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Stress (takotsubo) cardiomyopathy
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Stress (takotsubo) cardiomyopathy

Disclosures: Guy S Reeder, MD Nothing to disclose. Abhiram Prasad, MD Nothing to disclose. William J McKenna, MD Nothing to disclose. Susan B Yeon, MD, JD, FACC Nothing to disclose.

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All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Jul 2015. | This topic last updated: Apr 08, 2014.

INTRODUCTION — Stress cardiomyopathy, also called apical ballooning syndrome, broken heart syndrome, takotsubo cardiomyopathy, and stress-induced 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, but in the absence of obstructive coronary artery disease [1-15].

Stress 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 (image 1 and movie 1). In the most commonly described typical type of stress cardiomyopathy, the contractile function of the mid and apical segments of the LV are depressed, and there is hyperkinesis of the basal walls, producing a balloon-like appearance of the distal ventricle with systole. Less common (atypical) variants include ventricular hypokinesis restricted to the mid-ventricle with relative sparing of the apex [17], hypokinesis of the base with sparing of the mid-ventricle and apex (reverse or inverted Takotsubo), and global hypokinesis [18]. About a third of cases involve both right and left ventricles.

Stress cardiomyopathy is much more common in women than men [3,4,7,15,16]. In a review of 10 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 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) [4,5,7-10,19].

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 (LV) mid-cavity and apex are predominantly affected. Although the clinical presentation simulates that of an acute myocardial infarction (MI), coronary arteriography typically shows no obstructive lesions [4,7], and only a minority of patients display coronary spasm with acetylcholine provocation [4]. Studies comparing left ventricular systolic and diastolic function in patients with stress cardiomyopathy to function in patients with acute MI have reached differing conclusions [20,21]. Initial systolic function may be similar [20] or worse [21] with stress cardiomyopathy compared to acute MI, while diastolic function may be similar [20] or better [21] with stress cardiomyopathy.

Postulated mechanisms include catecholamine excess [8,22], coronary artery spasm, and microvascular dysfunction. Alternatively, there may be dynamic mid-cavity or LV outflow tract obstruction which may contribute to apical dysfunction. Analogous permanent (rather than transient) apical outpouchings develop in patients with hypertrophic cardiomyopathy with 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 five of five patients diagnosed with stress cardiomyopathy [23], other IVUS series found no evidence of culprit lesions in the LAD [24,25].

Physical or emotional stress — A number of features of stress cardiomyopathy, including its association with physical or emotional stress [4,5,7-10,19], suggest that this disorder may be caused by diffuse catecholamine-induced microvascular spasm or dysfunction, resulting in myocardial stunning [26], or by direct catecholamine-associated myocardial toxicity [27]. In some patients with stress cardiomyopathy, the only apparent stressor is exposure to catecholamine or beta-agonist drugs in routine clinical doses [28]. (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 [8,29-31]. Combining the results from these series, plasma norepinephrine levels were elevated in 26 of 35 patients (74 percent) [26]. Elevated catecholamine levels and reversible LV ballooning have also been observed in a rat model of immobilization-induced stress [32].

The magnitude of catecholamine excess associated with this disorder was illustrated in a report that measured plasma catecholamine levels in 13 patients with stress cardiomyopathy and seven patients with a Killip class III MI (table 1) [8]. Plasma catecholamines were significantly higher in the patients with stress 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 [33].

Further support for the catecholamine hypothesis is provided by observations of a similar reversible cardiomyopathy with global or focal dysfunction in patients with pheochromocytoma (see "Clinical presentation and diagnosis of pheochromocytoma") [34], and in the setting of acute brain injury, which has also been postulated to be related to catecholamine excess [35]. (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 [4,7,36].

Transient myocardial perfusion abnormalities that resolve with improvement in the myopathy [29].

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 [5,8,27] are consistent with histologic signs of catecholamine toxicity [37,38]. Findings have ranged from no evidence of myocarditis [36] 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 LV dysfunction revealed intracellular accumulation of glycogen, many vacuoles, disorganized cytoskeletal and contractile structure, contraction bands, and increased extracellular matrix proteins [27]. 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 [39]. It is speculated that the greater effect at the apical myocardium may be due to a higher density of beta-adrenoreceptors at this location [40].

Predisposing factors — Since stress cardiomyopathy occurs in a minority of postmenopausal women, it is likely that predisposing factors increase susceptibility in some individuals. 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 [41,42]. However, early genetic analyses have not identified a mutation or polymorphism [43,44]. Moreover, there may be a higher prevalence of chronic anxiety disorders preceding the illness in patients with stress induced cardiomyopathy [45].

Critical illness — The incidence of stress 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 [46]. 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 LV ejection fraction of 33 percent

LV 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) [10,29,30,47], which were included in a larger systematic review [26]. Each of these series included 10 to 16 patients with stress 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 cardiomyopathy is similar to that of an acute myocardial infarction (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 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 intraaortic balloon counterpulsation was used in 0 to 18 percent of patients [15]. Left ventricular outflow tract (LVOT) obstruction, induced by left ventricular basal hyperkinesis produces a late peaking systolic murmur, similar to that heard in patients with hypertrophic cardiomyopathy and can contribute to the development of shock and cause severe mitral regurgitation (see "Auscultation of cardiac murmurs", section on 'Subvalvular outflow obstruction') [48]. Apical thrombus formation and stroke have also been described [28,49].

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 [50]. 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 cardiomyopathy come from relatively small case series [3-5,7-10,29,48,51,52]. A systematic review of 14 series, including a total of 286 patients, provides a more comprehensive assessment of this disorder [26]. 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 [52,53]. 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 electrocardiogram (ECG) is normal at presentation [4,8,51].

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 [26]. In a later series of 136 patients, troponin T levels ranged from 0.01 to 5.2 ng/mL [28].

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 [3-5,7-9,26,29]. Overall systolic function is reduced, and the reported average LVEF has ranged from 20 to 49 percent [4,7,8,26]. Apical sparing variant accounts for a significant proportion of patients, though the clinical characteristics of typical and apical sparing forms appear to be similar [11,35-39]. 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 [26]. In some cases, this is accompanied by systolic anterior motion of the mitral valve, similar to that seen in hypertrophic cardiomyopathy [48].

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 [7,54].

Diagnosis — The diagnosis of stress 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 are usually seen on left ventriculography or echocardiography (image 1 and movie 1) [3-5,7,9,16,29]. 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 be seen in some patients with stress cardiomyopathy given the prevalence of coronary artery disease in the population at risk for stress cardiomyopathy [55,56]. 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 [23]. Transient occlusion in such a vessel, with subsequent spontaneous thrombus lysis, could produce apical stunning and wall-motion abnormalities that would improve over follow-up. However, in one series the prevalence of “wrap-around” left anterior descending artery in stress cardiomyopathy was found to be low (27 percent) and comparable to that in patients diagnosed with anterior ST elevation MI [55].

The following CMRI features may be helpful in the diagnosis of stress cardiomyopathy:

Late gadolinium enhancement (LGE) on CMRI is generally absent in stress cardiomyopathy in contrast to MI in which intense (ie, >5 standard deviations above the mean signal intensity of remote myocardium) subendocardial or transmural LGE is seen [5,11,17,43,44]. LGE is also useful in differentiating stress 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 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 cardiomyopathy. However, myocardial edema is also seen in acute MI 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 [28].

A number of other syndromes in addition to stress 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 [34,35]. These conditions are discussed in detail separately. (See "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' and "Cardiac syndrome X: Angina pectoris with normal coronary arteries".)

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 ECG 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 cardiomyopathy.

An important issue is how the possible diagnosis of stress 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 percutaneous coronary intervention (PCI) should proceed with angiography in the usual manner. If the patient has stress 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 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 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" non-ST elevation MI 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 angiography 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 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 [57]. The wall motion abnormalities in stress cardiomyopathy typically involve the distribution of more than one coronary artery.

Right ventricular involvement — Most reports of stress cardiomyopathy have focused on transient dysfunction of the left ventricle. However, there is emerging evidence that the right ventricle is also affected in some cases [58,59].

The frequency and significance of right ventricular involvement was illustrated in a series of 34 patients with stress cardiomyopathy who underwent CMRI [59]. Nine patients (26 percent) had right ventricular wall motion abnormalities. Patients with right ventricular dysfunction had lower LVEF compared to patients with normal right ventricular function (40 versus 48 percent), and were also more likely to have pleural effusions. At a mean of one year after presentation, follow-up CMRI showed improvement or resolution of right ventricular dysfunction in eight of nine patients.

TREATMENT AND PROGNOSIS — Despite the severity of the acute illness, stress 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 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 (LV) systolic dysfunction. These include angiotensin converting enzyme 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 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 heart failure 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 LV outflow tract (LVOT) obstruction is present, which has been described in 13 to 18 percent of cases [3,48].

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. Intraaortic 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 [7,48].

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 [3,48].

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. The utility of non-catecholamine agents such as vasopressin or terlipressin in this setting is unknown at this time, but these may be considered as alternative vasopressors.

Thromboembolism — The potential risk of intraventricular thrombus formation and systemic embolization should be addressed. Echocardiography (and cardiac magnetic resonance imaging 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 LV 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 myocardial infarction. 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 LV 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 [28]. Patients who survive the acute episode typically recover normal ventricular function within one to four weeks [4,7-9,36]. 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 [60]. 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 cardiomyopathy (The Basics)")

SUMMARY AND RECOMMENDATIONS — Stress cardiomyopathy is an increasingly reported syndrome characterized by transient regional left ventricular (LV) dysfunction in the absence of significant coronary artery disease.

Clinical manifestations and diagnosis

Stress 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 cardiomyopathy may account for approximately 2 percent of suspected acute coronary syndromes. (See 'Prevalence' above.)

Common presenting features include electrocardiographic (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 cardiomyopathy include acute heart failure, cardiogenic shock, transient left ventricular outflow tract (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 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 myocardial infarction, the suspicion of possible stress 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 percutaneous coronary intervention or with fibrinolytic therapy (Grade 1B). (See "Criteria for the diagnosis of acute myocardial infarction" and "Acute ST elevation myocardial infarction: Selecting a reperfusion strategy".)

We approach hemodynamically stable patients diagnosed with stress 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 angiotensin converting enzyme 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 LV 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 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 intraaortic balloon pump (Grade 2C). (See 'Hypotension and shock' above.)

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