Novel treatment (new drug/intervention; established drug/procedure in new situation)

CASE REPORT

Wearable cardioverter defibrillator in stress

cardiomyopathy and cardiac arrest
Francisco O Nascimento,1 Rama K Krishna,1 Hakop Hrachian,2 Orlando Santana3
1
Department of Cardiology, SUMMARY medical history was diagnosed with severe hypokal-
Mount Sinai Medical Center, A 57-year-old woman presented with nausea, vomiting aemia (2 mmol/dL) and hypomagnesemia (1.2 mg/
Miami Beach, Florida, USA
2
Department of and diarrhoea. She had severe hypokalaemia and dL), laboratory testing including stool samples
Electrophysiology, Mount Sinai hypomagnesemia with marked QTc (680 ms) testing and review of history were inconclusive for a
Medical Center, Miami Beach, prolongation after suspected viral diarrhoea. She then cause, and viral aetiology for diarrhoea was sus-
Florida, USA developed progressive dyspnoea with congestion. pected. The ECG is shown (figure 1). Besides, sinus
3
Division of Cardiology,
An echocardiogram was obtained and showed severe bradycardia and diffuse T wave inversion, the QTc
Columbia University, Mount
Sinai Medical Center, Miami hypokinesis with apical ballooning and hyperdynamic interval was 680 ms. On day 2 of admission, the
Beach, Florida, USA cardiac base, suggestive of stress cardiomyopathy. patient could not sleep due to anxiety. The diar-
A repeat ECG showed further prolongation of the QTc rhoea has resolved, but the patient started to have
Correspondence to (883 ms) and she rapidly developed polymorphic dyspnoea and signs of congestion for which an
Dr Francisco O Nascimento,
[email protected] ventricular tachycardia. She underwent cardiac arrest and echocardiogram and ECG were obtained. The echo-
was successfully resuscitated. A coronary angiogram cardiogram showed severe hypokinesis with apical
confirmed the diagnosis of stress cardiomyopathy. ballooning and hyperdynamic cardiac base, whereas
We had therapeutic dilemma at discharge to implant a the ECG demonstrated markedly prolonged QTc of
permanent automated implantable cardiac defibrillator in 883 ms. The electrolytes were back to normal range.
view of the high risk for recurrent ventricular tachycardia, Subsequently on the same day the patient developed
or follow-up for resolution of both reversible causes of the cardiac arrest due to polymorphic ventricular tachy-
prolonged QTc (stress cardiomyopathy and electrolytes cardia requiring defibrillation. There was no family
abnormalities). We suggested an alternate treatment for history of sudden death and she was not on any
sudden death prevention in high risk patients who have medication recently to have contributed to pro-
reversible cause for QT interval prolongation. longation of QT. She underwent emergent cardiac
catheterisation which showed no obstructive disease
in the coronary arteries. The left ventriculogram
BACKGROUND showed severe apical ballooning with an ejection
Stress cardiomyopathy (Takotsubo) is characterised fraction of 20%. The findings were consistent with
by reversible left ventricular wall motion abnormal- stress cardiomyopathy, although no stressors were
ities, extending beyond a single epicardial vascular identified other than severe malaise due to diarrhoea
distribution and in the absence of obstructive cor- and vomiting, and anxiety for being in the hospital.
onary artery disease. It usually affects elderly The patient was treated supportively, including a
women usually after a stressful event or severe temporary transvenous pacer for faster pacing and
acute illness. The ECG typically shows ST segment to shorten the QT interval during the subsequent
elevation or T wave inversions and QT interval pro- days of hospitalisation. On day 8, she had diffuse T
longation. The treatment is supportive and the wave inversion on ECG and a QTc of 521 ms, but
prognosis is usually favourable.1 This patient was was hemodynamically stable and able to be dis-
unusual with severe electrolyte abnormalities and charged out of the hospital. As she had an episode
QTc prolongation which led to cardiac arrest in of unstable Torsades de Pointes (TdP), β-blocker was
polymorphic ventricular tachycardia event during avoided to prevent eventual recurrence in case of
hospitalisation requiring secondary prevention for bradycardia.
sudden cardiac death (ie, AICD), as the QTc per- We believed that the severe prolongation of QT
sisted prolonged at the time of discharge. The was secondary to stress cardiomyopathy based on
AICD implantation however, would commit the the evidence published but we did have a thera-
patient to a permanent device for a potentially peutic dilemma at discharge whether to implant an
reversible (treatable) cause, where an implantable AICD in view of cardiac arrest and prolonged QTc,
hardware could be avoided. The temporary treat- or follow-up for resolution in view of associated
ment with wearable cardioverter defibrillator stress cardiomyopathy and severe electrolyte abnor-
(WCD) and close follow-up was effective and this mality. She remained at high risk for recurrent ven-
approach presents as an alternate therapy with no tricular tachycardia and sudden cardiac death.
To cite: Nascimento FO, additional risk. By writing this up evidence is pro-
Krishna RK, Hrachian H, vided for a very effective non-invasive treatment.
et al. BMJ Case Rep
OUTCOME AND FOLLOW-UP
Published online: [please The patient was discharged with a WCD after com-
include Day Month Year] CASE PRESENTATION plete orientation about the device and details
doi:10.1136/bcr-2013- A 57-year-old woman with 2-day history of nausea, regarding medications to avoid in future in view of
009789 vomiting and diarrhoea with unremarkable prior prolonged QTc were provided. The patient was

Nascimento FO, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-009789 1

 Novel treatment (new drug/intervention; established drug/procedure in new situation)

Figure 1 ECGs during disease and recovery.

able to follow the instructions and was able to wear the device during her hospitalisation, led her into cardiac arrest due to
as advised but admitted to the fear of possible defibrillation polymorphic ventricular tachycardia (Tdp), which was success-
anytime and had limited herself to a certain extent and made fully treated. A WCD was placed before committing the patient
sure that a family member was around her most of the time. She to a permanent intracardiac device, given the fully reversible
was followed up in the clinic 2 weeks later and the echocardio- nature of the process. If during the recovery period further
gram showed normal left ventricular ejection fraction. She was events were noted, then an AICD implantation should be con-
in sinus bradycardia at 52 bpm, and a QTc of 416 ms (figure 1). sidered for long-term management. Further studies are needed
The WCD was then discontinued. to confirm this hypothesis.
LifeVest (figure 2) is the WCD approved for sales in the USA
DISCUSSION and Europe. Unlike an AICD, the LifeVest is worn outside the
We report a case of a relatively young woman with a combin- body rather than implanted in the chest. Common indications
ation of predisposing factors for prolonged QT: stress cardiomy- for WCD are patients with early postmyocardial infarction with
opathy, hypomagnesemia and hypokalaemia. The QTc worsened severe left ventricular dysfunction and left ventricular ejection

2 Nascimento FO, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-009789

 Novel treatment (new drug/intervention; established drug/procedure in new situation)

Figure 2 LifeVest (Image courtesy

Zoll Medical Corp.).

fraction (LVEF) <35, newly diagnosed non-ischemic cardiomy-

opathy with LVEF <35, as a bridge to heart transplantation or a Learning points
temporary inability to implant an ICD due to infection. Patients
and family are explained to reduce unprotected situations off
▸ Temporary QT prolongation is noted with stress
WCD (like during shower) to a minimum and to be assisted by
cardiomyopathy and it reflects the transient myocardial insult
family. The WCD represents an alternative approach to prevent
and it usually resolves to normal in few weeks after recovery.
sudden arrhythmic death until either AICD implantation is
▸ When Torsades de Pointes (TdP) occurs during stress
clearly indicated or the arrhythmic risk is considered signifi-
cardiomyopathy, the risk for sudden cardiac death increases.
cantly lower or even absent.2
▸ A wearable cardioverter defibrillator is an effective
It is proposed that the temporary QT prolongation reflects
alternative to implanted automated implantable cardiac
the transient myocardial insult of stress cardiomyopathy.
defibrillator and its associated risks, especially given the
Although this phenomenon predisposes for ventricular tachy-
fully reversible nature of the process.
cardia, frequency of such event is as low as 1%.3 Recurrence
▸ This case is a learning example for complex presentation of
of stress cardiomyopathy can be up to 11.4% but survival
stress cardiomyopathy and its associated complications and
rates are no different to age-matched and gender*matched
management strategies.
population.4 Stress cardiomyopathy has been shown to be
associated with life-threatening ventricular arrhythmia in over
8% of cases as noted from a registry of consecutive patients
and should be recognised among the causes of acquired long Contributors FCN indentified the case, was involved in follow-up of the patient,
QT syndrome and can be associated with risk of Tdp.5 drafted and edited the report. RKK was involved in drafting the report. HH reviewed
Recently TdP and sudden death have emerged as complica- and edited the report. OS was involved in drafting the article and approved the final
tions associated with stress cardiomyopathy; however, version of the manuscript. All four authors were involved in managing the case and
are the contributors as being responsible for the overall content as guarantors.
described only in few case reports. It was suggested as an
association between the prolongation of the presenting and Competing interests None.
maximal QTc intervals in development of TdP. A cut-off QTc Patient consent Obtained.
of 500 ms during the acute phase of stress cardiomyopathy is Provenance and peer review Not commissioned; externally peer reviewed.
considered as a predictor for TdP, with sensitivity and specifi-
city greater than 80%.6 Findings from the WEARIT-II regis-
try suggest that in real world management strategies using
REFERENCES
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AICD therapy. ballooning: a syndrome that mimics ST-segment elevation acute myocardial infarction.
WCD can be as effective as AICD. In a US postmarket study Ann Intern Med 2004;141:858–65.
of 3569 patients, 80 sustained ventricular tachycardia/ventricu- 2 Klein HU, Meltendorf U, Reek S, et al. Bridging a temporary high risk of sudden
arrhythmic death. Experience with the wearable cardioverter defibrillator (WCD).
lar fibrillation (VT/VF) events occurred and a first shock success Pacing Clin Electrophysiol 2010;33:353.
in terminating VT/VF was 100% among unconscious patients 3 Mahida S, Dalageorgou C, Behr E. Long QT syndrome and Torsades de Pointes in a
and 99% for all patients.7 patient with Takotsubo cardiomyopathy: an unusual case. Europace 2009;11:376–8.

Nascimento FO, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-009789 3

 Novel treatment (new drug/intervention; established drug/procedure in new situation)

4 Elesber AA, Prasad A, Lennon RJ, et al. Four-year recurrence rate and prognosis of 6 Behr ER, Mahida S. Takotsubo cardiomyopathy and the long-QT syndrome: an insult
the apical ballooning syndrome. J Am Coll Cardiol 2007;50:448–52. to repolarization reserve. Europace 2009;11:697–700.
5 Madias C, Fitzgibbons TP, Alsheikh-Ali AA, et al. Acquired long QT syndrome from 7 Chung MK, Szymkiewicz SJ, Shao M, et al. Aggregate national experience with the
stress cardiomyopathy is associated with ventricular arrhythmias and torsades de wearable cardioverter-defibrillator: event rates, compliance, and survival. J Am Coll
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4 Nascimento FO, et al. BMJ Case Rep 2013. doi:10.1136/bcr-2013-009789