Is this heart broken

We present the case of a woman presenting with chest pain that illustrates the importance of recognizing an atypical presentation of coronary artery disease in women, as well as the role of myocardial perfusion imaging in the differential diagnosis. Case presentation. A 54-year-old black woman presented to the emergency department with atypical chest pain and left shoulder and arm pain of 8 hours’ duration. She was known to have fibromyalgia and an anxiety disorder, with a recent exacerbation related to domestic unrest. Her risk factors for coronary artery disease consisted of hypertension, hyperlipidemia, tobacco use, and a family history of myocardial infarction. The 12-lead electrocardiogram at presentation showed sinus bradycardia and T-wave inversion in the inferior and lateral leads. The cardiac troponin I level was elevated, at 0.5 g/L. Aspirin, -blocker, heparin, and intravenous nitrate therapy was initiated. Several hours later, the patient had worsening chest discomfort associated with diaphoresis, bradycardia, and nonsustained ventricular tachycardia. An electrocardiogram showed more pronounced and extensive T-wave inversion (Figure 1), and the second troponin I level was 9.8 g/L. Emergent cardiac catheterization was performed and revealed a left anterior descending coronary artery that tapered distally, with luminal irregularity ( 50% diameter stenosis) in its midportion. The left circumflex and right coronary arteries were normal (Figure 2). Contrast left ventriculography was remarkable for severe regional dysfunction localized to the left ventricular (LV) apex and the adjacent anterior and inferior walls, with hypercontractility of the basal portions of the ventricle (Figure 3). A clinical diagnosis of takotsubo cardiomyopathy, also referred to as transient LV apical ballooning or “broken heart” syndrome, was entertained. The cardiac troponin I level peaked at 32 g/L on serial testing. A resting technetium 99m sestamibi myocardial single photon emission computed tomography (SPECT) scan was performed the next day. It revealed a severe perfusion abnormality involving the apex and the adjacent anterior and inferior walls, with a complete absence of Tc-99m sestamibi uptake (Figure 4). A rest thallium 201/exercise Tc-99m sestamibi myocardial gated SPECT study was performed 2 months later and showed a large fixed defect associated with regional dysfunction, essentially unchanged from the initial scan (Figure 5). An echocardiogram obtained shortly thereafter showed persistent LV apical dyskinesis (Figure 6). Discussion. Several features of this patient’s initial presentation were typical of takotsubo cardiomyopathy. These include her gender, age, recent emotional distress, and severe regional LV dysfunction in the absence of epicardial coronary obstruction. Takotsubo cardiomyopathy, originally described in Japanese patients, is being increasingly recognized in the Western world, including the United States, and is characterized by acute, reversible LV apical dysfunction in the absence of obstructive epicardial coronary disease. The prevalence of this syndrome is reported to be 1% to 2% in patients presenting with clinical features suggestive of an acute coronary syndrome. The majority of patients are postmenopausal women, with the following features common to most accounts: (1) chest symptoms, electrocardiographic changes, and mild elevation in cardiac biomarkers consistent with acute myocardial ischemia; (2) a temporal relationship to a recent stressful event, giving this condition its alternate name of “broken heart” syndrome (some form of either emotional or physical stress preceded onset in about 65% of cases); (3) regional dysfunction localized to the LV apex, associated with hyperkinesis of the basal portions of the left ventricle with normal epicardial coronary arteries (Takotsubo is Japanese for octopus trap or pot and refers to the characteristic shape of the dysmorphic left ventricle seen on the ventriculogram of patients with this syndrome); and (4) complete resolution of the regional dysfunction in days to weeks after the acute event. The pathogenesis of myocardial perfusion and functional abnormalities in takotsubo cardiomyopathy remains speculative. Proposed mechanisms include (1) coronary spasm, (2) LV dysfunction induced by From the Department of Internal Medicine and Division of Cardiology, University of Pittsburgh Medical Center, Pittsburgh, Pa. Reprint requests: Prem Soman, MD, PhD, MRCP(UK), Division of Cardiology, University of Pittsburgh Medical Center, Room A 429, Scaife Hall, 200 Lothrop St, Pittsburgh, PA 15213; somanp@ upmc.edu. J Nucl Cardiol 2008;15:142-5. 1071-3581/$34.00 Copyright © 2008 by the American Society of Nuclear Cardiology. doi:10.1016/j.nuclcard.2007.11.011