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Seven days after admission, an echocardiogram showed persistent apical akinesia, no LV outflow tract obstruction (Fig. 3A), a LVEF of 40%, and mild mitral regurgitation. The septum presented a sigmoid morphology with a septal thickness of 12.5 mm (Fig. 3B). The patient was discharged home on bisoprolol, spironolactone, lisinopril, and simvastatin. One month later, the echocardiogram showed normal LVEF without regional motion abnormalities. A cardiac MRI performed at that time confirmed a normal LVEF, a sigmoid septum with a septal bulge of 13 mm (Fig. 3C and 3D) without subaortic LV obstruction or significant mitral regurgitation, and absence of late gadolinium enhancement. Comments In this patient with Tako-tsubo (TT) syndrome and mid LV obstruction leading to cardiogenic shock and acute pulmonary edema, we document for the first time and to the best of our knowledge, the intraventricular gradient with the recording of pressures within the apical LV, the intermediate LV chamber, and the aorta. In addition, we report for the first time the systolic collapse of septal branches in a TT syndrome with dynamic subaortic obstruction. The presence of mid LV obstruction in TT has been detected in some 20% of the patients with this syndrome 1. The relation between cardiogenic shock and subaortic LV obstruction is not well established in the literature. In the series of 32 TT patients reported on by El Mahmoud et al. there was mid LV obstruction in eight cases, two of whom had cardiogenic shock 2. It is tempting to suggest that in patients with TT syndrome and severe systolic LV dysfunction, the presence of subaortic obstruction adds an important hemodynamic burden that may lead to cardiogenic shock 3. A “sigmoid septum” has been previously linked to the development of LV outflow tract obstruction in patients with TT syndrome. Thus, in the study by El Mahmoud et al. all their cases with LV obstruction had a sigmoid septum, a finding only encountered in 29% of the patients without obstruction. Our patient had a sigmoid septum of 13 mm in the MRI study. In addition, our patient showed milking of the left anterior descending coronary artery and systolic collapse of the proximal septal branches reflecting a hypercontractile state of the basal left ventricle under the influence of endogenous and, in our case, exogenous, cathecholamines. The hemodynamic situation in our patient was worsened by the infusion of dopamine which increased the LV gradient, and most likely the severity of mitral regurgitation. The initial echocardiographic study did not identify the presence of LV outflow tract obstruction and the diagnosis of TT was not suspected. The coronariographic study demonstrating normal coronary arteries, myocardial bridging in the mid segment of the left anterior coronary artery, and the mid ventricular gradient was crucial to establish not only the diagnosis, but also to correct the wrong therapeutic approach. Treatment in patients with TT syndrome and cardiogenic shock or pulmonary edema in the very acute stages includes the avoidance of cathecholamines and vasodilating agents, and the use of beta-blockers 4,5. Verapamil, a drug that can reduce dynamic gradients in hypertrophic cardiomyopathy, should be avoided in the setting of TT syndrome because of the depressed LVEF and the frequently high LV end-diastolic pressures. Norepinephrine that increases afterload could be an option in patients with significant hypotension. Fluid therapy must be handled carefully in these patients who characteristically have very high LV filling pressures and in whom the shock state does not depend on a hypovolemic situation. IABP and extracorporeal cardiopulmonary support have been used in anecdotal cases 6. While in our case the mid LV gradient was not observed at the time of the initial echocardiographic study in which severe LV systolic dysfunction and heart failure with shock were present, the subaortic gradient would have been documented if a repeat echocardiogram had been obtained after initiating the infusion of dopamine. This was not done so the correct diagnosis was delayed until the cardiac catheterization was performed. The lesson to take home is that the combination of shock and TT syndrome is a deadly tramp if dopamine infusion is used for hemodynamic support. TT syndrome is seldom suspected in an emergency room, and under a marked hemodynamic impairment with severe systolic LV dysfunction, the use of dopamine support is understandable. When a TT syndrome is present, dopamine may further deteriorate the clinical situation through the creation of a subaortic dynamic gradient. 1 Chockalingam A, Tejwani L, Aggarwal K, Dellsperger KC. Dynamic left ventricular outflow tract obstruction in acute myocardial infarction with shock: cause, effect, and coincidence. Circulation. 2007 Jul 31;116(5):e110-3. 2 El Mahmoud R, Mansencal N, Pilliére R, Leyer F, Abbou N, Michaud P, Nallet O, Digne F, Lacombe P, Cattan S, Dubourg O. Prevalence and characteristics of left ventricular outflow tract obstruction in Tako-Tsubo syndrome. Am Heart J. 2008 Sep;156(3):543-8. 3 Brunetti ND, Ieva R, Rossi G, Barone N, De Gennaro L, Pellegrino PL, Mavilio G, Cuculo A, Di Biase M. Ventricular outflow tract obstruction, systolic anterior motion and acute mitral regurgitation in Tako-Tsubo syndrome. Int J Cardiol. 2008 Jul 21;127(3):e152-7 4 Yoshioka T, Hashimoto A, Tsuchihashi K, Nagao K, Kyuma M, Ooiwa H, Nozawa A, Shimoshige S, Eguchi M, Wakabayashi T, Yuda S, Hase M, Nakata T, Shimamoto K. Clinical implications of midventricular obstruction and intravenous propranolol use in transient left ventricular apical ballooning (Tako-tsubo cardiomyopathy). Am Heart J. 2008 Mar;155(3):526.e1-7. 5 Kyuma M, Tsuchihashi K, Shinshi Y, et al. Effect of intravenous propranolol on left ventricular apical ballooning without coronary artery stenosis (ampulla cardiomyopathy): Three cases. Circ J 2002;66:1181-4. 6 Bonacchi M, Maiani M, Harmelin G, Sani G. Intractable cardiogenic shock in stress cardiomyopathy with left ventricular outflow tract obstruction: is extra-corporeal life support the best treatment?. Eur J Heart Fail. 2009 Jul;11(7):721-7. Contact nuria.barron@siemens.com Cardiology Tako-Tsubo Syndrome 44  AXIOM Innovations | December 2013 | www.siemens.com/angiography


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