Official reprint from UpToDate

www.uptodate.com 2013 UpToDate

Authors
Guy S Reeder, MD
Abhiram Prasad, MD
Section Editor
William J McKenna, MD
Deputy Editor
Susan B Yeon, MD, JD, FACC
Stress-induced (takotsubo) cardiomyopathy
Disclosures
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: May 2013. | This topic last updated: Oct 19, 2012.
INTRODUCTION Stress-induced cardiomyopathy, also called apical ballooning syndrome, broken heart
syndrome, and takotsubo cardiomyopathy, is an increasingly reported syndrome generally characterized by
transient systolic dysfunction of the apical and/or mid segments of the left ventricle that mimics myocardial
infarction (MI), but in the absence of obstructive coronary artery disease [1-15].
Stress-induced cardiomyopathy was first described in Japan [1,2] and was subsequently reported in non-Asian
populations, including the United States [6,7,10] and Europe [9,16].
The term takotsubo is taken from the Japanese name for an octopus trap, which has a shape that is similar to the
apical ballooning configuration of the left ventricle (LV) in systole in the "typical" form of this disorder. In the more
commonly described "typical" type of stress-induced cardiomyopathy, the contractile function of the mid and apical
segments of the LV are depressed, and there is compensatory hyperkinesis of the basal walls, producing a balloon-
like appearance of the distal ventricle with systole. Less commonly (17 percent of cases in one series), the
ventricular hypokinesis is restricted to the midventricle ("atypical") with relative sparing of the apex [17]. About a
third of cases involve both right and left ventricles.
Stress-induced cardiomyopathy is much more common in women than men [3,4,7,15,16]. In a review of ten
prospective series, women accounted for 80 to 100 percent of cases, with a mean age of 61 to 76 years [15].
PATHOGENESIS The onset of stress-induced cardiomyopathy is frequently but not always triggered by an
acute medical illness or by intense emotional or physical stress (eg, death of relatives, particularly if unexpected,
domestic abuse, arguments, catastrophic medical diagnoses, devastating financial or gambling losses, natural
disasters) [4,5,7-10,18].
The pathogenesis of this disorder is not well understood. It is not known why this disorder affects postmenopausal
women disproportionately or why the left ventricular mid-cavity and apex are predominantly affected. Although the
clinical presentation simulates that of an acute MI, coronary arteriography typically shows no obstructive lesions
[4,7], and only a minority of patients display coronary spasm with acetylcholine provocation [4].
Postulated mechanisms include catecholamine excess [8,19], coronary artery spasm, and microvascular
dysfunction. Alternatively, there may be dynamic mid-cavity or left ventricular outflow tract obstruction which may
contribute to apical dysfunction. Analogous permanent (rather than transient) apical outpouchings develop in
patients with hypertrophic cardiomyopathy and mid-ventricular obstruction. (See "Types and pathophysiology of
obstructive hypertrophic cardiomyopathy", section on 'Midcavity obstructive HCM'.)
A potential role for plaque rupture and thrombosis with spontaneous thrombolysis has not been established and the
results of intravascular ultrasound (IVUS) studies are mixed. Although one IVUS study found evidence of mid left
anterior descending (LAD) coronary artery plaque rupture in 5 of 5 patients diagnosed with stress-induced
cardiomyopathy [20], other IVUS series found no evidence of culprit lesions in the LAD [21,22].
Physical or emotional stress A number of features of stress-induced cardiomyopathy, including its association
with physical or emotional stress [4,5,7-10,18], suggest that this disorder may be caused by diffuse catecholamine-
induced microvascular spasm or dysfunction, resulting in myocardial stunning [23], or by direct catecholamine-
associated myocardial toxicity [24]. In some patients with stress-induced cardiomyopathy, the only apparent
stressor is exposure to catecholamine or beta-agonist drugs in routine clinical doses [25]. (See "Clinical
syndromes of stunned or hibernating myocardium".)
Support for a possible pathogenic role for catecholamines comes from studies in which plasma catecholamines
were measured at presentation [8,26-28]. Combining the results from these series, plasma norepinephrine levels
were elevated in 26 of 35 patients (74 percent) [23]. Elevated catecholamine levels and reversible left ventricular
ballooning have also been observed in a rat model of immobilization-induced stress [29].
The magnitude of catecholamine excess associated with this disorder was illustrated in a report that measured
plasma catecholamine levels in 13 patients with stress-induced cardiomyopathy and seven patients with a Killip
class III MI (table 1) [8]. Plasma catecholamines were significantly higher in the patients with stress-induced
cardiomyopathy as compared to those with MI: epinephrine (1264 versus 376 pg/mL) and norepinephrine (2284
versus 1100 pg/mL). However, elevation in blood catecholamine levels is not uniformly present and some studies
have reported normal levels [30].
Further support for the catecholamine hypothesis is provided by observations of a similar reversible cardiomyopathy
with global or focal dysfunction in patients with pheochromocytoma [31] (see "Clinical presentation and diagnosis of
pheochromocytoma"), and in the setting of acute brain injury, which has also been postulated to be related to
catecholamine excess [32]. (See "Cardiac complications of stroke", section on 'Neurogenic cardiac damage'.)
The following observations support the hypothesis of vascular dysfunction that may be catecholamine-induced:
The occasional finding of multifocal coronary vasospasm on coronary angiography [4,7,33].
Transient myocardial perfusion abnormalities that resolve with improvement in the myopathy [26].
The presence of abnormal TIMI frame counts on angiography [10]. The TIMI frame count is the number of
cine frames required for dye to first reach standardized distal coronary landmarks. (See "Fibrinolytic
(thrombolytic) agents in acute ST elevation myocardial infarction: Markers of efficacy", section on 'TIMI frame
count'.)
The following observations support the hypothesis of catecholamine-induced myocardial effects:
Limited available endomyocardial biopsy data [5,8,24] are consistent with histologic signs of catecholamine
toxicity [34,35]. Findings have ranged from no evidence of myocarditis [33] to interstitial fibrosis with or
without slight cellular infiltration [5] to mononuclear infiltrates with contraction band necrosis [8]. In a series
of eight patients, acute biopsies obtained during the period of left ventricular dysfunction revealed intracellular
accumulation of glycogen, many vacuoles, disorganized cytoskeletal and contractile structure, contraction
bands and increased extracellular matrix proteins [24]. These alterations resolved nearly completely after
functional recovery.
In a mouse model, it has been demonstrated that a high level of epinephrine is negatively inotropic due to a
switch from beta-2 adrenoreceptor mediated Gs protein signaling, which is positively inotropic, to Gi protein
signaling which is negatively inotropic [36]. It is speculated that the greater effect at the apical myocardium
may be due to a higher density of beta-adrenoreceptors at this location [37].
Predisposing factors Since stress-induced cardiomyopathy occurs in a minority of postmenopausal women, it
is likely that predisposing factors increase susceptibility in some individuals. Recently, there have been two reports
of familial cases involving two sisters in one family and a mother and daughter in another family raising the
possibility of a genetic predisposition [38,39]. However, early genetic analyses have not identified a mutation or
polymorphism [40,41]. Moreover, there may be a higher prevalence of chronic anxiety disorders preceding the
illness in patients with stress induced cardiomyopathy [42].
Critical illness The incidence of stress-induced cardiomyopathy in a medical intensive care unit (ICU)
population was prospectively evaluated in a series of 92 patients with a non-cardiac diagnosis and no prior history of
cardiac disease [43]. All patients underwent serial echocardiography on admission and on hospital days three and
seven, specifically evaluated for LV apical ballooning. The following findings were reported:
26 patients had LV apical ballooning (21 on admission), with an average left ventricular ejection fraction
(LVEF) of 33 percent.
Left ventricular function normalized in 20 patients at a mean of seven days.
In multivariable analysis, sepsis was the only predictor of LV apical ballooning.
LV apical ballooning predicted lower two-month survival (52 versus 71 percent without this finding).
The high incidence of transient LV apical ballooning in this series requires validation in larger series, but it appears
that this phenomenon is not uncommon in a medical ICU population.
PREVALENCE Some of the best available estimates come from four small series of consecutive patients
presenting with a suspected acute coronary syndrome (ACS) [10,26,27,44], which were included in a larger
systematic review [23]. Each of these series included 10 to 16 patients with stress-induced cardiomyopathy,
accounting for approximately 1.7 to 2.2 percent of cases presenting with suspected ACS. A similar prevalence of
1.2 percent was reported from a registry of 3265 patients with troponin-positive ACS [16].
CLINICAL PRESENTATION The clinical presentation of stress-induced cardiomyopathy is similar to that of an
acute MI [3,4,7]. The most common presenting symptom is acute substernal chest pain, but some patients present
with dyspnea, syncope, shock, or electrocardiographic abnormalities.
Acute complications of stress-induced cardiomyopathy can include heart failure, tachyarrhythmias (including
ventricular tachycardia and ventricular fibrillation), bradyarrhythmias, mitral regurgitation and cardiogenic shock
[3,4,7,9]. In a review of 12 prospective series (with 13 to 88 subjects), pulmonary edema occurred in 0 to 44 percent
of cases and intra-aortic balloon counterpulsation was used in 0 to 18 percent of patients [15]. Left ventricular
outflow tract (LVOT) obstruction, induced by left ventricular basal hyperkinesis, can contribute to the development of
shock and cause severe mitral regurgitation or the murmur of hypertrophic cardiomyopathy (see "Auscultation of
cardiac murmurs", section on 'Subvalvular outflow obstruction') [45]. Apical thrombus formation and stroke have also
been described [25,46].
A preliminary report proposed a risk score to predict the likelihood of acute heart failure based on the presence or
absence of the following three variables: age >70 years, presence of a physical stressor, and left ventricular ejection
fraction (LVEF) <40 percent [47]. In the development cohort of 118 patients, the likelihood of developing acute heart
failure was <10 percent in the absence of these risk factors. With one, two, or three risk factors present, the risk
was approximately 28, 58, and 85 percent, respectively. A similar gradient of risk was observed when the risk score
was applied to the 52 patients in the validation cohort.
Data on the characteristics of stress-induced cardiomyopathy come from relatively small case series [3-5,7-
10,26,45,48,49]. A systematic review of 14 series, including a total of 286 patients, provides a more comprehensive
assessment of this disorder [23]. The range of findings from these reports is illustrated by the following:
Electrocardiographic abnormalities are the most common finding. ST segment elevation was present in 34 to
56 percent of patients in the systematic review [49,50]. Among these patients, ST segment elevation was
most common in the anterior precordial leads. The remaining patients had deep T wave inversion with QT
interval prolongation, abnormal Q waves, non-specific abnormalities, and in some cases the ECG is normal
at presentation [4,8,48].
Cardiac biomarker levels, particularly high-sensitivity troponin assays, are usually elevated. However, the
elevations are typically mild, which contrasts with the often severe hemodynamic compromise. In the
systematic review, among studies that measured cardiac troponins or creatine kinase MB, these levels were
elevated in 86 and 74 percent of patients, respectively [23]. In a later series of 136 patients, troponin T levels
ranged from 0.01 to 5.2 ng/mL [25].
Left ventriculography or echocardiography usually show the characteristic apical ballooning with akinesis or
dyskinesis of the apical one-half to two-thirds of the LV [3-5,7-9,23,26]. Overall systolic function is reduced,
and the reported average LVEF has ranged from 20 to 49 percent [4,7,8,23]. Apical sparing variant accounts
for a significant proportion of patients, though the clinical characteristics of typical and apical sparing forms
appear to be similar [11,32-36]. No obvious mechanism for the different patterns of regional wall motion
abnormality has been identified.
In the systematic review, transient LVOT obstruction was reported in 16 percent of the patients (21 of 133)
who underwent left ventriculography [23]. In some cases, this is accompanied by systolic anterior motion of
the mitral valve, similar to that seen in hypertrophic cardiomyopathy [45].
Reversible perfusion abnormalities in the left ventricular apex have been documented [3,5], while
cardiovascular magnetic resonance imaging (CMRI) typically shows mid and apical LV segmental wall
motion abnormalities, often in multiple coronary territories without delayed hyperenhancement of involved
regions [7,51].
Diagnosis The diagnosis of stress-induced cardiomyopathy should be suspected in postmenopausal women
who present with an acute coronary syndrome after intense psychologic stress in whom the clinical manifestations
and ECG abnormalities are out of proportion to the degree of elevation in cardiac biomarkers [11]. Apical ballooning
(typical variant) and/or midventricular hypokinesis is usually seen on left ventriculography or echocardiography [3-
5,7,9,16,26]. In a minority of cases, the transient left ventricular hypokinesis is restricted to the midventricular
segments ("atypical variant" or apical sparing variant) without involvement of the apex [16]. In one series of 256
patients, 82 percent were apical, 17 percent midventricular, and 1 percent basal, with 34 percent of cases
demonstrating right ventricular involvement [17].
Coronary angiography typically demonstrates either normal vessels or mild to moderate coronary atherosclerosis.
Obstructive coronary artery disease may rarely coexist by virtue of its prevalence in the population at risk [52].
Some investigators have hypothesized that stress cardiomyopathy is not a distinct clinical entity, but rather a
manifestation of aborted anterior MI in patients with a long wrap around left anterior descending artery [20].
Transient occlusion in such a vessel, with subsequent spontaneous thrombus lysis, could produce apical stunning
and wall-motion abnormalities that would improve over followup. However, in one series the prevalence of wrap-
around left anterior descending artery in stress-induced cardiomyopathy was found to be low (27 percent) and
comparable to that in patients diagnosed with anterior ST elevation MI [52].
The following cardiac magnetic resonance (CMR) imaging features may be helpful in the diagnosis of stress-
induced cardiomyopathy:
Late gadolinium enhancement (LGE) on CMR is generally absent in stress-induced cardiomyopathy in
contrast to myocardial infarction in which intense (ie, >5 standard deviations above the mean signal intensity
of remote myocardium) subendocardial or transmural LGE is seen [5,11,17,40,41]. LGE is also useful in
differentiating stress-induced cardiomyopathy from myocarditis, which is characterized by patchy late
gadolinium enhancement. However, when a low threshold for LGE is used (eg, three standard deviations
above the mean signal intensity of remote myocardium), LGE is occasionally detected in stress-induced
cardiomyopathy [17]. (See "Clinical utility of cardiovascular magnetic resonance imaging", section on 'Late
gadolinium enhancement'.)
CMR evidence of myocardial edema is commonly seen in stress-induced cardiomyopathy. However,
myocardial edema is also seen in acute myocardial infarction and myocarditis. In one series, 81 percent of
patients had evidence of focal myocardial edema on CMR and these regions corresponded to areas of wall
motion abnormality [17].
CMR may also enable identification of thrombus in the left or right ventricle, which may not be detected by
echocardiography [25].
A number of other syndromes in addition to stress-induced cardiomyopathy have been associated with ST segment
changes in the absence of significant coronary artery disease, including cardiac syndrome X, variant (Prinzmetal's)
angina, myocarditis, and cocaine abuse. As noted above, similar reversible cardiomyopathy with global or focal
dysfunction has been observed in patients with pheochromocytoma and in the setting of acute brain injury [31,32].
These conditions are discussed in detail separately. (See "Cardiac syndrome X: Angina pectoris with normal
coronary arteries", section on 'Acute coronary syndrome' and "Variant angina" and "Clinical manifestations and
diagnosis of myocarditis in adults" and "Evaluation and management of the cardiovascular complications of cocaine
abuse" and "Clinical presentation and diagnosis of pheochromocytoma" and "Cardiac complications of stroke",
section on 'Neurogenic cardiac damage'.)
The following are the proposed Mayo Clinic diagnostic criteria, all four of which are required for the diagnosis [3,14]:
Transient hypokinesis, akinesis or dyskinesis of the left ventricular mid segments with or without apical
involvement. The regional wall motion abnormalities typically extend beyond a single epicardial coronary
distribution. A stressful trigger is often, but not always present.
Absence of obstructive coronary disease or angiographic evidence of acute plaque rupture.
New electrocardiographic abnormalities (either ST-segment elevation and/or T wave inversion) or modest
elevation in cardiac troponin.
Absence of pheochromocytoma or myocarditis.
There are rare exceptions to these criteria such as patients in whom the regional wall motion abnormality is limited
to a single coronary territory, and the occasional patient with obstructive coronary atherosclerosis who develops
stress-induced cardiomyopathy.
An important issue is how the possible diagnosis of stress-induced cardiomyopathy should influence the evaluation
of a suspected acute coronary syndrome. We suggest the following approach:
Patients presenting with ST elevation who can undergo urgent cardiac catheterization for the purpose of
primary PCI should proceed with angiography in the usual manner. If the patient has stress-induced
cardiomyopathy, angiographic findings will suggest the diagnosis by showing no critical coronary disease
and the presence of apical ballooning (or mid wall hypokinesis) on left ventricular angiography. (See "Primary
percutaneous coronary intervention in acute ST elevation myocardial infarction: Determinants of outcome".)
Patients with ST elevation meeting criteria for reperfusion therapy presenting in a setting without availability
of urgent angiography and PCI are usually treated with fibrinolytic therapy. In such cases, suspicion of the
diagnosis of stress-induced cardiomyopathy is not a sufficient reason to withhold fibrinolytic therapy since
the majority of patients with acute ST elevation will have a critical coronary lesion. (See "Fibrinolytic therapy
in acute ST elevation myocardial infarction: Initiation of therapy".)
In the later scenario, the diagnosis of stress-induced cardiomyopathy may later be suggested by such clinical
features as the absence of critical stenoses on coronary angiography, modest cardiac enzyme elevations and
recovery of LV function. However, none of these features is diagnostic, as they may also reflect successful early
fibrinolysis.
Patients who present without ST elevation will usually fit into a "high risk" NSTEMI profile (positive troponin,
elderly, significant left ventricular dysfunction). Early (less than 48 hours) cardiac catheterization will be
performed in most such patients, which should suggest the correct diagnosis. (See "Coronary arteriography
and revascularization for unstable angina or non-ST elevation acute myocardial infarction".)
Patients for whom both cardiac catheterization and thrombolytic therapy are relatively contraindicated are
problematic. In these patients, the pattern of wall motion abnormality may differ between individuals with
stress-induced cardiomyopathy and those with acute obstruction of flow in the left anterior descending
coronary artery. The former had significantly more dysfunction of the right ventricular free and left ventricular
lateral walls than the latter in a small, retrospective echocardiographic study [53]. The wall motion
abnormalities in stress-induced cardiomyopathy typically involve the distribution of more than one coronary
artery.
Right ventricular involvement Most reports of stress-induced cardiomyopathy have focused on transient
dysfunction of the left ventricle. However, there is emerging evidence that the right ventricle (RV) is also affected in
some cases [54,55].
The frequency and significance of RV involvement was illustrated in a series of 34 patients with stress-induced
cardiomyopathy who underwent cardiac magnetic resonance (CMR) imaging [55]. Nine patients (26 percent) had
RV wall motion abnormalities. Patients with RV dysfunction had lower LV ejection fractions compared to patients
with normal RV function (40 versus 48 percent), and were also more likely to have pleural effusions. At a mean of
one year after presentation, follow-up CMR showed improvement or resolution of RV dysfunction in eight of nine
patients.
TREATMENT AND PROGNOSIS Despite the severity of the acute illness, stress-induced cardiomyopathy is a
transient disorder managed with supportive therapy. Conservative treatment with resolution of the physical or
emotional stress usually results in rapid resolution of symptoms.
General therapy Once the diagnosis of stress-induced cardiomyopathy has been made, therapy is based upon
the patient's overall clinical condition. There are no controlled data to define the optimal medical regimen, but it is
reasonable to treat these patients with standard medications for left ventricular systolic dysfunction. These include
ACE inhibitors, beta blockers, and diuretics as necessary for volume overload [3]. Aspirin is also suggested in the
presence of coexisting coronary atherosclerosis [3,14]. (See "Overview of the therapy of heart failure due to systolic
dysfunction".)
Stress-induced cardiomyopathy is a transient disorder. In the absence of clinical trial data, the appropriate duration
of therapy is not known. We usually treat patients with the standard HF medical regimen until there is recovery of
systolic function which occurs in one to four weeks in most cases. However, because the condition may recur, we
often continue adrenergic blockade with either beta-blockers or combined alpha and beta-blockers indefinitely in the
absence of contraindications or intolerance.
Hypotension and shock Patients who are in shock should undergo urgent echocardiography to determine if left
ventricular outflow tract (LVOT) obstruction is present, which has been described in 13 to 18 percent of cases
[3,45].
Without left ventricular outflow tract obstruction Patients without significant LVOT obstruction who are
hypotensive due to pump dysfunction can be treated cautiously with inotropes such as dobutamine and dopamine.
Since the condition is potentially caused by catecholamine excess, the impact of sympathomimetics remains to be
established. In a patient with hypotension due to pump failure, inotropic agents may induce LVOT obstruction, but
the degree is usually mild [7]. A change in the therapeutic approach is not necessary if mild LVOT obstruction
develops. Intra-aortic balloon counterpulsation (IABP) is the preferred therapy when there is marked LV dysfunction
associated with severe hypotension or shock [7].
With left ventricular outflow tract obstruction In contrast to hypotension due only to pump failure,
hypotension associated with LVOT obstruction should NOT be treated with inotropic agents, because they can
worsen the degree of obstruction [7,45].
The recommended approach to patients with moderate-to-severe LVOT obstruction includes the use of beta
blockers, which can improve hemodynamics by causing resolution of the obstruction. (See "Medical therapy in
hypertrophic cardiomyopathy".) In addition, in the absence of significant pulmonary congestion, the patient should
be fluid resuscitated [3,45].
As with patients in shock due to pump failure, those with LVOT obstruction may benefit from an IABP, although
there is a slight risk that afterload reduction from the IABP will worsen the degree of obstruction [7]. The initial
treatment in such patients, however, should be to treat the underlying pathophysiological mechanisms with fluid
replacement, beta blockers, and other negative inotropic agents. In patients with hypotension, whether with or
without significant LVOT obstruction, who do not respond to initial medical therapy and volume resuscitation, we
suggest the use of an IABP.
In patients with LVOT obstruction and severe hypotension who either do not tolerate or do not adequately respond
to beta blockers, an alpha agonist may be added with caution and close monitoring. Phenylephrine is a pure alpha-
adrenergic agonist that may reduce the gradient by increasing afterload, thereby improving overall hemodynamics.
This treatment may be helpful to support blood pressure while a beta blocker is administered to reduce inotropy.
However, the vasoconstrictive effects of alpha agonists may be harmful, particularly in these patients who can be
prone to coronary vasospasm. Thus, if phenylephrine is used, it should be done with a high degree of caution and
very close monitoring of hemodynamics and tissue perfusion.
Thromboembolism The potential risk of intraventricular thrombus formation and systemic embolization should
be addressed. Echocardiography (and CMR if available) should include evaluation for potential thrombus as well as
assessment of the extent of wall motion abnormality.
Data are scant to identify suitable criteria for use of anticoagulation to prevent thromboembolism in patients with
stress cardiomyopathy.
Indirect data are available from randomized trials of anticoagulation to prevent left ventricular thrombus in
patients with acute myocardial infarction. As discussed in detail separately, these trials found that about 10
days of anticoagulation reduced the incidence of LV thrombus. (See "Left ventricular thrombus after acute
myocardial infarction", section on 'Prevention of formation'.)
The use of anticoagulation to prevent embolization in patients with known LV thrombus is supported by
indirect data from observational studies in patients with LV thrombus after MI. In these studies,
anticoagulation during a period of four to six months was associated with a reduced rate of embolization.
(See "Left ventricular thrombus after acute myocardial infarction", section on 'Prevention of embolization'.)
We recommend approximately three months of anticoagulation if intraventricular thrombus is detected. The duration
of anticoagulation may be modified based on the rate of recovery of cardiac function and resolution of the thrombus.
For patients without thrombus but with severe left ventricular dysfunction, we suggest anticoagulation until akinesis
or dyskinesis has resolved or for three months, whichever is shorter.
Prognosis Reported in-hospital mortality rates have ranged from 0 to 8 percent [3,4,7,15]. In a large series of
136 patients, there were three in-hospital deaths [25]. Patients who survive the acute episode typically recover
normal ventricular function within one to four weeks [4,7-9,33]. In two series, for example, the mean LVEF
increased from 29 percent at presentation to 63 percent at a mean of six days [7] and from a median of 20 percent
at presentation to 60 percent at two to four weeks [8].
The longest follow-up data comes from a study of 100 patients [56]. Over a mean follow-up of 4.4 4.6 years, 31
patients continued to have episodes of chest pain and 10 patients had recurrence of apical ballooning syndrome.
Seventeen patients died, but there was no difference in survival compared to an age- and gender-matched
population.
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Basics topic (see "Patient information: Stress-induced cardiomyopathy (The Basics)")
SUMMARY AND RECOMMENDATIONS Stress-induced cardiomyopathy is an increasingly reported syndrome
characterized by transient regional LV dysfunction in the absence of significant coronary artery disease.
Clinical manifestations and diagnosis
Stress-induced cardiomyopathy is typically triggered by an acute medical illness or by intense emotional or
physical stress, although a triggering event is not always present. Postulated pathogenic mechanisms
include catecholamine excess, multivessel coronary artery spasm, and microvascular dysfunction. (See
'Pathogenesis' above.)
Stress-induced cardiomyopathy may account for approximately 2 percent of suspected acute coronary
syndromes. (See 'Prevalence' above.)
Common presenting features include ECG abnormalities (often anterior ST elevations), elevated cardiac
biomarkers, substernal chest pain, and dyspnea. (See 'Clinical presentation' above.)
Proposed diagnostic criteria include presence of transient regional wall motion abnormalities (typically not in
a single coronary distribution), absence of angiographic evidence of obstructive coronary disease or acute
plaque rupture, presence of new ECG abnormalities or modest troponin elevation, AND absence of
pheochromocytoma or myocarditis. (See 'Diagnosis' above.)
Acute complications of stress-induced cardiomyopathy include acute heart failure, cardiogenic shock,
transient LVOT obstruction, tachyarrhythmias, and bradyarrhythmias (See 'Clinical presentation' above.)
In-hospital mortality is approximately 2 percent. Patients who survive the acute episode typically recover
normal LV function within one to four weeks. (See 'Prognosis' above.)
Management The initial management of stress-induced cardiomyopathy is largely supportive, including
hydration and an attempt to alleviate the triggering physical or emotional stress. The role of additional medications
and the appropriate duration of therapy are not established. Most experts favor at least the short-term use of
standard medications for heart failure due to systolic dysfunction. (See "Overview of the therapy of heart failure due
to systolic dysfunction".)
In patients who present with a clinical picture consistent with an ST elevation MI, the suspicion of stress-
induced cardiomyopathy is not a reason to alter management. The significant majority of these cases are
due to occlusion of a coronary artery and revascularization therapy should not be delayed. We recommend
that such patients be managed in the conventional manner, either with urgent catheterization and PCI or with
fibrinolytic therapy (Grade 1B). (See "Criteria for the diagnosis of acute myocardial infarction" and "Selecting
a reperfusion strategy for acute ST elevation myocardial infarction".)
We approach hemodynamically stable patients diagnosed with stress-induced cardiomyopathy in the following
manner (see 'General therapy' above):
We suggest the initiation of a beta blocker (Grade 2C).
In patients who do not have an LVOT gradient, we suggest the initiation of an ACE inhibitor or an angiotensin
receptor blocker (Grade 2C).
In patients with heart failure who do not have an LVOT gradient, we suggest diuresis (Grade 2C).
We suggest treatment with aspirin in the presence of coexisting coronary atherosclerosis (Grade 2C).
We recommend anticoagulation if intraventricular thrombus is detected (Grade 1B). We typically
anticoagulate for three months. The duration of anticoagulation may be modified based on the rate of
recovery of cardiac function and resolution of the thrombus.
For patients without thrombus but with severe left ventricular dysfunction, we suggest anticoagulation until
the akinesis or dyskinesis has resolved or for three months, whichever is shorter (Grade 2C).
Hypotension Some patients with stress-induced cardiomyopathy will develop hypotension and shock, which
could be due either to severe systolic dysfunction or to LVOT obstruction. Because it will influence the choice of
treatment, patients who develop severe hypotension should undergo urgent echocardiography to determine if LVOT
obstruction is present.
In patients with hypotension, whether with or without significant LVOT obstruction, if significant pulmonary
congestion is not present we suggest cautious fluid resuscitation (Grade 2C). (See 'Hypotension and shock'
above.)
In patients with significant hypotension who do not have significant outflow obstruction, we suggest
intravenous inotropes, such as dopamine (Grade 2C). (See 'Hypotension and shock' above.)
In patients with hypotension and moderate-to-severe LVOT obstruction, we suggest that inotropic agents not
be used because they can worsen the degree of obstruction (Grade 2C). (See 'Hypotension and shock'
above.)
In patients with hypotension and moderate-to-severe LVOT obstruction, we suggest beta blockers, which
can improve hemodynamics by causing resolution of the obstruction (Grade 2C). In patients with LVOT
obstruction and severe hypotension who either do not tolerate or do not adequately respond to beta
blockers, an alpha agonist may be added with caution and close monitoring. (See 'Hypotension and shock'
above.)
In patients with hypotension, whether with or without significant LVOT obstruction, who do not respond to
initial medical therapy and volume resuscitation, we suggest the use of an IABP (Grade 2C). (See
'Hypotension and shock' above.)
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Topic 3494 Version 6.0
GRAPHICS
Killip classification of acute myocardial infarction
Class
I
No evidence of heart failure
Class
II
Findings consistent with mild to moderate heart failure (S3 gallop, lung rales less
than one-half way up the posterior lung fields, or jugular venous distension)
Class
III
Overt pulmonary edema
Class
IV
Cardiogenic shock