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Cardiomyopathies
JohnC.Klick andRachelCarlson
16
Learning Objectives
1. Review how bedside echocardiography can
identify and help manage patients in the ICU
with cardiomyopathies.
2. Describe the various cardiomyopathies specic echocardiographic diagnostic criteria.
3. Discuss stress-induced cardiomyopathy.
Cardiomyopathy is dened as a disease of the
myocardium. The term encompasses a variety
of myocardial disorders where the heart muscle
is structurally and functionally abnormal in the
absence of coronary artery disease, hypertension, valvular disease, or congenital disorder
adequate to explain the anomalies. These disorders are frequently genetic in origin. This chapter will discuss the echocardiographic
identication of the major subtypes of cardiomyopathy [1].
Supplementary Information The online version contains supplementary material available at https://doi.
org/10.1007/978- 3- 031- 80038- 2_16.
J. C. Klick (*)
Department of Anesthesiology, University of Vermont
Medical Center, UVM Larner College of Medicine,
Burlington, VT, USA
R. Carlson
Department of Anesthesiology, Tufts University
Medical Center, Boston, MA, USA
e-mail: rcarlson@tuftsmedicalcenter.org
The World Health Organization (WHO) in
1980 dened cardiomyopathies as “heart disease
of unknown cause.” This was to distinguish cardiomyopathies from cardiac dysfunction due to
known pathologies such as myocardial ischemia,
hypertension, or valvulopathies [2]. However, in
clinical practice, the terms “ischemic cardiomyopathy” and “hypertensive cardiomyopathy”
remain common parlance.
In 1995, the WHO/International Society and
Federation of Cardiology (ISFC) Task Force on
the Denition and Classication of
Cardiomyopathies expanded the classication
according to anatomy and physiology, dening
them as “diseases of the myocardium associated
with cardiac dysfunction.” The following types
were outlined, each with multiple different
causes: dilated cardiomyopathy (DCM),
Hypertrophic cardiomyopathy (HCM),
Restrictive cardiomyopathy (RCM),
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D), and unclassied cardiomyopathies. This classication system did
include ischemic, valvular, and hypertensive diseases among the causes [3].
A statement from the American Heart
Association (AHA) in 2006 proposed a contemporary denition and classication of cardiomyopathies. An expert consensus panel
proposed that “cardiomyopathies are a heterogeneous group of diseases of the myocardium
associated with mechanical and/or electrical
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2025
M. J. Lanspa, A. T. Levinson (eds.), Echocardiography and Ultrasonography in the ICU,
Respiratory Medicine, https://doi.org/10.1007/978-3-031-80038-2_16
211
212
J. C. Klick and R. Carlson
dysfunction that usually (but not invariably)
exhibit inappropriate ventricular hypertrophy
or dilation and are due to a variety of causes
that frequently are genetic. Cardiomyopathies
either are conned to the heart or are a part of
generalized systemic disorders, often leading to
cardiovascular death or progressive heart failure-related disability” [4].
In 2008, the European Society of
Cardiology (ESC) presented an update to the
WHO/ISFC classification. They defined cardiomyopathy as “a myocardial disorder in
which the heart muscle is structurally and
functionally abnormal in the absence of coronary artery disease, hypertension, valvular
disease, and congenital heart disease sufficient to explain the observed myocardial
abnormality.” This classification emphasizes
the distinction between familial/genetic and
nonfamilial/nongenetic causes of cardiomyopathy and excludes heart disease secondary
to valvular, ischemic, or congenital disorders.
It also differs from the AHA definition by
excluding ion channelopathies [5].
In 2013, the World Heart Federation (WHF)
endorsed and published the MOGE(s) classication as a phenotype-genotype-based nomenclature for cardiomyopathies. It is inspired by
the TNM staging of malignancy and excludes
ion channelopathies [6]. The system incorporates ve aspects: the morphofunctional notation (M), organ involvement (O), genetic
inheritance (G), etiology (specic gene mutation (E)), and an optional functional status (S)
[6]. By incorporating ve aspects, the nomenclature system attempts to classify cardiomyopathy based on etiology and highlight the
complex genetics for increasing specicity in
registry data. However, this system has not
been widely accepted into clinical practice and
the applicability remains to be dened [7]. The
remainder of this chapter is focused on discussing cardiomyopathy from the descriptive
phenotypes commonly encountered in the clinical setting.
Dilated Cardiomyopathy
Dilated cardiomyopathy is a disease of the
myocardium involving left ventricular dilation
and systolic dysfunction. This dysfunction must
be in the absence of abnormal loading conditions including hypertension, valve disease, or
coronary artery disease, which lead to pathologic dilation [8]. Dilated cardiomyopathy is
the most common cardiomyopathy, with a variable etiology that may include viral infection,
toxins, parasites, alcohol, peripartum, or autoimmune disease. Up to 25% of idiopathic cases
have a genetic basis and these patients may
have pathologic dilation that precedes systolic
dysfunction [9].
Clinically, patients present with symptoms of
heart failure. The approach to echocardiography
involves assessment of cardiac structures from
standard TTE windows [10, 11]. Diagnostic cri-
teria include evidence of left ventricular systolic dysfunction with EF < 45%, and/or
fractional shortening < 25% in association
with left ventricular end-diastolic dimension > 112% corrected for age and BSA. In
addition to diagnostic criteria, echocardiography
can offer information on the severity and sequelae
of dilated cardiomyopathy. When present, right
ventricular fractional area of change (FAC<35)
indicates RV dysfunction and is predictive of a
worse long-term prognosis [10, 12]. It is common
for valvular pathology to occur in the setting of
dilated cardiomyopathy [13]. Right ventricular
systolic pressures can be derived from tricuspid
regurgitation Doppler velocities and left atrial and
ventricular end-diastolic pressures can be estimated from mitral and aortic regurgitant Doppler
velocities respectively [14]. Left ventricular
thrombus in the setting of poorly functioning
chambers can be identied with the assistance of
contrast [10]. Echocardiography rarely alludes to
the specic cause; thus patient history, family history, and imaging all contribute to the determination of cardiomyopathy etiology (i.e., infectious,
alcoholic, peripartum) (see Figs.16.1 and 16.2).
16 Cardiomyopathies
Fig. 16.1 Apical
four-chamber view of a
patient with dilated
cardiomyopathy (see
Video 16.1)
Fig. 16.2 Parasternal
long-axis view of a
patient with dilated
cardiomyopathy
secondary to
methamphetamine (see
Video 16.2)
213
Additional techniques including, evaluation
with 3D echocardiography, TDI-derived strain
imaging, and 2D-derived speckle-tracking
strain analysis, may help assess ventricular
dysfunction. Treatment remains supportive.
ICD placement and cardiac resynchronization
therapy are commonly used for suitable
patients at risk for life-threatening arrhythmias [15, 16].
Hypertrophic Cardiomyopathy
Hypertrophic cardiomyopathy is a heterogeneous
disease that develops from protein mutations in
the cardiac sarcomere resulting in left ventricular
hypertrophy with disorganized myocardial architecture in the absence of hypertension or valvular
disease [17]. Asymmetric hypertrophy of the septum and anterior wall is the most common vari-
214
J. C. Klick and R. Carlson
ant; however, there are variations including apical
hypertrophy and mid-ventricular hypertrophy
[18]. Diagnostic criteria include [19–21]:
• Unexplained maximal wall thickness>15mm
in any myocardial segment or
• Septal/posterior wall thickness ratio>1.3in
normotensive patients or
• Septal/posterior wall thickness ratio>1.5in
hypertensive patients
Systolic function generally remains preserved
as remodeling occurs; however, the diastolic left
ventricular function gradually is impaired due to
decreased compliance [17]. The presence of septal hypertrophy narrows the left ventricular outow tract (LVOT) and may lead to systolic
anterior motion (SAM) of the mitral valve and
ultimately LVOT obstruction [22]. Up to twothirds of patients display dynamic outow tract
obstruction with evidence of a gradient
>30mmHg. About 25% of patients experience a
resting gradient, while the remainder require
changes in volume and contractility to provoke a
gradient indicating outow tract obstruction [21].
Clinically, patients present with angina, dyspnea, exercise intolerance, or syncope. The
approach to echocardiography involves the
assessment of cardiac structures from standard
TTE windows. It is important to assess interventricular septal width in diastole for asymmetric
septal hypertrophy. Parasternal long- and shortaxis views assess left ventricular wall thickness
from the mitral, papillary, and apical levels,
allowing evaluation of segmental hypertrophy
[19, 21]. Systolic function is qualitatively and
quantitatively normal to supranormal early in the
disease process but may be reduced in the end
stages. Evaluation of the mitral valve in apical
four-chamber and apical ve-chamber views
allows determination of structure and function.
Systolic anterior motion is considered mild if
contact between the anterior mitral leaet and
the septum is <10% in systole and severe if
>30% in systole [21]. In obstructive hypertrophic cardiomyopathy with SAM, a mitral regurgitation jet is usually present and is posteriorly
directed [23]. The apical three- and ve-chamber
views allow for the evaluation of LVOT dynamic
ow gradient. Continuous wave Doppler will
often demonstrate a dagger-like prole with a
peak velocity of 5m/s or a gradient of 100mmHg
in the setting of left ventricular outow tract
obstruction. Wall thickness>3mm or a left ventricular outow gradient >30 mmHg are independent predictors of sudden cardiac death [22]
(see Figs.16.3 and 16.4).
Evolving imaging techniques include using
tissue Doppler imaging, one-dimensional strain
imaging, and two-dimensional speckle tracking.
These have shown evidence of longitudinal
strain, twists, and torsion changes which can be
subtle and emerge early in the disease course for
hypertrophic cardiomyopathy [24–26]. This is
particularly useful in assessing early disease in
family members and identifying patients who
may be at higher risk of developing the phenotype of hypertrophic cardiomyopathy.
16 Cardiomyopathies
Fig. 16.3 Hypertrophic
cardiomyopathy apical
four-chamber
demonstrating SAM (see
Video 16.3)
215
Fig. 16.4 Hypertrophic
cardiomyopathy
parasternal long-axis
view (see Video 16.4)
Restrictive Cardiomyopathies
Restrictive Cardiomyopathy is a cardiac muscle
disease characterized by increased stiffness, elevated end-diastolic pressure, and ultimately diastolic dysfunction of one or both ventricles [27].
Despite abnormal diastolic lling, the systolic
function of the ventricles remains preserved.
Idiopathic restrictive cardiomyopathy is uncommon, however restrictive physiology can be seen
in constrictive pericarditis, and inltrative processes including amyloidosis, hemochromatosis,
216
J. C. Klick and R. Carlson
sarcoidosis, and metabolic disorders [27]. It is
important to identify the restrictive physiology,
and thoroughly assess for its etiology to determine opportunities for intervention [28].
Clinically, patients present with evidence of
heart failure including dyspnea, edema, and elevated jugular venous pressure. Inltrative processes can disrupt the conduction system
resulting in AV conduction delay/block, atrial
brillation, or atrial arrhythmias [27]. The
approach to echocardiography involves looking
at the structure and both systolic and diastolic
functions. Specic attention is paid to diastolic
function. On initial exam, normal ventricular size
and wall thickness are common with biatrial
enlargement [27]. Left ventricular systolic function is typically preserved. Doppler
echocardiography reveals diastolic lling abnormalities. Increased mitral E velocity, increased
ratio of mitral early to late lling (E/A ratio>2),
and shortened deceleration time (<150 ms) are
commonly seen [28]. Tissue Doppler is signicant for decreased early mitral annular velocity
(E′) [14] (see Figs.16.5 and 16.6).
Limitations to dening restrictive cardiomyopathy involve challenges in evaluating dia-
stolic dysfunction. Accurate Doppler evaluation
of diastolic function relies on normal sinus
rhythm, normal PR interval, and varies with the
amount of valvular regurgitation and the phase
of respiration [28–30]. The diastolic lling pattern also changes with disease progression. In
early disease, the LV inow curve shows
reduced E velocity and increased A velocity,
and the pulmonary vein ow curve has reduced
diastolic lling and normal systolic lling. As
the restrictive process progresses, there is
increased E velocity, rapid early diastolic deceleration of the LV inow, and the A velocity is
reduced due to increased diastolic pressure and
reduced atrial contraction. There is normal pulmonary venous inow in diastole with decreased
systolic lling. During atrial contraction, the
increased resistance to left ventricular lling
allows atrial ow reversal in the lower pulmonary veins. In late disease, there is increased E
velocity, decreased A velocity, and steep early
diastolic deceleration. Over time, end-stage
restrictive cardiomyopathy can lead to biventricular enlargement and transition to echocardiographic features of a dilated cardiomyopathy
[31] (see Fig.16.7).
Fig. 16.5 Parasternal
short-axis of a patient
with restrictive
cardiomyopathy
secondary to
amyloidosis (see Video
16.5)
16 Cardiomyopathies
Fig. 16.6 Parasternal
long-axis view of a
patient with restrictive
cardiomyopathy
secondary to
amyloidosis (see Video
16.6)
217
Fig. 16.7 Mitral inow Doppler tracing and tissue Doppler tracing from a patient with restrictive cardiomyopathy
demonstrating restrictive diastology
Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D)
bility and the development of ventricular
arrhythmias. Adrenergic stimulation and catecholamine release associated with exercise are
particularly stimulating for inducing ventricular
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is characterized by
progressive brofatty replacement of the right
ventricular myocardium resulting in right ventricular dilation and dysfunction [32]. ARVC/D
is genetically inherited and remains the second
most common cause of sudden cardiac death in
patients <35years old [33]. The right ventricular structural changes result in electrical insta-
arrhythmias [34].
Patients present with nonspecic symptoms
including chest pain, syncope, and right heart
failure. Sudden death by ventricular arrhythmia
may be the presenting symptom with a diagnosis of ARVC/D postmortem [34]. A 1994
International Task Force created criteria for the
clinical diagnosis of ARVC/D with revision in
2010. These criteria classify structural, histo-
218
J. C. Klick and R. Carlson
logical, electrocardiographic, arrhythmic, and
genetic features with major and minor criteria
based on the specicity of diagnosis [35, 36].
The full criteria for diagnosis of ARVC/D can
be referred to by Marcus etal. and is beyond the
scope of this chapter [36]. Further focus will be
on the specic structural components identied
by echocardiography.
For patients with ventricular arrhythmia and/
or undifferentiated heart failure, TTE assessment
can be used to assess for ARVC/D.Updated 2010
criteria for the echocardiography structural
assessment include [36]:
Major
• Regional RV akinesia, dyskinesia, or aneu-
rysm and
• (One of the following measured at
end-diastole)
– PLAX RVOT >32mm
– PSAX RVOT >36mm
– Fractional area change ≤33%
Minor criteria include
• Regional RV akinesia or dyskinesia and
• (One of the following measured at end-diastole)
– PLAX RVOT ≥29 to <32mm or
– PSAX RVOT ≥32 to <36mm
– Or fractional area change >33% to <40%
(see Fig.16.8)
Of note, only the echocardiography ndings
are summarized in the review above. Full diagnostic criteria for ARVC/D rely on echocardiography, tissue characterization of myocardium,
repolarization/depolarization/conduction abnormalities, arrhythmia, and family history, requiring major and minor criteria from each category.
Please refer to Marcus etal. for a more detailed
description of diagnostic criteria [36].
Beyond these criteria, tricuspid annular plane
systolic excursion (TAPSE), doppler tissue imaging (DTI)-derived tricuspid lateral annular systolic
velocity (S′-wave), RV index of myocardial perfor-
Fig. 16.8 Mid-esophageal four-chamber view of a patient with arrhythmogenic RV dysplasia (see Video 16.7)
16 Cardiomyopathies
219
mance (RIMP), and fractional area change (FAC)
can be helpful to dene the systolic and diastolic
function of the right ventricle [37–39]. TAPSE
<17 mm indicates right ventricular systolic dysfunction focused on right ventricular longitudinal
function. RV wall thinning, aneurysm formation,
outow tract dilation, and trabecular disarray are
inconsistently present [14]. While echocardiographic ndings do not establish a diagnosis for
ARVC/D, when right ventricular dysfunction is
observed and is consistent with these parameters,
ARVC/D remains an important etiology to consider. Treatment includes beta blockers, avoidance
of catecholamine surges (exercise, stress, iatrogenic), and consideration of ICD placement for
those at risk of life-threatening arrhythmias [34].
Stress-Induced Cardiomyopathy
Takotsubo Cardiomyopathy
Initially described in 1990, stress cardiomyopathy or “Takotsubo Syndrome,” is characterized
by transient ventricular systolic and diastolic
dysfunction with gradual recovery in the weeks
and months following diagnosis [40, 41].
Takotsubo refers to a Japanese octopus trap,
which resembles the apical ballooning shape of
the heart when affected by the most common
variant of stress cardiomyopathy [41]. Over 90%
of cases are diagnosed in postmenopausal
women, and a majority experience intense emotional (close death, assault, violence) or physical
(illness, surgery, trauma, stroke, or central nervous system disorder) discomfort prior to presentation [41–43]. Patients present with chest pain,
shortness of breath, or evidence of cardiogenic
shock, particularly in the setting of neurogenic or
other acute illness [43]. Revised criteria by the
Mayo Clinic are widely used for the diagnosis of
stress cardiomyopathy and include [44, 45]:
(a) Transient dyskinesis, hypokinesis, or akine-
sis with abnormalities in regional wall
motion of the left ventricular wall extending
beyond epicardial vascular distribution usually in the presence of a stress trigger
(b) Absence of CAD on angiography
(c) New ECG ST-segment elevation or T wave
inversions
(d) Elevation in cardiac troponin
(e) Absence of pheochromocytoma or myocar-
ditis
Transthoracic echocardiography provides
visualization of left ventricular function on presentation and allows monitoring of the ventricular recovery from the transient dysfunction.
During acute illness, myocardial wall motion
abnormalities exist beyond any specic coronary
artery distribution [42, 46]. The classic Takotsubo
Cardiomyopathy denition of “apical ballooning” involves apical hypokinesis with preserved/
hyperkinetic basal segment wall motion [47].
Two additional patterns of dysfunction involve
mid-ventricular and/or basal wall motion abnormalities [48, 49]. While atypical, they can occur.
Diastolic dysfunction is evident with increased
E/e′ ratio and has been found to be an independent predictor of heart failure and in-hospital
mortality [50]. Two-dimensional strain imaging
depicts decreased longitudinal strain values from
base to apex in stress cardiomyopathy. Contrast
echocardiography also allows for improved visualization of endocardial borders and evaluation of
thrombus formation [44]. Left ventricular outow tract obstruction (LVOTO), acute mitral
regurgitation, and ventricular thrombus with distal embolization are all sequelae of cardiac dysfunction and can be periodically reevaluated
throughout the course of illness by echocardiography [47] (see Fig.16.9).
Stress cardiomyopathy is notable for the rapid
recovery of LVEF; however, there is increased
evidence that some degree of dysfunction remains
despite supposed “normalization” of function.
Months after ejection fraction recovery, patients
may endorse ongoing heart failure symptoms as
well as evidence of dysfunction on strain imaging
with reduced global longitudinal strain, apical
circumferential strain, left ventricular twist, and
untwist [51, 52]. With increased evidence of cardiac dysfunction, despite recovered ejection fraction, the long-term prognosis and follow-up for
these patients continue to evolve [43, 53].
220
Fig. 16.9 Apical
four-chamber view of a
patient with Takotsubo
cardiomyopathy (see
Video 16.8)
J. C. Klick and R. Carlson
Neurogenic Stress Cardiomyopathy
as cardiac dysfunction in patients with end-stage
liver disease of any source [58, 59]. The complex
Cardiac abnormalities can be observed in patients
who experience a severe grade subarachnoid
hemorrhage, traumatic brain injury, stroke, or
central nervous system infection and are considered a Neurogenic Stress Cardiomyopathy (NSC).
One hypothesis attributes etiology to massive catecholamine release and has been compared to the
stress response involved with Takotsubo
Cardiomyopathy. NSC can be asymptomatic or
have symptoms of heart failure, pulmonary
edema, hypotension, or arrhythmia.
Echocardiography is signicant for left ventricular dysfunction with wall motion abnormalities of
pathophysiology of cirrhosis, including hepatocellular dysfunction, portal hypertension, and
portosystemic shunting, results in increased production of vasodilators and resistance to vasoconstrictors [60]. This delicate balance between
the distribution of preload and afterload results in
hyperdynamic cardiac function, low systemic
vascular resistance, and an overall high cardiac
output. Cardiac dysfunction for many patients is
only uncovered during episodes of stress, including infection, surgery, or TIPS procedures, and
can have a signicant impact on morbidity and
mortality [59].
apical, basal, and mid-ventricular walls without
correlation with coronary artery distribution, similar to Takotsubo cardiomyopathy. NSC is more
likely to be associated with severe SAH and predicts cerebral vasospasm. Further research is necessary to determine outcome associations with
NSC.Treatment remains supportive [54, 55].
Cardiomyopathy were developed in 2005 during
the World Congress of Gastroenterology in
Montreal [60]. While the guidelines classied the
diagnosis based on impaired contractility, systolic dysfunction, and electromechanical abnormalities, challenges emerged with identifying
systolic dysfunction for patients in a hyperdynamic state [61]. A new set of guidelines were
Cirrhotic Cardiomyopathy
established in 2019 by the Cirrhotic
Cardiomyopathy Consortium to use updated
There has long been observation of hemodynamic changes in patients with end-stage liver
disease [56, 57]. Initially attributed to alcohol
use, cirrhotic cardiomyopathy is now identied
techniques to assess cardiac function including
strain imaging and tissue Doppler [61, 62].
Systolic function analysis should utilize strain
imaging to assess circumferential, longitudinal,
Diagnostic criteria for Cirrhotic