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Transient Stress Cardiomyopathy

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Transient Stress Cardiomyopathy

BASICS

DESCRIPTION

2.2% of patients presenting to a referral hospital with ST-segment MIs were found to have TSC.

No genetic associations have been described to date.

Death from TSC is rare, and most cases resolve rapidly, within 2 to 3 days. Reported complications include:

Characteristic pathologic findings of involved myocardium have not been described.

After diagnostic cardiac catheterization, empirical treatment goals are as follows:

Short-term anticoagulation should be considered in patients with severely reduced LV function to prevent LV thrombus formation. Unfractionated heparin 80 U/kg IV bolus followed by 18 U/kg/hr IV or Lovenox 1 mg/kg SC BID.

All patients with TSC generally should be comanaged with cardiology while inpatient and referred to cardiology as an outpatient.

Admission Criteria/Initial Stabilization

Normal saline infusion to support BP, if necessary, and no evidence of heart failure

11 Bybee KA, Prasad A. Stress-related cardiomyopathy syndromes. Circulation. 2008;118(4):397 " 409.22 Dote K, Sato H, Tateishi H, et al. Myocardial stunning due to simultaneous multivessel coronary spasms: a review of 5 cases [in Japanese]. J Cardiol. 1991;21(2):203 " 214.33 Fitzgibbons TP, Madias C, Seth A, et al. Prevalence and clinical characteristics of right ventricular dysfunction in transient stress cardiomyopathy. Am J Cardiol. 2009;104(1):133 " 136.44 Templin C, Ghadri JR, Diekmann J, et al. Clinical features and outcomes of takotsubo (stress) cardiomyopathy. N Engl J Med. 2015; 373(10): 929 " 935.55 Paur H, Wright PT, Sikkel MB, et al. High levels of circulating epinephrine trigger apical cardiodepression in a ²2-adrenergic receptor/Gi-dependent manner: a new model of takotsubo cardiomyopathy. Circulation. 2012;126(6): 697 " 706.66 Madias C, Fitzgibbons TP, Alsheikh-Ali AA, et al. Acquired long QT syndrome from stress cardiomyopathy is associated with ventricular arrhythmias and torsades de pointes. Heart Rhythm. 2011;8(4):555 " 561.77 Randhawa MS, Dhillon AS, Taylor HC, et al. Diagnostic utility of cardiac biomarkers in discriminating takotsubo cardiomyopathy from acute myocardial infarction. J Card Fail. 2014;20(1):2 " 8.88 Medeiros K, O 'Connor MJ, Baicu CF, et al. Systolic and diastolic mechanics in stress cardiomyopathy. Circulation. 2014;129(16):1659 " 1667.99 Hunt SA, Abraham WT, Chin MH, et al. 2009 focused update incorporated into the ACC/AHA 2005 guidelines for the diagnosis and management of heart failure in adults: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines developed in collaboration with the International Society for Heart and Lung Transplantation. J Am Coll Cardiol. 2009;53(15):e1 " e90.

I51.81 Takotsubo syndrome

429.83 Takotsubo syndrome

  • Transient stress cardiomyopathy (TSC) is a unique cause of reversible left ventricle (LV) dysfunction with a presentation indistinguishable from the acute coronary syndromes (ACS), particularly ST-segment elevation myocardial infarction (MI) (1).
  • Typically, the patient is a postmenopausal woman who presents with acute chest pain or dyspnea after an identifiable "trigger "  (i.e., an acute emotional or physiologic stressor).
  • First reported by authors from Japan, TSC was known initially as the takotsubo syndrome because the typical LV morphology (i.e., apical ballooning) resembled that of a Japanese octopus trap or takotsubo (2)[B].
  • Presenting clinical features include the following:Chest symptoms and/or dyspneaECG changes, including ST-segment elevations or diffuse T-wave inversionsMild elevation in cardiac biomarkers (creatine kinase [CK], troponin)Transient wall motion abnormalities that may involve the base, midportion, and/or lateral walls of the LV.The apex of the right ventricle (RV) may be affected in up to 25% of cases (3)[B].
  • Clinical features may vary on a case-by-case basis, and formal diagnostic criteria have not been established.
  • Authors from the Mayo Clinic have proposed that 3 of the 4 following criteria establish the diagnosis (1)[A]:Transient akinesis or dyskinesis of the LV apical and midventricular segments with regional wall motion abnormalities extending beyond a single epicardial vascular distributionAbsence of obstructive coronary artery disease (CAD) or angiographic evidence of acute plaque ruptureNew ECG abnormalities, either ST-segment elevation or T-wave inversionAbsence ofRecent significant head traumaIntracranial bleedingPheochromocytomaObstructive epicardial CADMyocarditisHypertrophic cardiomyopathy
  • Synonym(s): takotsubo cardiomyopathy; apical ballooning syndrome; stress cardiomyopathy; broken heart syndrome; ampulla cardiomyopathy
  • Chest symptoms and/or dyspnea
  • ECG changes, including ST-segment elevations or diffuse T-wave inversions
  • Mild elevation in cardiac biomarkers (creatine kinase [CK], troponin)
  • Transient wall motion abnormalities that may involve the base, midportion, and/or lateral walls of the LV.
  • The apex of the right ventricle (RV) may be affected in up to 25% of cases (3)[B].
  • Transient akinesis or dyskinesis of the LV apical and midventricular segments with regional wall motion abnormalities extending beyond a single epicardial vascular distribution
  • Absence of obstructive coronary artery disease (CAD) or angiographic evidence of acute plaque rupture
  • New ECG abnormalities, either ST-segment elevation or T-wave inversion
  • Absence ofRecent significant head traumaIntracranial bleedingPheochromocytomaObstructive epicardial CADMyocarditisHypertrophic cardiomyopathy
  • Recent significant head trauma
  • Intracranial bleeding
  • Pheochromocytoma
  • Obstructive epicardial CAD
  • Myocarditis
  • Hypertrophic cardiomyopathy

EPIDEMIOLOGY

  • TSC accounts for a small percentage (1 " “3%) of ACS.
  • In a recent prospective evaluation of patients admitted to the ICU, as many as 28% had apical ballooning, often in association with sepsis.
  • Predominant sex: 82 " “100% of cases occur in women.
  • Predominant age: Mean age of patients is 62 to 75 years.

ETIOLOGY AND PATHOPHYSIOLOGY

  • The exact pathophysiology is not known.
  • A perturbation in the brain-heart axis, originating in the insular cortex, may be the inciting event (4).
  • Subsequent overwhelming activation of the sympathetic nervous system initiates a cascade of events, including the following:Catecholamine-induced LV dysfunction: "biased agonism "  of epinephrine for Ž ²2-adrenergic receptors, located predominantly at the cardiac apex (5)Endothelial dysfunction and vasospasmCellular metabolic injuryMyocardial norepinephrine releaseCalcium overloadContraction band necrosis
  • Catecholamine-induced LV dysfunction: "biased agonism "  of epinephrine for Ž ²2-adrenergic receptors, located predominantly at the cardiac apex (5)
  • Endothelial dysfunction and vasospasm
  • Cellular metabolic injuryMyocardial norepinephrine releaseCalcium overloadContraction band necrosis
  • Myocardial norepinephrine release
  • Calcium overload
  • Contraction band necrosis

RISK FACTORS

  • Female sex
  • Postmenopausal state
  • Emotional stress (i.e., argument, death of family member)
  • Physiologic stress (i.e., acute medical illness)
  • Chronic neurologic or psychiatric disease (4)

COMMONLY ASSOCIATED CONDITIONS

  • Left-sided heart failure
  • Pulmonary edema
  • Cardiogenic shock and hemodynamic compromise
  • Dynamic LV outflow tract gradient complicated by hypotension
  • Mitral regurgitation
  • Ventricular arrhythmias
  • LV thrombus formation
  • LV free wall rupture
  • Death (rare, 0 " “8%)

DIAGNOSIS

  • Activate emergency medical services or report to emergency department.
  • Oxygen, IV access, and ECG monitoring
  • Urgent cardiology consultation

HISTORY

  • In ¢ ”; of patients, there is exposure to a "trigger event. " Emotional stress: argument, death of family member, divorce, public speaking, and so forthPhysiologic stress: acute medical condition such as head trauma, asthma attack, seizure, and so forth
  • In ¢ “; of patients, there is no identifiable trigger (4).
  • Acute onset of dyspnea or chest pain
  • Palpitations
  • Syncope
  • Emotional stress: argument, death of family member, divorce, public speaking, and so forth
  • Physiologic stress: acute medical condition such as head trauma, asthma attack, seizure, and so forth

PHYSICAL EXAM

  • Tachypnea
  • Tachycardia
  • Hypotension
  • Jugular venous distension
  • Bibasilar rales
  • S3 gallop
  • Systolic ejection murmur due to dynamic LV outflow tract gradient
  • Holosystolic murmur of mitral regurgitation

DIFFERENTIAL DIAGNOSIS

  • Acute ST-segment elevation MI
  • Pulmonary embolism
  • Myopericarditis
  • Pheochromocytoma
  • Hypertrophic cardiomyopathy
  • Subarachnoid hemorrhage or stroke

DIAGNOSTIC TESTS & INTERPRETATION

  • ECG should be done urgently and may show the following:Diffuse ST-segment elevationsDiffuse and often dramatic T-wave inversionsQTc interval prolongation (6)[B]Q waves
  • Laboratory tests typically reveal a mild elevation in cardiac biomarkers such asCK (rarely >500 U/mL)Troponin IB-type natriuretic peptide (BNP)Markers of high filling pressures (e.g., BNP) tend to be higher than markers of necrosis (e.g., CK, troponin).TSC can be distinguished from AMI with 95% specificity using a BNP/TnT ratio ≥1,272 (sensitivity 52%) (7)[B].
  • Chest radiographCardiomegalyPulmonary edema
  • EchocardiogramReduced LV systolic functionAbnormal diastolic function, including evidence of increased filling pressuresRegional wall motion abnormalities in one of the following patterns:Classic or "takotsubo-type "  ballooning of the apex with a hypercontractile base "Reverse takotsubo " : apical hypercontractility with basal akinesis "Midventricular "  akinesis with apical and basal hypercontractilityFocal or localized akinesis of an isolated segmentDynamic intracavitary LV gradientMitral regurgitationVariable involvement of the RV
  • Cardiac MRIReduced LV functionWall motion abnormalities as described for transthoracic echocardiographyAbsence of delayed hyperenhancement with gadolinium
  • Diffuse ST-segment elevations
  • Diffuse and often dramatic T-wave inversions
  • QTc interval prolongation (6)[B]
  • Q waves
  • CK (rarely >500 U/mL)
  • Troponin I
  • B-type natriuretic peptide (BNP)
  • Markers of high filling pressures (e.g., BNP) tend to be higher than markers of necrosis (e.g., CK, troponin).
  • TSC can be distinguished from AMI with 95% specificity using a BNP/TnT ratio ≥1,272 (sensitivity 52%) (7)[B].
  • Cardiomegaly
  • Pulmonary edema
  • Reduced LV systolic function
  • Abnormal diastolic function, including evidence of increased filling pressures
  • Regional wall motion abnormalities in one of the following patterns:Classic or "takotsubo-type "  ballooning of the apex with a hypercontractile base "Reverse takotsubo " : apical hypercontractility with basal akinesis "Midventricular "  akinesis with apical and basal hypercontractilityFocal or localized akinesis of an isolated segment
  • Dynamic intracavitary LV gradient
  • Mitral regurgitation
  • Variable involvement of the RV
  • Classic or "takotsubo-type "  ballooning of the apex with a hypercontractile base
  • "Reverse takotsubo " : apical hypercontractility with basal akinesis
  • "Midventricular "  akinesis with apical and basal hypercontractility
  • Focal or localized akinesis of an isolated segment
  • Reduced LV function
  • Wall motion abnormalities as described for transthoracic echocardiography
  • Absence of delayed hyperenhancement with gadolinium
  • Because ST-segment elevation MI is the diagnosis of exclusion, patients typically are referred for urgent cardiac catheterization.
  • Coronary angiographyNonocclusive CADRarely, epicardial coronary spasmEndothelial dysfunction as measured by fractional flow reserve or TIMI frame counts
  • Left-sided heart catheterization: increased LV end-diastolic pressure to a similar degree as AMI (8)[B]
  • Ventriculography: wall motion abnormalities as described for transthoracic echocardiography
  • Right-sided heart catheterizationIncreased pulmonary capillary wedge pressureSecondary pulmonary hypertensionIncreased right ventricular filling pressuresReduced cardiac output or cardiogenic shock (cardiac index <2 and mean arterial pressure [MAP] <60 mm Hg)
  • Nonocclusive CAD
  • Rarely, epicardial coronary spasm
  • Endothelial dysfunction as measured by fractional flow reserve or TIMI frame counts
  • Increased pulmonary capillary wedge pressure
  • Secondary pulmonary hypertension
  • Increased right ventricular filling pressures
  • Reduced cardiac output or cardiogenic shock (cardiac index <2 and mean arterial pressure [MAP] <60 mm Hg)

TREATMENT

  • Activation of emergency medical services
  • Advanced cardiac life support therapies as needed
  • Oxygen
  • IV access
  • ECG monitoring

MEDICATION

  • Management of hypotension: differentiation between cardiogenic shock and dynamic LV cavity gradient
  • Management of increased filling pressures and congestive states
  • Attenuation of sympathetic drive
  • There are no evidence-based treatment recommendations for TSC.
  • Although Ž ²-blockers are of theoretical benefit, their use has not been associated with improved outcomes in observational cohorts (4)[B].
  • If there is evidence of left ventricular systolic dysfunction or pulmonary edema, consider the following:Furosemide: 20 to 40 mg IV/PO BID as needed to reduce LV filling pressures and dyspnea (9)ACE inhibitors or angiotensin receptor blockers: lisinopril 10 to 40 mg/day PO or equivalent or valsartan 80 to 160 mg PO BID have been associated with improved outcomes in observational cohorts (4)[B].
  • Furosemide: 20 to 40 mg IV/PO BID as needed to reduce LV filling pressures and dyspnea (9)
  • ACE inhibitors or angiotensin receptor blockers: lisinopril 10 to 40 mg/day PO or equivalent or valsartan 80 to 160 mg PO BID have been associated with improved outcomes in observational cohorts (4)[B].

ISSUES FOR REFERRAL

ADDITIONAL THERAPIES

  • Urgent cardiology consultation and consideration of cardiac catheterization
  • Hypotension may require the following:Vasopressors (e.g., dopamine or Levophed) if there is no LV outflow tract gradient (9)[C]Phenylephrine and IV fluids to increase afterload in the presence of an LV outflow tract gradient (9)[C]Cardiogenic shock that is not due to an LV outflow tract gradient may require placement of an intra-aortic balloon pump.
  • Vasopressors (e.g., dopamine or Levophed) if there is no LV outflow tract gradient (9)[C]
  • Phenylephrine and IV fluids to increase afterload in the presence of an LV outflow tract gradient (9)[C]
  • Cardiogenic shock that is not due to an LV outflow tract gradient may require placement of an intra-aortic balloon pump.

INPATIENT CONSIDERATIONS

  • 12-lead ECG
  • Chest radiograph
  • Laboratory testing
  • Echocardiography
  • Patients with TSC usually are admitted for observation because the differential diagnosis includes ACS.
  • Congestive state
  • Hypotension
  • Profound impairments of systolic function

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Impairments in systolic function typically resolve in 2 to 3 days but may last as long as 1 month.
  • Patients should follow up with cardiology and serial echocardiography to document improved LV function.

PROGNOSIS

  • Prognosis is excellent. Inpatient mortality is rare and ranges from 0% to 8%.
  • Recurrence is rare; it also has been reported in 0 " “8% of patients.

REFERENCES

SEE ALSO

CODES

ICD10

ICD9

SNOMED

CLINICAL PEARLS

  • TSC is a cause of reversible LV dysfunction with a clinical presentation indistinguishable from the ACS, particularly ST-segment elevation MI.
  • Echocardiography may strongly suggest the diagnosis.
  • Treatment is supportive and should include diuretics and ACE inhibitors in patients with CHF.