EpidemiologyIncidence is unknown although some

have estimated 1-2% of all patients presenting with “ACS”

Mean age is 58-75 and rarely has been reported in patients less than 50 years of age

Ninety percent of the cases have occurred in women

Clinical PresentationEssentially present as ACS patients

~50% with angina-like chest pain at restDyspnea~66% have a preceding physical or emotional

stressor (reports have described various stressful events such as a surprise party, public speaking, and the death of loved one)

Rarely present with syncope or cardiac arrest

PathophysiologyCatecholamine induced?

Very high levels of circulating catecholaminesContraction band necrosis on endomyocardial

biopsy

Coronary spasmMyocarditis

Findings on workupEKG: Typically STE in precordial leads

although reports have been described where no STE were observed. Rarer findings include nonspecific T wave

abnormalities or a bundle branch blockBiomarkers: Elevated Troponin and CK-MB

Levels are not as high as expected given degree of cardiac dysfunction.

Cardiac catheterization: Normal or insignificant disease

Left ventriculogram

Prasad, A. et al. American Heart Journal. 2008; 155: 408-417

EchocardiogramHypokinesis or akinesis of mid and apical

segments of the LVMotion abnormality involves more than one

coronary artery distributionFunction at the base is normal

RV may show similar findings in ~30% of patients (seen in sicker patients)

Cardiac MRIDocuments degree of wall motion abnormalities

No delayed hyperenhancementMI and myocarditis show hyperenhancement

Proposed Mayo Clinic criteria for Diagnosis

Transient hypokinesis, akinesis, or dyskinesis of the left ventricular mid segments with or without apical involvement; the regional wall motion abnormalities extend beyond a single epicardial vascular distribution; a stressful trigger is often, but not always present. ⁎

Absence of obstructive coronary disease or angiographic evidence of acute plaque rupture.

New electrocardiographic abnormalities (either ST- segment elevation and/or T-wave inversion) or modest elevation in cardiac troponin.

Absence of: Pheochromocytoma or MyocarditisPrasad, A. et al. American Heart Journal. 2008; 155: 408-417

TreatmentIn short, nobody really knows optimal therapy.Patients will initially be treated as ACS

(anticoagulation, asa, b-blocker, tele, etc.)Beta-blockers and ACEI are reasonable at

discharge (no asa necessary if cath revealed no CAD)

Some argue for several weeks of warfarin therapy if LV dysfunction is severe

CHF can be seen in ~20% of patients which responds well to diuretics

OutcomesIn hospital mortality is exceedingly low

Typically complete recovery is seen within 4-8 weeks. ACEI can be discontinued at that time