

 Severe MS (MVA ≤ 1.0cm2) and severe PHT (PASP IIa

>60mmHg) with NYHA I-II symptoms who are not
considered candidates for PTMC or MV repair
 Mitral Regurgitation Overview
• Backflow of blood from the LV to the LA
during systole
• Competence of the mitral valve may be
compromised by disease affecting any
component of the mitral apparatus from
the annulus, leflets, chordae and papillary
muscles to the function of LV
• Mild (physiological) MR is seen in 80% of
normal individuals.
 Acute MR
• Causes
 Endocarditis
 Acute MI:
 Malfunction or disruption of prosthetic valve

• In acute severe severe MR, the heart does not

have time to develop compensatory
mechanisms and this often results in cardiogenic
shock and pulmonary oedema
 Etiologies of Chronic MR

• Leaflet abnormality
- Mitral Valve Prolapse (PMVL more common than
AMVL)
- Rheumatic Disease
- Endocarditis
• Congenital – cleft of AMVL
• Annular dilatation from LV dilatation
• Mitral Annulus Calcification
- degenerative disorder most commonly seen in the
elderly
- accelerated by HPT and DM
- chronic renal failure
• Chordal and pappilary muscle abnormality
 Pathophysiology of MR
• Pure Volume Overload
• Compensatory Mechanisms: Left atrial
enlargement, LVH and increased contractility
– Progressive left atrial dilation and right
ventricular dysfunction due to pulmonary
hypertension.
– Progressive left ventricular volume
overload leads to dilation and progressive
heart failure.
 Clinical Features of MR

• Exertion Dyspnea: ( exercise intolerance)

• Heart Failure: May coincide with increased
hemodynamic burden e.g., pregnancy,
infection or atrial fibrillation
• Cardiomegally: by displacement of apex beat
(thrusting quality)
• Auscultation: soft S1 and a holosystolic
murmur at the apex radiating to the axilla
– S3 (CHF/LA overload)
– In chronic MR, the intensity of the murmur
does correlate with the severity
 The Natural History of MR

• Compensatory phase: 10-15 years

• Patients with asymptomatic severe MR
have a 5%/year mortality rate
• Once the patient’s EF becomes <60%
and/or becomes symptomatic, mortality
rises sharply
• Mortality: From progressive dyspnea and
heart failure
 Initial Investigation of MR
• ECG: May Confirm atrial fibrillation, show LV
hypertrophy or previous myocardial infarction

• CXR: LA enlargement, central pulmonary artery

enlargement. Cardiomegally and features of
congestive cardiac failure

• ECHO: Quantify the extend and severity of MR,

Estimation of LA, LV size and function. Valve
structure assessment
Mild MR

Moderate MR
Severe MR Eccentric MR jet
 Treatment of Acute MR

• In the setting of Myocardial infarction:

Coronary angioplasty or thrombolytics
• Balloon Pump
• Emergency Surgery
 Management of chronic MR

• Medications
a) Vasodilator
b) Rate control for atrial fibrillation with -
blockers, CCB, digoxin
c) Anticoagulation in atrial fibrillation and
flutter
d) Diuretics for fluid overload
 Indication for Mitral valve surgery

• Symptomatic patients with chronic severe mitral

regurgitation in the absence of severe LV dysfuntion(EF
less than 0.3 or end systolic dimension > 55mm)

• Asymptomatic patients with chronic severe mitral

regurgitation and mild to moderate LV dysfunction (EF
0.3-0.6)

• When feasible mitral valve repair is prefer over mitral

valve replacement
 Tricuspid Valve Disease
• Triscupid regurgitation is rare. It is usually secondary to
dilatation of right ventricle and tricuspid annulus rather
than intrisic disease of tricuspid valve.
• Symptoms of right sided heart failure predominate with
peripheral oedema and ascites
• On exam;
 Peripheral oedema
 Jaundice from hepatic congestion
 Elevated JVP
 RV heave
 PSM loudest at the 4th parastenal intercostal space
loudest during inspiration
 Pulsatile liver
 Pulmonary valve Disease
• Pulmonary stenosis is predominanly a
congenital disease. Survival into adulthood is
common. O/E ESM loudest over pulmonary area
• Pulmonary regurgitation is rare and occurs
secondary to dilatation of the pulmonary valve
ring from pulmonary hypertension or any other
dilatation of the main pulmonary artery.
Presented with symptom of RV failure and
fatigue. An early diastolic murmur in the left 2nd
to 4th ICS resembling the murmur of AR
Questions