Two Cases of Apical Ballooning Syndrome Masking Apical Hypertrophic Cardiomyopathy

Apical akinesis and dilation in the absence of obstructive coronary artery disease is a typical feature of stress-induced (takotsubo) cardiomyopathy, whereas apical hypertrophy is seen in apical-variant hypertrophic cardiomyopathy. We report the cases of 2 patients who presented with takotsubo cardiomyopathy and were subsequently found to have apical-variant hypertrophic cardiomyopathy, after the apical ballooning from the takotsubo cardiomyopathy had resolved. The first patient, a 43-year-old woman with a history of alcohol abuse, presented with shortness of breath, electrocardiographic and echocardiographic features consistent with takotsubo cardiomyopathy, and no significant coronary artery disease. An echocardiogram 2 weeks later revealed a normal left ventricular ejection fraction and newly apparent apical hypertrophy. The 2nd patient, a 70-year-old woman with pancreatitis, presented with chest pain, apical akinesis, and a left ventricular ejection fraction of 0.39, consistent with takotsubo cardiomyopathy. One month later, her left ventricular ejection fraction was normal; however, hypertrophy of the left ventricular apex was newly noted. To our knowledge, these are the first reported cases in which apical-variant hypertrophic cardiomyopathy was masked by apical ballooning from stress-induced cardiomyopathy.     

Recurrent takotsubo with prolonged QT and torsade de pointes and left ventricular thrombus

Takotsubo cardiomyopathy, also known as “takotsubo syndrome,” refers to transient apical ballooning syndrome, stress cardiomyopathy, or broken heart syndrome and is a recently recognized syndrome typically characterized by transient and 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 to be fully determined. At present, there have been very few reports of recurrent takotsubo cardiomyopathy. In this case report, we present a patient with multiple recurrences of takotsubo syndrome triggered by severe emotional stress that presented with recurrent loss of consciousness, QT prolongation, and polymorphic ventricular tachycardia (torsade de pointes) and left ventricular apical thrombus.     

Takotsubo Cardiomyopathy as a Sequela of Elective Direct-Current Cardioversion for Atrial Fibrillation

In takotsubo cardiomyopathy, the clinical appearance is that of an acute myocardial infarction in the absence of obstructive coronary artery disease, with apical ballooning of the left ventricle. The condition is usually precipitated by a stressful physical or psychological experience. The mechanism is unknown but is thought to be related to catecholamine excess. We present the case of a 67-year-old woman who experienced cardiogenic shock caused by takotsubo cardiomyopathy, immediately after undergoing elective direct-current cardio-version for atrial fibrillation. After a course complicated by left ventricular failure, cardiogenic shock, and ventricular tachycardia, she made a complete clinical and echocardiographic recovery. In addition to this case, we discuss the possible direct effect of cardioversion in 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.     

Left anterior descending coronary artery stenosis: in a patient with takotsubo cardiomyopathy

Takotsubo cardiomyopathy is characterized by chest pain, electrocardiographic abnormalities such as ST-segment elevation or depression, and elevated cardiac enzyme levels. Left ventriculography reveals transient akinesis of the involved segment of the myocardial wall (usually the left ventricular apex) and compensatory hyperkinesis of the noninvolved myocardium, which appears as apical ballooning during systole. Existing criteria for the diagnosis of takotsubo cardiomyopathy include the absence of obstructive coronary artery disease. Indeed, previous investigators have found incidental stenosis in only a minority of patients.Herein, we present the unusual case of an 84-year-old woman who sustained 4 episodes of takotsubo cardiomyopathy in 18 years. At the time of the initial episode, coronary angiography revealed no substantial stenosis. Concomitant with the 2nd episode, stenosis in the 1st obtuse marginal branch was treated with stenting. No new lesions were apparent after the patient's 3rd presentation, and the previously placed stent was patent. During the 4th (current) presentation, we detected and percutaneously treated severe stenoses in the patient's left anterior descending coronary artery and 2nd obtuse marginal branch.Although this report is of a single patient only, it definitively illustrates that severe coronary artery disease can occur in patients who have takotsubo cardiomyopathy. We recommend the thorough evaluation of possible coronary artery disease in high-risk patients, even upon the strong clinical suspicion of takotsubo cardiomyopathy.     

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 Report and General Discussion

In recent years, our understanding of the physiologic mechanisms of transient takotsubo cardiomyopathy has improved because of the growing use of emergent heart catheterization in patients who present with severe ischemic syndromes. However, even this procedure has revealed only that, in most patients with takotsubo syndrome, the sudden onset of ventricular dysfunction is not due to fixed coronary artery occlusions. We present a case of transient takotsubo cardiomyopathy with an exceptional feature—uneven impairment of both right and left ventricular function, or biventricular takotsubo—and we discuss a novel, comprehensive theory that we have devised to explain the pathophysiology of this syndrome''s many manifestations.Key words: Acetylcholine/diagnostic use, angina pectoris, variant, coronary endothelial dysfunction, coronary vasospasm/physiopathology, takotsubo cardiomyopathy/classification/diagnosis/physiopathology, ventricular dysfunction, left/etiology/diagnosis, ventricular dysfunction, right/etiology/diagnosisOnly during the last 2 decades have Japanese authors^1,2 specifically categorized transient takotsubo cardiomyopathy (TTC) as an entity in itself. Before that time, TTC was often called “acute myocardial infarction with normal coronary arteries.”³ Its prevalence is probably as low today as in the remote past. However, acute coronary artery syndromes are now studied aggressively with emergent heart catheterization, which documents better than any previous means the transience of the myopathy and the presence of apparently normal coronary arteries. These circumstances have stimulated the quest to generate a pathophysiologic concept broad enough to encompass the full clinical spectrum of TCC.Apical ballooning (resulting in a systolic takotsubo or “octopus trap”) is the most frequent and emblematic feature of TTC. The use of this term has successfully promoted awareness of the disease in the cardiology community at large, but it has also impeded clinicians'' understanding of the breadth of this entity''s clinical manifestations. Our persistently inadequate knowledge of the nature and spectrum of TTC seems to warrant an update on the subject.Here, we present a case of right ventricular (in union with left ventricular) TTC. In addition, we discuss a pathophysiologic theory that our group has recently proposed, which might explain the newly discovered and broad spectrum of TTC clinical manifestations.     

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).     

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.     

Takotsubo cardiomyopathy followed by neurogenic stunned myocardium in the same patient: gradations of the same disease?

Takotsubo cardiomyopathy is a phenomenon of transient acute left ventricular dysfunction without obstructive coronary disease seen predominantly in postmenopausal women in the setting of acute emotional or physical stress. Neurocardiogenic injury from acute neurologic events such as intracranial bleeding can precipitate transient left ventricular dysfunction (termed 'neurogenic stunned myocardium') that may be indistinguishable from takotsubo cardiomyopathy. There is controversy about the diagnosis of takotsubo cardiomyopathy in the setting of acute neurologic disorders. We describe a case of a 67-year-old female who initially presented with takotsubo cardiomyopathy due to an acute gastrointestinal illness and 4 years later developed a recurrence in the setting of an ischemic cerebrovascular accident that was associated with more prominent EKG changes and much higher cardiac biomarker release but similar degree of left ventricular dysfunction. This case suggests that susceptibility to this disorder is likely due to patient-specific factors rather than etiology, and acute neurologic disorders should be included as precipitants of takotsubo cardiomyopathy. We also theorize that there may be patients with milder forms of stress-related cardiac injury who do not develop left ventricular dysfunction, being similar to the wide range of cardiac manifestations in patients with acute neurologic disorders. We review published literature on neurologic precipitants of takotsubo cardiomyopathy.     

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.     

Takotsubo Cardiomyopathy in a 22-Year-Old Single-Ventricle Patient

We report a highly unusual case of a 22-year-old woman with single-ventricle anatomy and a history of Fontan palliation who developed takotsubo cardiomyopathy. To our knowledge, takotsubo cardiomyopathy has not previously been described in a patient with single-ventricle anatomy, or more generally in any patient with congenital heart disease. The patient presented at our clinic for urgent examination after a 2-day history of chest pain that had begun upon the death of her dog. Invasive evaluation was refused by the patient; instead, she underwent electrocardiogram-gated coronary computed tomographic angiography, to rule out obstructive coronary disease.A physician who suspects takotsubo cardiomyopathy in a patient should look for new-onset chest pain and dyspnea coupled with these features: immediate onset of segmental akinesia in the midventricular or apical aspects of the heart, elevation of brain natriuretic peptide level 12 to 24 hours after onset of akinesia, elevation of troponin level 24 to 48 hours after onset, and disappearance of the segmental akinesia a few days after onset.     

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.     

Takotsubo cardiomyopathy associated with titration of duloxetine

Takotsubo cardiomyopathy is characterized by transient multisegmental left ventricular dysfunction, dynamic electrocardiographic changes that mimic acute myocardial infarction, and the absence of obstructive coronary disease. Takotsubo cardiomyopathy has been solidly associated with antecedent emotional and physical stressors that trigger catecholamine surges, which lead to coronary vasospasm or direct myocardial injury. Some medications can also cause catecholamine surges, although this phenomenon is not as well described. Duloxetine is a combined serotonin and norepinephrine reuptake inhibitor (SNRI). The basic goal of SNRIs is to increase catecholamine levels in neuronal tissue. However, the increased catecholamine levels may also affect the cardiovascular system.Herein, we report the case of a 59-year-old woman whose takotsubo cardiomyopathy was temporally associated with the titration of duloxetine. The duloxetine therapy was subsequently discontinued, and the patient's left ventricular function recovered completely 1 month after the index event. The purpose of this report is to alert clinicians to a possible association between SNRI medications and takotsubo cardiomyopathy.     

Pathophysiology of Takotsubo Cardiomyopathy: Reopened Debate

Takotsubo cardiomyopathy (TTC), a persistently obscure dysfunctional condition of the left ventricle, is uniquely transient but nevertheless dangerous. It features variable ventricular patterns and is predominant in women. For 30 years, pathophysiologic investigations have progressed only slowly and with inadequate focus.It was initially proposed that sudden-onset spastic obliteration of coronary flow induced myocardial ischemia with residual stunning and thus TTC. Later, it was generally accepted without proof that, in the presence of pain or emotional stress, the dominant mechanism for TTC onset was a catecholamine surge that had a direct, toxic myocardial effect.We think that the manifestations of TTC are more dynamic and complex than can be assumed from catecholamine effects alone. In addition, after reviewing the recent medical literature and considering our own clinical observations, especially on spasm, we theorize that atherosclerotic coronary artery disease modulates and physically opposes obstruction during spasm. This phenomenon may explain the midventricular variant of TTC and the lower incidence of TTC in men. We continue to recommend and perform acetylcholine testing to reproduce TTC and to confirm our theory that coronary spasm is its initial pathophysiologic factor. An improved understanding of TTC is especially important because of the condition''s markedly increased incidence during the ongoing COVID-19 pandemic.     

An angiographic and intravascular ultrasound study of the left anterior descending coronary artery in takotsubo cardiomyopathy

The precise cause of takotsubo cardiomyopathy (TC) remains controversial. Plaque rupture with transient thrombotic occlusion of a transapical left anterior descending coronary artery (LAD) has been advanced as a potential mechanism. To explore this hypothesis, the investigators analyzed data from 11 patients prospectively enrolled in the Rhode Island Takotsubo Cardiomyopathy Registry who underwent coronary angiography and intravascular ultrasound evaluation of the LAD during their initial presentation. Despite the presence of nonobstructive coronary artery disease, no culprit lesion was identified in any patient. Similarly, the course of the LAD failed to account for the characteristic left ventricular apical ballooning seen in TC. In conclusion, an atherosclerotic coronary lesion in the LAD causing an aborted myocardial infarction may not be the primary underlying cause of TC, and nonobstructive coronary artery disease and TC may coexist without a direct causal association.     

Takotsubo cardiomyopathy complicated by high-grade atrioventricular block: A report of two cases

The present report describes two female patients aged 39 and 57 years who experienced loss of consciousness and chest pain due to high-grade atrioventricular block. Both patients demonstrated noncontraction centred on the cardiac apex and excessive contraction at the cardiac base on cardiac ultrasonography and left ventriculography, but neither of them demonstrated any significant stenotic lesions on coronary angiography. Furthermore, neither patient showed elevated serum biomarkers of cardiac injury or serum viral antibodies. In a repeat left ventriculogram two weeks later, the left ventricular wall motion disorder had improved in both patients. Based on these findings, the patients were diagnosed with takotsubo cardiomyopathy. Because the high-grade atrioventricular conduction disorder did not improve in spite of the improvement of left ventricular wall motion disorder, permanent pacemaker implantation was performed. It is extremely rare for takotsubo cardiomyopathy to be complicated by high-grade atrioventricular block. In the present study, both patients had takotsubo cardiomyopathy complicated by high-grade atrioventricular block and eventually underwent permanent pacemaker implantation.     

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.     

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.