Apical Ballooning Syndrome

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Transient Left VentricularTransient Left Ventricular

Apical Ballooning SyndromeApical Ballooning Syndrome

TakoTako--Tsubo / AmpullaTsubo / Ampulla

CardiomyopathyCardiomyopathy


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CharacteristicsCharacteristics First described in 1991 by Sato & Dote et al in Japanese literatureFirst described in 1991 by Sato & Dote et al in Japanese literature

AnginaAngina

ST segment elevation / diffuse T wave inversion / abnormal Q wavesST segment elevation / diffuse T wave inversion / abnormal Q waves

RWMA in lower anterior wall and apexRWMA in lower anterior wall and apex

Myocardial enzyme release (mild to mod.)Myocardial enzyme release (mild to mod.)

Hemodynamically insignificant stenosis on CAGHemodynamically insignificant stenosis on CAG

Psychological / physical stress within minutes to hours of onset ofPsychological / physical stress within minutes to hours of onset ofsymptomssymptoms

Hemodynamic compromise, cardiac arrestHemodynamic compromise, cardiac arrest

Rapid resolution of RWMA & normalization of EF in 7Rapid resolution of RWMA & normalization of EF in 7--30 days30 days


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Systematic Review of 7 case seriesSystematic Review of 7 case seriesAnn Intern Med 2004

Demographics and Presenting Symptoms

82% to 100% were women

Mean age was 62 to 75 years

Presenting symptom chest pain (33% to 71%)

Dyspnea & rarely syncope


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ECG findings on admission

ST-segment elevation (46% to 100%)

Mostly precordial leads and more marked in V4-V6

Isolated inferior or lateral ST elevation unusual

New LBBB and RBBB

Almost all patients developed T-wave inversions usually present inmost leads.

New pathologic Q waves (6% to 31%)

The corrected QT interval (mean 0.45 s to 0.50 s)

Systematic ReviewSystematic Review


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Cardiac Enzyme and Biomarker Release

Most patients had a modest increase in cardiac enzyme levels.

56% incidence of increase in creatine kinase

100% incidence of increase in cardiac troponin

Peak levels are often those drawn at the time of initial

presentation

Do not follow the kinetics observed with conventional MI.



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Angiographic Data and LV Function

No angiographically detectable lesion(25% to 100%)

Nonobstructive coronary disease (0% to 75%)

None of the patients had epicardial stenosis greater than 50%

Mean LVEF at presentation 0.39 to 0.49

LVEF improved rapidly over a period of days to weeks to mean0.60 to 0.76

Apical and mid-ventricular RWMA completely resolved in mostpatients



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Endothelial Function and Coronary Microcirculation

Either spontaneous or provocable multivessel epicardial spasmwas present in 0% to 43%

Coronary flow reserve (coronary microvascular function)showed conflicting results

Abnormal TIMI frame counts in all 3 major epicardial coronary

arteries during the acute phase of the syndrome



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Clinical Complications and Outcome n-hospital mortality rates - 0% to 8%

Significant LV failure during the acute phase in 3% to 46%

A transient, dynamic intraventricular pressure gradient due toobstruction in the LV cavity in 13% to 18% with MR

AV block, sinus bradycardia, paroxysmal atrial fibrillation, VT and Vfib

Isolated cases of LV mural thrombus formation

1 case of LV freewall rupture occurred 3 days after presentation

Cardiogenic shock

Death



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Preceding Stressors, Incidence, and Recurrence

Most patients presented immediately after an episode of acute

emotional (14% to 38%) or physiologic (17% to 77%)

In 1 series, this syndrome represented 2.2% of ST elevation

ACS presenting to a referral hospital during 2002 and 2003

A separate series, this syndrome was diagnosed in 1.5% of

patients who had presented with an Qwave ACS

Recurrence of the syndrome seems to be rare (0% to 8%)



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Possible triggering factorsPossible triggering factors

Systemic disordersSystemic disorders

CVACVA

epileptic attacksepileptic attacks

exacerbation of bronchialexacerbation of bronchialasthmaasthma

acute abdomenacute abdomen

noncardiac surgery/procedurenoncardiac surgery/procedure

PnuemothoraxPnuemothorax

Emotional/physical problemsEmotional/physical problems

death/funeral of family memberdeath/funeral of family member

Surprise party / reunionSurprise party / reunion

fear of procedurefear of procedure

public speakingpublic speaking

court appearancecourt appearance

tragic newstragic news

sudden accidentssudden accidentsinexperienced exerciseinexperienced exercise

quarrelingquarreling

excessive alcohol consumptionexcessive alcohol consumption

vigorous excitationvigorous excitation


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MechanismMechanism Several mechanisms have been proposed

Catecholamines mediated myocardial stunning

neurogenically mediated

direct metabolic injury

multivessel epicardial spasm

microvascular coronary spasm/dysfunction

Abnormal LAD anatomy

Intraventricular gradient

Genetic


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Neurogenic

Acute mental stress, a time of enhanced sympathetic outflow

The distribution of apical WMA is similar to the distribution reportedwith catecholamine-induced cardiomyopathy (pheochromocytoma,

GBS, SAH)

Increased adrenergic receptor density and responsiveness in theapical segments

In a rat model the RWMA could not be reproduced after pretreatmentwith adrenoreceptor antagonist

Measurements of circulating catecholamine levels have showninconsistent results suggesting that activation of cardiacadrenoceptors maybe the primary cause.


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Direct metabolic injury

Elevated catecholamine level decrease the viability of myocytesthrough cAMPmediated calcium overload and formation of freeradicals causing necrosis

Transient exposure to excessive catecholamines, may resultin stunning with cellular metabolic abnormalities rather than necrosis

Pulmonary sympathetic stimulation increases the pore size in thepulmonary capillaries, leading to alveolar transudation

Studies have demonstrated a reduction of the fatty acid metabolismand mitochondrial function in the apical myocardium by usingisotopes, suggesting direct myocardial toxicity


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Coronary spasmCoronary spasm

In an angiographic studies,

spontaneous spasm seen very occasionally

70 % had coronary spasm (single/multivessel) in response to

provocative maneuvers (ergonovine or acetylcholine)

ST elevation was common at presentation

Multivessel coronary vasospasm (which could probably produce

diffuse wall-motion abnormalities), was found in 18% patients testedacross series

Transient multivessel epicardial spasm may be responsible for some

cases of the syndrome


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Microcirculatory spasm/dysfunctionMicrocirculatory spasm/dysfunction

Coronary microvascular function has been shown to be diffuselyabnormal when assessed using CFR and TIMI frame counts

IC nicorandil during the acute phase acutely reduced the extent

of ST elevation

Regional defects on cardiac imaging suggests sympatheticallymediated microcirculatory dysfunction.

? primary mechanism or secondary phenomenon


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Abnormal LAD anatomyAbnormal LAD anatomy

In a series of 5 tako-tsubo patients,the LAD had a long course around the LV apex, supplying an

extensive portion of the diaphragmatic LV aspect

Transient coronary artery occlusion/reperfusion episodes may lead tostunning and akinesia also the minimal release of biomarkers withtotal recovery of cardiac function

Pathogenesis of this disorder could be similar to that of AMI:

coronary atherosclerotic plaque complicated by thrombotic occlusion

Evidence supporting the hypothesis is weak and hardly explainscharacteristics of this syndrome


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ntraventricular gradientIntraventricular gradient

Clinical and hemodynamic situation improved when the gradientdisappeared and in some cardiogenic shock persisted until thedynamic obstruction was present

Dynamic obstruction raise intraventricular pressures in the distal

chamber and reduce myocardial perfusion, leading to ischemia anddyskinesia

Exposure to an exogenous catecholamine (dobutamine), can provokedynamic LVOT obstruction even in normal hearts

Some women might have a geometric predisposition (sigmoidseptum, narrow LVOT, reduced LV volume) to the development ofdynamic subaortic obstruction that would only manifest with intenseadrenergic stimulation


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Proposed pathophysiologicalmechanism of TLVABS


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Female PreponderanceFemale Preponderance

Sex hormones exert important influences on the sympatheticneurohormonal axis as well as on coronary vasoreactivity.

Women are more vulnerable to sympathetically mediated myocardialstunning (transient LV dysfunction after SAH)

Explanation - alterations of endothelial function and microcirculatoryvasomotor reactivity due to reduced estrogen levels

Estradiol supplementation attenuates emotional stressinducedchanges in LV function in ovariectomized female rats.


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PATHOLOGICAL FINDINGS

Interstitial fibrosis was a common finding and no significant

inflammation necrosis during the acute phase

Later myocytolysis, with cell infiltrates including mononuclear

cells and increase of loose connective tissue.

The histologic findings in this heart syndrome, were similar toThe histologic findings in this heart syndrome, were similar to

those observed in catecholaminethose observed in catecholamine--induced cardiomyopathyinduced cardiomyopathy

There was no evidence of acute myocarditis in any series.


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Proposed Mayo Criteria for the ClinicalDiagnosis

1. Transient akinesis or dyskinesis of the left ventricular apical and mid-ventricular segments with regional wall-motion abnormalities extendingbeyond a single epicardial vascular distribution

2. Absence of obstructive coronary disease or angiographic evidence of acute

plaque rupture

3. New electrocardiographic abnormalities (either ST-segment elevation or Twave inversion)

4. Absence ofRecent significant head traumaIntracranial bleedingPheochromocytomaObstructive epicardial coronary artery diseaseMyocarditis

Hypertrophic cardiomyopathy * All 4 criteria must be met


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Diagnosis of TLVABS Prior conditions (both obligatory):

Evidence of transient apical dysfunction of LV with a typical shape inthe systole, diagnosed by angio, echo, isotopes, or cardiac MRI. Normalventricular mobility returns in 2-3 weeks

Absence of other conditions associated with transient regional systolicdysfunction of the LV: SAH, pheochromocytoma, cardiac ischemia, toxicsubstances (cocaine), myocarditis, etc.

Diagnostic CriteriaMajorcriteria

Early coronary angiography (first24 h) withoutanatomical lesions

Minorcriteria1. Early coronary angiography with non-significant lesions2. Late coronary angiography(2 to7 days) withoutsignificant lesions3. Disordertriggered by physical oremotional stress4. Typical ECG changes ST segmentelevation in acute phase, Q waves

thatdisappearafteracute phase, T inversion prominentin V1-V6

and prolongation of QTc5. Woman aged >50 years


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ManagementManagement Beta-blockers

ACEI (in patients without an intracavitary gradient)AspirinIV diuretics

Pts with hypotension must be evaluated for a dynamic intraventricularpressure gradient in the LV cavity and LVOT which is managed by

beta-blockers,phenylephrinefluid resuscitation

Beta-blockers and phenylephrine are not recommended in documentedepicardial coronary vasospasm (diltiazem or verapamil)

Short-term anticoagulation if significant LV dysfunction and continued untilLV function has improved

Monitoring for atrial and ventricular arrhythmias, heart failure, andmechanical complications.


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69-year-old womanacute chest pain

CPKMB was elevatedurgent CAG - normal coronaries

LV angio-apical ballooning insystole

LVEF of 33% & severe MRMidcavity gradient of 25

mmHg

Supportive therapyClinical recovery within 1 weekLV function normal at 40 days


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8080--yearyear

--old woman withold woman withhypertensionhypertension

33--hour history of dyspnoea andhour history of dyspnoea andprecordial discomfortprecordial discomfort

trop T, CPK and CPKtrop T, CPK and CPK--MB wereMB werenormalnormal

patient developed haemodynamicpatient developed haemodynamicinstability, cardiac cath was doneinstability, cardiac cath was done

The patients condition improvedThe patients condition improvedafterafter an IABP was placedan IABP was placed

Echo 3Echo 3 weeks later showedweeks later showednormal LV size and function.normal LV size and function.


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78-year-old woman withhypertension

acute epigastric pain since 12hours

melena and Hb of8g/L.

After transfusion of 2 units ofpacked RBCs, the symptomsdisappeared and the ECGevidenced extensive anteriorsubepicardial ischemia.

The trop I was 8.5 ng/mL.

Echo showed anteroapicalakinesia and dynamicsubaortic obstruction with apeak gradient of 42 mm Hg

Cath performed 5 days latershowed normal coronariesand apical ballooning

She has remainedasymptomatic after 15 monthsof follow-up, with normalizedECG and LV wall motion


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64 year old woman

No coronary risk factors

continuous atypical chest pain-2d

Maximal CPK and trop I valueswere 159 U/l (normal 0170) and7.4 ng/ml (00.1)

CAG-normal coronaries

LV angio - apical ballooning, apicalthrombus. LVEF 40%. Confirmedwith TTE (IV gradient 75mmHg)

Pathologic Q waves developedfrom V1-V3

discharged under anticoagulants

3 months later T in V3V6TTE - normal LVwall motion, LVEF62%, complete resolution of theapical thrombus and IV pressuregradient

Apical thrombus in TLVABSApical thrombus in TLVABS


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Indian Heart JournalIndian Heart Journal

5 cases (all females) aged 225 cases (all females) aged 2240 years40 years

Presenting with severe transient LV dysfunction, and cardiogenicPresenting with severe transient LV dysfunction, and cardiogenic

shock following surgery (cesarean section 2, laparoscopicshock following surgery (cesarean section 2, laparoscopic

cholecystectomy 3)cholecystectomy 3)

All the patients had ECG changes in multiple leads, suggestive ofAll the patients had ECG changes in multiple leads, suggestive of

STEMI in 2 cases, and NSTE MI in 3 cases, raised cardiac enzymes,STEMI in 2 cases, and NSTE MI in 3 cases, raised cardiac enzymes,

severe LV dysfunction, and normal coronariessevere LV dysfunction, and normal coronaries

IABP support was required in 3 cases.IABP support was required in 3 cases.

All the patients made a complete recovery, and are asymptomatic atAll the patients made a complete recovery, and are asymptomatic at

11--month to 6month to 6--year followyear follow--up.up.


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Unanswered QuestionsUnanswered Questions

Why do middleWhy do middle--aged/elderly women appearaged/elderly women appearsusceptible to this disorder?susceptible to this disorder?

Exactly how does psychological stress trigger itsExactly how does psychological stress trigger itssudden onset?sudden onset?

Why is the LV apex selectively vulnerable to regionalWhy is the LV apex selectively vulnerable to regionalballooning?ballooning?

Is this really a new disease with increasing incidence,Is this really a new disease with increasing incidence,or has it previously been unrecognized?or has it previously been unrecognized?

Should pharmacological therapy be continuedShould pharmacological therapy be continued

indefinitely?indefinitely?


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Clinical RelevanceClinical Relevance

Although rare, TLVABS must be considered as a d/d ofAlthough rare, TLVABS must be considered as a d/d ofACS or STEMIACS or STEMI

Especially when extent of ECG abnormaliries exceedEspecially when extent of ECG abnormaliries exceedbiomarker evidence for myocardial necrosis and CAGbiomarker evidence for myocardial necrosis and CAG

confirms noncritical CAD.confirms noncritical CAD.

Prompt recognition and aggressive t/tPrompt recognition and aggressive t/t(pharmacological / mechanical)(pharmacological / mechanical)


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THANKTHANK YOUYOU


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ST-Segment Elevation in Various Conditions

ConditionCondition FeatureFeature

Normal (male pattern)Normal (male pattern) 90 percent of healthy young men

Concave, 13mm, most marked in V2

Early repolarizationEarly repolarization Most marked in V4, notching at J point

Tall, upright T waves

Reciprocal ST depression in aVR

ST elevation normal variantST elevation normal variant V3- V5, inverted T waves

Short QT, high QRS voltage

LVHLVH Concave, Other features of LVH

LBBBLBBB Concave

ST deviation discordant from the QRS

Acute PericarditisAcute Pericarditis Diffuse ST elevation, Reciprocal STdepression in aVR, Elevation

seldom>5mm, PR-segment depression


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ConditionCondition FeatureFeature

HyperkalemiaHyperkalemia Other features of hyperkalemia presentOther features of hyperkalemia present

Brugada SyndromeBrugada Syndrome rSR' in V1-2, ST- elevation in V1-2,

typically downsloping

Pulmonary embolismPulmonary embolism Changes simulating MI in both inferior

and anteroseptal leads

CardioversionCardioversion ST elevation, often >10 mm, lasting

only a minute or two immediately after

DC shock

Prinzmetal AnginaPrinzmetal Angina Same as ST-segment elevation in

infarction, but transient

Apical Ballooning Syndrome

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