Takotsubo Cardiomyopathy Transient Left Ventricular Apical Ballooning Mimicking Acute Myocardial Infarction

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1 CSE REPORT Takotsubo Cardiomyopathy Transient Left Ventricular pical allooning Mimicking cute Myocardial Infarction Yung-Lung Chen, Tung-Hong Yu, Morgan Fu* Takotsubo cardiomyopathy is characterized by transient left ventricular dysfunction with clinical symptoms of chest pain, electrocardiographic changes of ST-segment elevation or T wave inversion, which mimics acute myocardial infarction in patients without angiographically significant coronary artery stenosis. We report a 75-year-old woman with a history of chest tightness who presented with typical pictures of takotsubo cardiomyopathy. cute myocardial infarction was initially diagnosed based on the electrocardiographic changes and elevated troponin. pical akinesis and ballooning with basal hyperkinesis were noted during left ventriculography. Coronary angiography, however, did not show significant coronary artery stenosis. Electrocardiography was normal 3 months later. Follow-up echocardiography did not show any wall motion abnormality. This patient remained well without chest pain or dyspnea over 24 months of follow-up. Optimal medical management of takotsubo cardiomyopathy remains unclear. This patientt received diltiazem to prevent possible coronary artery spasm. The prognosis of this syndrome seems to be favorable except for occasional mortality due to left ventricular rupture or ventricular arrhythmia. Recurrence of this syndrome is rare. [J Formos Med ssoc 2006;105(10): ] Key Words: takotsubo cardiomyopathy, transient left ventricular apical ballooning Takotsubo cardiomyopathy is a state of transient left ventricular dysfunction characterized by chest pain or dyspnea associated with electrocardiographic changes of ST-segment elevation or T wave inversion and minor elevation of cardiac enzymes, which mimics acute myocardial infarction. However, these patients have no significant stenosis of the coronary artery. The transient wall motion abnormalities may present with hypokinesis or akinesis of the left ventricular apex and midportion with hyperkinesis of the basal segments. Left ventriculography in end-systole looks like a takotsubo, which is an instrument with a round bottom and narrow neck used for trapping octopuses in Japan. This syndrome was first reported by Dote et al in 1991 and subsequently recognized and further reported in the Japanese population. 1 5 It has also recently been documented in the United States 6 and elgium. 7 lthough there are many proposed mechanisms, the actual etiology of takotsubo cardiomyopathy remains unclear. We report a 75-year-old woman with a history of chest tightness who presented with the typical characteristics of takotsubo cardiomyopa- thy. To our knowledge, this condition has not been previously reported in Taiwan Elsevier & Formosan Medical ssociation Section of Cardiovascular Disease, Chang Gung Memorial Hospital, Kaohsiung Medical Center, Kaohsiung, Taiwan. Received: June 14, 2005 Revised: July 12, 2005 ccepted: October 4, 2005 *Correspondence to: Dr Morgan Fu, Section of Cardiovascular Disease, Chang Gung Memorial Hospital, Kaohsiung Medical Center, 123 Ta-Pei Road, Niao-Sung, Kaohsiung 807, Taiwan. fumorgan@adm.cgmh.org.tw J Formos Med ssoc 2006 Vol 105 No

2 Y.L. Chen, et al Case Report 75-year-old woman was admitted to our hospital on June 28 th, 2003 due to acute onset of chest pain, cold sweating and dyspnea. She had a history of bilateral knee osteoarthritis and had recently undergone total knee arthroplasty. She had a history of hypertension and asthma in the past few years. This patient also reported emotional stress due to family problems. On arrival at the emergency room (ER), physical examination showed clear consciousness, conjunctiva was not pale and jugular venous pressure was approximately 6cmH 2 O. Mild rales were heard over both lung bases. Cardiac examination showed regular heartbeat without murmur. Neither third nor fourth heart sound was audible. There were no other noteworthy findings. Chest X-ray showed pulmonary congestion. Electrocardiography (ECG) in the ER showed: slight ST-segment elevation over leads I,, avf, V2 6; inverted T wave over leads V1 6, I,, avl; and prolonged QT interval with QTc of 522 ms (Figure 1). There was mild elevation of the cardiac enzyme troponin-i (5.6 ng/dl) but not CK-M (0 U/L). Under the impression of acute coronary syndrome, cardiac catheterization was performed on the 2 nd day of hospitalization. No significant coronary artery lesion was found on coronary angiography (Figure 2). Left ventriculography in end-systole showed apical akinesis and ballooning with basal hyperkinesis (Figure 3). Subsequent echocardiography on the next day of cardiac catheterization confirmed apical wall hypokinesis, mild mitral regurgitation and thickened interventricular septum. The patient was discharged 5 days later in a stable condition without chest pain. ECG at discharge showed that T wave inversion had decreased and the QTc interval was Figure 1. () Initial electrocardiography (ECG) during attack shows inverted T wave over V1 6 and I,, avl. () ECG 3 months later shows resolution of T wave. I avr June 28, 2003 V1 V4 avl V2 V5 I avf V3 V6 October 6, 2003 I avr V1 V4 avl V2 V5 I avf V3 V6 840 J Formos Med ssoc 2006 Vol 105 No 10

3 Transient left ventricular apical ballooning Figure 2. No significant stenosis is seen on coronary angiogram: () left coronary artery (left anterior oblique view); () right coronary artery (right anterior oblique view). Figure 3. Left ventriculography (right anterior oblique view): () end-diastole; () end-systole shows apical ballooning with hyperkinesis of basal segments. within the normal range. Some nonspecific ST T changes still persisted. ECG was normal 3 months later (Figure 1). Follow-up echocardiography did not show any wall motion abnormality, with global ejection fraction of 76%. Discussion Transient left ventricular apical ballooning syndrome is also known as takotsubo or ampulla cardiomyopathy. It is characterized by wall motion abnormalities involving hypokinesis or akinesis of the left ventricular apex and midportion with hyperkinesis of the basal segments. This syndrome clinically presents as chest pain or dyspnea associated with ST-segment elevation or T wave inversion on ECG. Cardiac enzymes and biomarkers are often slightly elevated. It mimics acute ST T-segment elevation myocardial infarction but coronary angiography shows no significant obstructive epicardial coronary atherothrombosis. The impaired left ventricular systolic function and abnormal wall motion in the left ventricularr J Formos Med ssoc 2006 Vol 105 No

4 Y.L. Chen, et al apex and midcavity generally resolve within days to weeks after initial presentation. 1 7 This syndrome occurs most often in females and in the elderly. 6 Initially, the differential diagnosis between this syndrome and acute anterior wall myocardial infarction is difficult to make. One report proposed that the combination of the absence of reciprocal changes and the ratio of ST-segment elevation in leads V4 6 to V1 3 1 had a greater specificity and overall accuracy for takotsubo cardiomyopathy. 8 Diagnostic criteria are not universally accepted. ybee et al 6 proposed the Mayo criteria for clinical diagnosis of transient left ventricular apical ballooning syndrome. This case fulfilled their criteria and the whole course was well documented by ECG changes, coronary angiography, left ventriculogram and echocardiography. t the 3-month follow-up, the apical wall hypokinesis had disappeared and the ECG had normalized. The cause of transient left ventricular ballooning syndrome is still unknown, but several mechanisms have been proposed. Inducible or spontaneous coronary vasospasm in at least one coronary artery has been reported in some series. 2 5 However, as in this case, many patients with persistent ST-segment elevation do not have identifiable epicardial coronary spasm or stenosis at the time of coronary angiography. Previous reports have noted coronary microvascular dysfunction measured by coronary flow reserve and TIMI frame count. 4,9 However, it is not clear if this is the primary mechanism or a secondary associated phenomenon. Some researchers have suggested that neurogenic stunned myocardium may be related to transient apical regional wall motion abnormalities caused by emotional or physical stress. 10 In this syndrome, apical wall motion abnormalities are similar to catecholamineinduced cardiomyopathy. 11 The phenomenon of the affected apical segment and the spared basal segment may be partly due to increased adrenergic receptor density or responsiveness to adrenergic stimulation in cardiac apical segments. 12 The findings of 123 I-metaiodobenzylguanidine myocardial scintigraphy in patients with takotsubo cardiomyopathy strongly suggest that neurogenic myocardial stunning may play a role in this syndrome. 13 This patient was under mental and emotional stress before the episode, which is consistent with this possibility. The strong female predominance in this syndrome may be related to alterations of endothelial function in response to reduced estrogen levels in postmenopausal women. In studies of ovariectomized female rats, estradiol supplementation attenuated changes in left ventricular function associated with emotional stress. 14 This phenomenon has not been clarified in humans. Medical management of takotsubo cardiomyopathy is uncertain. Use of β-blockers, angiotensin converting enzyme inhibitors, aspirin, calcium channel blockers and intravenous diuretics may be considered. Dynamic intraventricular obstructions in some patients with this syndrome are managed by administration of β-blockers to in- crease diastolic ventricular filling time and left ventricular end-diastolic volume, 6,7,15 administration of phenylephrine to increase afterload 6,7 and administration of fluid resuscitation if no pulmonary congestion is present. 6 Use of non- dihydropyridine calcium channel blockers may be considered if coronary artery vasospasm is documented, while β-blocker and phenylephrine would not be indicated in this condition. Patients should be monitored for arrhythmias, heart failure and mechanical complications as encountered in patients with myocardial infarction. The prognosis of this syndrome is, on the whole, fa- vorable, although there is occasional mortality due to left ventricular rupture or ventricular arrhythmia. Recurrence of this syndrome has been reported to be rare. 3,6,7 Our patient received the calcium channel blocker diltiazem under the impression of possible non-st T-elevation myo- cardial infarction. nother reason for the use of diltiazem was to prevent possible coronary spasm. This patient remained well without chest pain or dyspnea over 24 months of follow-up. In conclusion, this is the first report of takotsubo cardiomyopathy from Taiwan. Characteristics are transient left ventricular apical ballooning 842 J Formos Med ssoc 2006 Vol 105 No 10

5 Transient left ventricular apical ballooning without significant coronary stenosis. The etiology of this condition is not well established. Further investigation is indicated to define the causes and effective treatment of this syndrome. References 1. Dote K, Sato H, Tateishi H, et al. Myocardial stunning due to simultaneous multivessel coronary spasms: a review of 5 cases. J Cardiol 1991;21: Kawai S, Suzuki H, Yamaguchi H, et al. mpulla cardiomyopathy (Takotsubo cardiomyopathy)-reversible left ventricular dysfunction: with ST segment elevation. Jpn Circ J 2000;64: Tsuchihashi K, Ueshima K, Uchida T, et al. Transient left ventricular apical ballooning without coronary artery stenosis: a novel heart syndrome mimicking acute myocardial infarction. ngina Pecoris-Myocardial Infarction Investigations in Japan. J m Cardiol 2001;38: Kurisu S, Sato H, Kawagoe T, et al. Takotsubo-like left ventricular dysfunction with ST-segment elevation: a novel cardiac syndrome mimicking acute myocardial infarction. m Heart J 2002;143: be Y, Kondo M, Matsuoka R, et al. ssessment of clinical features in transient left ventricular apical ballooning. J m Coll Cardiol 2003;41: ybee K, Kara T, Prasad, et al. Systematic review: transient left ventricular apical ballooning: a syndrome that mimics ST-segment elevation myocardial infarction. nn Intern Med 2004;141: Desmet WJ, driaenssens F, Dens J. pical ballooning of the left ventricle: first series in white patients. Heart 2003;89: Ogura R, Hiasa Y, Takahashi T, et al. Specific findings of the standard 12-lead ECG in patients with Takotsubo cardiomyopathy: comparison with the findings of acute anterior myocardial infarction. Circ J 2003;67: Kume T, kasaka T, Kawamoto T, et al. Relationship between coronary flow reserve and recovery of regional left ventricular function in patients with takotsubo-like transient left ventricular dysfunction. J Cardiol 2004;43: Kono T, Morita H, Kuroiwa T, et al. Left ventricular wall motion abnormalities in patients with subarachnoid hemorrhage: neurogenic stunned myocardium. J m Coll Cardiol 1994;24: Scott IU, Gutterman DD. Pheochromocytoma with reversible focal cardiac dysfunction. m Heart J 1995;130: Mori H, Ishikawa S, Kojima S, et al. Increased responsiveness of left ventricular apical myocardium to adrenergic stimuli. Cardiovasc Res 1993;27: kashi YJ, Nakazawa K, Sakakibara M, et al. 123 I-MIG myocardial scintigraphy in patients with takotsubo cardiomyopathy. J Nucl Med 2004;45: Ueyama T, Hano T, Kasamatsu K, et al. Estrogen attenuates the emotional stress-induced cardiac response in the animal model of Takotsubo (mpulla) cardiomyopathy. J Cardiovasc Pharmacol 2003;42:S Kyuma M, Tsuchihashi K, Shinshi Y, et al. Effect of intravenous propranolol on left ventricular apical ballooning without coronary artery stenosis (ampulla cardiomyopathy): three cases. Circ J 2002;66: J Formos Med ssoc 2006 Vol 105 No