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Cardiomyopathy, Takotsubo
Updated: Mar 31, 2009
Introduction
Background
Takotsubo cardiomyopathy (TCM) is a transient cardiac syndrome that involves left ventricular apical akinesis and mimics acute coronary syndrome (ACS). It was first described in Japan in 1990 by Sato et al. Patients often present with chest pain, have ST-segment elevation on electrocardiogram, and elevated cardiac enzyme levels consistent with a myocardial infarction.1 However, when the patient undergoes cardiac angiography, left ventricular apical ballooning is present and there is no significant coronary artery stenosis.
The Japanese word takotsubo translates to "octopus pot," resembling the shape of the left ventricle during systole on imaging studies. Although the exact etiology is still unknown, the syndrome appears to be triggered by a significant emotional or physical stressor.
Example of takotsubo or octopus pot.
The modified Mayo Clinic criteria for diagnosis of takotsubo cardiomyopathy can be applied to a patient at the time of presentation and must contain all 4 aspects:
- Transient hypokinesis, dyskinesis, or akinesis of the left ventricular mid-segments with or without apical involvement; the regional wall motion abnormalities extend beyond a single epicardial vascular distribution; a stressful trigger is often, but not always, present.
- Absence of obstructive coronary disease or angiographic evidence of acute plaque rupture
- New electrocardiographic abnormalities (either ST-segment elevation and/or T-wave inversion) or modest elevation in cardiac troponin level
- Absence of pheochromocytoma or myocarditis2
Pathophysiology
The exact etiology of takotsubo cardiomyopathy (TCM) is still unknown, but several theories have been proposed and are being investigated. These include multivessel coronary artery spasm, impaired cardiac microvascular function, impaired myocardial fatty acid metabolism, acute coronary syndrome with reperfusion injury, and endogenous catecholamine-induced myocardial stunning and microinfarction.3
Normal myocardium utilizes approximately 90% of its energy from fatty acid metabolism at rest and with aerobic activity. During ischemia, this pathway is suppressed and instead glucose is largely utilized, resulting in impaired cardiac function. Patients with takotsubo cardiomyopathy are found to shift toward the glucose pathway despite relatively normal myocardial perfusion and lack of ischemia in left ventricular segments.4
The most commonly discussed possible mechanism for takotsubo cardiomyopathy (TCM) is stress-induced catecholamine release, with toxicity to and subsequent stunning of the myocardium. Endomyocardial biopsy of patients with takotsubo cardiomyopathy demonstrates reversible focal myocytolysis, mononuclear infiltrates, and contraction band necrosis. The sympathetic/catecholamine theory is gaining momentum as takotsubo cardiomyopathy has been induced in rats exposed to physical stress and, in some instances, was prevented when pretreated with an alpha- or beta-blocker. Other evidence for this theory has been demonstrated through myocardial imaging studies using catecholamine analogues that evaluate cardiac sympathetic activity.
Cases of takotsubo cardiomyopathy (TCM) have been reported in the literature following cocaine, methamphetamine, and excessive phenylephrine use.3,4 Exercise stress testing, which is known to cause increased levels of catecholamines, has resulted in false positives attributable to TCM.5 Studies have found that patients with TCM have statistically significant higher levels of serum catecholamines (norepinephrine, epinephrine, and dopamine) than patients with myocardial infarctions.6 The apical portions of the left ventricle have the highest concentration of sympathetic innervation found in the heart and may explain why excess catecholamines seem to selectively affect its function.4
Frequency
United States
No data are available for incidence or prevalence of takotsubo cardiomyopathy (TCM) in the United States.
International
Studies have reported that 1.7-2.2% of patients who had suspected acute coronary syndrome were subsequently diagnosed with takotsubo cardiomyopathy.7,8
Mortality/Morbidity
Acute complications occur in approximately 20% of patients, including cardiogenic shock, heart failure, pulmonary edema, dysrhythmias, left ventricular thrombus formation, left ventricular free wall rupture, and death. Estimates of mortality rates have ranged from 1-3.2%.9,10
Race
Patients are typically Asian or Caucasian. In a literature review of cases in which race was reported 57.2% were Asian, 40% were Caucasian, and 2.8% were other races.10
Sex
Nearly 90% of reported cases involve postmenopausal women.9
Age
Literature reviews report a mean patient age of 67 years, although cases have occurred in children and young adults3,6
Clinical
History
The clinical presentation of patients ultimately diagnosed with takotsubo cardiomyopathy is usually indistinguishable from that of acute coronary syndrome.
- The most common presenting symptoms are chest pain and dyspnea, although palpitations, nausea, vomiting, syncope, and rarely, cardiogenic shock have been reported.
- One of the more unique features of TCM is the association with a preceding emotionally or physically stressful trigger event, occurring in approximately two thirds of patients.
- A recent large systematic review found patients with TCM tend to have a lower incidence of traditional cardiac risk factors such as hypertension, hyperlipidemia, diabetes, smoking, or positive family history for cardiovascular disease.11
Physical
Physical examination findings are nonspecific and often normal, but the patient may have the clinical appearance of having acute coronary syndrome or acute congestive heart failure.
- Patients may appear anxious and diaphoretic.
- Both tachydysrhythmias and bradydysrhythmias have been reported, but average heart rate in one review was 102 bpm.4
- Hypotension can occur from a reduction in stroke volume because of acute left ventricular systolic dysfunction or outflow tract obstruction.
- Murmurs and rales may be present on auscultation in the setting of acute pulmonary edema.
Causes
A significant emotional or physical stressor typically precedes the development of the takotsubo cardiomyopathy (TCM). Stressors include learning of a death of a loved one, bad financial news, legal problems, natural disasters, motor vehicle collisions, exacerbation of a chronic medical illness, newly diagnosed significant medical condition, surgery, intensive care unit stay, and use of or withdrawal from illicit drugs. Recently, TCM has been reported after near drowning episodes.12
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References
Sato H, Tateishi H, Uchida T, et al. Kodama K, Haze K, Hon M, eds. Clinical Aspect of Myocardial Injury: From Ischaemia to Heart Failure. Tokyo: Kagakuhyouronsya; 1990:56-64.
Kawai S, Kitabatake A, Tomoike H. Guidelines for diagnosis of takotsubo (ampulla) cardiomyopathy. Circ J. Jun 2007;71(6):990-2. [Medline].
Afonso L, Bachour K, Awad K, Sandidge G. Takotsubo cardiomyopathy: pathogenetic insights and myocardial perfusion kinetics using myocardial contrast echocardiography. Eur J Echocardiogr. Nov 2008;9(6):849-54. [Medline].
Dorfman TA, Iskandrian AE. Takotsubo cardiomyopathy: State-of-the-art review. J Nucl Cardiol. Jan-Feb 2009;16(1):122-34. [Medline].
Dhoble A, Abdelmoneim SS, Bernier M, Oh JK, Mulvagh SL. Transient left ventricular apical ballooning and exercise induced hypertension during treadmill exercise testing: is there a common hypersympathetic mechanism?. Cardiovasc Ultrasound. Jul 18 2008;6:37. [Medline].
Buchholz S, Rudan G. Tako-tsubo syndrome on the rise: a review of the current literature. Postgrad Med J. Apr 2007;83(978):261-4. [Medline].
Bybee KA, Prasad A, Barsness GW, et al. Clinical characteristics and thrombolysis in myocardial infarction frame counts in women with transient left ventricular apical ballooning syndrome. Am J Cardiol. Aug 1 2004;94(3):343-6. [Medline].
Ito K, Sugihara H, Katoh S, Azuma A, Nakagawa M. Assessment of Takotsubo (ampulla) cardiomyopathy using 99mTc-tetrofosmin myocardial SPECT--comparison with acute coronary syndrome. Ann Nucl Med. Apr 2003;17(2):115-22. [Medline].
Gianni M, Dentali F, Grandi AM, Sumner G, Hiralal R, Lonn E. Apical ballooning syndrome or takotsubo cardiomyopathy: a systematic review. Eur Heart J. Jul 2006;27(13):1523-9. [Medline].
Donohue D, Movahed MR. Clinical characteristics, demographics and prognosis of transient left ventricular apical ballooning syndrome. Heart Fail Rev. Dec 2005;10(4):311-6. [Medline].
Pilgrim TM, Wyss TR. Takotsubo cardiomyopathy or transient left ventricular apical ballooning syndrome: A systematic review. Int J Cardiol. Mar 14 2008;124(3):283-92. [Medline].
Citro R, Previtali M, Bossone E. Tako-tsubo cardiomyopathy and drowning syndrome: is there a link?. Chest. Aug 2008;134(2):469. [Medline].
Sharkey SW, Lesser JR, Menon M, Parpart M, Maron MS, Maron BJ. Spectrum and significance of electrocardiographic patterns, troponin levels, and thrombolysis in myocardial infarction frame count in patients with stress (tako-tsubo) cardiomyopathy and comparison to those in patients with ST-elevation anterior wall myocardial infarction. Am J Cardiol. Jun 15 2008;101(12):1723-8. [Medline].
Kolkebeck TE, Cotant CL, Krasuski RA. Takotsubo cardiomyopathy: an unusual syndrome mimicking an ST-elevation myocardial infarction. Am J Emerg Med. Jan 2007;25(1):92-5. [Medline].
Scheffel H, Stolzmann P, Karlo C, et al. Tako-tsubo phenomenon: dual-source computed tomography and conventional coronary angiography. Cardiovasc Intervent Radiol. Jan-Feb 2008;31(1):226-7. [Medline].
Prasad A, Lerman A, Rihal CS. Apical ballooning syndrome (Tako-Tsubo or stress cardiomyopathy): a mimic of acute myocardial infarction. Am Heart J. Mar 2008;155(3):408-17. [Medline].
Merchant EE, Johnson SW, Nguyen P, Kang C, Mallon WK. Takotsubo cardiomyopathy: a case series and review of the literature. WestJEM. 2008;9:104-11.
Sealove BA, Tiyyagura S, Fuster V. Takotsubo cardiomyopathy. J Gen Intern Med. Nov 2008;23(11):1904-8. [Medline].
Kurisu S, Inoue I, Kawagoe T, Ishihara M, Shimatani Y, Nakama Y. Incidence and treatment of left ventricular apical thrombosis in Tako-tsubo cardiomyopathy. Int J Cardiol. Feb 2 2009;[Medline].
Bybee KA, Kara T, Prasad A, et al. Systematic review: transient left ventricular apical ballooning: a syndrome that mimics ST-segment elevation myocardial infarction. Ann Intern Med. Dec 7 2004;141(11):858-65. [Medline].
Khallafi H, Chacko V, Varveralis N, Elmi F. "Broken heart syndrome": catecholamine surge or aborted myocardial infarction?. J Invasive Cardiol. Jan 2008;20(1):E9-13. [Medline].
Terefe YG, Niraj A, Pradhan J, Kondur A, Afonso L. Myocardial infarction with angiographically normal coronary arteries in the contemporary era. Coron Artery Dis. Dec 2007;18(8):621-6. [Medline].
Further Reading
Keywords
takotsubo cardiomyopathy, cardiac syndrome, myocardial infarction, TCM, broken heart syndrome, stress-induced cardiomyopathy, transient left ventricular apical ballooning syndrome, ampulla cardiomyopathy, acute coronary syndrome, ACS, symptoms, treatment, causes
