Case Report

Takotsubo Cardiomyopathy and CanalithRepositioning Procedure for Benign ParoxysmalPositional VertigoDOI: 10.3766/jaaa.21.2.2

Selmin Karatayli-Ozgursoy*

Larry B. Lundy*

David A. Zapala†

Keith R. Oken‡

Abstract

Background: Takotsubo cardiomyopathy, also known as left ventricular apical ballooning syndrome,

ampulla cardiomyopathy, or transient left ventricular dysfunction is characterized by chest pain, electro-cardiographic changes, transient left ventricular apical aneurysm, and normal coronary arteries. Tako-

tsubo is a round-bottomed, narrow-necked Japanese octopus trap and lends its name to takotsubocardiomyopathy because of its resemblance to echocardiographic and ventricular angiographic images

of the left ventricle in this condition. This appearance takes its source from peculiar, transient regionalsystolic dysfunction involving the left ventricular apex and mid-ventricle with hyperkinesis of the basal left

ventricular segments. Benign paroxysmal positional vertigo (BPPV) is the most common cause of vertigocaused by peripheral vestibular dysfunction. The symptoms of BPPV are attributed to intralabyrinthine

particles, presumed displaced otoconia. Thus, the treatment recommended for BPPV is head reposition-ing maneuvers.

Purpose: To present the first takotsubo cardiomyopathy case in the English literature related to BPPVundergoing canalith repositioning procedure.

Conclusion: This report will provide additional information for physicians encountering acute-onsetchest pain and vertigo. It will also expand the spectrum of clinical correlates of the increasingly well rec-

ognized but poorly understood syndrome, takotsubo cardiomyopathy.

Key Words: Benign paroxysmal positional vertigo, canalith repositioning procedure, complication,takotsubo cardiomyopathy, vertigo

Abbreviations: BPPV 5 benign paroxysmal positional vertigo

Takotsubo cardiomyopathy, also known as left

ventricular apical ballooning syndrome, ampulla

cardiomyopathy, or transient left ventricular

dysfunction, is characterized by peculiar, transientregional systolic dysfunction involving the left ven-

tricular apex and midventricle with hyperkinesis

of the basal left ventricular segments. Takotsubo

cardiomyopathy was first described in 1991 by Dote

and colleagues (1991) in Japan and named tako-

tsubo-like left ventricular dysfunction because of the

resemblance of the left ventricle to the appearance

of a round-bottomed, narrow-necked Japanese octopustrap on echocardiogram. Patients with takotsubo car-

diomyopathy usually present with sudden-onset chest

pain associated with ST segment elevation in the ante-

rior electrocardiographic leads (V1–V4), relatively

*Department of Otolaryngology, Mayo Clinic Florida; †Department of Audiology, Mayo Clinic Florida; ‡Division of Cardiovascular Diseases, MayoClinic Florida

Larry Lundy, M.D., Department of Otolaryngology, Mayo Clinic Florida, 4500 San Pablo Road, Jacksonville, FL 32224; E-mail: lundy.larry@mayo.edu

J Am Acad Audiol 21:73–77 (2010)

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minor elevation in cardiac enzymes, and transient api-

cal systolic left ventricular dysfunction despite the

absence of obstructive coronary artery disease (Bybee

et al, 2004b). The majority of patients with takotsubocardiomyopathy are women older than 60 yr of age.

Physiological or emotional stress usually precedes

the onset of symptoms (Akashi et al, 2003b). Cur-

rently, the incidence of takotsubo cardiomyopathy in

the setting of acute coronary syndrome is reported

to be between 1.5 and 2.2% (Akashi et al, 2003a; Bybee

et al, 2004a). The pathogenesis of the syndrome has

still been unclear; however, several mechanisms in-cluding multivessel epicardial spasm, catecholamine-

induced myocardial damage, microvascular coronary

spasm or dysfunction, and neurogenic myocardial

stunning have been suggested as potential causes

(Bybee et al, 2004b).

Benign paroxysmal positional vertigo (BPPV) is the

most common cause of vertigo caused by peripheral

vestibular dysfunction (Froehling et al, 2000). Thesymptoms of BPPV are attributed to intralabyrinthine

particles, presumed displaced otoconia (Schuknecht,

1969). Thus, the treatment recommended for BPPV

is head repositioning maneuvers that were introduced

by Semont (Semont et al, 1988) and modified by Epley

(1992). These maneuvers include rotational head and

body movements in order to relocate the floating par-

ticles from the involved semicircular canal into the ves-tibule of the labyrinth. The repositioning therapies

might be repeated in recurrent or refractory cases.

We present the first case with takotsubo cardiomyop-

athy in the English literature that was triggered by

and presented during canalith repositioning procedures

applied for the treatment of the patient’s existing

BPPV.

CASE REPORT

A 76-yr-old female patient presented to the Depart-

ment of Otolaryngology at Mayo Clinic Florida for

evaluation of her dizziness. The dizziness first started

1 yr ago with a sudden onset of hearing loss in the right

ear. Her brain MRI revealed no abnormality, and she

was diagnosed with acute right labyrinthitis with sec-ondary BPPV. She was initially treated with oral ste-

roids and subsequently has undergone a number of

canalith repositioning maneuver sessions with no sign

of improvement of her vertigo. Our otolaryngological

examination did not reveal any abnormality. Her audio-

gram showed a bilateral sensorineural hearing loss,

moderate and sloping in high frequencies on the left

and severe on the right with very poor discrimination.Her vestibular work-up included video electronystag-

mography, computerized dynamic posturography, rota-

tional testing, and vestibular evokedmyogenic potentials,

which demonstrated a right posterior canal BPPV and

no vestibulopathy otherwise. She underwent repeated

canalith repositioning procedures for the treatment

of her BPPV. Although the patient appeared to have

a classic case of right posterior canal BPPV, it seemedto be particularly resistant to treatment with tradi-

tional repositioning procedures. One week later, the

patient underwent another trial of canalith reposition-

ing procedure. When brought upright, she was vio-

lently dizzy and reported extreme nausea and chest

pain. As the chest pain persisted, she was taken to

the emergency room after a soft collar was placed

and phenergan (25mg intramuscular) was given forher nausea.

In the emergency room, the patient’s electrocardiog-

raphy revealed ST segment elevation in the anterolat-

eral leads without sign of reciprocal changes. Chest X

ray revealed a normal cardiac silhouette and no evi-

dence of pulmonary edema. Creatinine kinase-total,

creatinine kinase-MB, and troponin-T were elevated.

Her only cardiac risk factor was hyperlipidemia, forwhich she had been taking 20mg of simvastatin per

day. Her family history was negative for cardiac dis-

eases. With the elevated cardiac enzymes and electro-

cardiography implying acute myocardial infarction,

the patient was immediately referred for cardiac

catheterization. Coronary angiography revealed no sig-

nificant coronary artery disease. Echocardiography re-

vealed abnormal systolic function with anterior/septal/apical wall motion abnormalities, with left ventricular

ejection fraction in the range of 36–49% (Fig. 1). The

constellation of an unremarkable coronary angiogram,

antero-apical hypokinesis, elevated cardiac biomarkers,

electrocardiographic changes suggestive of anterior

injury, and a precipitating stressful experience sug-

gested a diagnosis of takotsubo cardiomyopathy secon-

dary to her profound vestibular vertigo. The patientwasadmitted to the hospital for monitoring and treated

with 3.125mg of carvedilol po BID and aspirin; angio-

tensin converting enzyme inhibitor could not be started

due to hypotension. The next day, her electrocardiogra-

phy revealed persistent ST–T wave elevation and pro-

longed QT intervals. She was discharged with 3.125mg

carvedilol po twice a day, simvastatin, and aspirin.

In postevent fourth-month follow-up, the patient wasasymptomatic, and echocardiogram revealed no abnor-

mality except some mild mitral regurgitation (Fig. 2).

DISCUSSION

Takotsubo cardiomyopathy was first recognized and

described in the Japanese population (Dote et al,

1991; Kawai et al, 2000; Tsuchihashi et al, 2001; Kurisuet al, 2002) as being characterized by chest pain, elec-

trocardiographic changes, transient left ventricular

apical aneurysm, and normal coronary arteries. Typical

presentation is usually in postmenopausal women

Journal of the American Academy of Audiology/Volume 21, Number 2, 2010

74

experiencing an inciting stressful event. In 2004, Bybee

and colleagues (2004b) proposed Mayo criteria for diag-

nosis of this syndrome (Table 1). Most takotsubo cardi-omyopathy cases present with acute-onset chest pain,

and initial work-up reveals electrocardiographic

changes such as ST segment elevation followed by T

wave inversions and QT prolongation and a mild but

rapid increase in cardiac enzymes (Bybee et al,

2004b). The diagnosis of takotsubo cardiomyopathy is

made by the absence of significant epicardial coronary

artery stenosis despite the presence of transient apicaland left midventricular systolic dysfunction. Case

reports suggest that the vast majority of patients are

women older than 60 yr of age presenting with sud-

den-onset chest pain after an episode of acute emotional

or physiological stress (Bybee et al, 2004b). Reversibil-

ity of the left ventricular dysfunction is a hallmark of

the syndrome (Akashi et al, 2003a).

Our patient was a 76-yr-old woman referred to the

emergency department with acute-onset chest pain

immediately after undergoing a canalith repositioningprocedure for BPPV. Her clinical presentation and sub-

sequent course were entirely consistent with takotsubo

cardiomyopathy. The incidence of takotsubo cardiomy-

opathy within patients diagnosed with acute myocar-

dial infarction has been reported to be from 1.5 to

2.2% (Akashi et al, 2003a; Bybee et al, 2004a). Although

the exact cause of takotsubo cardiomyopathy remains

unknown, many theories about the underlying mecha-nisms have been proposed. Among these are abnormal

fatty acid metabolism in the apex, coronary microcircu-

latory dysfunction, increased catecholamine in response

to stress inducing myocardial dysfunction, and diffuse

epicardial spasm. However, neurogenic myocardial

stunning is the leading candidate mechanism (Bybee

et al, 2004b; Brenner and Powers, 2008). It is not known

Figure 2. Echocardiographic apical four-chamber view at follow-up, fourmonths later. A. End-diastolic frame reveals normal leftventricular size and contour. B. End systolic frame reveals normal regional and global systolic function with resolution of the apicaldyskinesis.

Figure 1. Echocardiographic apical four-chamber view at presentation. A. End-diastolic frame reveals normal left ventricular size andcontour. B. End systolic frame reveals apical dyskinesis and typical “tako-tsubo” configuration caused by basal hyperkinesis (arrows) andapical akinesis/dyskinesis (arrow heads).

Takotsubo Cardiomyopathy and BPPV/Karatayli-Ozgursoy et al

75

why the apex of the heart is affectedwhile the basal seg-

ments are spared. However, it may partly be explained

by increased adrenergic receptor density, increased

sympathetic innervation, or increased adrenergic sensi-tivity in the apical myocardium (Mori et al, 1993). The

explanation for the strong female predominance of the

syndrome is also still unclear; however, it is speculated

to be related to reduced postmenopausal estrogen levels

leading to alterations of endothelial function (Celermajer

et al, 1994) and catecholamine-mediated microvascular

reaction (Ueyama et al, 2003).

Japanese physicians identified that the predominantand significant factor present in all cases of takotsubo

cardiomyopathy is internal (emotional) or external

(physiological) stress. They found that the death or

funeral of a family member, argument with a spouse

or partner, or intense excitation as well as medical dis-

orders such as acute asthma and noncardiac medical

procedures are common presenting events (Park et al,

2005). In our patient, the precipitating factor was thecanalith repositioning procedure applied for her existing

BPPV. It was noted that the patient was anxious that

day because of her persisting symptoms despite repeti-

tive canalith repositioning procedures. She expressed

skepticism that canalith repositioning would really help.

Presumably this emotional stress or the physiologic and/

or psychological stress induced by her adverse response

to the maneuver triggered the onset of takotsubo cardi-omyopathy at that particular session: despite the fact

that she had undergone repetitive canalith repositioning

procedures previously without complications.

Appropriate management of takotsubo cardiomyop-

athy is supportive medical care with angiotensin con-

verting enzyme inhibitor and beta-blockade, aspirin,

and intravenous diuretics as needed. Treatment should

be continued until left ventricular function returns tonormal on echocardiogram (Bybee et al, 2004b; Sealove

et al, 2008). Anticoagulation is generally deferred unless

apical thrombi are detected on imaging studies or embolic

events are suspected.

The short-term and long-term prognosis of patientswith takotsubo cardiomyopathy is quite favorable (Sealove

et al, 2008). However, reported in-hospital mortality

rates range from 0 to 8% (Bybee et al, 2004b). The most

frequently reported complication is left-sided heart fail-

ure with or without pulmonary edema (Bybee et al,

2004b). Infrequently reported complications include

cardiogenic shock, ventricular arrhythmias, left ven-

tricular thrombus,mitral valve dysfunction, pulmonaryembolism, and left ventricular rupture (Tsuchihashi

et al, 2001; Bybee et al, 2004b). Our patient did not have

any immediate or late cardiac complications; at poste-

vent fourth-month follow-up, the patient was asympto-

matic, and echocardiogram revealed no abnormality

except some mild mitral regurgitation.

The misdiagnosis of takotsubo cardiomyopathy as an

acute myocardial infarction may result in unnecessaryadministration of fibrinolytic medications, with their

attendant risk of bleeding complications. It is important

for emergency physicians as well as cardiologists to

recognize a suggestive clinical history of takotsubo

cardiomyopathy, especially in elderly female patients,

so that these patients may be referred for emergent

coronary angiography, which offers optimal triage

and management. However, if an emergency coronaryangiogram cannot be obtained, patients should be trea-

ted with standard acute myocardial infarction therapy.

To our knowledge this is the first takotsubo cardio-

myopathy case in the English literature related to

BPPV undergoing canalith repositioning procedure. We

believe that this report will provide additional informa-

tion for physicians encountering acute-onset chest pain

and vertigo. It will also expand the spectrum of clinicalcorrelates of the increasingly well-recognized but poorly

understood syndrome, takotsubo cardiomyopathy.

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