Cardiomyopathies (Non-Ischemic),
Hypertensive and Pulmonary Heart Disease
Susan A. Raaymakers, MPAS,PA-C, RDCS (AE)(PE)
Radiologic and Imaging Sciences - Echocardiography
Grand Valley State University, Grand Rapids, Michigan
raaymasu@gvsu.edu
Basic Review of Heart Walls

Three Heart Walls



Epicardium
Myocardium
Endocardium
Basic Review of Heart Walls

Three Heart Walls



Epicardium
Myocardium
Endocardium
Basic Review of Heart Walls

Three Heart Walls



Epicardium
Myocardium
Endocardium
Basic Review of Heart Walls
Epicardium

Includes:


Nerves to heart and coronary blood vessels
Connective tissues
Basic Review of Heart Walls
Myocardium

Muscular layer composed of bands of cardiac
fibers
Basic Review of Heart Walls
Endocardium


Contains branches for electrical conduction
system
Pathway for blood supply to the valves
Definition of Cardiomyopathy

Primary disease of the myocardium
Three Basic Types of Cardiomyopathies



Dilated
Hypertrophic
Restrictive
Overlap particularly between
dilated and restrictive
Dilated Cardiomyopathy
(DCM)
Dilated Cardiomyopathy

Four-chamber enlargement with impaired
systolic function of both ventricles

Physiology is characterized by:



Impaired left ventricular contractility
Reduced cardiac output
Elevated left ventricular end-diastolic pressure
Dilated Cardiomyopathy
Potential Causes


Idiopathic
Toxins





Alcohol
Medications
Cobalt
Snake bites
Metabolic



Thiamine deficiency
Acromegaly
Sickle cell anemia
Non-dynamic
Dilated Cardiomyopathy
Potential Causes-continued



Peripartum
Non-compaction
Infectious


Systemic diseases


e.g. Chagas’ disease
Immune-mediated injury
Inherited disorders


Duchenne’s muscular dystrophy
Sick cell anemia
Dilated Cardiomyopathy
Non-Compaction

(“spongy myocardium”)

Congenital cardiomyopathy of children and adults
resulting from arrested myocardial development
during embryogenesis.
Circulation
May 11, 1999 vol. 99 no. 18 2475
Dilated Cardiomyopathy
Non-Compaction

Prior to formation of the epicardial coronary
circulation (about 8 weeks of life)

The myocardium


Increased surface area permits


Meshwork of interwoven myocardial fibers that form trabeculae
and deep trabecular recesses.
Perfusion of the myocardium by direct communication with the left
ventricular cavity.
Normally, as the myocardium undergoes gradual
compaction, the epicardial coronary vessels form.
.
Dilated Cardiomyopathy
Non-Compaction (“spongy myocardium”)

Echocardiographic results
 A thin epicardium with extremely hypertrophied
endocardium and prominent trabeculations with
deep recesses.
 Often apically localized

Compaction would normally proceed from base to apex, and from
epicardium to endocardium.
http://www.youtube.com/watch?v=vWnlSIonta0&feature
=related
Echocardiographic Approach
Non-Compaction Cardiomyopathy (CM)

Left ventricular systolic function




Qualitative global and regional function
Qualitative end-diastolic and end-systolic
dimensions or volumes
Ejection fraction
Right ventricular systolic function


Qualitative size and systolic function
Pulmonary artery systolic pressure
Dilated Cardiomyopathy
Chagas Disease (American trypanosomiasis)

Parasitic infection

Rare in North America and Europe
(Endemic in South and Central
America)

Early acute symptoms: mild local
swelling at site of swelling

Serious chronic symptoms as many
as 20 years later: heart disease and
malformation of intestines
Chagas Disease

LV apical aneurysm with
minimal involvement of the
ventricular septum

Apical obliteration with a small
ventricular chamber

Atrial enlargement

Atrioventricular valve
regurgitation
Non-dynamic image
Circulation. 2007; 115: 1124-1131
Echocardiographic Approach
Clinical Utility

Echocardiography considerations




Systolic and diastolic function
Chamber sizes
Associated valvular disease
Pulmonary artery pressures

Periodic echocardiograms essential for optimal care
for medications and for need of cardiac
transplantation

Cardiac catheterization is indicated for pulmonary
vascular resistance in heart transplant candidates
Echocardiographic Approach to Dilated
Cardiomypathies

Increased E-Point Septal Separation (EPSS)


“B-Bump” (AC shoulder)


Left ventricular dilation & reduced mitral leaflet motion D/T low transmitral flow
rates
Delayed mitral valve closure
Decreased aortic root motion with early closure of the aortic valve

Reduced left atrial filling and emptying
Echocardiographic Approach to Dilated
Cardiomypathies

Doppler
 Reduced aortic ejection velocity

Reduced stroke volume,


Compensatory mechanisms (including left ventricular dilation)
result in normal stroke volume at rest in many individuals
Associated mitral regurgitation

Often moderate in severity

Due to ventricular dilation and abnormal alignment of the
papillary muscles
Echocardiographic Approach to
Dilated Cardiomyopathies

Doppler
 Reduced dP/dt

Slow rate of rise in velocity of the MR jet


Reduced rate of increase in left ventricular pressure in early
systole (dP/dt)
Diastolic Dysfunction

Early in disease course

Grade I E<A
Echocardiographic Approach
Limitations/Technical Considerations
Usually cannot establish the etiology of dilated
cardiomyopathies

Fairly uniform regardless of etiology of disease

Exception: Chagas’ Heart Disease
Dilated Cardiomyopathy
Large LA and LV, All Walls are Equally Hypokinetic
17-01A
Feigenbaum
Dilated Cardiomyopathy
Same Patient
17-01B
Feigenbaum
Tricuspid Regurgitation Due
To Dilated Cardiomyopathy
12-030a
Feigenbaum
Tricuspid Regurgitation Due To Dilated
Cardiomyopathy – Same Patient
12-030b
Feigenbaum
Dilated CM with Preserved BulletShaped Geometry
(Long Axis Significantly > Short Axis)
17-02
Feigenbaum
Dilated CM without Preserved BulletShaped Geometry
(Long Axis Not Significantly > Short Axis)
17-03
Feigenbaum
Dilated Cardiomyopathy With
Apical Thrombi
17-21
Feigenbaum
Mobile Thrombus Connected
to Laminar Thrombus
17-22
Feigenbaum
Papillary Muscle Displacement Due To
Dilated Cardiomyopathy
17-23
Feigenbaum
Papillary Muscle Displacement Due To
Dilated Cardiomyopathy
17-24
Feigenbaum
Hypertrophic
Cardiomyopathy (HCM)
2011 Hypertrophic Cardiomyopathy
Association Definition of HCM

HCM is characterized by left ventricular (LV)
hypertrophy

without dilatation

in the absence of another cardiac or
systemic condition that could be
responsible for the extent of hypertrophy
that is present.

Mutations involving the gene encoding proteins in
the sarcomere are responsible for the majority of
genetically mediated cases.
https://www.4hcm.org/2011_accf_aha_guidelines.html
Basic Principles

Autosomal dominant inherited disease of the
myocardium

Related to abnormalities in the ß-myosin heavychain gene
HCM Predominant Features




Asymmetric hypertrophy of the LV
Normal ventricular systolic function
Impaired diastolic LV function
Subaortic dynamic obstruction

in some individuals
Non-dynamic
Other Important Clinical
Features




High risk of sudden death (especially during
exertion)
Symptoms of angina
Syncope
Systolic murmur on auscultation
Pattern of Left Ventricular Hypertrophy

Quite variable
 “Classic” septal hypertrophy: Anterior segment of the ventricular
septum (Type I)

Anterior and posterior segments of the septum (Type II) with
sparing of the lateral, posterior and inferior walls

Extensive thickening with normal wall thickness seen only in basal
segment of the posterior wall (Type III)

Isolated apical hypertrophy (Type IV)
Echocardiographic Approach


Multiple tomographic views
Attention in parasternal long axis view to
inferolateral/inferoseptal basal wall between
papillary muscle and mitral annulus

Wall is in this region is not thickened in patients with HCM
Non-dynamic
Left Ventricular Diastolic Pressure
Impaired diastolic relaxation and elevation of
left ventricular end-diastolic pressure
 Increased duration & velocity of the
pulmonary vein a-reversal
 Prolonged IVRT
 Reduced E velocity
 Enhanced A velocity
Subaortic Obstruction
-Dynamic Outflow Tract Obstruction

Apposition of the anterior leaflet of the mitral valve against the
hypertrophied ventricular septum in systole
Subaortic Obstruction - Dynamic Outflow Tract
Obstruction

Dynamic rather than fixed
 Occurs only in mid to late systole

Maximum gradient occurs in late systole
Subaortic Obstruction
-Dynamic Outflow Tract Obstruction






Systolic anterior motion of the mitral leaflet
Mid-systolic closure of the aortic valve
Late peaking high velocity flow in the outflow tract
Variability in the severity of the obstruction with certain maneuvers
Aortic leaflets may be sclerotic because of the long-term effect of a
turbulent jet
http://www.youtube.com/watch?v=Y7JUVTXHBs0&feature=endscreen&
NR=1
Subaortic Obstruction
-Dynamic Outflow Tract Obstruction
 Post PVC or PAC (increases contractility)
 Valsalva maneuver (decreased preload)
 Stress Echocardiography with CW before and after
exercise
 Inhalation of amyl nitrate


Brief decrease in preload (venodilation)
Decrease in afterload (arterial dilation)
Subaortic Obstruction
-Dynamic Outflow Tract Obstruction
Inhalation of amyl nitrate (decreased afterload
and preload





Direct Physician supervision
CW Doppler before, during and after
administration while maintain parallel intercept
angle
Moderate degree of tachycardia may occur but
duration of action of medication is brief
Physician monitors HR, rhythm, symptoms, and
BP
http://www.justoneshot.org.uk/drug_amyl.htm
Dynamic Outflow Tract Obstruction
M-Mode


“Contact lesion” on the ventricular septum
Midsytolic closure of the aortic valve followed by
course fluttering of the leaflets
Left: SAM (arrows)
Right: Mid-systolic closure of the aortic valve (arrow) following by coarse
fluttering of the leaflets
Systolic Anterior Motion of the
Mitral Valve (SAM)
Dynamic Outflow Tract Obstruction
Doppler
 Provide more direct evaluation than imaging
techniques of:



Presence,
Location, and
Degree of dynamic obstruction
Dynamic Outflow Tract Obstruction Doppler
Apical Views
 Using PW Doppler sample volume


Proximal to outflow obstruction


Slowly moved from apex toward the base recording the velocity at
each step
Velocity is normal
Site of obstruction

Velocity increases abruptly reflecting the degree of the obstruction
(Bernoulli Equation 4V2)
Dynamic LVOT
Apical Views
 CW Doppler


Shows a late-peaking high velocity
systolic jet in patient with dynamic
outflow tract obstruction
Remember CW measures velocities
along entire length of the ultrasound
beam
 The obstruction may be due apex
rather than SAM
 Need PW to rule this out
Systolic Motion of the Anterior
Mitral Leaflet (SAM)

Note the relatively narrow width of the turbulent jet at the level
of the mitral valve (arrows).
17-54
Feigenbaum
Mitral Valve Abnormalities

MR results from SAM leading to:



Late systolic failure of coaptation
Posteriorly directed jet
Autopsy & echo studies:




Increased surface area & longer length (particularly the anterior
leaflet)
Degree of coaptation: excessive
Posteriorly displaced coaptation plane
10%: anomalous papillary muscle anatomy with direct insertion of the
papillary muscle into the leaflet
Anaesthesia
Volume 63, Issue 10,
Limitations/Technical Considerations
 HCM can be mimicked

Chronic hypertension (especially patients with
renal failure)

Cardiac amyloid (covered under Restrictive
Cardiomyopathies)
Non-dynamic
Limitations/Technical Considerations

HCM can be mimicked- continued

Pheochromocytoma




Friedreich’s ataxia






Noncancerous (benign) tumor in adrenal
gland releasing adrenaline
Rare cause of HTN.
Concentric LVH (20%)
Autosomal recessively inherited
spinocerebellar degeneration
Concentric LVH (most common)
Decreased function
Asymmetric septal thickening (uncommon)
Increased RV mass (uncommon)
Inferior MI with Previous LVH
Limitations/Technical Considerations

Coexisting aortic stenosis

Accurate measure of pressure
drop may not be possible

AS and LVH may present with
dynamic subaortic obstruction
only after the aortic valve
replacement

HCM that is “unmasked” by the
afterload reduction of the valve
replacement
Clinical Utility

Diagnosis and Screening



Echo: procedure of choice for accurate diagnosis
of HCM
Inherited disorder: screening is indicated for all
first degree relatives of the affected individual
even in asymptomatic due to high risk of sudden
death with exertion
Evaluation of Medical Therapy

Echo: used to assess impact of medical therapy
specifically diastolic function but also dynamic
outflow obstructions
Clinical Utility - continued
On right: contrast in septum defining
area perfused by the septal branch
that will be injected
Non-dynamic

Monitory of Percutaneous Alcohol Septal Ablation

Patient selection
 Extent, distribution and curvature of septal hypertrophy

Cath Lab
 Catheter in septal coronary branch, contrast is injected during
echo to show specific location before delivering of the agent
 Monitoring procedure in cardiac catheterization laboratory:
baseline and post procedure Doppler data are used in
conjunction with invasive hemodynamics to assess the
reduction in the outflow obstruction
Clinical Utility - continued

Surgical Therapies

Interoperative monitoring of myotomy allows
evaluation of the adequacy of the procedure
Non-dynamic
Before and After Myectomy
Prior
Post
17-67a
17-67b
Feigenbaum
Feigenbaum
Review of HCM
Septal Hypertrophy
Type I
17-45a
Feigenbaum
Septal Hypertrophy
Type I – Same Patient
17-45b
Feigenbaum
HCM Type III with Massive
Septal Hypertrophy and SAM
17-49a-49b
Feigenbaum
HCM Type III, Same Patient
17-50a-50b
Feigenbaum
Apical Hypertrophy
Type IV
03-004b
Feigenbaum
Left Ventricular Opacification Agent
Apical Hypertrophy
Type IV
04-027b
Feigenbaum
No Classification. Focalized
Hypertrophy Inferior Wall Only
17-51
Feigenbaum
Pulmonary Hypertension with RVH
Mimicking HCM


Hypertrophy: right ventricular trabeculation
True septal dimension


noted by the double-headed arrow,
septal and trabeculation dimension noted by the two inwardpointing arrows.
17-66
Feigenbaum
Additional Notes on Hypertrophic
Cardiomyopathies

IHSS/ASH WITH SAM/HOCM


Idiopathic hypertrophic subaortic stenosis = LVOT
obstruction
Shape of CW Doppler tracing in HOCM is
described as “dagger”
Restrictive
Cardiomyopathy (RCM)
Basic Principles



Normal LV systolic fxn
Impaired diastolic fxn dt a stiff, hypertrophied LV
Heart failure symptoms are due:


Resultant elevation in LV end-diastolic mmHg
Inability to increase CO w/exercise dt impaired diastolic filling
Heart Failure Definition

Congestive heart failure

Life-threatening condition


Inability to maintain a normal cardiac output
Can occur with normal systolic function
Heart Failure and Restrictive
Cardiomyopathy (RCM)

RCM


Right-sided failure
predominates
Initially with symptoms of
peripheral edema and
ascites
Restricted Cardiomyopathy
(RCM) History

Congestive Heart Failure
(CHF)








Dyspnea
Paroxysmal nocturnal
dyspnea
Orthopnea
Fatigue
Weakness
Anorexia
Angina
Poor exercise tolerance
Restrictive Cardiomyopathy

Infiltrative




Amyloidosis
Hemochromatosis
Glycogen storage disease
Inflammatory


Sarcoidosis
Idiopathic (Löffler’s) Hypereosinophilic syndrome
Restrictive - Infiltrative
Infiltrative - Amyloidosis

Multi-system disease of unknown cause



Extracellular deposition of amyloid protein in
the kidney, heart, liver, nerve, skin and
tongue
Results in tissue damage and organ
malfunction
2D “Ground Glass” appearance

(may also be seen in renal failure)

Concentric LV and RV thickening

May have asymmetric septal thickening
(mimics HCM)
Infiltrative – Amyloidosis
continued

Segmental wall motion abnormalities: common
(septum and inferior wall)

Global LV systolic function may be normal
(early) or decreased (late)

Usual presentation


Male >40 years old with progressive biventricular failure
Biopsies

Amyloid found in myocardium, endocardium, pericardium and
walls of intramural coronary arteries within the conduction
system
Infiltrative - Amyloidosis

Classic cardiac
amyloidosis.

Note the uniform
hypertrophy of the walls
with abnormal myocardial
texture.

Myocardium: substantially
brighter and may take on a
speckled appearance
(ground-glass)

Secondary biatrial
enlargement
17-39a
Feigenbaum
Infiltrative –Amyloidosis
Same Patient
17-39b
Feigenbaum
Infiltrative - Hemochromatosis
Infiltrative
- Hemochromatosis

Iron storage disease:

Affects multiple organ and tissue systems w


Iron


May result in tissue damage and organ malfunction
stored within the cardiac cell rather than extracellular
Types
 Primary (idiopathic)
 Secondary


Iron overload due to multiple blood transfusions in patients with
chronic anemia, abnormal erythropoiesis
Alcohol liver disease
Infiltrative - Hemochromatosis
Primary
 Clinical presentation: usually male >40 years with
evidence of clinical tetrad (DM, liver, skin, and heart
dz, with 1/3 manifesting congestive heart failure)

“Bronze-diabetes”

Liver function abnormalities

CHF
Infiltrative - Hemochromatosis
Primary
 Chest x-ray: cardiomegaly

Cardiac cath: increased LV filling pressures and
reduced ventricular function (global or segmental)

Echo/M-mode:



LV cavity normal size to dilated
Normal LV and RV wall thickness
Dilated Cardiomopathy
Infiltrative - Hemochromatosis
Secondary
 Physical/History


High output cardiac failure from anemia
M-Mode/2D




Increased LV wall thickness
LV dilation
Left atrial dilation
May demonstrate normal or depressed systolic LV function
with depressed fx indicating a worse prognosis
Infiltrative - Hemochromatosis

Parasternal short-axis view recorded in a patient with
documented cardiac hematochromatosis.

Note the increased wall thickness and the abnormal myocardial
texture with modest reduction in systolic function.
22-34
Feigenbaum
Infiltrative – Glycogen Storage
Disease (Pompe’s)
Infiltrative – Glycogen Storage
Disease (Pompe’s)

A group of inheritable disorders of glycogen
metabolism, resulting from a specific
enzymatic defects

Echo

Severe thickening of the IVS, free wall, and
posterior left ventricle wall



Tumor-like appearance of the papillary muscles
Small left ventricular cavity
Poor global left ventricular systolic function
Inflammatory
Inflammatory - Sarcoidosis

Multi-system granulomatous disease;


Involves the heart in about 25% of cases
Females:males 2:1

Unknown etiology

Progressive heart failure

Diffuse fibrosis of the lungs/pulmonary hypertension

Can cause sudden death due to electrical
dysrhythmias
Inflammatory - Sarcoidosis

Echo/M-mode







Increased LV wall thickness (early)
Asymmetric septal hypertrophy
LV dilation with decreased EF (late)
Pericardial effusion
MVP
RV dysfunction due to pulmonary hypertension
Diagnosis by echo is difficult.

Suspect when a young pt presents w/increased LV dimensions,
segmental wall motion abnormalities and/or ventricular
aneurysms
Inflammatory - (Löffler’s) Hypereosinophilic
Syndrome
 Systemic illness characterized by
persistently elevated blood
eosinophil counts

Unknown etiology

Leads to endomyocardial/myocardial
fibrosis and thrombosis

Chest X-ray: cardiomegaly, linear
calcification along left heart border
(calcification of endocardial
thrombus)
dense fibrosis of ventricle in
a postmortem dissected
heart
Inflammatory - (Löffler’s) Hypereosinophilic
Syndrome

Echo:

Apical obliteration,


Global LV systolic function



May extend basally to the inflow portion of the ventricular
wall
Usually well preserved (including apical wall)
Biatrial enlargement
Posterior atrioventricular valves and subvalvular
apparatus

May become involved: massive mitral and/or tricuspid
regurgitation
Inflammatory –Loeffler’s

Hypereosinophilic
syndrome and
obliteration of the LV
apex.

Homogeneous apical
mass
22-35a
Feigenbaum
Review of Cardiomyopathies
Hypertensive Heart
Disease
Hypertensive Heart Disease


End-organ consequence of systemic hypertension
Chronic systemic pressure overload


Initially



Left ventricular hypertrophy
Diastolic function is impaired
Systolic function is normal
Long standing-chronic


Systolic and diastolic dysfunction
Ventricular dilation
Hypertensive Heart Disease
Typical echocardiographic findings
 Left ventricular hypertrophy
 Diastolic dysfunction
 Aortic root dilation
 Aortic valve sclerosis
 Mitral annular calcification
 Left atrial enlargement
Non-dynamic
 Atrial fibrillation
Hypertensive Heart Disease
Echocardiographic approach
 Standard views
 Symmetrically increased wall thickness




Increased end-diastolic wall thickness (>11 mm)
Non-dilated/dilated chamber
LV mass calculations
Diastolic function
Hypertensive Heart Disease
Diastolic function
 Often first evidence of end-organ damage
 Antedating clear evidence of anatomic
hypertrophy
 Impaired early diastolic dysfunction



Prolonged IVRT
E/A ratio reduction
Prolonged deceleration time
Hypertensive Heart Disease
Systolic function
 Preserved early in disease course
 Mid cavity obliteration


May occur in small, hypertrophied LV with
normal function
Associated Doppler velocity curve



Brief, late-systolic high-velocity signal
Duration of signal is shorter than with HCM
Level of obstruction is mid-cavitary
http://www.echojournal.org/vid
eo/58/Ridiculous-LVH-1-of-3
Hypertensive Heart Disease
Mid cavity obliteration

Associated Doppler velocity curve



Brief, late-systolic high-velocity signal
Duration of signal is shorter than with HCM
Level of obstruction is mid-cavitary
Hypertensive Heart Disease
Additional Echocardiography Findings
 Aortic root dilation


Associated with increased tortuosity of the
ascending aorta, arch, and descending
aorta
May have increased irregular
echogenicity of the aortic walls

Represents atherosclerosis

Mitral annular calcification common in
chronic hypertension (HTN)

Left atrial pressure

Combination of chronically elevated LV
end-diastolic pressure and MR
Non-dynamic
Hypertensive Heart Disease
Non-dynamic
Limitations/Technical Considerations

LV mass measurements



Adequate endocardial and epicardial border
definitions
2D vs. M-mode optimal controversial
Differentiation of restrictive and hypertrophic
heart disease
Clinical Utility
 LV mass

Strong predictor of clinical outcome in patient with
hypertension

Borderline hypertension:

Degree of LVH reflects the chronic elevation of
systemic pressure

Index of the temporally average blood pressure over long
periods
 LV mass may be more accurate for assessing the
severity of hypertension than occasional BP recordings
Left Ventricular Mass

Determined by the left ventricular muscle volume
and specific gravity of the muscle

LV muscle volume

Equal to total ventricular volume contained within the
epicardial boundaries of the ventricle

minus the chamber volume contained by the endocardial
surfaces
Left Ventricular Mass

LV mass is calculated by
 Multiplying the left ventricular muscle volume by
the specific gravity of muscle (1.04 g/ml)

specific gravity
n. (Abbr. sg, sp gr) The ratio of the mass of a solid or liquid to the
mass of an equal volume of distilled water at 4°C (39°F) or of a gas
to an equal volume of air or hydrogen under prescribed conditions
of temperature and pressure.
Left Ventricular Mass
Penn-Cube Method
 LV measurements are made during diastole just below the tips of
the mitral valve
LV mass
= 1.04 [(LVID + IVST + PWT)3 – (LVID)3 ] – 13.6
1.04 = specific gravity of
myocardium g/ml
IVST=Interventricular
septal thickness (cm)
LVID = Left ventricular
internal diameter (cm)
PWT= Posterior wall
thickness (cm)
Left Ventricular Mass
ASE Method
 Overestimate LV mass by approximately 25%
Regression equation has been derived for the calculation based on ASE
method
LV Mass =
1.04([LVID +PWT+IVST]3 – LVID3) *0.8 + 0.6

1.04 = specific gravity of
myocardium g/ml
IVST=Interventricular
septal thickness (cm)
LVID = Left ventricular
internal diameter (cm)
PWT= Posterior wall
thickness (cm)
Left Ventricular Mass

Medical Therapy

Monitor efficacy of medical therapy using
echocardiographic measurements

Effective antihypertensive therapy should reverse
end-organ changes (i.e. result in regression of
LVH)
Evaluation of Heart Failure Symptoms

Chronic hypertension

Heart failure symptoms may be due to
 Diastolic or systolic left ventricular dysfunction
 Superimposed coronary artery disease
 Superimposed valvular disease

Hypertensive hypertrophic cardiomyopathy
 Extreme form of preserved LV systolic function and heart
failure symptoms
 Normal to hyperdynamic LV systolic function
 Concentric LVH
 Diastolic dysfunction
 Midventricular late systolic gradient d/t cavity obliteration
 Technically not a cardiomyopathy
Evaluation of Heart Failure Symptoms

Chronic hypertension

Impairment of LV contractility can occur


Even in absence of LV contractility can occur
End-stage hypertensive heart disease has
echocardiographic appearance of end-stage
cardiomyopathy
Evaluation of the PostCardiac-Transplant
Patient
Post-Cardiac-Transplant
Basic Principle

Three goals
1.
2.
3.
Assessment of cardiac anatomy and physiology prompted by
specific clinical problem
The elusive goal of noninvasive diagnosis of early rejection of
the transplanted heart
Diagnosis of post-transplant coronary artery disease
Post-Cardiac-Transplant
Clinical Challenges
Pericardial effusion


Particularly early postoperative
RV systolic dysfunction due to:




Inadequate myocardial preservation at the time of
transplantation
Persistently elevated pulmonary vascular resistance
Transplant rejection
LV systolic dysfunction due to:




Inadequate myocardial preservation
Acute rejection early after transplantation
Superimposed coronary artery disease at a longer interval
after transplantation
Post-Cardiac-Transplant
Normal Findings

Normal
RV and LV size
Wall thickness
Systolic function
Valvular anatomy






Trivial MR, TR, and PR
Abnormal septal motion


Anterior motion of septum in systole
Slight decrease in extent of systolic thickening of
septum
Post-Cardiac-Transplant :Normal Findings

Suture lines in aorta and pulmonary artery: difficult to assess

Small pericardial effusion in early postoperative period
Rarely persists beyond a few weeks
Pericardial effusions often loculated dt postoperative pericardial
adhesions



May have some degree of persistent elevation of pulmonary
artery pressures

Bi-atrial enlargement-if anastomosis of nl and donor atrium
If anastomosis is of SVC and IVC for RA AND cuff of tissue with
pulmonary veins for LA = little bi-atrial enlargement (BAE) and
suture lines may not be evident

Post-Cardiac-Transplant
Abnormal Findings
Acute rejection
 Increased LV mass
 Decreased systolic function
 Increase in echogenicity of the myocardium
 New or increasing pericardial effusion
Post-Cardiac-Transplant
Abnormal Findings
Mild/Early rejection
 2D echocardiographic changes are subtle


Not accurate or reproducible enough for
adjustments of medications
Diastolic dysfunction seen with Doppler
Post-Cardiac-Transplant
Abnormal Findings
Proposed echocardiographic approaches

Diastolic function measurements using patient as
baseline



Decreased pressure half-time (increased early diastolic
deceleration slope)
Decreased isovolumetric relaxation time
Increased E velocity
Significant changes




>20% E velocity
>15% for pressure half-time
>15% isovolumetric relaxation time
Post-Cardiac-Transplant
Abnormal Findings
Transplant coronary artery disease
 Disease is often accelerated relative to CAD
in non-transplant patients


Epicardial vessels and microvasculature or
diffusely involved
Stress echocardiography=higher prevalence of
false-negative


Diffuse disease process masks regional wall motion
abnormalities
Dobutamine stress echocardiography = more
accurate in this patient population
Post-Cardiac-Transplant
Limitations/Technical Considerations/Alternate
Approaches
Standard evaluation for rejection:
transvenous endomyocardial biopsy


Some centers utilize echocardiography guidance
Bi-Atrial Enlargement (BAE)

Several years post cardiac
transplantation.

Biatrial enlargement
Echo density along the atrial
septum, which represents a
prominent atrial suture line
Residual right ventricular and
right atrial dilation are also
present


19-69
Feigenbaum
Left Ventricular Assist Device (LVAD)
Heartmate II
Pulmonary Heart
Disease
Pulmonary Heart Disease
Chronic vs. Acute
Chronic pulmonary heart
disease

Results in group of clinical
signs and symptoms
called cor pulmonale

Underlying pathophysiology
of cor pulmonale

Chronic pressure overload of
the RV ejecting into a highresistance pulmonary
vascular bed
Pulmonary Heart Disease
Chronic vs. Acute
Chronic pulmonary heart disease

Results in group of clinical signs and symptoms
called cor pulmonale

Initially compensatory RVH with preserved RV
function

Progressively RV systolic function deteriorates
leading to RV enlargement (RVE), moderate to
severe TR and consequent right atrial enlargement
(RAE)
Pulmonary Heart Disease
Chronic vs. Acute
Acute pulmonary embolism
 Sudden onset of elevated pulmonary
vascular resistance
Pulmonary Heart Disease
Echocardiographic approach
Non-invasive evaluation of pulmonary artery pressures
 Tricuspid regurgitation
 VSD and PDA assessment
 Pulmonic regurgitant Doppler curve
 M-mode through pulmonic valve
 Right ventricular hypertrophy
 Dilation of right ventricle
 Ventricular septal motion : “paradoxical”
Pulmonary Heart Disease
Echocardiographic approach
Non-invasive evaluation of pulmonary artery
pressures
 Tricuspid regurgitation
Estimation of Cardiac Pressures
Systolic Right Heart Pressures

Normal right heart pressures
25/10
mmHg
0-5
mmHg
25/5
mmHg
Estimation of Cardiac Pressures
Systolic Right Heart Pressures



Tricuspid regurg. + right atrial pressure = Systolic RV
Systolic BP – Ventricular septal defect = Systolic RV
Systolic BP – patent ductus arteriosis = Systolic RV
IVC Reactivity
Estimation of Right Atrial Pressure
IVC Reactivity
Estimation of Right Atrial Pressure
Inferior vena cava (IVC) view. Measurement of the
IVC. The diameter (solid line) is measured
perpendicular to the long axis of the IVC at endexpiration, just proximal to the junction of the hepatic
veins that lie approximately 0.5 to 3.0 cm proximal to
the ostium of the right atrium (RA).
http://www.asecho.org/files/rhfinal.pdf
IVC Reactivity
Estimation of Right Atrial Pressure

4V2 + right atrial pressure = Systolic right heart pressure
Ventricular Septal Defect
Patent Ductus Arteriosus
Estimation of Pulmonary Artery End Diastolic
Pressure
Normal 10 mmHg

Pressure gradient between the pulmonary
artery and the right ventricle during diastole
Mean Pulmonary Artery Pressure
Normal 16 mmHg

Estimated using


Peak pulmonary regurgitant velocity and
From the right ventricular artery acceleration time
Pulmonary Heart Disease
Indirect signs
 M-mode


High specificity (>90%)
Low sensitivity (30-60%)
Note:Absence of A wave
Pulmonary hypertension
Normal
Pulmonary Heart Disease
Doppler
 Rapid early systolic
flow with abrupt
midsystolic
deceleration of flow
indicates severe
pulmonary
hypertension
Pulmonary HTN vs. Vol Overload
Pulmonary Heart Disease
Right Ventricular Pressure Overload


Hypertrophy
Dilation
07-049
Feigenbaum
Pulmonary Heart Disease
Right Ventricular Pressure Overload

RVH on right ventricular free wall best seen
in subcostal four chamber
07-056
Feigenbaum
Pulmonary Heart Disease
Right Ventricular Pressure Overload
Dyskinetic septal motion
07-058a
Feigenbaum
Pulmonary Heart Disease
Right Ventricular Pressure Overload
 Characterized by systolic flattening of
interventricular septum reversed in late
diastole
07-058b
Feigenbaum
Pulmonary Heart Disease
Right Ventricular Pressure Overload
07-060a
Feigenbaum
Pulmonary Heart Disease
Note
 Heavily trabeculated RV
 Dilated RV and RA
 RVH
 Pericardial effusion
 McConnell Sign


Observed in acute pulmonary embolism
http://www.echojournal.org/video/132/McC
onnells-Sign-RV-dysfunction-inpulmonary-embolus
07-061 Feigenbaum
Pulmonary Heart Disease
Long standing pulmonary hypertension or acute
pulmonary hypertension (PHTN)
 RV systolic dysfunction with secondary dilation


Dilation: compensatory mechanism to maintain forward
stroke volume
Tricuspid regurgitation due to annular dilation and
malalignment of papillary muscles (pap msc)
 Leads to further dilation and malalignment of papillary msc
 Tricuspid regurgitation leads to RA enlargement dt
increased pressure and volume
Pulmonary Heart Disease
Secondary Tricuspid Regurgitation

Careful attention to rule out other etiologies of
TR





Vegetation
Rheumatic
Carcinoid
Ebstein’s anomaly
Severe tricuspid regurgitation results in
retrograde flow in hepatic and inferior vena
cava
Pulmonary Heart Disease
Secondary Tricuspid Regurgitation

Severe tricuspid regurgitation
12-029a
Feigenbaum
Pulmonary Hypertension
Tricuspid Regurgitation Severity
Pulmonary Hypertension
Limitations/Technical Considerations

Evaluation of cor pulmonale




Poor ultrasound resolution resultant of obscuring
hyperexpanded lung tissue
Assessment of tricuspid regurgitation for
qualification of pulmonary artery pressure
dependent upon intercept angle
Absence of TR does not translate to normal
pulmonary pressures
Overestimation of tricuspid regurgitation
Graham Steell’s Murmur

High-pitched and blowing murmur during early diastole with a
decrescendo configuration

Noted over the left upper-to-left midsternal area

Result of high-velocity regurgitant flow across an incompetent
pulmonic valve

May be present during the whole of diastole
 Due to pulmonary-to-right ventricular pressure gradient
throughout this time period

Typically occurs in severe PHTN when the PA systolic pressure
is >60 mm Hg
Tricuspid Regurgitation
Overestimation

Avoid overestimation

Measure outer edge of spectral Doppler and not
spectral broadening at peak velocity resultant from
transit time effect
Pulmonary Heart Disease
Clinical Utility

Confirmation of clinical diagnosis of cor pulmonale
in patient with right ventricular failure and chronic
lung disease

Essential to exclude other causes of pulmonary
hypertension



Atrial septal defect
Mitral regurgitation
Noninvasive measurements of pulmonary
pressure used for assessment of medical therapy
routinely used
http://www.amicusvisua
lsolutions.com/obrasky/
06029_01W.jpg
Pulmonary Heart Disease
Clinical Utility-continued
Acute pulmonary embolism
 May visualize residual thrombus originating from
or in transit in the right side of the heart (DVT)

TEE/TTE imaging can demonstrate thrombi in
main, right or left pulmonary arteries

Sensitivity is low

Usually thrombi are located more distally in pulmonary
vasculature

Adequate visualization of pulmonary artery bifurcation in
TTE is not possible in all patients due to interposition of the
air-filled trachea and bronchi
Pulmonary Heart Disease
Clinical Utility-continued
22-45
Feigenbaum
Pulmonary Heart Disease
Clinical Utility-continued
Indirect signs of pulmonary embolism
 Elevated pulmonary artery pressures

Evidence of acute right ventricular pressure
overload


“D-shaped” PSAX-PM level
Right ventricular dilation and dysfunction
Non-dynamic images
http://www.4ventavis.com/img/
patient/diagnosingpah_05.jpg
Pulmonary Heart Disease
Clinical Utility-continued
Indirect signs of pulmonary embolism

Tricuspid regurgitation

Similar findings in patient w/chronic recurrent
pulmonary emboli

Reasons for study may be:

Chest pain, dyspnea or heart failure
Pulmonary Heart Disease- Pulmonary Emboli
Alternate Approaches

Cardiac catheterization allows direct


Measurement of RV and PA pressures
Calculation of pulmonary vascular resistance

Angiography can evaluate RV size and systolic
function utilizing contrast

Standard approach

Radionuclide lung ventilation-perfusion scan
Sources

As noted in individual slides and the following:

Feigenbaum H, Armstrong W. (2004). Echocardiography. (6th Edition).
Indianapolis. Lippincott Williams & Wilkins.

Goldstein S., Harry M., Carney D., Dempsey A., Ehler D., Geiser E.,
Gillam L., Kraft C., Rigling R., McCallister B., Sisk E., Waggoner A., Witt
S., Gresser C.. (2005). Outline of Sonographer Core Curriculum in
Echocardiography.

Otto C. (2004). Textbook of Clinical Echocardiography. (3rd Edition).
Elsevier & Saunders.

Reynolds T. (2008). The Echocardiographer's Pocket Reference. (3rd
Edition). Arizona. Arizona Heart Institute.