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Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023
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From Suzuki R, Kudo T, Kurazumi H, et al. J Cardiothorac Surg. 2013 May 26;8:135. [Open access] PMID: 23705797; PMCID: PMC3720530.
Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023 | Contributor Information
In the above image, a left ventricular (LV) thrombus (black arrows) is revealed in a 58-year-old woman with takotsubo cardiomyopathy (TCM).
TCM, first described in 1990 by Sato et al,[1,2] is a transient cardiac syndrome that typically involves LV apical akinesis and basal hypercontraction; it may symptomatically mimic acute coronary syndrome (ACS).[1,3]
A significant emotional stressor (eg, unexpected death of a loved one, divorce, bad financial news) or physical stressor (eg, motor vehicle collision, significant surgery, a stay in the intensive care unit [ICU]) typically precedes the development of TCM in two thirds of patients; thus, the condition is also known as broken heart syndrome. In the end-systolic phase, aneurysmal dilatation (ballooning) of the anterior, apical, and inferior segments of the left ventricle is apparent; accordingly, another alternative term for TCM is apical ballooning syndrome.[1,3]
Left image from Koeth O, Mark B, Zahn R, Zeymer U. Cases J. 2008;1(1):331. [Open access.] PMID: 19019232, PMCID: PMC2599899. Right image from Richard C. Ann Intensive Care. 2011;1(1):39. [Open access.] PMID: 21933374, PMCID: PMC3224539.
Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023 | Contributor Information
Pathophysiology
TCM is named for the resemblance of the LV apical ballooning (left) to the round bottom and narrow neck of traditional Japanese octopus traps (takotsubo; right). This syndrome occurs more commonly in postmenopausal women and is thought to be due in part to the effects of stress-induced catecholamine release on the myocardium (myocardial stunning, microinfarction[4]).[1,3] Other proposed mechanisms include multivessel epicardial coronary artery spasm, impairment of coronary microvascular function, and impaired myocardial fatty acid metabolism.[1,3] Myocardial regional differences in response to high levels of catecholamine (eg, apical segments more responsive to negatively inotropic epinephrine) appear to support the role of catecholamines in the pathogenesis of TCM.[1]
A study by Khan et al (published in JACC: Heart Failure, March 2023) suggested that higher-level function centers in the brain are involved in TCM, as evidenced by abnormalities in the thalamus-amygdala-insula and basal ganglia. The investigators stated that patients may benefit from treatment to modulate these abnormalities.[23]
Images from Wassmuth R, Prothmann M, Utz W, et al. J Cardiovasc Magn Reson. 2013;15:27. [Open access.] PMID: 23537111, PMCID: PMC3627620.
Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023 | Contributor Information
The systolic cine magnetic resonance imaging (MRI) scan (A) shows apical ballooning in an 81-year-old patient with TCM. Cardiac MRI (CMRI) T2 (transversal relaxation time) mapping in the same patient (B) reveals elevated apical myocardial T2-signal intensity (orange). Both images are in two-chamber orientation and were obtained 3 days after the initial presentation of TCM, with transient electrocardiographic (ECG) changes and elevated troponin levels following a generalized seizure.
Endomyocardial biopsies have revealed interstitial infiltrates of mononuclear lymphocytes, leukocytes, and macrophages as well as the presence of myocardial fibrosis and contraction band necrosis.
Image courtesy of Medscape.
Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023 | Contributor Information
The coronary angiogram reveals normal coronary arteries in a patient with TCM.
Clinical Features
The actual prevalence of TCM is unknown, but this syndrome likely accounts for 1-2% of all cases of suspected acute MI.[1,3,5-7] Patients with TCM often present with chest pain, ECG ST-segment elevation, and elevated cardiac enzyme levels, all of which are consistent with an acute myocardial infarction (MI). However, when the patient undergoes coronary angiography, no significant obstructive coronary artery stenosis is evident, and when left ventriculography is performed, LV apical ballooning is present.[8]
Image courtesy of Medscape.
Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023 | Contributor Information
This left ventriculogram, taken during systole, shows TCM's characteristic apical ballooning with apical akinesis (arrow).
As noted earlier, the clinical presentation of patients ultimately diagnosed with TCM is usually indistinguishable from that of patients with ACS (ie, chest pain, dyspnea, palpitations, nausea). However, unlike ACS, the peak occurrence of which is in the morning hours, TCM events are most prevalent in the afternoon, when stressful triggers may be more likely to take place.[9] Moreover, patients with TCM may have a lower incidence of traditional cardiac risk factors (eg, hypertension, hyperlipidemia, diabetes, smoking, positive family history for cardiovascular disease).[10] Hypotension can occur from a reduction in stroke volume owing to acute LV systolic dysfunction or outflow tract obstruction.[1]
Image from Keskin A, Winkler R, Mark B, et al. J Med Case Rep. 2010;4:280. [Open access.] PMID: 20727147, PMCID: PMC2933634.
Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023 | Contributor Information
The ventriculograms reveal hypokinesia of the midventricular segment (white arrows) in diastole (left) and systole (right).
Diagnostic Criteria
Multiple diagnostic criteria and recommendations exist for TCM. According to the modified Mayo Clinic criteria, all four of the following must be present for the diagnosis of TCM to be made[1,3,11]:
Transient hypokinesis, dyskinesis, or akinesis of the LV midsegments, with or without apical involvement; the regional wall-motion abnormalities extend beyond a single epicardial vascular distribution; a stressful trigger may or may not be present
There is no obstructive coronary artery disease (CAD) or angiographic evidence of an acute plaque rupture
New ECG abnormalities (ST-segment elevation and/or T-wave inversion) or modestly elevated levels of cardiac troponin
Absence of pheochromocytoma or myocarditis
Image courtesy of Medscape.
Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023 | Contributor Information
The above electrocardiogram, from a patient with TCM, demonstrates ST-segment elevation in the anterior/anterolateral and inferior leads.
Electrocardiography
The most common ECG finding in acute TCM is ST-segment elevation in the precordial leads (usually V2-V3),[1,3,12] although an initially normal or nonspecific ECG finding is seen in 20-30% of affected patients.[1] The ST-segment elevation is then followed by diffuse T-wave inversions (as the ST segments normalize) along with QT prolongation. Thus, on the basis of ECG findings alone, there is no reliable way to differentiate TCM from ST-elevation acute MI (STEMI).[13]
Levels of cardiac biomarkers (eg, troponin) are usually elevated in TCM, but the rise tends to be lower than that seen in ACS (including STEMI).[1] Therefore, coronary angiography is usually required for the diagnosis.[1,3,14,15]
Image courtesy of Medscape.
Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023 | Contributor Information
The above echocardiogram demonstrates apical akinesis during systole in a patient with TCM. The ejection fraction (EF) is 40%.
Echocardiography
Transthoracic echocardiography (TTE) provides a quick method of diagnosing the wall-motion abnormalities typically seen in TCM, specifically LV hypokinesis or akinesis of the midsegment and apical segment. These wall-motion abnormalities extend beyond the distribution of any single coronary artery. The LV ejection fraction (LVEF) can be estimated by means of echocardiography, CMRI, or left ventriculography.
Images from Eitel I, Friedrich MG. J Cardiovasc Magn Reson. 2011;13:13. [Open access.] PMID: 21332972, PMCID: PMC3060149.[14]
Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023 | Contributor Information
Top row: The CMRIs in images A-C demonstrate the typical apical contractile dysfunction seen in patients with stress-induced TCM (C is without late gadolinium enhancement). Bottom row (T2-weighted images): The basal myocardium has normal T2-signal intensity in image D, whereas in images E and F, the presence of global apical and midventricular myocardial edema corresponds to the LV wall-motion abnormalities.
Cardiac Magnetic Resonance Imaging
CMRI may provide new insight into the pathophysiology of TCM and may potentially be useful at a patient's acute presentation,[1,3,16] thereby broadening recognition of the condition and improving clinical outcomes.[16] In patients with TCM, delayed enhancement on four-chamber CMRI shows no abnormal LV enhancement. However, in a patient with an acute MI due to coronary occlusion or with acute myocarditis, contrast enhancement may be seen, which can be useful for differentiating between TCM and these other conditions.
Adapted images from Ferreira VM, Piechnik SK, Dall'Armellina E, et al. J Cardiovasc Magn Reson. 2012;14:42. [Open access.] PMID: 22720998, PMCID: PMC3424120.
Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023 | Contributor Information
Representative short-axis CMRI slices from five imaging modalities, as taken in three individuals, are shown for comparison. Top row: Patient with regional edema in the anterior wall; the anterior, anterolateral wall, and anterior septum demonstrate high T2-signal intensity and T1 (longitudinal relaxation time) values. Middle row: Patient with TCM. Bottom row: Normal volunteer.
Column A: Dark-blood T2 images
Column B: Bright-blood T2 images
Column C: Color shortened modified look-locker inversion recovery (ShMOLLI) T1 maps (Green = normal myocardium; red = increased T1 values)
Column D: R2 (relaxation rate) maps of ShMOLLI fit (for quality verification of images in column C)
Column E: Imaging with late gadolinium enhancement
Images from Wassmuth R, Prothmann M, Utz W, et al. J Cardiovasc Magn Reson. 2013;15:27. [Open access.] PMID: 23537111, PMCID: PMC3627620.
Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023 | Contributor Information
These two-chamber CMRIs were obtained 3 days after the initial presentation in an 81-year-old patient with TCM (same patient as in slide 3). Left image: Conventional T2 short tau inversion recovery (STIR) image. There is increased myocardial apical signal intensity that could be difficult to differentiate from an intraluminal blood signal in cases of slow flow. Right image: Late gadolinium enhancement image that excludes the presence of a myocardial scar.
Image from Patankar GR, Choi JW, Schussler JM. J Med Case Rep. 2013;7:84. [Open access.] PMID: 23510078, PMCID: PMC3668300.
Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023 | Contributor Information
TCM Variants
Variants to the typical pattern of TCM have been recognized, but they occur much less commonly than the conventionally identified TCM.[17] The left ventriculograms are from a patient with reverse TCM, in which there is apical hyperkinesis and basal akinesis rather than the characteristic TCM pattern of apical dilatation (ballooning) and hypokinesis with basal hyperkinesis. Image A was obtained during diastole. Image B was taken during systole. The arrows in the apical segments show where normal ventricular movement in this patient occurred.
Images from Romano M, Zorzoli F, Bertona R, Villani R. Case Rep Med. 2013;2013:946378. [Open access.] PMID: 24327812, PMCID: PMC3847958.
Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023 | Contributor Information
Left images: Normal coronary angiograms from a patient whose TCM was induced by multidrug toxicity in a suicide attempt. Right images: Left ventriculograms in diastole (top) and systole (bottom) show apical hypokinesis in the same patient.
Management
No standard treatment guidelines exist for TCM;[3,18] all patients with suspected TCM should be treated as having ACS until this has been proven not to be the case. TCM therapy is mainly empirical and supportive.[1,3,18] When the patient's hemodynamic status permits, beta blockers may be helpful. In the 5-8% of patients with TCM who have an LV thrombus, anticoagulation is needed.[19]
Despite the lack of treatment guidelines, the prognosis for patients with TCM is excellent (95-98% inpatient survival),[1,3] with complete recovery often occurring in 4-8 weeks. However, as many as 10% of patients will have a recurrence.[1,3]
Table courtesy of Medscape. Information sources: Lyon A, Bossone E, Schneider B, et al,[20] and Peters MN, George P, Irimpen AM.[21]
Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)
Mark P Brady, PA-C | March 13, 2023 | Contributor Information
Complications
Although management of TCM is mainly supportive and the outcomes are typically good (with a full recovery achieved by about 95% of patients[22]), there are a variety of complications that can increase mortality and prolong time to recovery. Patients may present in heart failure and cardiogenic shock, which may be severe. No definitive data provide guidance in avoiding complications; in general, it is advised to avoid catecholaminergic inotropic agents such as dobutamine because these may serve to exacerbate the theorized stress caused by endogenous epinephrine.
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Contributor Information
Author
Mark P Brady, PA-C
Chief Physician Assistant
Department of Emergency Medicine
Emerson Hospital
Concord, MA
Disclosure: Mark P Brady, PA-C, has disclosed no relevant financial relationships.
Editor
Olivia Wong, DO
Section Editor
Medscape Drugs & Diseases
New York, NY
Disclosure: Olivia Wong, DO, has disclosed no relevant financial relationships.
Reviewer
Yasmine S Ali, MD, MSCI, FACC, FACP
President
LastSky Writing, LLC
Ashland City, TN;
Assistant Clinical Professor of Medicine
Vanderbilt University School of Medicine
Nashville, Tennessee;
Chief Editor
Atherosclerosis section and Congenital Heart Disease section
Medscape from WebMD
New York, NY
Disclosure: Yasmine S Ali, MD, MSCI, FACC, FACP, serve(d) as a director, officer, partner, employee, advisor, consultant, or trustee for:
LastSky Writing, LLC; M Health; Philips Healthcare; MDLinx; Verywell Health and Health.com (DotDash Media); Edanz Group; Learnroll, LLC; Eradigm; WellnessVerge; Corvidane.
Received income in an amount equal to or greater than $250 from: Prose Media; Pharmaceutical Training Institute; Athena Health; AKH, Inc.; MedLearning Group; MDLinx; Elsevier; Future USA; MedStudy Corporation; Remedy Health Media.
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References
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