Tako-tsubo cardiomyopathy in two sisters: a chance finding or familial predisposition?

A 54-year-old woman was admitted to our coronary care unit for prolonged chest pain and dizziness that began while she was performing allergological tests. She had a family history of myocardial infarction and no other additional cardiovascular risk factors. She was undergoing skin prick test for allergic asthma, which resulted positive for oat grass (Poaceae family). The physical examination was unremarkable. Her blood pressure was 120/80 mmHg and the heart rate was 114 bpm. The electrocardiogram (ECG) showed sinus tachycardia and ST elevation in the anterior and inferior leads with a QS wave in the anterior leads. Echocardiography showed a dilatation of left ventricle due to a dyskinesia of all distal segments. Basal segments were hypercontractile and no left ventricular dynamic obstruction was described. Laboratory tests showed a significant increase in cardiac enzymes, reaching a peak approximately 10 h after the beginning of symptoms (troponin I 9.91 ng/ml, upper reference limit 0.03 ng/ ml; CK-MB 23.06 ng/ml, upper reference limit 5.00 ng/ ml). In the suspect of an acute coronary syndrome, the patient was treated with low weight molecular heparin, dual antiplatelet therapy and nitroglycerin. Coronary angiography excluded the presence of obstructive coronary artery disease. Left ventriculography showed an extensive apical akinesis with a moderately reduced left ventricular systolic function (LVEF 42 %). Atrial fibrillation occurred during hospitalization, which resolved with infusion of amiodarone after 48 h. Because of a history of asthma, a calcium channel blocker was preferred to beta-blocker therapy. The presence of underlying disorders predisposing to a coronary thrombosis or myocarditis was excluded. In addition, screening for cardiotropic viral agents was negative as well as the urinary catecholamine metabolites amount. Cardiac biomarkers showed progressive normalization in the next days, with a curve consistent with an acute cardiac injury at the time of admission. Our diagnosis was tako-tsubo cardiomyopathy (TTC) and the patient was discharged after 5 days with oral anticoagulant therapy and a calcium channel blocker. An echocardiogram performed 10 days after discharge showed a normalization of the left ventricular systolic function (Fig. 1a, b). One year later, a 44-year-old female presented to our department with a 1-week history of mild dyspnoea on exertion, atypical chest pain, fatigue, and lightheadedness. She had prior diagnosis of mild mitral valve prolapse and cigarette smoking as unique cardiovascular risk factor. She reported an emotional stress event (a dispute with a close person) 1 week before. This woman was the sister of the patient we previously described. The ECG showed sinus rhythm with negative T waves in the anterior, lateral and inferior leads. Transthoracic echocardiography demonstrated akinesis of all the mid-ventricular and apical segments, and hyperkinesia of the basal portions. The left ventricular systolic function was significantly reduced (LVEF 38 %). Serial cardiac markers showed an elevated troponin I at the admission (0.97 ng/ml, upper reference limit 0.03 ng/ml) and slightly elevated CK-MB (7.14 ng/ ml, upper normal limit 5.0 ng/ml). The patient was treated with an antiplatelet drug, low molecular weight heparin and ACE inhibitor. Cardiac catheterization revealed G. Caretta (&) D. Robba Department of Cardiology, A.O. ‘‘Istituti Ospitalieri’’ di Cremona, Cremona, Italy e-mail: giorgio.caretta@gmail.com