Epidemiology Incidence is unknown although some have estimated 1-2% of all patients presenting with “ACS” Mean age is and rarely has been reported in patients less than 50 years of age Ninety percent of the cases have occurred in women

Clinical Presentation Essentially present as ACS patients ~50% with angina-like chest pain at rest Dyspnea ~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

Pathophysiology Catecholamine induced? Very high levels of circulating catecholamines Contraction band necrosis on endomyocardial biopsy Coronary spasm Myocarditis

Findings on workup EKG: 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 block Biomarkers: 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:

Echocardiogram Hypokinesis or akinesis of mid and apical segments of the LV Motion abnormality involves more than one coronary artery distribution Function at the base is normal RV may show similar findings in ~30% of patients (seen in sicker patients)

Cardiac MRI Documents degree of wall motion abnormalities No delayed hyperenhancement MI 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 Myocarditis Prasad, A. et al. American Heart Journal. 2008; 155:

Treatment In 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

Outcomes In hospital mortality is exceedingly low Typically complete recovery is seen within 4-8 weeks. ACEI can be discontinued at that time