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Image of Takotsubo cardiomyopathy with myocardial rupture from Mitchell A, Marquis F. BMC Clin Pathol. 2017;17:4. [Open access.] PMID: 28396614; PMCID: PMC5382367.

Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)

Mark P Brady, PA-C | January 27, 2021 | Contributor Information

Takotsubo cardiomyopathy (TCM), first described in 1990 by Sato et al,[1,2] is a transient cardiac syndrome that typically involves left ventricular (LV) apical akinesis and basal hypercontraction that may symptomatically mimic acute coronary syndrome (ACS).[1,3]

A significant emotional (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 LV 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 | January 27, 2021 | Contributor Information

Pathophysiology

TCM is named for the resemblance of the LV apical ballooning (left) to the round bottoms and narrow necks 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 and 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 catecholamines (eg, apical segments more responsive to negatively inotropic epinephrine) appear to support the role of catecholamines in the pathogenesis of TCM.[1]

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 | January 27, 2021 | Contributor Information

The systolic cine magnetic resonance image (MRI) (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. These findings are in contrast to coagulation necrosis, which is seen in myocardial infarctions (MIs) as a result of coronary artery occlusion.[5-8] Animal models of catecholamine toxicity have also shown these pathologic patterns.[9,10]

Image courtesy of Medscape.

Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)

Mark P Brady, PA-C | January 27, 2021 | 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.[1,3,11-13] Patients with TCM often present with chest pain, ECG ST-segment elevation, and elevated cardiac enzyme levels that are consistent with an acute MI. However, when the patient undergoes coronary angiography, no significant obstructive coronary artery stenosis is evident (shown), and when left ventriculography is performed, left ventricular apical ballooning is present.[14]

Image courtesy of Medscape.

Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)

Mark P Brady, PA-C | January 27, 2021 | Contributor Information

The left ventriculogram during systole shows the characteristic apical ballooning with apical akinesis of TCM (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 and dyspnea, palpitations, nausea). However, unlike ACS, for which peak occurrence is the morning hours, TCM events are most prevalent in the afternoon, when stressful triggers may be more likely to take place.[15] 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).[16] 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 | January 27, 2021 | 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[1,3,17]:

  • Transient hypokinesis, dyskinesis, or akinesis of the LV midsegments, with or without apical involvement; the regional wall-motion abnormalities (RWMAs) 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
Images from Gangadhar TC, Von der Lohe E, Sawada SG, Helft PR. J Med Case Rep. 2008;2:379. [Open access.] PMID: 19063741, PMCID: PMC2633360.

Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)

Mark P Brady, PA-C | January 27, 2021 | Contributor Information

The echocardiograms are from a 64-year-old woman and show (A) dilatation of the LV in the acute phase and (B) resolution of LV function on repeat echocardiography 6 days later.

In January of 2016, the Task Force on Takotsubo Syndrome of the Heart Failure Association of the European Society of Cardiology released a position statement on the diagnosis and treatment of TCM. The following diagnostic criteria for takotsubo syndrome were presented[18]:

  • A stressful trigger that is followed by transient regional wall-motion abnormalities of the LV or right ventricular (RV) myocardium (these regional wall-motion abnormalities usually extend beyond a single epicardial vascular distribution)
  • The absence of culprit atherosclerotic CAD
  • During the acute phase, new and reversible ECG abnormalities are present
  • Elevated natriuretic peptide level
  • Elevation in cardiac troponin levels measured with a conventional assay
  • At follow-up, cardiac imaging shows recovery of ventricular systolic function
Images from De Backer O, Debonnaire P, Gevaert S, Missault L, Gheeraert P, Muyldermans L. BMC Cardiovasc Disord. 2014;14:147. [Open access.] PMID: 25339604, PMCID: PMC4210484.

Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)

Mark P Brady, PA-C | January 27, 2021 | Contributor Information

(A) Doppler echocardiogram identifies severe LV outflow tract obstruction. (B) Echocardiogram shows apical ballooning. These images are from a 74-year old woman who was admitted in cardiac shock and with ST-segment elevation in the precordial leads.

The Task Force on Takotsubo Syndrome further differentiates between primary and secondary TCM.[18] In primary TCM, patients present for care primarily because of cardiac symptoms, in the presence or absence of a stressful trigger. Secondary TCM occurs in patients already hospitalized for other medical, surgical, or psychiatric indications. In this group, the sudden rise of catecholamines, occurring as a result of a complication of the primary medical, surgical, or psychiatric issue, serves as the trigger for TCM.[18,19]

Images from Parsai C, O'Hanlon R, Prasad SK, Mohiaddin RH. J Cardiovasc Magn Reson. 2012;14:54. [Open access.] PMID: 22857649, PMCID: PMC3436728.

Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)

Mark P Brady, PA-C | January 27, 2021 | Contributor Information

MRI short tau triple inversion-recovery fast spin-echo (STIR) images are shown. (A) Acute viral myocarditis revealing edema. (B) TCM with acute LV apical ballooning (a, diastole; b, systole).

Once ischemic heart disease has been excluded, one of the principal challenges in the diagnosis of TCM is differentiating between TCM and infectious myocarditis. Both TCM and infectious myocarditis can present with symptoms of heart failure or ACS, ST elevation, mild troponin release, and decreased LV ejection fraction (LVEF). Infectious myocarditis and TCM may also appear very similar on multiple imaging modalities, including CMRI (with the exception of direct contrast uptake, which is usually seen in acute myocarditis and ischemia) and echocardiography.

Elevations of acute phase inflammatory molecules, such as C-reactive protein (CRP), may be more pronounced in infectious myocarditis. Furthermore, elevations of brain natriuretic peptide (BNP) may be more notable in TCM. Viral serologies, as well as polymerase chain reaction (PCR), may assist in clarifying the diagnosis, in that these tests may be positive in the setting of infectious myocarditis.[18,19]

Image courtesy of Medscape.

Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)

Mark P Brady, PA-C | January 27, 2021 | Contributor Information

ECG 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),[1,3,20] though 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).

In addition, 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.[1,3,21,22]

Image from Lisi M, Zaca V, Maffei S, et al. Cardiovasc Ultrasound. 2007;5:18. [Open access.] PMID: 17417970, PMCID: PMC1852545.

Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)

Mark P Brady, PA-C | January 27, 2021 | Contributor Information

This is an admission ECG from a 68-year-old patient with TCM. In leads V2-V3, 2- to 3-mm ST-segment elevation is evident, in conjunction with sinus tachycardia. Leads V5-V6 demonstrate 1-mm ST-segment depression.

Image from Lisi M, Zaca V, Maffei S, et al. Cardiovasc Ultrasound. 2007;5:18. [Open access.] PMID: 17417970, PMCID: PMC1852545.

Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)

Mark P Brady, PA-C | January 27, 2021 | Contributor Information

This is an ECG on postadmission day 1 from the same patient as in the previous slide. In leads V2-V3, evolving T-wave inversion can be seen. In leads V5-V6, the ST-segment depressions that were noted on the admission ECG have now normalized.

Image courtesy of Medscape.

Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)

Mark P Brady, PA-C | January 27, 2021 | Contributor Information

The 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 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.[21]

Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)

Mark P Brady, PA-C | January 27, 2021 | 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,[1,3,23] thereby broadening recognition of the condition and improving clinical outcomes.[23] 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 diagnoses.

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 | January 27, 2021 | Contributor Information

Representative short-axis CMRI slices from five imaging modalities are shown in three individuals 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 (green = normal myocardium; red = increased T1 values): Color shortened modified look-locker (ShMOLLI) T1 maps. Column D: R2 (relaxation rate) maps of ShMOLLI inversion recovery fit (for quality verification of images in column C). Column E: Imaging with late gadolinium enhancement (LGE).

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 | January 27, 2021 | 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 STIR image. There is increased myocardial apical signal intensity that could be difficult to differentiate from intraluminal blood signal in cases of slow flow. Right image: LGE 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 | January 27, 2021 | 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.[24] 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 normal ventricular movement.

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 | January 27, 2021 | Contributor Information

Left images: Normal coronary angiograms from a patient with TCM 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[3,25]; all patients with suspected TCM should be treated as having ACS until proved otherwise. TCM therapy is mainly empirical and supportive.[1,3,25] When the patient's hemodynamic status permits, beta blockers may be helpful. In the 5-8% of TCM patients with an LV thrombus, anticoagulation is needed.[26]

Close follow-up care with a cardiologist, usually including serial echocardiograms, is advised in the weeks after the diagnosis is made in order to ensure resolution of the cardiomyopathy. Thereafter, annual monitoring is warranted because the long-term effects and natural history of TCM are unknown. Still, 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,[18] and Peters MN, George P, Irimpen AM.[19]

Keys to Diagnosing Broken Heart Syndrome (Takotsubo Cardiomyopathy)

Mark P Brady, PA-C | January 27, 2021 | Contributor Information

Complications

Although management of TCM is mainly supportive and the outcomes are typically good, 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 to provide guidance exist; in general, it is advised to avoid catecholaminergic inotropic agents such as dobutamine because such agents may serve to exacerbate the theorized stress caused by endogenous epinephrine.

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