Atypical takotsubo cardiomyopathy: dobutamine-precipitated apical ballooning with left ventricular outflow tract obstruction

Takotsubo cardiomyopathy, or transient apical ballooning syndrome, is a recently recognized form of transient left ventricular dysfunction that is presumably caused by stress. Takotsubo cardiomyopathy can clinically resemble an acute coronary syndrome. Reported cases have been preceded by emotional stress or medical illness. Herein, we report a fatal case of takotsubo cardiomyopathy that followed a dobutamine stress test. We believe that the dobutamine infusion led to stress-induced cardiomyopathy with a dynamic left ventricular outflow tract obstruction. To our knowledge, there is only 1 other report of an association between dobutamine infusion and the development of takotsubo cardiomyopathy.     

Recurrent long QT syndrome and syncope in transient apical ballooning syndrome (takotsubo cardiomyopathy)

Transient apical ballooning syndrome, or takotsubo cardiomyopathy is a syndrome characterized by reversible dilation of the left ventricular apex. It usually occurs in response to stress, and resolves completely in a few weeks. The present report describes a 55-year-old woman who presented with chest pain and syncope in response to emotional stress. Her electrocardiogram suggested acute coronary syndrome with prolonged QT. However, cardiac catheterization showed normal coronary arteries, with apical akinesis of the left ventricle. The patient’s symptoms recurred 18 months later; subsequently, she was diagnosed with recurrent takotsubo cardiomyopathy with prolonged QT and syncope. The treatment, etiology and pathophysiology of takotsubo cardiomyopathy are discussed.     

Repeat recurrence of takotsubo cardiomyopathy related to inhaled beta-2-adrenoceptor agonists

Takotsubo cardiomyopathy (also referred to as transient apical ballooning syndrome, broken heart syndrome or stress cardiomyopathy) is an increasingly recognized entity in the western world typically characterized by reversible left ventricular dysfunction that develops in the setting of acute severe emotional or physical stress. Increased catecholamine levels have been proposed to play a central role in the pathogenesis of the disease, although the specific pathophysiology of this condition remains elusive at the present moment. In recent times, there have been reports of takotsubo cardiomyopathy (TC) following medical interventions such as invasive or surgical procedures or specific medical regimens. In the current report, we present a patient with multiple recurrences of TC triggered by the same medical therapeutic intervention; in our particular case, repetitive exposure to inhaled beta-2-adrenoceptor agonist.     

Recurrent Takotsubo Cardiomyopathy Related to Recurrent Thyrotoxicosis

Takotsubo cardiomyopathy, or transient left ventricular apical ballooning syndrome, is characterized by acute left ventricular dysfunction caused by transient wall-motion abnormalities of the left ventricular apex and mid ventricle in the absence of obstructive coronary artery disease. Recurrent episodes are rare but have been reported, and several cases of takotsubo cardiomyopathy have been described in the presence of hyperthyroidism. We report the case of a 55-year-old woman who had recurrent takotsubo cardiomyopathy, documented by repeat coronary angiography and evaluations of left ventricular function, in the presence of recurrent hyperthyroidism related to Graves disease. After both episodes, the patient''s left ventricular function returned to normal when her thyroid function normalized. These findings suggest a possible role of thyroid-hormone excess in the pathophysiology of some patients who have takotsubo cardiomyopathy.     

Takotsubo cardiomyopathy in a patient with pulmonary embolism

Takotsubo cardiomyopathy is an acute cardiac syndrome characterized by transient regional wall motion abnormalities of the left ventricular apex or midventricle. Patients often present with chest pain or dyspnoea, ST-segment elevation, and minor elevations of cardiac enzyme levels. Takotsubo cardiomyopathy has been associated with severe emotional or physical stress such as severe burns, spinal cord injury, subarachnoid haemorrhage, and multiple traumas. We report a case of takotsubo cardiomyopathy in a 79-year-old woman with pulmonary embolism. Although pulmonary embolism has been listed as a potential cause of takotsubo cardiomyopathy, this is the first case reported with this association.     

Acute stress cardiomyopathy

A unique syndrome of heart failure and transient left ventricular systolic dysfunction precipitated by acute emotional or physical stress has recently emerged in the medical literature. The syndrome is referred to by several names, including stress cardiomyopathy, takotsubo cardiomyopathy, left ventricular apical ballooning syndrome, and broken heart syndrome. Because most patients with stress cardiomyopathy present with chest pain, electrocardiographic abnormalities, elevated cardiac enzymes, and focal left ventricular wall motion abnormalities, it is not surprising that for years this syndrome went relatively unrecognized because physicians mistook it for acute myocardial infarction. As reports of this condition have increased worldwide during the past 5 years, it has become clear that stress cardiomyopathy has unique clinical features that can be readily distinguished from those of an acute myocardial infarction. This article reviews the clinical features of stress cardiomyopathy and discusses potential pathophysiologic mechanisms of this disorder.     

Inverted takotsubo cardiomyopathy

Takotsubo cardiomyopathy is a transient acute left ventricular dysfunction characterized by left ventricular apical akinesis and ballooning without obstructive coronary disease described predominantly in post-menopausal women in the setting of acute emotional or physical stress. Recent reports have described isolated transient basal akinesis (inverted takotsubo cardiomyopathy) in mostly female patients with acute neurologic disorders or pheochromocytoma. We describe a rare case of a 78-year-old male with inverted takotsubo cardiomyopathy in the setting of acute abdominal pain attributed to biliary colic. A review of published literature reveals that inverted takotsubo cardiomyopathy precipitated by acute stress rather than an acute neurologic disorder appears to be an extremely rare presentation in a male patient. We discuss the relevant literature regarding incidence and reported gender distribution of inverted takotsubo cardiomyopathy.     

Systematic review: transient left ventricular apical ballooning: a syndrome that mimics ST-segment elevation myocardial infarction

The transient left ventricular apical ballooning syndrome, also known as takotsubo cardiomyopathy, is characterized by transient wall-motion abnormalities involving the left ventricular apex and mid-ventricle in the absence of obstructive epicardial coronary disease. In this paper, we review case series that report on patients with the transient left ventricular apical ballooning syndrome to better characterize patients presenting with the syndrome. We identified 7 case series that reported on at least 5 consecutive patients with the transient left ventricular apical ballooning syndrome. The syndrome more often affects postmenopausal women (82% to 100%) (mean age, 62 to 75 years). Patients commonly present with ST-segment elevation in the precordial leads, chest pain, relatively minor elevation of cardiac enzyme and biomarker levels, and transient apical systolic left ventricular dysfunction despite the absence of obstructive epicardial coronary disease. An episode of emotional or physiologic stress frequently precedes presentation with the syndrome. The in-hospital mortality rate seems to be low, as does the risk for recurrence.     

Takotsubo cardiomyopathy, or broken-heart syndrome

Takotsubo cardiomyopathy mimics acute coronary syndrome and is accompanied by reversible left ventricular apical ballooning in the absence of angiographically significant coronary artery stenosis. In Japanese, "takotsubo" means "fishing pot for trapping octopus," and the left ventricle of a patient diagnosed with this condition resembles that shape. Takotsubo cardiomyopathy, which is transient and typically precipitated by acute emotional stress, is also known as "stress cardiomyopathy" or "broken-heart syndrome."Herein, we describe the clinical angiographic characteristics of 4 patients who exhibited this syndrome, and we review the existing literature and propose reasons to conduct prospective studies.     

Reverse takotsubo cardiomyopathy: after an episode of serotonin syndrome

Stress-induced cardiomyopathy is characterized by transient left ventricular dysfunction, usually followed by complete resolution. It is precipitated by severe stress, and the most common variant (takotsubo) is marked by apical hypokinesis and ballooning with basal hyperkinesis. Serotonin syndrome is best understood as excess serotonergic activity in the central and peripheral nervous system. This imposes significant stress on the body. We report what we believe is the 1st case of serotonin syndrome as an indirect cause of stress-induced cardiomyopathy with a reverse takotsubo profile.     

Acute subarachnoid haemorrhage as a precipitant for takotsubo cardiomyopathy: a case report and discussion

Takotsubo cardiomyopathy is a recently recognised acute cardiac entity. It involves transient left ventricular apical ballooning, which is usually preceded by an episode of physiological or emotional stress. Its presentation may mimic acute myocardial infarction but there is no evidence of obstructive disease at coronary angiography. The aetiology of this condition has not been clearly defined, though a number of hypotheses have been put forward. Precipitating factors vary widely, including acute medical conditions and emotional stressors. We present a case of takotsubo cardiomyopathy precipitated by acute subarachnoid haemorrhage, the first report of this association in a Caucasian patient, and discuss the implications for clinicians involved in the management of such a case.     

Not takotsubo: a different form of stress-induced cardiomyopathy--a case series

The diagnosis of stress-induced cardiomyopathy requires wall motion abnormality, most typically apical ballooning. The authors report 3 cases of transient global left ventricular systolic dysfunction that developed over the course of severe medical illnesses. The cardiomyopathy that developed in each patient had all the features of takotsubo cardiomyopathy, including reversibility, mild troponin elevation, nonspecific electrocardiographic abnormalities, and a negative work-up for ischemia. The only difference was the absence of regional wall motion abnormalities. No patients developed sepsis or myocarditis. Stress-induced cardiomyopathy may present in different forms, including regional or global left ventricular dysfunction. Classic takotsubo syndrome may represent only part of the spectrum of this reversible condition.     

Takotsubo Syndrome (Stress Cardiomyopathy): An Intriguing Clinical Condition in Search of Its Identity

Takotsubo syndrome is a relatively frequent clinical entity presenting typically as an acute coronary syndrome in the absence of obstructive coronary artery disease and characterized angiographically by transient left ventricular systolic dysfunction, sparing the basal segments of the left ventricle (“apical ballooning”). Takotsubo syndrome characteristically affects peri- or postmenopausal women, albeit recent series show that men also are at risk. Takotsubo syndrome is characteristically triggered by severe emotional or physical stress, which suggests a pathogenic role for increased sympathetic activity leading to myocardial perfusion abnormalities and ventricular dysfunction. The reasons why severe emotional and physical stress result in the development of takotsubo syndrome in certain individuals but not others is still a matter of speculation, but strongly suggests the existence of predisposing factors/mechanisms in certain subjects. The present article reviews the different factors that can play a role in the development of takotsubo syndrome in different patients. We propose that triggers (ie, emotional stressors, physical stressors, iatrogenic stressors, and neurologic triggers), pathogenic mechanisms (ie, increased catecholamine levels, coronary vasomotor abnormalities leading to myocardial ischemia), and predisposing factors (ie, cardiovascular risk factors, endothelial dysfunction, comorbidities) all interact in a complex fashion and possibly differently in different patients to cause takotsubo syndrome. Identifying these factors may help in preventing and managing the condition more effectively.     

Inverted Takotsubo Cardiomyopathy and the Fundamental Diagnostic Role of Echocardiography

Takotsubo cardiomyopathy is characterized by the development of transient focal wall-motion abnormalities that involve the apical and midventricular segments, in the absence of obstructive coronary artery disease. A variant, inverted takotsubo cardiomyopathy, was described in 2010. We report 3 cases in which each patient''s transthoracic echocardiogram revealed the characteristic basal and midventricular segmental akinesis of this variant. This pattern is not associated with coronary artery distribution, and it therefore can be differentiated from coronary artery disease with the use of echocardiography, by evaluating the distribution and temporal changes of akinetic areas.Key words: Cardiomyopathies/diagnosis/physiopathology, echocardiography, takotsubo cardiomyopathy/diagnosis, ventricular dysfunction, left/diagnosisTakotsubo cardiomyopathy is a syndrome characterized by transient apical and midventricular akinesis that is typically precipitated by acute stress. A variant, with akinesis of the mid and basal left ventricular (LV) segments and sparing of the apex, has been called inverted (or atypical) takotsubo cardiomyopathy. We describe 3 cases of this variant in which the transthoracic echocardiograms (TTEs) and temporal evolution of the condition were diagnostic. Each patient was emergently admitted to Barnes-Jewish Hospital in St. Louis and examined there; the TTEs were reviewed by a visiting cardiologist (AM).     

The "broken heart syndrome": State of the art

Takotsubo cardiomyopathy is a reversible condition, characterized by transient left ventricular systolic dysfunction, that mimics an acute coronary syndrome. It usually occurs after physical or emotional stress, predominantly in postmenopausal women, although it also can affect younger age groups and males. It often presents as chest pain or dyspnea with electrocardiographic changes and mild elevation of cardiac enzymes suggesting acute myocardial infarction. Coronary angiography excludes obstructive coronary disease, and imaging reveals ventricular apical akinesia and compensatory hypercontractility of the basal segments. Various pathophysiological mechanisms have been proposed for the syndrome, such as occult atherosclerotic disease, multivessel spasm and/or microvascular dysfunction. However, the most widely accepted hypothesis at present is an excess of catecholamines causing calcium overload in cardiac myocytes, leading to disruption of contraction and ventricular function. Treatment is essentially supportive, with spontaneous and complete reversal of the changes within days or weeks. However, the presence of complications and comorbidities may predict a more adverse prognosis. As much is still unknown about takotsubo cardiomyopathy and the number of reported cases is growing, we present a literature review.     

Two Cases of Reversible Left Ventricular Hypertrophy during Recovery from Takotsubo Cardiomyopathy

We report 2 cases of reversible ventricular hypertrophy in patients with takotsubo cardiomyopathy (stress‐induced cardiomyopathy) during recovery of cardiac function. The first case involved a 72‐year‐old woman who presented with cerebral infarction. On admission, an elevated troponin I and decreased apical wall motion were observed with normal myocardial perfusion imaging. The second case involved a 79‐year‐old woman who presented with angina, anxiety resulting from emotional stress, slightly decreased apical wall motion, and normal epicardial arteries. In both cases, apical hypertrophy of the left ventricle was observed at approximately 3 weeks after onset, when the wall motion had improved. The ventricular wall gradually became thinner over time. To our knowledge, this is the first report of reversible ventricular hypertrophy in patients with takotsubo cardiomyopathy. We hypothesize the hypertrophic signaling in the myocardium was stimulated by catecholamines, which are the suggested etiology of takotsubo cardiomyopathy, and the hypertrophied myocardium gradually returned to normal as the syndrome receded.     

Recurrence of stress-induced (takotsubo) cardiomyopathy

Takotsubo cardiomyopathy is a recently recognized form of transient left ventricular dysfunction that is presumably precipitated by stress and may clinically resemble an acute coronary syndrome. These patients have an akinetic left ventricular apex in an unusual shape that resembles a takotsubo. Although reports of single episodes of takotsubo cardiomyopathy are not infrequent in recent medical literature, we report a case of recurrence that may provide more insight into the nature of this syndrome.     

Pathophysiology of the Syndrome: Biventricular Takotsubo Cardiomyopathy: Case Study and Review of Literature

Biventricular takotsubo cardiomyopathy is associated with more hemodynamic instability than is isolated left ventricular takotsubo cardiomyopathy; medical management is more invasive and the course of hospitalization is longer.In March 2011, a 62-year-old woman presented at our emergency department with abdominal pain, nausea, and vomiting. On hospital day 2, she experienced chest pain. An electrocardiogram and cardiac enzyme levels suggested an acute myocardial infarction. She underwent cardiac angiography and was found to have severe left ventricular systolic dysfunction involving the mid and apical segments, which resulted in a left ventricular ejection fraction of 0.10 to 0.15 in the absence of obstructive coronary artery disease. Her hospital course was complicated by cardiogenic shock that required hemodynamic support with an intra-aortic balloon pump and dobutamine. A transthoracic echocardiogram revealed akinesis of the mid-to-distal segments of the left ventricle and mid-to-apical dyskinesis of the right ventricular free wall characteristic of biventricular takotsubo cardiomyopathy. After several days of medical management, the patient was discharged from the hospital in stable condition.To the best of our knowledge, this is the first review of the literature on biventricular takotsubo cardiomyopathy that compares its hemodynamic instability and medical management requirements with those of isolated left ventricular takotsubo cardiomyopathy. Herein, we discuss the case of our patient, review the pertinent medical literature, and convey the prevalence and importance of right ventricular involvement in patients with takotsubo cardiomyopathy.Key words: Cardiomyopathies/radiography, hemodynamic instability/takotsubo, magnetic resonance angiography, takotsubo cardiomyopathy, biventricular/diagnosis, transient apical ballooning syndrome, ventricular dysfunction, left, ventricular dysfunction, rightTransient left ventricular apical ballooning syndrome (TLVABS) is an acute cardiac syndrome that typically is characterized by transient left ventricular (LV) wall-motion abnormalities, electrocardiographic (ECG) findings of ST-T segment changes, and minimal release of cardiac enzymes in the absence of significant stenosis of the coronary arteries (<50% luminal stenosis).^1–8 This syndrome was first described in Japan in 1991 and named takotsubo-like LV dysfunction in reference to the asynergy, which consists of hypokinesis or akinesis of the mid-to-apical LV and hyperkinesis of the basal LV extending over more than one coronary artery region.^8–11 Since then, this asynergy has been called takotsubo cardiomyopathy (TC), stress cardiomyopathy, ampulla syndrome, broken-heart syndrome, and neurogenic stunned myocardium.^12–14 Takotsubo cardiomyopathy presents clinically with chest pain and dyspnea associated with ECG changes, thereby mimicking ST-segment elevation myocardial infarction that is often preceded by emotional or physical stress.^1-7,9,10 Indeed ST-segment elevation is the most common ECG abnormality, reported in approximately 82% of patients with TC, followed by T-wave inversion in 64%.^8,15 This peculiar cardiac syndrome results in severe impairment of systolic function.There are several variants of TC. Isolated LV involvement is the most common variant, but right ventricular (RV) involvement is gaining increased recognition. Although there is only one documented case of isolated RV stress cardiomyopathy,¹⁶ the recognition of biventricular TC is increasing. It has been reported that RV involvement affects approximately 25% to 42% of patients diagnosed with TC.^12,13,17,18 In addition to isolated LV, isolated RV, and biventricular involvement, a variety of ballooning patterns has been observed. The typical apical-ballooning shape, with akinesis of the apical and mid-ventricular LV segments, is the most common; however, a mid-ventricular variant with apical sparing and a variant with isolated basal ballooning have been recognized.¹⁸ Left ventricular systolic dysfunction and heart failure—with simultaneous RV dysfunction—identifies a patient population with an extremely poor prognosis.¹⁹ Therefore, it is not surprising that TC with RV involvement appears to be associated with more severe LV dysfunction that results in significantly longer hospitalization and hemodynamic instability that is associated with a worse prognosis.^1-4,13,18 Clinicians need to be aware of the prevalence and complications of RV involvement and dysfunction, because it can affect patient management, morbidity, and, ultimately, mortality rates in substantial ways.The data concerning RV involvement in TC are sparse, because they are derived from several case reports and case series studies. This review of the literature documents the prevalence, pathophysiology, diagnostic imaging characteristics, clinical findings, management, and prognosis of patients with biventricular TC. Throughout this article, TC with RV involvement will be referred to as biventricular TC, whereas classic left ventricular TC (with apical ballooning) will be referred to as TLVABS. All of the documented cases of biventricular TC have been evaluated and analyzed in regard to how the patient presented, how the TC was diagnosed and managed, and how we can improve in managing the even more hemodynamically unstable biventricular TC, in comparison with TLVABS.     

Takotsubo cardiomyopathy in a cardiology department

Takotsubo cardiomyopathy, also known as transient left ventricular apical ballooning syndrome, stress-induced cardiomyopathy and broken heart syndrome, is characterized by transient left ventricular dysfunction in the absence of obstructive coronary artery disease. It was first described in 1990 in Japan, and gained worldwide recognition following the publication of several series of case reports. Its prevalence is estimated to be 1.7-2.2% of suspected acute coronary syndromes. Although takotsubo cardiomyopathy has been progressively better characterized, certain aspects remain to be clarified, and it is still under study. In this article, we report a series of ten cases of takotsubo cardiomyopathy admitted to a cardiology department, and compare the clinical, laboratory, electrocardiographic and imaging characteristics, therapeutic regimens and follow-up of these patients with those described in the latest scientific reviews.     

It’s a Trap! Clinical Similarities and Subtle ECG Differences between Takotsubo Cardiomyopathy and Myocardial Infarction

We describe a 65-year-old woman with a history of hypertension and smoking who presented with an acute episode of chest pain precipitated by severe emotional stress. Her initial electrocardiogram done in the emergency room showed non-specific T wave changes in the lateral leads and her cardiac troponin levels were mildly elevated. Because of her clinical presentation, she was admitted with a presumptive diagnosis of acute myocardial infarction and managed with antiplatelet and anticoagulant therapy. Coronary angiogram did not reveal coronary artery disease and left ventriculography showed findings consistent with apical ballooning syndrome or takotsubo cardiomyopathy. Subsequent electrocardiograms displayed dramatic changes including T wave inversions, QT interval prolongation and U waves. The patient remained asymptomatic and recovered uneventfully. Three weeks post-discharge, an echocardiogram documented resolved left ventricular dysfunction. We describe the clinical features and highlight the electrocardiographic findings that may help differentiate takotsubo cardiomyopathy from myocardial infarction.