Principle

of
Cardiac Hemodynamic
and
Cardiac Hemodynamic Disturbance
dr Herman Mulijadi MS, SpKP,

Lecturer :
 Principle of cardiac hemodynamic

Principle of cardiac hemodynamic

1. Describe mechanism of cardiac contraction and relaxation
2. Describe pressure and volume changes during the cardiac cycle (
Wigers diagram ) and related herth sound
3. Describe cardiac hemodynamicand body compensation
- venous return
- strokes volume
- cardiac out put
- pre load
- after load
- LV end diastolic and end systolic pressure
- contractility (Frank - starling Law)
 Cardiac hemodynamic disturbance

Cardiac hemodynamic disturbance

1. Describe hemodynamic diturbance and related heart murmur due
to valvular problem
a. aortic stenosis - regurgitation -
b. mitral stenosis -reguritation
c. Pulmonal stenosis - regurgitation
d. Tricuspidalis stenosis - regurgitation

2. Describe hemodynamic disturbance due to myocardial problem

a. Dilated cardiomyophaty
(hypertropic obstructive cardiomyopathy)
b. Restrictive cardiomyopathy
4
INTRODUCTION

Hemodynamic - Blood circulation in

the Human Body

Cardiac Hemodynamic - Blood

circulation in the Heart

Cardiovascular hemo-dynamic :
Blood Circulation to the heart and in
turn the blood circulation regulated by
the heart

Hypertension and congestive

heart failure are two best known
systemic hemodynamic disorder
5
The Blood Vessels and the Cardiovascular System

Heart : as the Pump

Vessels: as the Canal
Blood : as the vehicle
6

CARDIAC FUNCTIONAL
ANATOMY

•Low pressure system

–Right heart
–Pulmonary
•High pressure
system
–Left heart
–Systemic
7
STRUCTURE OF THE HEART AND COURSE OF BLOOD FLOW
THROUGH THE HEARTCHAMBER AND HEART VALVE

NO VALVES BETWEEN THE ATRIA AND VEINS

8
CARDIAC CONDUCTION
8
 Normal rhythmical impulse Review. CARDIAC
is generated in CONDUCTION
S-A node, therefore called the primary
pacemaker.
 Atrial Depolarization
 Internodal pathways conduct impulse
from the sinus node to the
atrioventricular (A-V) node;
 The impulse from the atria is delayed
in A-V node, before passing into the
ventricles;
 Ventricular depolarization
 A-V bundle conducts Impulse from the
atria into ventricles;
 Left and right bundle branches of
purkinje fibers, conduct the cardiac
impulse to all parts of ventricles.
 Atrial repolarizartion
 Ventricular repolarization.
 9
Cardiac Muscle
3 Types of Muscle
1. Skeletal
2. Cardiac
Skeletal and Cardiac Striated Alternating light and dark
bands.
Skeletal muscles are the most of the voluntary muscle
in body
3. Smooth
– not striated
– Located in walls of blood vessels and viscera
Muscle type Smooth Cardiac Skeletal
# nuclei 1 1-2 Many
Position of Central Central Periphery
nucleus
Striations No Yes Yes
Shape Spindle Short, Long, cylindrical
branched
Size (diam) 5-10μm 10-15μm 30-150 μm
Function Peristalsis Pumping Movement/
stabilization
- Involuntary Involuntary Voluntary 9
 10 Cardiac Muscle
Tissue Features:
 Striated (same contractile machinery)
 Self-excitatory and electrically coupled
 Rate of contractions modulated by autonomic
nervous system
– innervation is neuroendocrine in nature (i.e.
no “motor end plates”)
Cell Features:
 1 or 2 centrally placed nuclei
 Branched fibers with intercalated discs
 Numerous mitochondria (up to 40% of cell volume)
 Sarcoplasmic reticulum
- Sarcoplasmic reticulum does not form terminal
cisternae
 T-tubules crosses at the Z-line
- T tubules are about 2x larger in diameter than in
skeletal muscle
– transport Ca2+ into fibers
 Transverse Tubular System 11
(T-tubules)

Gap junctions exist

Transverse Tubular System (T-tubules)
between cardiac muscle
 T tubule crosses at the Z-line
cells, providing low
 The sarcolemma of the myocyte invaginates to form an
resistance pathways for the
extensive tubular network
spread of excitation from
 Extends the extracellular space into the interior of the cell.
one cell to another.
 Transmit the electrical stimulus rapidly (well developed in
ventricular myocytes but is scanty in atrial and purkinje cells).
 , the action potential is propagated down the T tubule.
 Adjacent cardiac myocytes are joined end-to-end at
structures known as intercalated disks
Sarcolemma contains a number of ion channels and pumps that
contribute to overall Ca 2+ levels within the myocyte
12 Sarcoplasmic Reticulum (SR):

Sarcoplasmic Reticulum (SR):

 A fine network spreading throughout the myocytes.
 Demarcated by its lipid bilayer.
 Close apposition to the T tubules.
 Junctional SR.
 Longitudinal SR.

Subsarcolemmal Cisternae Junctional SR:

 The tubules of the SR expand into bulbous swellings.
 Contains a store of ca2+ ions.
 Release calcium from the calcium release channel
(ryanodine receptor) to initiate the contractile cycle.

Longitudinal or Network SR:

 Consists of ramifying tubules.
 Concerned with the uptake of calcium that initiates
relaxation.
 Achieved by the ATP-requiring calcium pump
(SERCA= sarcoendoplasmic reticulum Ca2+ -
ATPase)
 13
Resting membrane and action potentials

Cardiac myocytes can be divided into work cells and pacemaker cells.
 The work cells have a large stable resting membrane potential and display a
prolonged action potential with a plateau phase.

 The pacemaker cells have smaller unstable resting potentials and

spontaneously depolarize, generating the intrinsic electrical activity of the heart.
 Pacemaker cells are found in the sinoatrial (SA) and atrioventricular (AV) nodes.
 The cells of the bundle of His and some Purkinje cells are also capable of
spontaneous firing.
14
The cardiac action potential.
The cardiac action potential.
Phase 0—depolarization because of the opening of
fast sodium channels. Potassium flux also decreases.
Phase 1—partial repolarization because of a rapid
decrease in sodium ion passage as fast sodium
channels close.
Phase 2—is the plateau phase of the cardiac action
potential. Membrane permeability to calcium
increases during this phase, maintaining
depolarization and prolonging the action potential and
the plateau phase is partially maintained by an inward
sodium current. Sodium flows into the cell through the
sodium–calcium exchanger. The exchanger transfers
three sodium ions into the cell in exchange for one
calcium ion flowing out, and so produces a net inward
positive current
 Phase 3—repolarization, sodium, and calcium
channels all close and membrane potential returns to
baseline.
 Phase 4—resting membrane potential (−90 mV),
resulting from the activity of the Na+/K+ ATPase pump
which creates a negative intracellular potential
because of the exchange of three sodium ions for only
two potassium ions.
 15 The pacemaker potential

The pacemaker potential is :

 produced by a decrease in membrane permeability to potassium,
 a slow inward current because of calcium influx via T-type (transient) calcium
channels,
 and an increased sodium current because of sodium– calcium exchange.
 Once the threshold potential is reached, L-type calcium channels open, calcium
ions enter the cell, and depolarization occurs.

0 3

4
16

The pacemaker potential.

 Phases 1 and 2 do not occur.
 Phase 4—pacemaker potential. Because of slow inward current of sodium and avoltage
gated increase in calcium conductance (via T channels)
 Phase 0—depolarization. As opposed to the ventricular muscle action potential, this
occurs because of voltage gated calcium channels opening.
 Phase 3—repolarization. This is because of a decrease in potassium.

0 3

4
17
1. Pacemaker potential
Have unstable resting potentials
(pacemaker potentials or
prepotentials) due to open slow
Na+ channels. This slow
depolarization is due to both
opening of Na+ channels and
closing of K+ channels. Notice
that the membrane potential is
never a flat line.
2. Depolarization The
action potential begins when
the pacemaker potential
reaches threshold.
Depolarization is due to Ca2+
Explosive influx through Ca2+
channels.
3. Repolarization is due to
Ca2+ channels inactivating and
K+ channels opening. This
allows K+ efflux, which brings
the membrane potential back
to its most negative voltage.
18
Extrinsic Innervation of the Heart
19
Cardiac muscle contraction
 Heart is
 endowed with a special system for generating rhythmical electrical
impulses
 Cause rhythmical contraction of heart muscle and
 conducting these impulses rapidly through heart.

 Atria
 contract about one sixth of a second ahead of ventricular
contraction,
 Which allows filling of ventricles before they pump blood through lungs
and peripheral circulation
 20 Excitation contraction coupling & relaxation
This is the process linking electrical excitation to contraction.
Calcium has an essential role in this process; a raised intracellular calcium concentration
is the trigger that activates contraction.

 Depolarization opens voltage-gated fast Na+

channels in the sarcolemma
 Reversal of membrane potential from –90 mV to
+30 mV
 The action potential spreads to the interior of the
cardiac muscle fiber along the membranes of the
transverse (T) tubules
 Depolarization wave in Longitudinal
sarcoplasmicsT tubules membrane causes the
SR to release Ca2+
 In addition Depolarization wave also opens slow
Ca2+ channels in the sarcolemma Ca2+ surge
prolongs the depolarization phase (plateau)
 During the plateau phase of the action potential,
calcium ions flow down this steep concentration
gradient and enter the myocyte. Most of this
calcium enters through the L-type channels,
 The influx of calcium triggers the release of
further calcium from the sarcoplasmic reticulum
via ryanodine receptors
 21 Excitation contraction coupling & relaxation

 Free intracellular calcium interacts with the C

subunit of troponin
 This leads to a configuration change in the
troponin/tropomyosin complex,
 allowing actin to interact with myosin.
 Cross bridge cycling occurs,
 leading to a shortening of the sarcomere and
resultant muscular contraction.
 As intracellular calcium concentrations decrease
during repolarization,
 calcium dissociates from troponin as intracellular
calcium concentration decreases, resulting in
relaxation.
 Diastolic relaxation is an active (ATP-dependent)
process. Calcium transport out of the cytosol
occurs via a sarcoplasmic reticulum Ca2+-ATPase,
through sarcolemmal Na+/Ca2+ exchange, via a
sarcolemmal Ca2+-ATPase, and finally by utilizing
a mitochondrial Ca2+ uniport.
 Na gradient is maintained by the Na＋- K ＋ ATP ase
22
23 Heart Sounds
• There are optimal recording sites for the various heart sounds or PCG signals.
Because of the acoustical properties of the transmission path, heart sound waves
are attenuated but not reflected. Figure shows four basic chest locations at which
the intensity of sound from the four valves is maximized.
• Auscultatory areas on the chest A, aortic; P, pulmonary; T, tricuspid; and M, mitral
areas.
 24 Heart Sounds
• Heart sounds are vibrations or sounds due to the acceleration or deceleration of
blood during heart muscle contractions,
• '(1st sound) Lubb’ - Closure of A-V valves
• ‘(2nd sound) Dupp’ - Closure of Semi-Lunar valve, when pressure in the
ventricles falls below pressure in the arteries.Caused by Turbulence on closing.
• ‘(3th sound) corresponds to the sudden cessation of the ventricular rapid filling.
This low-amplitude, low frequency vibration is audible in children and in some
adults.
• ‘(4th sound) occurs when the atria contracts and propel blood into the ventricles.
This sound with very low amplitude and low frequency is not audible, but may be
recorded by the phonocardiography (PCG).
 25
Heart Sounds

Anything extra ’Murmur’ 1st

(swishing of blood) (a type of heart sounds)
are abnormal heart sounds produced by
abnormal patterns of blood flow in the
heart. And considered as vibrations or
sounds due to blood turbulence. Increases
Could be due to: Pressure on
• Defective heart valves;Stenosis of Valves heart
(calcification)
• Valves not closing properly (Incompetence,
Insufficiency)
• Mitral stenosis
• Phonocardiographyis the recording of
heart sounds
 26
The cardiac cycle is the contraction and relaxation
cycle of the heart
The heart is the driver of the circulatory system generating cardiac output (CO)
by rhythmically contracting and relaxing.

– Systole - Phase of contraction.

– Diastole - Phase of relaxation.
– End-diastolic volume (EDV) - Total volume of blood in the ventricles at the
end of diastole.
– Stroke volume (SV) - Amount of blood ejected from ventricles during
systole.
– End-systolic volume (ESV) - Amount of blood left in the ventricles at the
end of systole.
• Average rate is 75 bpm.
• Each cycle is ~ 0.8 s : 0.5 s for diastole & 0.3 s for systole..
 27 MECHANICS OF CARDIAC CYCLE (Wiggers Diagram)
• Step 1: Isovolumetric contraction:
– QRS just occurred.
– Contraction of the ventricle 1 2
causes ventricular pressure to rise
above atrial pressure & AV valves
close.
– Ventricular pressure is less than
aortic pressure.
• Semilunar valves are closed.
– Volume of blood in
ventricle is EDV.
• Step 2: Ventricular Ejection (Rapid):
– Contraction of the ventricle
causes ventricular pressure to rise
above aortic pressure.
• Semilunar valves open.
– Ventricular pressure is greater
than atrial pressure.
• AV valves are closed.
– Volume of blood ejected:
SV..
 28 MECHANICS OF CARDIAC CYCLE (Wiggers Diagram)

• Step 3: T wave occurs: 4

– Ventricular pressure drops below 5 6
aortic pressure. Ventricular ejection
(reduced)
• Step 4: Isovolumetric relaxation:
– Back pressure causes semilunar
valves to close.
• AV valves are still closed.
– Volume of blood in the
ventricle: ESV.
• Step 5: Rapid filling of ventricles:
– Ventricular pressure decreases
below atrial pressure.
• AV valves open.
– Rapid ventricular filling
occurs.
• Step 6: Atrial systole : Atrial
contraction.
• Push 10-30% more blood into
the ventricle.. 3
29
30
Cardiac Output (CO)
• Volume of blood pumped by each ventricle in one minute
• CO = heart rate (HR) x stroke volume (SV)
= heart rate (HR) x (EDV - ESV)
– HR = number of beats per minute
– SV = volume of blood pumped out by a ventricle with each beat

• At rest
• CO (ml/min) = HR (75 beats/min)  SV (70 ml/beat) = 5.25 L/min
• Maximal CO is 4–5 times resting CO in nonathletic people
• Maximal CO may reach 35 L/min in trained athletes
• Cardiac reserve: difference between resting and maximal CO
31 Regulation of Stroke Volume

• SV = EDV – ESV
• Three main factors affect SV
– Preload
– Contractility
– Afterload

• Preload: degree of stretch of cardiac muscle cells before they

contract (Frank-Starling law of the heart)
• Closely related to LV end-diastolic volume
• Cardiac muscle exhibits a length-tension relationship
• At rest, cardiac muscle cells are shorter than optimal length
• Slow heartbeat and exercise increase venous return
• Increased venous return distends (stretches) the ventricles and
increases contraction force
32 Regulation of Stroke Volume

Contractility: contractile strength at a given muscle length,

independent of muscle stretch and EDV
• A Fundamental property of cardiac muscle reflects the level of
activation of cross-bridge formation
• Positive inotropic agents increase contractility
– Increased Ca2+ influx due to sympathetic stimulation
– Hormones (thyroxine, glucagon, and epinephrine)
• Negative inotropic agents decrease contractility
– Acidosis
– Increased extracellular K+
– Calcium channel blockers
33 Regulation of Stroke Volume

• Afterload: pressure that must be overcome for ventricles to

eject blood
• closely related to aortic impedance (i.e., the sum of the external
factors that oppose ventricular ejection);
• Hypertension increases afterload, resulting in increased ESV
and reduced SV

Ejection Fraction :
Basic parameter for evaluation of the systolic function of the heart

EDV = ± 110 - 120 cc -> 150 - 180

cc
ESV = ± 40 - 50 cc
SV = ± 70 cc -> contract strongly = 10 -20 cc
CO = SV x HR = 70 cc x 70/min = 4900 cc/min
Ejection Fraction = SV ( EDV - ESV) : EDV = ± 60%
 the change in volume and pressure in the heart and in
34
the blood vessels

Blood volume  Venous Return EDV

• Return of blood to the
heart via veins.
• Venous pressure is
driving force for return of
blood to the heart.
• Veins have thinner walls,
thus higher compliance.
– Capacitance vessels.
• 2/3 blood volume is
in veins.
• EDV, SV, and CO are
controlled by factors
which affect venous
return.
 35

End Diastolic Volume (EDV)

Workload on the heart prior to contraction (preload).
Volume of blood in the ventricles at the end of diastole.is about 130 mL.
 SV directly proportional to preload.
Increase in EDV results in an increase in SV.
 SV directly proportional to contractility.
Strength of contraction varies directly with EDV.
Ejection fraction: - SV/ EDV.
- Normally is 60%.
- Clinical diagnostic tool.

End-systolic ventricular volume, (ESV)

The ventricle does not empty completely during ejection
 blood remains in each ventricle at the end of systole when
ejection is complete is about 50 mL
36
Frank-Starling Law of the Heart
36
Relationship between EDV, contraction strength, and SV.

• Intrinsic mechanism:
– Varying degree of stretching of
myocardium by EDV.
– As EDV increases:
• Myocardium is increasingly
stretched.
• Contracts more forcefully.
• As the ventricles fill, the
myocardium stretches; This
increases the number of interaction
between actin and myosin.
• Allows more force to develop.
 37 Factors Controlling MAP
Total peripheral resistance

Mean arterial
pressure After load End Systolic Volume

60
38 The role of the heart in producing
the blood pressure
 Factors Controlling MAP :
39 The Driving Pressure for Blood Flow

cardiac output peripheral vessel

blood volume resistance elasticity

Venous SVR = systemic

return vascular resistance
CO = cardiac output
SV = stroke volume
Q = Blood Vol flow rate
40
Cardiac Hemodynamic Disturbance

A. Describe hemodynamic disturbance

and related heart murmur due to valvular
problem :
1.Aortic stenosis-regurgitation
2. Mitral stenosis-regurgitation
3.Pulmonal stenosis-regurgitation
4. Tricuspid stenosis-regurgitation

B. Describe hemodynamic disturbance

due to myocardial problem :
1.Dilated cardiomyopathy
2. Hyperthrophic obstructive
cardiomyopathy
3.Restrictive cardiomyopathy
41
INTRODUCTION
324

Cardiac Hemodynamic Disturbance

STENOTIC AND INSUFFICIENT VALVES
Pressures in heart valve diseases
The most common cause of turbulence is valve malfunction,
either a stenotic or an insufficient valve.
A stenotic valve:
 a stiff , narrowed valve that does not open completely.
 resulting in turbulence that produces an abnormal whistling
sound
An insufficient, or incompetent valve
one that cannot close completely,
usually because the valve edges are scarred and do not fit together
properly.
produced when blood flows backward known as regurgitation.
creating a swishing or gurgling murmur.
42
Aortic stenosis
 Obstruction of blood flow across the aortic
valve during left ventricular systole Due to
stenosis Aortic valve
 The pressure in LV increases and becomes
higher than pressure in aorta (Ao)
 Pressure gradient results (normally both
pressure peaks equal)
 Important hypertrophy of LV

Etiology of Aortic Stenosis

Calcified AS
• Congenital
• Rheumatic
• Degenerative/Calcific
• Patients under 70: >50% have
a congenital cause
• Patients over 70: 50% due to
degenerative
43
Pathophysiology of Aortic Stenosis

• A pressure gradient develops between the left ventricle and the aorta.
(increased afterload)
• LV function initially maintained by compensatory pressure hypertrophy
• When compensatory mechanisms exhausted, LV function declines.

increased after load

44
Three Cardinal Symptoms of AS
• Exertional dyspnea, on exertion due to heart failure
(systolic and diastolic), Is a result of elevation of the pulmonary
capillary pressure secondary to reduced compliance and/or LV
dilatation
• Exertional angina,Usually develops later and reflects an
imbalance between the augmented myocardial oxygen
requirements and reduced oxygen availability (increased
myocardial oxygen demand; demand/supply mismatch)
• Exertional syncope, Caused by arrhythmias (usually
ventricular tachycardia and bradycardias), hypotension, or
decreased cerebral perfusion resulting from increased blood flow
to exercising muscles without compensatory increase in cardiac
output
 45
Aortic stenosis
Physical Findings in Aortic Stenosis
• Slow rising carotid pulse (pulsus tardus) & decreased pulse amplitude
(pulsus parvus)
• Heart sounds- soft and split second heart sound, S4 gallop due to LVH.
• Systolic ejection murmur- cresendo-decrescendo character. This peaks
later as the severity of the stenosis increases.
– Loudness does NOT tell you anything about severity

Evaluation of AS
• Echocardiography is the most valuable test for diagnosis,
quantification and follow-up of patients with AS.
• Two measurements obtained are:
a) Left ventricular size and function: LVH, Dilation, and EF
b) Doppler derived gradient and valve area (AVA)
46
Imaging Studies

• ECG
• Chest radiography
• Echocardiography
• Dobutamine echocardiography
• Cardiac catheterization

ECG
• LV hypertrophy – classic finding
• Other nonspecific changes are left
atrial enlargement, left axis
deviation, and left bundle-branch
block
 Large S wave in V1
• Not a reliable test because of the  Large R wave in V5
wide variations seen in AS and
other cardiac conditions
47
Imaging Studies

Chest Radiograph
• Normal or enlarged cardiac Arrow points out dilated shadow
silhouette of the ascending aorta

• Calcification of aortic valve

• Dilatation and calcification
of ascending aorta

Echocardiography
• Useful in assessing the severity of AS, the
degree of coexisting aortic regurgitation, LV size
and function
• Helpful in estimating pulmonary systolic pressure
and in identifying other cardiac abnormalities
• TEE (Transehageal echocardiogram) – displays
the obstructive orifice extremely well
48
Imaging Studies

Dobutamine Echocardiography
• Indicated in patients with moderate aortic stenosis and LV dysfunction
to predict the reversibility of LV dysfunction after AVR
• Pts. With AS, LV dysfunction, and relatively low gradients have better
outcome when management decisions are based on the results of
dobutamine echocardiogram (Schwammenthal, et al, 2001)

Cardiac Catheterization
• Indicated for hemodynamic evaluation whenever there is discrepancy
between the clinical picture and echocardiography
• Indicated for young, asymptomatic patients with noncalcific congenital
AS, to define the severity of obstruction to LV outflow
• Indicated for patients in whom it is suspected that the obstruction to
LV outflow may not be at the aortic valve but rather in the sub or
supra-valvular regions
• Also indicated to evaluate the coronaries in AS patients
at risk for coronary artery disease
49
Management of Aortic Stenosis

Grading of aortic stenosis

• The aortic valve area must be
reduced to one-fourth of its
normal size before significant
changes in the circulation occur
• AS is graded based on the aortic
valve area
– Mild - >1.5 cm²
– Moderate – 1.1 to 1.5 cm²
– Severe - <0.75 to 1 cm²
50
Aortic Regurgitation

• Definition: Leakage of blood into LV during diastole due to ineffective

coaptation of the aortic cusps or due to backward flow the aortic
pressure declines more rapidly to compensate (to maintain
normal mean pressure)

Etiology of acute AR
 Endocarditis
 Aortic Dissection

Etiology of chronic AR
Bicuspid aortic valve
Rheumatic
Infective endocarditis
51 Pathophysiology of AR

Combined pressure and volume overload

Compensatory Mechanisms: LV dilation, LVH. Progressive dilation
leads to heart failure
52

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 (lateral Mitral
valve) causing it to vibrate
• Systolic ejection murmur: due to
increased flow across the aortic valve
systolic pressure increases
• Florid pulmonary oedem
increased pressure amplitude
53
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 ( diastolic aortic pressure and increased LVEDP thus 
coronary artery diastolic flow)
– Palpitations: due to increased force of contraction
– With extreme reductions in diastolic pressures (e.g. < 40) may see
angina
54
Peripheral Signs of Severe Aortic Regurgitation

• Quincke’s sign: capillary pulsation (Exaggerated reddening and bleaching of

nail beds)
• Corrigan’s sign: water hammer pulse (High amplitude, abruptley collapsing
pulse)
• Bisferiens pulse (AS/AR > AR)
• De Musset’s sign: systolic head bobbing (visible boobing of the ead)
• Mueller’s sign: systolic pulsation of uvula (visible pulsation of the uvula)
• Austint Flint Murmur Low pitched apical mid-systolic rumble)
• Durosier’s sign: femoral retrograde bruits (to-and-fro bruit over femoral artery)
• Traube’s sign: pistol shot femorals (Loud systolic “pistol shoot) over femoral
artery
• Hill’s sign:BP Lower extremity >BP Upper extremity by
– > 20 mm Hg - mild AR
– > 40 mm Hg – mod AR ( Between popliteal and brachial pressure )
– > 60 mm Hg – severe AR
© Continuing Medical
Implementation
55
Assessing Severity of AR

• Assess severity by impact on peripheral signs and LV

–  peripheral signs =  severity
–  LV =  severity
– S3
– Austin -Flint
– LVH
– radiological cardiomegaly

© Continuing Medical
Implementation
56
Management Aortic Regurgitation

Aortic
Valve
Replacment
 57
Mitral Stenosis

• Definition: Obstruction of LV
inflow that prevents proper filling
during diastole
• Normal MV Area: 4-6 cm2
• Transmitral gradients and
symptoms begin at areas less
than 2 cm2
• Progressive fibrosis
• Calcification of valve leaflets
• Fusion of the cusp and Etiology of Mitral Stenosis
subvalvular apparatus  Rheumatic heart disease: 77-
• Rheumatic carditis is the 99% of all cases
predominant cause
 Infective endocarditis: 3.3%
• Prevalence and incidence:
decreasing due to a reduction of  Mitral annular calcification:
rheumatic heart disease. 2.7%
 58
MS Pathophysiology
 The flow of blood from LA to LV is restricted
and left arterial pressure rises
 Increased Transmitral Pressures: Leads to
left atrial enlargement and atrial fibrillation
and lead hypertrophy of LA
 LV filling becomes more dependent on left
atrial pressure
 Pulmonary oedema : accompanying a
tachycardia and loss of atrial contraction
and Leading to pulmonary venous
congestiom and breathlessness
 Right heart failure symptoms: due to
Pulmonary venous HTN
 All these lead to marked haemodynamic
deterioration
59

Natural History of MS
• Disease of plateaus:
– Mild MS: 10 years after initial RHD insult
– Moderate: 10 years later
– Severe: 10 years later
• 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
60

Auscultation MS
• Loud first heart sound (S1)
• Opening snapible and move closer to S2 with increaase in severity
• Mid-Diastolic murmur:
– Low-pitched diastolic rumble most prominent at the apex.
– Heard best with the patient lying on the left side in held
expiration
– accentuated by exercise
• Crepitations pulmonary oedema, effusion ( raised pulmonary
capillary pressure)
61

Symptom MS
 Patients usually remain asymptomatic until the stenosis is<
2 cm2
 Breathlessness - reduced lung compliance dur to chronic
pulmonary congestion
 Fatique - low cardiac out put - exercise tolerance typically
diminishes very slowly over many years
 Oedema ascites ( right heart failure)
 Palpitation ( atrial fibrilation)
 Haemotysis ( pulmonary congestion, pulmonary embolism)
 Cough ( pulmonary congestion)
 Chest pain ( pulmonary hypertension )
 Thromboembolic complication ( e.g. strokes, ischemic limb )
62
Mitral Stenosis
324
 Serial echocardiography:
63 Mild: 3-5 years
Echocardiography MS Moderate:1-2 years
Severe: yearly
 64 Management MS
 Patients with the minor symptom should be treated medically
 Systemic embolism : anticoagulant
 Atrial Fibrilation : ventricular rate control by digoxin, β Bloker or rate-limiting
antagonis
 Pulmonary congestion : Diuretic theraphy
 Antibiotic prophylaxis againts infective endocarditis is no longer routinly
recommended
 Surgical Management :
 Patient remains symptomatic despite medical treatment or if pulmonary
hypertension develops
 Ballon valvuloplasty
 Mitral valvotomy
 Mitral valve replacement
 Mitral valvuloplasty : Treatment of choice if specific criteria are fulfilled
 Significant symptom
 Isolated mitral stenosis
 No ( or trivial ) mitral regurgitation
 Mobile, non calcified valve/subvalve apparatus on each
 LA free of thrombus
 65 Mitral regurgitation
• Definition: Backflow of blood from the LV to the LA during systole
through a defective hear valve
• Mild (physiological) MR is seen in 80% of normal individuals.
• Any one or more of the five functional componenets of the mitral valve
apparatus ( leaflets, annulus, chordae tendineae, papillary muscle,
subjacent myocardium )
Acute MR
• Endocarditis
• Acute MI:
• Malfunction or disruption of prosthetic valve
Chronic MR
• Myxomatous degeneration (MVP)
• Ischemic MR
• Rheumatic heart disease
• Infective Endocarditis
• Dilated cardiomyopathy
66
Pathophysiology of MR
• Pure Volume Overload
• Compensatory Mechanisms:
Left atrial enlargement, LVH
and increased contractility
- Increase of pressure in LA
during ventricle contraction
(part of the blood returns to
the atrium) LA dilation and
hypertrophy
– Progressive left atrial
dilation and right ventricular
dysfunction due to
pulmonary hypertension.
– Progressive left ventricular
volume overload leads to
dilation and progressive
heart failure.
67
Clinical feature MR

Symptoms depend on how suddenly the regurgitation develops

 Acute : usually presents with acute pulmonary oedema
 Chronis: symptom complex that is similiar to that of MS
 Symptom
 Dyspnoea or orthopnea (pulmonary venous congention)
 Fatique (low cardiac out put)
 Palpitation ( atrial fibrilation, increase SV)
 Oedema, ascites ( Right heart failure)
 Signs
 Atrial fibrilation / flutter
 Cardiomegali displaced hyperdynamic apex beat
 Sign of pulmonary hypertensiom and right heart faikure
 Floppy mitral valave - mid systolic click
 Prominent third heard sound- sudden rush of blood back to the
dilated left ventricle in early diastole
 68

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

Physical Exam findings in MR

• 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.
• Exertion Dyspnea: ( exercise intolerance)
• Heart Failure: May coincide with increased
hemodynamic burden e.g., pregnancy, infection or
atrial fibrillation
69

324

Imaging studies in MR
• ECG: May show, LA enlargement, atrial fibrillation and LV hypertrophy
with severe MR
• Chest XRay : LA enlargement, LV enlargement, Pulmonary venous
congestion, pulmonary oedema ( if acute)
• ECHO: Estimation of LA, LV size and function
Valve structure assessment
• Doppler: Detects and quatifies regurgitation
• Cardiac catherisation
 Dilated LA, dilated LV, mitral refurgitation
 Pulmonary hepertension
 Coexisting coronary artery disease
 70
Management of MR
Serial Echocardiography:
 Mild: 2-3 years
 Moderate: 1-2 years
 Severe: 6-12 months
 Mild MR can be treated medically
 Diuretica
 Vasodilatator
 Digoxin if atrial fibrilation is present
 Anticoagulation if atrial fibrilation is present
 Evidence of progressive cardiac enlargement early surgical intervention
 valvotomy, valvoplasty, and valve replacement
 Repair rhe valve and restore mitral valve function by inserting an annuoplasty
ring
 Overcome annular dilatation
 bring the valve leaflets closer together
71 Clinical Practice
72
Pulmonary Stenosis

Definitions.
 A form of right ventricular out flow tract
obstruction in which stenosis is usually
valvar or infundubular or both ( rarely,
supravalvar)
 Simple, pure, isolated pulmonary valvar
stenosis ( 70%)
 Pulmonary stenosis with normal aortic root

Pathophysiology
 Usually invoves a stenotic valve that obstruct right
ventriculau empting and pulmonary blood flow, producing
right ventricular hypertrophy and cyanosis
 Subvalvar and supravalvar stenosis are other form of this
defect
73
Pulmonary Stenosis
Pattern of pulmonary stenosis
1. Critical valvar pulmonary stenosis in neonatus
2. Pulmonary stenosis in infant, children and adults
Auscultation
 A heart murmur is simply a noise caused by the
turbulance of blood flowing through the obstruction from
RV to Pulmonary artery - left 3rd intercostal space
 Delayed closure of pulmonary valve = splitting of 2nd
heart sound
Clinical features and dianosis (neonatus)
1. Critical ill, irritable, tachypneic, hypoxic
2. Tachycardia & heart failure, tricuspid insufficiency may
present
3. Chest X-ray, ECG show less evidence
4. Echocardiography provide precise information,
5. Tricuspid valve is competent in 10% & the ther 90%
show incompetence
74
Pulmonary Stenosis

Clinical features & diagnosis ( infant, children, adult)

1.Symptoms
 Symptomatic, ut less severe than neonatus
 30%-40% are asymptomatic
 Effort dyspnea & cyanosis may appear
2.Signs
 Systolic murmur with a thrill
 Pulmonary component of second sound is decreased
3.ECG
 RAE, RAD,RVH
 T- waves in right precordial lead
4. Echocardiography, catherization & cineangiography
 75 Management PS
Mild disease
 This form is asymptomatic and rarely progresses
 Sequential follow - up into annualy until 4 years of age, after which clinical
visits can range from every 3 to 5 years imto adulthood
Moderate disease
 1st. Percutaneous ballon pulmonary valvuloplast (PBPV)
 2nd. Surgical valvuloplasty
Severe to critical disease
Without respiratory distress or cyanosis
1st. Percutaneous ballon pulmonary valvuloplast (PBPV)
2nd. Surgical valvuloplasty
With respiratory distress or cyanosis
8 to 10 L/ minute in infant up to 15 L/ minute in adult
Pulse oximetry saturation 92%-96% in term infant and 88% to 92%
in preterm infant
Cyanotic neonates who are unresponsive to oxygen can be treated
with alprostadil (prostaglandin) Doses 0.01 - 0.02
microgram/kg/min
76
Pulmonic Regurgitation

 Definition: Pulmonic (pulmonary) regurgitation (PR) is

incompetency of the pulmonic valve causing blood flow from
the pulmonary artery into the right ventricle during diastole. The
most common cause is pulmonary hypertension.
 PR is usually asymptomatic.
 Signs include a decrescendo diastolic murmur.
 Diagnosis is by echocardiography.
 Usually, no specific treatment is necessary except for
management of pulmonary hypertension.
77
Pathophysiology

 Severe pulmonic regurgitation is rare

and most often results from an isolated
congenital defect involving dilation of the
pulmonary artery and pulmonary valve
annulus
 PR may contribute to development of
right ventricular (RV) dilatation and
eventually RV dysfunction–induced heart
failure (HF),
 but in most cases, pulmonary
hypertension contributes to this
complication much more significantly.
 Rarely, acute RV dysfunction–induced
HF develops when endocarditis causes
acute PR.
78
Pulmonic Regurgitation

The most common cause of pulmonic regurgitation :

 Secondary pulmonary hypertension
Less common causes :
 Infective endocarditis
 Surgical repair of tetralogy of Fallot
 Idiopathic pulmonary artery dilation
 Congenital valvular heart disease
Rare causes are
 Carcinoid syndrome
 Rheumatic fever
 Catheter-induced trauma
79

Symptoms and Signs

 Pulmonic regurgitation is usually asymptomatic.

 A few patients develop symptoms and signs of RV dysfunction–
induced HF.
 Palpable signs are attributable to pulmonary hypertension and RV
hypertrophy. They include a palpable pulmonic component (P2) of
the 2nd heart sound (S2) at the left upper sternal border and a
sustained RV impulse that is increased in amplitude at the left
middle and lower sternal border.
 Auscultation. High-pitched, early diastolic decrescendo murmur
80
Diagnosis PR

Diagnosis test
 Echocardiography
 Pulmonic regurgitation is usually incidentally detected during a
physical examination or Doppler echocardiography done for
other reasons. Mild PR is a normal echocardiographic finding that
requires no action.
 An ECG and chest x-ray are usually obtained.
 ECG may show signs of RV hypertrophy;
 Chest x-ray may show RV enlargement and evidence of
conditions underlying pulmonary hypertension.
81
Treatment PR

Treatment
 Treatment is management of the condition causing pulmonic
regurgitation
 Rarely valve replacement
 Pulmonic valve replacement is an option if symptoms and
signs of RV dysfunction–induced heart failure develop, but
outcomes and risks are unclear because the need for
replacement is so infrequent.
82

Tricuspid Stenosis

Definition TS:
Narrowing of the tricuspid orifice that
obstructs blood flow from the right
atrium to the right ventricle

Tricuspid stenosis:
 Almost always due to rheumatic fever;
 Tricuspid regurgitation is almost always also present, as is rheumatic
mitral valvulopathy (usually mitral stenosis).
 Rare causes of tricuspid stenosis include SLE, right atrial (RA) myxoma,
congenital malformations, and metastatic tumors.
 The RA becomes hypertrophied and distended, and sequelae of right
heart disease–induced heart failure develop but without right ventricular
(RV) dysfunction; the RV remains underfilled and small.
 Uncommonly, atrial fibrillation occurs.
83
Tricuspid Stenosis

Symptoms and Signs

 The only symptoms of severe tricuspid stenosis are fluttering discomfort
in the neck (due to giant a waves in the jugular pulse),
 fatigue and cold skin (due to low cardiac output),
 and right upper quadrant abdominal discomfort (due to an enlarged liver).
 The primary visible sign is a giant flickering a wave with gradual y
descent in the jugular veins. Jugular venous distention may occur,
increasing with inspiration (Kussmaul sign).
 The face may become dusky and scalp veins may dilate when the patient
is recumbent (suffusion sign). Hepatic congestion and peripheral edema
may occur.
84
Auscultation TS

 Soft opening snap

 Mid-diastolic rumble with presystolic accentuation
 On auscultation, tricuspid stenosis is often inaudible but may produce
a soft opening snap and a mid-diastolic rumble with presystolic
accentuation.
 The murmur becomes louder and longer with maneuvers that
increase venous return (exercise, inspiration, leg-raising, Müller
maneuver) and softer and shorter with maneuvers that decrease
venous return (standing, Valsalva maneuver).
 Findings of tricuspid stenosis often coexist with those of mitral
stenosis and are less prominent.
The murmurs can be distinguished clinically
Feature
Tricuspid Stenosis Mitral Stenosis

Character Scratchy Rumbling, low-pitched

Duration Short Long
85
Diagnosis test TS

Diagnosis of tricuspid stenosis is suspected based on history and physical

examination and confirmed by Doppler echocardiography
 Echocardiography. showing a pressure gradient across the tricuspid
valve. Severe tricuspid stenosis is signified by a mean forward gradient
across the valve > 5 mm Hg. Two-dimensional echocardiography shows
thickened leaflets with reduced movement and RA enlargement.
 ECG may show RA enlargement out of proportion to RV hypertrophy
and tall, peaked P waves in inferior leads and V1.
 Chest x-ray may show a dilated superior vena cava and RA
enlargement, indicated by an enlarged right heart border.
 Liver enzymes are elevated because of passive hepatic congestion.
 Cardiac catheterization is rarely indicated for evaluation of tricuspid
stenosis. When catheterization is indicated (eg, to evaluate coronary
anatomy), findings include elevated RA pressure with a slow fall in early
diastole and a diastolic pressure gradient across the tricuspid valve.
86 Echocardiographic and Doppler Parameters Used
in Grading Pulmonary Regurgitation Severity-
87
Treatment. TS

 A low-salt diet
 Diuretics and aldosterone antagonists
 Rarely valve repair or replacement
 Patients with severe tricuspid stenosis should
undergo intervention if they are symptomatic or if
cardiac surgery is being done for other reasons.
 Percutaneous balloon tricuspid commissurotomy
might be considered for severe TS without
accompanying tricuspid regurgitation.
88

Tricuspid Regurgitation

Tricuspid regurgitation (TR)

 Definition: is insufficiency of the tricuspid
valve causing blood flow from the right
ventricle to the right atrium during systole.
 The most common cause is dilation of the
right ventricle.

 Symptoms and signs are usually absent, but severe TR can

cause neck pulsations, a holosystolic murmur, and right
ventricular–induced heart failure or atrial fibrillation.
 Diagnosis is by physical examination and echocardiography.
 Treatment. TR is usually benign and does not require treatment,
but some patients require annuloplasty or valve repair or
replacement.
89
Etiology TR

 Primary tricuspid regurgitation is less common.

It can be due to valvular abnormalities caused by
 infective endocarditis in users of illicit IV drugs,
 carcinoid syndrome,
 blunt chest trauma,
 rheumatic fever,
 idiopathic myxomatous degeneration,
 congenital defects (eg, cleft tricuspid valve, endocardial cushion
defects), Ebstein anomaly (downward displacement of a congenitally
 malformed tricuspid cusp into the RV),
 Marfan syndrome,
 and use of certain drugs (eg, ergotamine, fenfluramine, phentermine).
 Iatrogenic causes include pacemaker leads that cross the tricuspid
 valve and valve damage sustained during RV endomyocardial biopsy.
90
Etiology TR

Secondary tricuspid regurgitation :

 most commonly caused by dilation of the right ventricle (RV) with
malfunction of a normal valve,
 as occurs in pulmonary hypertension,
 RV dysfunction–induced heart failure (HF),
 and pulmonary outflow tract obstruction.
 Long-standing severe TR may lead to RV dysfunction–induced HF
and atrial fibrillation (AF).
91

Symptoms and Signs TR:

Symptoms and Signs :
Tricuspid regurgitation usually causes no
symptoms,
 Some patients experience neck pulsations
due to elevated jugular pressures.
Signs of moderate to severe tricuspid
regurgitation include jugular venous distention,
with a prominent merged c-v wave and a steep y
descent,
 Sometimes enlarged liver and peripheral edema.
 Symptoms of severe TR include fatigue, abdominal bloating, and
anorexia.
 Patients may also develop symptoms of AF or atrial flutter.
 In severe TR, a right jugular venous thrill may be palpable, as may
systolic hepatic pulsation and an RV impulse at the left lower sternal
border.
92
Auscultation TR

Auscultation
The murmur of tricuspid regurgitation is frequently not heard.
 When evident, it is a holosystolic murmur heard best at the
left middle or lower sternal border or at the epigastrium with the
bell of the stethoscope when the patient is sitting upright or
standing.
The murmur varies with respiration, becoming louder with
inspiration (Carvallo sign).
The murmur may be high-pitched if TR is trivial and due to
pulmonary hypertension,
 or it may be medium-pitched if TR is severe and has other
causes.
 When the murmur is not present at all, the diagnosis is best
made by the appearance of the jugular venous wave pattern
and the presence of hepatic systolic pulsations.
93

Tricuspid Regurgitation

Diagnosis
More moderate or severe TR may be :
Suggested by history and physical examination.
Diagnostic test
Confirmation is by echocardiography.
 Echocardiography.Mild tricuspid regurgitation is most
often detected on echocardiography done for other
reasons.
Cardiac MRI is now the preferred method for evaluating RV
size and function, which typically should be done when
echocardiographic image quality is inadequate.
ECG is usually normal but, in advanced cases, may show tall
peaked P waves caused by right atrial enlargement, a tall R or
QR wave in V1 characteristic of RV hypertrophy, or AF.
94

Tricuspid Regurgitation

Diagnosis test
 Chest x-ray is usually normal but, in advanced cases with RV
hypertrophy or RV dysfunction–induced HF, may show an enlarged
superior vena cava, an enlarged right atrial or RV silhouette
(behind the upper sternum in the lateral projection), or pleural
effusion.
 Laboratory testing is not needed but if done may show hepatic
dysfunction in patients with severe TR.
 Cardiac catheterization is indicated for accurate measurement of
pulmonary pressure when TR is severe and to evaluate coronary
anatomy when surgery is planned. Catheterization findings include
a prominent right atrial c-v pressure wave during ventricular
systole.
95

Tricuspid Regurgitation

Prognosis
 Severe tricuspid regurgitation ultimately has a poor prognosis, even
if it is initially well-tolerated for years.
 As with left-sided valvular regurgitation, the volume-overloaded
ventricle eventually decompensates irreversibly.

Treatment
 Treatment of cause
 Sometimes annuloplasty or valve repair or replacement
 Very mild tricuspid regurgitation is a normal finding and
requires no action.
 Medical treatment of causes (eg, HF, endocarditis) is
indicated.
96

Cardiomyopathy
Cardiomyopathy
 Irreversible primary - Progressive deterioration
 Progressive disease of the heart muscle in which the heart
loses its ability to pump blood effectively
 Predominant affect is on the myocardium
 Damage to the myocardial cells
 The heartmuscle becomes enlargeed or abnormality
thick or rigid
 In rare cases, the muscle tissue in heart is replace with
scar tissue
 As cardiomyopathy progresss -> the heart becomes
weaker and less able to pump blood through the body ->
heart failure, arrhythmias, sytemic and pulmonary edema
and more rarely endoarditis
 “A diverse group of conditions whose final, common
pathway is myocardial dysfunction”
97

Cardiomyopathy

Classification
 Arrhythmogenic Right Ventricular CM
 Dilated
 Hypertrophic
 Restrictive
 Unclassified CM

Restrictive pericarditis
98
Arrhythmogenic Right Ventricular Cardiomyopathy

 Symptoms early teens to second decade

 Etiology unknown (familial). Incidence: 1:3000-
 10,000
 Arrhythmia most prominent
 CHF, tricuspid regurgitation
 Symptoms
 Palpitations
 Syncope
 Fatigue
 Heart failure
 Ventricular arrhythmias
99

Dilated Cardiomyopathy (DCM)

 Most prominent CM
 Incidence – 36 cases/100,000 per year
 (Diagnostic criteria are lacking)
 Males and Africans
 Middle age
 IDCM - accounts for 25% of all heart
failure cases
 EF less than 40% in the presence of
increased LV dimensions
 Dilation and impaired contraction
 Cardiac enlargement
 Hypertrophy?
 Impaired systolic function of either or both
ventricles
100
DCM: Etiologies

Primary
 Idiopathic
 Autosomal dominant
Secondary:
 Electrolyte abnormalities
 Endocrine abnormalities
 Hypertension*
 Infectious causes
 Infiltrative diseases
 Ischemia*
 Neuromuscular diseases
 Nutritional abnormalities
 Rheumatologic diseases
 Tachyarrhythmias
 Toxins
 Valvular heart disease*
*WHO classified as specific cardiomyopathies
101
Pathophysiology DCM

Pathophysiology DCM
1. The heart muscle begins to dilated or strech and
begin thinner

2. Ventricular chamber size >>

3. Over time, the heart becomes weaker

4. Heart failure
 Heart failure valve problem ( regurgitation)
 Arrythmias
 Blood clot in the heart (poor blood flow)
 Emboli formation
102
DCM: Clinical Presentation

 Fatigue/weakness  Orthopnea
 Weight loss  Chest pain
 Dyspnea on exertion  Abdominal pain
 Peripheral edema  Emboli
 Pulsus alternans  Dysrhythmias
 Pulsatile jugular veins  Syncope
 Apical displacement  Sudden death
 S3 / S4
 Murmurs
103
DCM: Diagnostic Tests

DCM: Diagnostic Tests

 CXR - enlargement
 EKG - tachyarrhythmias, Q waves, R-wave
 Echocardiography - diffuse global dysfunction. (MV?)
 Catheterization
104
DCM: Management

DCM: Management
 Sodium restriction
 Vasodilators (arterial/venous)
 ACE, Angiotensi Receptor Blocker (ARB)*
 Beta-Blockers
 Cardioversion
 Pacemakers
 Diuretics
 Anticoagulation
 Antiarrhythmics (amiodarone)
 Heart transplant
*Adrenergic and renin-angiotensin systems
105
Hypertrophic Cardiomyopathy

Hypertrophic Cardiomyopathy
 Stiffness of the LV with resultant impaired ventricular filling
 Disproportionate thickening of the of the intraventricular septum.
 Greater hypertrophy of the ventricular septum than of the
ventricular chambers
 Excessive thickening of the heart muscle.
 Myocardial disarray - normal alignment of muscle cells is absent
 Abnormalities of collagen deposition and altered contractile proteins in
the myocytes (whole structure changes)
 Fibrosis – visible scar
 Myocardial ischemia - abnormal intramural coronary arteries
106
Etiology HCM

Etiology HCM
 Rare genetic disease
 IHSS - Idiopathic hypertrophic subaortic stenosis
 Asymmetric septal hypertrophy
 Muscular subaortic stenosis
 Aortic stenosis
 Develop over time because of High blood presure
(Hypertension) or aging (the elderly)
 HCM vs. physiological hypertrophy
 often the causes is unknown
107

HCM Type
1. Obstructive type. The septum thicken and bulges into the left
ventricle -> blocks the flow of blood into aorta --> the ventricle must
work much harder to pump blood past the blockage and out to the body.
2. Non Obstructive type. The entire ventricle may become thicker (
symmetric ventricular hypertrophy) or it may happend only at the bottom
of the heart ( apical hypertrophy) .The right ventricle also may be
affected

Morbidity / Mortality HCM

 Mortality – 1%-3% per year
 Some remain stable or improve. Clinical deterioration is slow
 Sudden death – higher in children/ adolescents
 Patients with gradients are more likely to deteriorate
 Atrial fibrillation – may lead to increase symptoms (LA dilation)
 LV dilation and dysfunction (DCM) occurs in 5- 10%.
Wall thinning and scar formation
108

Pathophysiology HCM

Pathophysiology HCM
Hyperdynamic state – septal thickening
Leftventricular hypertrophy ( thick entricular wall ) -> ventricular
chamber size << (Diastolic dysfunction) -> Hold less blood (SV<<) ->
CO << (Higher pressures result to allow expansion for the inflow of
blood)
Pressure in ventricular and lung >> -> Pulm venous pressure>> ->
exertional dyspneoa
 Possible outflow obstruction (~25%): MV involvement (MR)
Change in cardiac muscle -> interfere with the heart's electrical
signal, leading to arrythmias -> sudden cardiac arrest
Myocardial ischemia
109
Clinical Manifestations HCM

Clinical Manifestations HCM

 Mild to asymptomatic – screenings
 Sudden death
 Dyspnea – most common from diastolic dysfunction
 Angina, fatigue,
 syncope, dysrhythmias (more common)
 Palpitations, PND, CHF, dizziness (less common)
 S4 / S3 may be heard with outflow obstructions as
well as a systolic murmur
 Ventricular arrhythmias – ¾ of patients
 SVT – ¼ to ½ of patients. Less tolerated
 EP testing – limited predictive value
 CXR – normal to cardiomegaly
 Squat position
110

Echocardiogram – screening and diagnosis HCM

 Cardinal sign is LV hypertrophy of septum and anterolateral free wall
 Variability in hypertrophy
 Dilated left atrium
 Normal to near-normal EF
 Septum at least 1.3 to 1.5 times the thickness of the posterior wall (15
mm). Average finding is 20 mm
 Outflow tract obstructions; MV / pressure gradient changes
 Diastolic dysfunction
111

ECG HCM

ECG HCM
R-wave in AVL >11mm;
R wave height in Lead I plus the S wave depth in Lead III > 25 mm
*S wave depth in V1 plus the height in V5 that exceeds 35 mm
112
HCM: Management

HCM

No symptoms - Mild symptoms -

No treatment Drug treatment

Moderate / severe
symptoms

Obstructive:
Non-obstructive – Drug treatment,
Beta blockers alcohol ablation,
Calcium antagonists myectomy,
(Diuretics) pacemaker
113

Restrictive Cardiomyopathy

 Myocardium becomes rigid,

noncompliant
 Diastolic dysfunction
 Ventricular filling
 Systolic function preserved
 Resembles constrictive
pericarditis
 Prevalence: <5% of CM in
western world

Restrictive pericarditis
114
Clinical feature and diagnosis RCM

Etiology RCM
 Idiopathic
 Noninfiltrative: Scleroderma
 Infiltrative: Amyloidosis, Sarcoidosis
 Storage Disease: Hemochromatosis
 Endomyocardial: Metastatic cancers, Radiation

Clinical heart failure – right failure prominent

 JVD (Jugular Venous Distention)  Exercise intolerance
 S3, S4, or both  Weakness
 Elevation in CVP  Dyspnea
 Peripheral edema,  AV block
liver enlargement, ascites  Symptomatic bradycardia
 Atrial fibrillation
115

Diagnostic test RCM

 CXR
 CT / MRI
 Echocardiogram – dilated atria, increased early LV filling velocity,
decreased atrial filling velocity, and
decreased isovolumetric relaxation time
 Endomyocardial biopsy

Treatment RCM
 No satisfactory medical therapy (treat secondary causes)
 Drug therapy must be used with caution:
 Diuretics for extremely high filling pressure
 Vasodilators may decrease filling pressure
 ?Calcium channel blockers to improve diastolic compliance
 Digitalis and other inotropic agents are not indicated
 Thanks for your attention

Any Question?
 Reference

Lauralee Sherwood: Human Physiology;. Department of Physiology and

Pharmacology School of Medicine West Virginia University. rooks/Cole
10 Davis Drive Belm
Arthur C. Guyton, M.D., John E. Hall, Ph.D:Text Book of Medical Physiology;.
Department of Physiology and Biophysics University of Mississippi Medical
Center Jackson, Mississippi, 11th ed. Philadelphia, Saunders.

W.F.: Ganong MD: Review of Medical Physiology, 12th ed , Lange

Medical Publications
Steven R. Bruhl MD, MS : Valvular Heart Disease, Internal Medicine
Didactics , 2009

Denise Antle, ARNP, MSN, CCRN, CCNS: Cardiomyopathy, Genesis Medical

Center Davenport, Iowa
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