Stress-Induced Cardiomyopathy: Broken Heart Syndrome

Cardiomyopathy characterized by transient apical and
midventricular LV dysfunction in the absence of significant
coronary artery disease that is triggered by emotional or
physical stress.
− In setting of depressed/abnormal function of distal and apical LV segments
there is compensatory hyperkinesis of basal walls  “ballooning” of apex
during systole.
copyright@drsudebmukherjee

1st
described in Japan in 1991
Named after the tako-tsubo, which is an octopus trap
− Shape of the trap is similar to the appearance of LV apical
ballooning noted in patients with this form of
cardiomyopathy
Was later described elsewhere as well and is being
increasingly recognized.

Kurisu, S., et al. 2002. American Heart Journal. 143: 448-455.

The Name
A Japanese term, named after the jar used for trapping octopus

In Popular Culture
• A mechanism of being scared to death?
o Ananias and Sapphira
o Legal
 Larry Whitfield – on trial for 1st degree murder
while holding 79F at gunpoint – “fear induced
heart attack”
 Willie Ingram – convicted of murder for
“emotional upset” causing heart attack, a 64M
 Mark Fisher – convicted of murder of 89F for
“fear induced heart attack”

Takotsubo cardiomyopathy
Stress-induced cardiomyopathy
Transient left ventricular apical ballooning syndrome
Apical ballooning syndrome
Broken heart syndrome
Ampulla cardiomyopathy
Different Names:

May account for up to 2% of suspected ACS
In-hospital mortality ranges 0-8%
Much more common in women (~90%), especially
postmenopausal women (>80% of cases)
Mean age 58-75 years
Triggers: death of loved one, other catastrophic news,
devastating financial losses, natural disasters, physical
illness/ICU, etc.copyright@drsudebmukherjee

CHARACTERISED BY-
• An acute completely reversible systolic heart failure
• Typical: Apical akinesia [ballooning] and hypercontractile
base
• Atypical: Midventricular akinesia and hypercontractile base
• No relevant CAD
• Mimics symptoms of ACS

CLASSIFICATION
1. Takotsubo type: apical akinesia and basal hypercontraction;
2. Reverse takotsubo: basal akinesia and apical
hypercontraction;
3. Mid ventricular type: mid ventricular ballooning and
basal/apical hypercontraction
4. Localized type: any other segmental ballooning when
Takotsubo-like LV dysfunction is present.

ESC/HFA STATEMENT
CLASSIFICATION

Causes/Epidemiology
• Triggered by extreme emotional or physical stress
o Deaths, accidents, surprise party, procedure,
arguments, legal, public speaking, armed robbery
• Strong predominance in postmenopausal women
• Under-recognized, ~2% of all ACS

Reported triggers
• Emotional
• Death of a loved one (including pets)
• Surprise party
• Family member being arrested
• Fierce argument
• Robbery
• Public speaking
• Surgery – Hysterectomy, Cholecystectomy
• Stress echo with dobutamine
• Opiate withdrawal
• Thyrotoxicosis
• Physical exhaustion ( triathlon, sexual, gym)- males

Pathophysiology
1. Coronary Spasm or Stunned myocardium:
1. Not favoured
2. Wall involvement extends beyond single vascular territory
3. Few patients demonstrate spasm with provocation during
catheterization
4. CEs only slightly elevated, not high enough
2. Microvascular Impairment:
1. Certainly present (As evident from several studies)
2. Correlative, but causation doubted

Pathophysiology
3. Catecholamine Cardiotoxicity
1. Plasma levels of Epi/NE increased, even higher than
in pt’s w/ similar HF.
2. Not uniformly present, but close
3. Histological findings simliar to in other forms of
catecholamine cardiotoxicity
4. Pheochromocytoma can cause similar cardiac event

MECHANISM:
1) Apical-basal gradients of β-adrenergic receptors (βARs) and sympathetic
innervation in mammalian LV, where apex contains the highest βAR and
the lowest sympathetic nerve density. The presence of ventricular βAR
gradient results in increased apical responsiveness to catecholamines
predominantly epinephrine
2) Epinephrine, at high levels can have negative inotropic impact and
trigger a switch from intracellular trafficking, from Gs (stimulatory)
protein to Gi (inhibitory) protein signaling through the β2AR. This negative
inotropic affect is greatest in apex where the density of βARs is highest.

Pathophysiology
4) Transient coronary artery occlusion/reperfusion
episodes:
Some investigators observed that transients LV dysfunction can
be caused by atheromatous ruptured plaque that is not clearly
visible on coronary angiography ( animal model has failed to
support this hypothesis.)

Pathophysiology
5) Oxidative stress response to excess catecholamine
Recent studies suggest that oxidative stress in response to excess of
catecholamines may be the underlying mechanism of LV dysfunction in TC.
( lack of evidence - how oxygen free radicals are released: )
 Directly in response to increased concentration of catecholamines ?
 As a result of catecholamines provoked microvascular changes?
 As a result of myocyte injury caused by various other mechanisms?

Pathophysiology
6) Relative deficiency of estrogen:
 Estrogen deficiency may be responsible for the development of TC.
 Ueyama et al. showed that ovariectomized rats were more prone to a stress
and demonstrated higher increase of the heart rate and reduction in LV function
comparing to the rats that had estradiol supplementation .
 Postmenopausal women lose the protective effect of estrogens which make
them more prone to the excess of circulating catecholamines.

Why Predominantly Women?
Stress induces upregulation of--
 Immediate early genes (IEGs),
Certain proto-oncogenes
Heat shock proteins
(transiently activated to rapidly adapt to a stressor BUT detrimental for CV
physiology)
Oestrogen minimises these factors.

Oestrogen?
Oestrogen may also play a role in enhancing the -adrenoreceptorβ
sensitivity and in promoting vasodilation.
Post-menopausal women have a decreased -adrenoreceptorβ
responsiveness to catecholamine stimulation than younger females.
However, their -adrenoreceptor vasoconstriction response toα
catecholamines remains the same.
 There is more -adrenoreceptor stimulation in relation to -β β
adrenoreceptor responsiveness thus leading to more vasoconstriction,
which in the setting of endothelial dysfunction may trigger TTC.

Why Less In Male?
Males are better protected biologically against stress (strossberg et al)
METABOLIC THEOREY:
 Oestrogen has also been implicated in maintaining appropriate glucose uptake
for cardiac energy .
 Female heart depends on glucose as its energy source more than the male
heart
 The relative lack of oestrogen as women age, therefore, may potentiate this
attenuated glucose uptake thus predisposing postmenopausal females to TTC.
 Males are not predisposed to TTC despite their relative lack of oestrogen
because they are not as dependent on glucose as their preferential cardiac
energy substrate.

Pathophysiology
7) Infective agent such as a viral illness
Infiltration by mononuclear lymphocytes and macrophages are usually
observed in histological examination of TC patients, nevertheless, no
infective agent has been successfully isolated from TC patients.
8) Genetic predisposition
Some authors suggested genetic predisposition due to a reported familial
association, nevertheless no genetic studies supported genetic basis of this
disorder.

Pathophysiology

1. Transient a/dyskinesis of apical and midventricular segments
in association with regional wall motion abnormalities that
extend beyond the distribution of a single epicardial vessel
2. Absence on angiography of obstructive coronary artery
disease or evidence of acute plaque rupture
3. New ST segment elevation or T wave inversions on ECG
4. Absence of recent significant head trauma, intracranial
bleeding, pheochromocytoma, myocarditis, or hypertrophic
cardiomyopathy
Proposed by Bybee, et al. 2004. Annals of Internal Medicine. 141: 858-865.

Mayo clinic criteria:
1. Typical LV contraction pattern: transient hypokinesia, akinesis or dyskinesia
in the LV mid segments with or without apical involvement accompanied with
hypercontraction in the basal segments; RWMA that extend beyond a single
coronary artery vascular distribution; stressful trigger is usually but not always
present;
2. Absence of obstructive CAD or angiographic evidence of acute plaque rupture;
3. Newly developed ECG abnormalities (ST segment elevation and/or T-wave
inversion) or modest elevation in cardiac troponin;
4. Absence of recent head trauma, intracranial hemorrhage, pheochromocytoma,
myocarditis or hypertrophic cardiomyopathy

Signs / Symptoms
• Think ACS
o CP, dyspnea, syncope, palpitaion, nausea , vomiting.
• Complications
o Pulmonary edema and respiratory failure
o Cardiogenic shock
o Ventricular tachyarrhythmias
o Ventricular wall rupture
o Mural thrombus

 Substernal chest pain
 ECG abnormalities
− ST elevation (usually anterior precordial leads)- 82%
− ST depression
− T wave inversion
− QT prolongation
− Abnormal Q waves
 Elevated cardiac biomarkers
Clue: Devoid of any traditional Risk Factors

Differential diagnosis
• Acute Coronary Syndromes
• Acute Myocarditis
• Angina Pectoris
• Aortic Dissection
• Boerhaave Syndrome
• Cardiac Tamponade
• Cardiogenic Shock
• Cardiomyopathy (Cocaine/ Dilated /Hypertrophic)
• Coronary Artery Vasospasm

 Tachyarrhythmias, bradyarrhythmias
 Pulmonary edema
 Cardiogenic shock
 Transient LV outflow tract obstruction
 Mitral valve dysfunction
 Acute thrombus formation and stroke
 Death

COMPLICATION/MORTALITY

 Because presentation is similar to ACS, proceed Accordingly.
 LV ventriculogram and/or echocardiography can both be used to visualize
apical ballooning with a/dyskinesis of apical ½ to ⅔ of the LV.
− Average LV EF range 20-49%
− Can have “atypical” ballooning of the middle or basal portions of the
LV (much less common)
− Wall motion abnormalities typically involve the distribution of more
than one coronary artery
 Ventriculography and echocardiography also allow evaluation for LV
outflow tract obstruction (~16%).
 Cardiac catheterization reveals lack of flow limiting coronary lesions or
evidence of plaque rupture.

Lab Evaluation:
• CE: Normal or slightly elevated
• Elevated BNP
• High serum catecholamines.

EKG
ST Elevation, TWI, Prolonged QT

Copyright ©2007 BMJ Publishing Group Ltd.
Nef, H. M et al. Heart 2007;93:1309-1315
Figure 1 Selective coronary angiography. Left (A) and right (B) coronary arteries in a
patient presenting with tako-tsubo cardiomyopathy, excluding coronary artery
disease. Left ventriculography during diastole (C) and systole (D) demonstrate the
typical left ventricular apical ballooning and a hypercontractile base.

Typical
Atypical

Copyright ©2007 BMJ Publishing Group Ltd.
Nef, H. M et al. Heart 2007;93:1309-1315
Figure 4 Transthoracic echocardiogram showing four-chamber views during diastole
(A) and systole (B) in a patient presenting with tako-tsubo cardiomyopathy. Real time
three-dimensional echocardiography shows the typical contractile pattern of tako-
tsubo cardiomyopathy with akinesia of apical segments and hypercontractility of the
basal segments (diastole, C; systole, D).

Cardiac MRI
 The most characteristic finding is ventricular edema that appears as
high signal intensity with a diffuse or transmural distribution.
 Moreover, the location of the edema is not related to a vascular territory
of coronary arteries, and edema is distributed in both the apical and mid
planes of the LV.
 The area of edema shows dysfunction in the ventricular contraction
observed with cine MRI sequence.

MRI:

MRI In MI:

 Supportive, conservative therapy
Hydrate, remove stress (if possible)
 Treat LV dysfunction with standard heart failure regimen- including
beta blocker, ACE inhibitor, diuretics (if volume overloaded), aspirin
Usually treated for ~6 months
 For pts who are hypotensive with shock, perform echo to evaluate for
LVOT obstruction.
− No LVOT obstruction inotropes, IABP if needed
− +LVOT obstruction NO inotropes (can worsen obstruction), use
beta blockers (+/- α agonist Phenylephrine), IABP if needed
− +/- fluid resuscitation (evaluate pulmonary status)

Treatment
Supportive care according to
complications
• Arrhythmias
• Cardiogenic shock
• Pulmonary edema
Careful use of pressors

Prognosis
• 95% complete recovery within 4-8 weeks.
• 3% recurrence
• Complications
• Death 1%
• Left heart failure with and without pulmonary edema
• Cardiogenic shock
• Left ventricular outflow obstruction
• Mitral regurgitation
• Ventricular arrhythmias
• Left ventricular mural thrombus formation
• Left ventricular free-wall rupture

 Takotsubo cardiomyopathy is a syndrome of transient dysfunction of
apical/midventricular LV with compensatory hyperkinesis of basal
segment resulting in apical ballooning.
 It is triggered by significant emotional or physical stress.
 It is more common in post-menopausal women.
 Presentation is similar to MI (symptoms, ECG changes, and biomarker
elevations). Accounts for ~1-2% of suspected ACS cases.
 No significant coronary artery disease or evidence of plaque rupture can
be identified.
 LV function recovers, typically within 4 weeks.

A 75 year old woman presents with pneumonia and chest
pain
Case study
In the last 24 hours, she
has experienced:
 Dyspnea
 Sputum production
 Fever
 Cough
She presents to the ER
for evaluation

T 101.2, BP 100/65, HR 110 (sinus tachycardia)
Diffuse rhonchi, decreased breath sounds left base
S1, S2 normal, No murmur, S3, S4
CXR: Infiltrate left lower lobe c/w pneumonia

ECG

Nef HM, et al. Tako-tsubo
Cardiomyopathy (Apical
Ballooning). Heart. 2007; 93:1309-
1315.

Case
a) She will need symptom limited stress test in 3-4 weeks
to determine prognosis
b) Biventricular pacing will improve prognosis.
c) Overall prognosis is poor given the severe LV
dysfunction.
d) Overall prognosis is good. She should have significant
improvement in LV function within 4 weeks.

Answer
d. Overall prognosis is good. She should have
significant improvement in LV function within 4
weeks.
 She has stress induced cardiomyopathy.
Takotsubo Cardiomyopathy, Apical ballooning
syndrome

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Stress-Induced Cardiomyopathy: Broken Heart Syndrome

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