Circulation. 2007;115:e56-e59
doi: 10.1161/CIRCULATIONAHA.106.669341
(Circulation. 2007;115:e56-e59.)
© 2007 American Heart Association, Inc.
Apical Ballooning Syndrome
An Important Differential Diagnosis of Acute Myocardial Infarction
Abhiram Prasad, MD, FRCP, FESC
From the Division of Cardiovascular Diseases and Department of Internal Medicine, and Mayo Clinic and Mayo Foundation, Rochester, Minn.
Reprint requests to Abhiram Prasad, MD, Cardiac Catheterization Laboratory, Mayo Clinic, 200 First St SW, Rochester, MN 55905. E-mail prasad.abhiram{at}mayo.edu
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Introduction
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Case presentation: A 60-year-old woman presented to the emergency
department 2 hours after the onset of severe retrosternal chest
pain that started soon after she was told that her son had died
in a car accident. A 12-lead ECG demonstrated ST-elevation in
the precordial leads (
Figure 1), and the plasma troponin T level
was elevated at 0.07 ng/mL. A diagnosis of acute ST-elevation
myocardial infarction was made, and the patient was admitted
for emergency coronary angiography, which revealed normal coronary
arteries. The left ventriculogram showed severe systolic dysfunction
involving the mid and apical segments (Data Supplement Movie
I).
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Recognition of Clinical Syndrome
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Physicians have long been aware of the possible association
between stress and cardiovascular events. Awareness has increased
of a distinct cardiac syndrome that was originally described
in the Japanese population and was called Takotsubo cardiomyopathy,
named after the octopus-trapping pot with a round bottom and
narrow neck that resembles the left ventriculogram during systole
in these patients.
1,2 Other names used to describe the condition
include apical ballooning syndrome (ABS), broken heart syndrome,
and stress or ampulla cardiomyopathy. The precise incidence
of ABS is unknown, but it may account for 1% to 2% of patients
who present with an acute myocardial infarction.
3
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Clinical Features
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The majority of patients have a clinical presentation that is
indistinguishable from an acute coronary syndrome. Most present
with chest pain at rest, although some patients have dyspnea
alone as their initial presenting symptom. Rarely, patients
present with syncope or an out-of-hospital cardiac arrest.
4 ABS appears to occur almost exclusively in postmenopausal women;
however, a few cases have been reported in younger women and
males.
4 The patients are usually hemodynamically stable, but
clinical findings of mild-to-moderate congestive heart failure
commonly coexist. In a minority of patients, hypotension may
occur from reduced stroke volume and occasionally from dynamic
left ventricular outflow tract obstruction. Cardiogenic shock
may be a rare complication. A unique feature of ABS is the occurrence
of a preceding emotionally or physically stressful event in
approximately two thirds of patients. Importantly, such a trigger
is not observed in all individuals, and its absence does not
exclude the diagnosis.
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Diagnosis
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The most frequent finding on the admission ECG is mild ST-segment
elevation, which occurs in approximately 50% to 60% of patients.
It is typically present in the precordial leads, but the ECG
can be normal or can show nonspecific T-wave abnormality or
major ST elevation across the precordial and limb leads. In
general, the 12-lead ECG alone is not helpful in differentiating
ABS from an ST-elevation myocardial infarction.
5 Characteristic
evolutionary changes that occur over 2 to 3 days include resolution
of the ST-segment elevation and subsequent development of diffuse
and often deep T-wave inversion that involves most leads (
Figure 2).
New pathological Q waves may be seen occasionally, and there
is frequent prolongation of the corrected QT interval. Most,
if not all, patients have a small early increase in cardiac
biomarkers, especially when cardiac troponins are measured.
Transthoracic echocardiography can detect the regional wall-motion
abnormality, but the diagnosis is frequently made in the cardiac
catheterization laboratory because the patients are initially
suspected of suffering from an acute coronary syndrome and are
referred for urgent or emergency coronary angiography. Patients
with ABS typically do not have obstructive coronary artery disease.
The left ventriculogram shows characteristic regional wall-motion
abnormalities involving the mid and usually the apical segments.
There is sparing of the basal systolic function, and the wall-motion
abnormality extends beyond the distribution of any 1 single
coronary artery (Data Supplement Movie I). Apical sparing variants
have also been described (Data Supplement Movie II).
6 Cardiac
magnetic resonance imaging may be helpful in excluding myocardial
infarction, because delayed gadolinium hyperenhancement is not
a feature of ABS.
7
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Figure 2. Twelve-lead ECG demonstrating resolution of ST-segment elevation and development of diffuse T-wave inversion.
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On the basis of our experience, we have previously proposed criteria for making a clinical diagnosis of ABS, and all 4 criteria must be present. The Table illustrates a modified version of the criteria that incorporates contemporary knowledge about the syndrome.4 The diagnosis of ABS is most likely to be made at institutions where primary percutaneous coronary intervention and an early invasive strategy are practiced for the management of acute coronary syndromes. The absence of obstructive coronary artery disease and the characteristic regional wall-motion abnormality are likely to lead to the diagnosis. Diagnosis of ABS in patients who present at hospitals without cardiac catheterization laboratories requires a high index of suspicion. The diagnosis should be considered in a postmenopausal woman who presents with cardiac symptoms that are temporally related to an emotional or physical stressor, with positive cardiac biomarkers or an abnormal ECG. Establishing the diagnosis is particularly important if fibrinolytic therapy is being considered for a presumed diagnosis of ST-elevation myocardial infarction. Inappropriate administration of fibrinolytics to a patient with ABS may lead to harm, and it would be appropriate to transfer a patient suspected of having ABS for emergency coronary angiography.
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Management
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The optimal management of ABS has not been established, but
supportive therapy invariably leads to spontaneous recovery.
Because the presentation in these patients is indistinguishable
from an acute coronary syndrome, initial management should be
directed toward the treatment of myocardial ischemia, with continuous
ECG monitoring and administration of aspirin, intravenous heparin,
and ß-blockers. Once the diagnosis of ABS has been
made, aspirin can be discontinued unless there is coexisting
coronary atherosclerosis. ß-Blocker therapy may be
continued, if tolerated, especially because an excess of catecholamines
may play a role in precipitating the conditions. Diuretics are
effective for the treatment of congestive heart failure. The
right ventricle is also involved in approximately one third
of cases, and these patients are sicker and more likely to develop
congestive heart failure (Data Supplement Movie III).
8 On rare
occasions, when there is significant hypotension, it is important
to exclude dynamic left ventricular outflow tract obstruction
with echocardiography. If present, a cautious trial of ß-blockers
may help by reducing the hypercontractility of the base of the
left ventricle. Alternatively, an infusion of phenylephrine
may be effective by increasing the afterload and left ventricular
cavity size. Inotropes would be contraindicated in this situation.
In contrast, cardiogenic shock owing to pump failure is treated
with standard therapies that include inotropes and intra-aortic
balloon counterpulsation. Anticoagulation should be considered
in cases of severe left ventricular systolic dysfunction to
prevent thromboembolism until there has been recovery.
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Prognosis
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Patients with ABS generally have a good prognosis in the absence
of significant underlying comorbid conditions. The systolic
dysfunction and the regional wall-motion abnormalities are transient
and resolve completely within a matter of days to a few weeks.
This is such a uniform finding that an alternative diagnosis
should be considered in patients in whom the cardiomyopathy
does not resolve. Typically, assessment of the ejection fraction
should be performed at approximately 4 to 6 weeks after discharge
from the hospital to document recovery of function. At this
time, the ECG usually shows complete resolution, although T-wave
inversion may persist for a longer period of time. In-hospital
mortality from ABS is very low and unlikely to be greater than
1% to 2%. The recurrence rate of ABS is no more than 10%.
9 In
our practice, in the absence of contraindications, we recommend
chronic ß-blocker therapy, with the aim of reducing
the likelihood of a recurrent episode. Annual clinical follow-up
is advisable because the natural history of ABS remains unknown.
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Pathophysiology
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The pathophysiology of ABS is poorly understood. Several mechanisms
for the reversible cardiomyopathy have been proposed, including
multivessel epicardial spasm, coronary microvascular spasm,
catecholamine-induced myocardial stunning, and myocarditis.
Multivessel epicardial coronary artery spasm has not been observed
in any of the patients at our institution, but it is possible
that the routine administration of nitrates for ischemic chest
pain may obscure its presence. Microvascular dysfunction is
present in at least two thirds of patients at the time of presentation,
and its severity correlates with the magnitude of troponin elevation
and ECG abnormalities.
10 It remains to be established whether
impairment in microvascular function is the primary mechanism
for the injury or is an epiphenomenon. Catecholamines may play
a role in triggering the syndrome.
11
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Case Conclusion
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The left ventricular end-diastolic pressure was 30 mm Hg, and
the patient was markedly hypotensive. An intra-aortic balloon
was inserted. Her symptoms and hypotension resolved over 48
hours. She was discharged on the fourth day with a prescription
for an ACE inhibitor and ß-blocker. An echocardiogram
4 weeks later revealed normal left ventricular function. The
ACE inhibitor was discontinued, and she remains well 1 year
after her admission.
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Acknowledgments
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Disclosures
None.
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Footnotes
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The online-only Data Supplement, consisting of movies, is available with this article at http://circ.ahajournals.org/cgi/content/full/115/5/e56/DC1.
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References
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1. Sato H, Tateishi H, Uchida T, Dote K, Ishihara M, Sasaki K. Tako-tsubo-like left ventricular dysfunction due to multivessel coronary spasm. In: Kodama K, Haze K, Hori M, eds.
Clinical Aspect of Myocardial Injury: From Ischemia to Heart Failure [in Japanese]. Tokyo, Japan: Kagakuhyoronsha Publishing Co; 1990: 5664.
2. Tsuchihashi K, Ueshima K, Uchida T, Oh-mura N, Kimura K, Owa M, Yoshiyama M, Miyazaki S, Haze K, Ogawa H, Honda T, Hase M, Kai R, Morii I. Transient left ventricular apical ballooning without coronary artery stenosis: a novel heart syndrome mimicking acute myocardial infarction: Angina Pectoris-Myocardial Infarction Investigations in Japan. J Am Coll Cardiol. 2001; 38: 1118.[Abstract/Free Full Text]
3. Bybee KA, Prasad A, Barsness G, Wright RS, Rihal CS. Clinical characteristics, outcomes, and impaired myocardial microcirculation in patients with transient left ventricular apical ballooning syndrome: a case-series from a U.S. medial center. Am J Cardiol. 2004; 94: 343346.[CrossRef][Medline]
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4. Bybee KA, Kara T, Prasad A Lerman A, Barsness GW, Wright RS, Rihal CS. Transient left ventricular apical ballooning syndrome: a mimic of ST-segment elevation myocardial infarction. Ann Intern Med. 2004; 141: 858865.[Abstract/Free Full Text]
5. Bybee K, Motiei A, Syed I, Kara T, Prasad A, Lennon RJ, Murphy JG, Hammill SC, Rihal CS, Wright RS. Electrocardiography cannot reliably differentiate transient left ventricular apical ballooning syndrome from anterior ST-segment elevation myocardial infarction. J Electrocardiol. 2007; 40: 38.e1e6.
6. Hurst RT, Askew JW, Reuss CS, Lee RW, Sweeney JP, Fortuin FD, Oh JK, Tajik AJ. Transient midventricular ballooning syndrome: a new variant. J Am Coll Cardiol. 2006; 48: 579583.[Abstract/Free Full Text]
7. Sharkey SW, Lesser JR, Zenovich AG, Maron MS, Lindberg J, Longe TF, Maron BJ. Acute and reversible cardiomyopathy provoked by stress in women from the United States. Circulation. 2005; 111: 472479.[Abstract/Free Full Text]
8. Elesber A, Prasad A, Bybee KA, Valeti U, Motiei A, Lerman A, Chandrasekaran K, Rihal CS. Transient cardiac apical ballooning syndrome: prevalence and clinical implications of right ventricular involvement. J Am Coll Cardiol. 2006; 47: 10821083.[Free Full Text]
9. Elesber AA, Prasad A, Wright S, Lerman A, Rihal C. Recurrence rate and long term prognosis of the apical ballooning syndrome. Circulation. 2006; 114 (suppl II): II-625. Abstract.
10. Elesber A, Lerman A, Bybee KA, Murphy JG, Barsness G, Singh M, Rihal CS, Prasad A. Myocardial perfusion in apical ballooning syndrome: correlate of myocardial injury. Am Heart J. 2006; 152: 469.e9e13.
11. Wittstein IS, Thiemann DR, Lima JA, Baughman KL, Schulman SP, Gerstenblith G, Wu KC, Rade JJ, Bivalacqua TJ, Champion HC. Neurohumoral features of myocardial stunning due to sudden emotional stress. N Engl J Med. 2005; 352: 539548.[Abstract/Free Full Text]
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