238 Hypertrophic cardiomyopathy and tako tsubo syndrome with left ventricular outflow tract obstruction: a thin line

A 75-years-old Caucasian man presented to the Emergency Department due to worsening dyspnoea and leg oedema in the previous days. His past medical history was significant for a minor surgical operation performed a few days earlier. A previous echocardiography described a hypertrophic left ventricular septum (IVSd 14 mm) in the absence of significant hypertension, with an intraventricular pressure gradient of 10 mmHg at rest and a prolapse of the anterior mitral leaflet. At the time of the admission, tachycardia and a 2/6 cardiac murmur were observed and blood tests showed elevated high-sensitivity Troponin T and N-terminal-pro-B-type natriuretic peptide. The EKG showed synus rhythm and sign of ventricular hypertrophy. Therefore ,the patient was transferred to the Cardiology department and therapy for a subacute coronary syndrome was initiated. A transthoracic echocardiogram revealed a hypertrophic left ventricle (IVSd 15 mm), with apical ballooning shape resulting in a moderately reduced ejection fraction. Moderate mitral regurgitation with systolic anterior movement of the anterior mitral leaflet (SAM), was observed, and an estimated left intraventricular gradient of 108 mmHg at rest was recorded. The patient underwent a coronary angiography: no critical obstructive coronary disease was observed. During ventriculography a typical apical ballooning was revealed and a Tako-Tsubo Syndrome was confirmed, besides this an intraventricular gradient of 34 mmHg was measured. A beta-blocker therapy was promptly initiated (metoprolol), since the patient was haemodynamically stable and QTc was only mildly prolonged. A cardiac magnetic resonance (CMR) performed at day 20, excluded both ischaemic pattern and signs of previous myocarditis. Furthermore, CMR showed an only mildly hypertrophic left ventricle (IVSd 12 mm) and no fibrosis, further supporting the idea that in our patient LVOTO was part of TTS physiopathology and not the sign of an underlying hypertrophic cardiomyopathy. One month later echocardiography showed a completely restored left ventricular systolic function; LVOTO was no more detectable and a complete normalization of left ventricular thickness was observed, in accordance with many papers describing a reversible hypertrophy induced by TTS. Early LVOTO, the one experienced by our patient, is a quite common complication of TTS. It is more often observed among the elderlies, in patient with redundant mitral valve leaflets and in those with septal hypertrophy. Besides this, late onset LVOTO is also described: it is only partially reversible and requiring a previous underlying cardiopathy. In both cases, LVOTO is an established bad prognostic factor. The existing evidence discourages the administration of inotropic agents and nitrates, supporting instead the use of beta-blockers aiming at reducing intraventricular gradient. In patients with haemodynamic instability and significant LVOTO, short acting beta blockers should be preferred, although concomitant hypotension may impose the use of mechanical support therapy. On the contrary, clinically stable patients often benefit from oral administration of beta blockers. In conclusion, LVOTO is a common complication of TTS that must be discerned from a probable underlying hypertrophic cardiomyopathy, that can be excluded only after observing a complete reversibility.

533 Uncommon ECG presentation in Tako-tsubo syndrome

Aims We present the case of an 80-year-old woman without prior cardiovascular history, recent instrumental diagnosis of peritoneal carcinomatosis and ongoing oncologic diagnostic work up. Methods and results The patient was admitted to our ED for acute-onset worsening dyspnoea. On first clinical evaluation, she denied typical angina, remarkable clinical features were dyspnoea, tachycardia and hypotension. Admission ECG showed sinus rhythm with posterior and inferior ST elevation (leads DII, aVF, V5, V6) with reciprocal ST segment depression in leads V1–V2. Echocardiography confirmed infero-postero-lateral akinesia determining moderate reduction of LVEF (35–40%), normal aortic root, no pericardial effusion. Laboratory tests revealed normal WBC count, mild anaemia (HB 10.7 g/dl), normal renal function, elevated C-reactive protein (139 mg/l, n.v. < 8). Cardiac troponin I (cTnI) was normal on admission, with significant delta on second determination (0.012 > 2.5 ng/ml, nv < 0.023). ST elevation persisted after BP normalization and hypoxia treatment; so, taken into account the increased procedural risk due to patient’s age and comorbidities, however we decided to perform urgent coronary angiography. Surprisingly, coronary angiography revealed absence of any significant stenosis, with TIMI 3 flow in any coronary segment. The patient was then admitted to the ICU with diagnosis of MINOCA. The next day ECG revealed normalization of ST segment and Q wave in V2–V3. On day 2 new ECG showed new ventricular repolarization abnormalities with T wave inversion in precordial leads. Peak hs-cTnI was >15 000 ng/l. Repeat echocardiography on day 2 reported complete akinesia of all the apical segments of the LV with normo-hyperkinesia of the mid-basal segments (apical ballooning pattern) and severely depressed systolic function (FE 32–35%). During the following days patient’s symptoms improved, with rapid weaning from oxygen therapy and stable haemodynamic parameters. After 10 days the patient repeated echocardiography, which revealed improvement of global LVEF and persisting mild apical hypokinesia, suggesting the diagnostic hypothesis of Tako-Tsubo Syndrome (TTS) or TTS-phenocopy (unfortunately cardiac MRI was not performed). The patient was therefore transferred to oncology department to complete the diagnostic work-up; primary mammary neoplasia was identified, moreover associated with metastasis in the liver and the brain. Unfortunately, the patient died a month later due to non-cardiac causes. This is the case report of an uncommon MINOCA, which presented mimicking inferolateral acute STEMI, but subsequent ECG and echocardiographic evolution showed the more typical TTS pattern, with apical ballooning on echo and deep negative T waves in anterior leads. Conclusions The prevalence of MINOCA is estimated to be 6% to 8% among patients diagnosed with MI, especially women, however it is more common in patients with NSTEMI compared with STEMI; moreover in cases of TTS presenting with ST elevation, usually the elevation is found in anterior leads without reciprocal ST depression; in this patient instead ST elevation was inferolateral with reciprocal anterior ST depression. Absence of obstructive CAD and clinical/echocardiographic evolution allowed us to confirm the diagnosis of MINOCA/TTS.

649 Clinicians miss diagnosis: it wasn’t a tako-tsubo syndrome, nowadays it is a recurrent tako-tsubo syndrome

Aims Tako-Tsubo syndrome is an usual form of acute cardiomyopathy characterized by reversible left ventricle apical ballooning which occurs in the absence of significant coronary artery disease. Rarely an increase in catecholamines due to pheochromocytoma could lead to Tako-Tsubo syndrome. Here we report a clinical case of undetected pheochromocytoma which led to Tako-Tsubo syndrome in an adult female patient. Methods and results A 60 years old female presented at our emergency department with chest pain, increase in cardiac troponin levels and hypo-akinesia of the apical segments at the echocardiography. She was admitted with acute coronary syndrome suspicious. He had history of acute coronary syndrome with non-obstructive coronary arteries (MINOCA) in the 2017. Coronary angiography performed at 24 h from admission, again showed non-obstructive coronary disease, whereby we reviewed the previous MINOCA diagnosis into recurrence Tako-Tsubo syndrome diagnosis. During hospitalization she presented parossistic sinus tachycardia refractory to beta-blocker and ivabradine combined therapy. Cardiac magnetic resonance was performed and confirmed Tako-Tsubo syndrome, furthermore showed a surrenalic mass. Contrast-enhanced abdomen computed tomography and high levels of metanephrines suggested pheochromocytoma diagnosis. One month later she underwent left surrenectomy surgery. Histological examination confirmed pheochromocytoma. The last echocardiography didn’t show kinesia abnormalities with normal left ventricular function. Conclusions Our case illustrates the importance of understanding the correlation between Tako-tsubo syndrome and pheochromocytoma, especially in patients with increased activity of the sympathetic nervous system. Until recently, according to Tako-Tsubo syndrome Mayo Clinic criteria, our patient wouldn’t had receive a Tako-Tsubo syndrome diagnosis because pheochromocytoma was an exclusion criteria. Since 2018, Inter-TAK criteria replaced Mayo Clinic criteria including Tako-Tsubo syndrome expression of pheochromocytoma. Referring to our annoying title we always have to consider Tako-Tsubo syndrome into MINOCA differential diagnosis.

711 A rare case of takotsubo syndrome induced by pacemaker implantation

Aims Takotsubo syndrome (TTS) is an acute cardiac condition characterized by a temporary wall motion abnormality of the left ventricle that mimics an acute coronary syndrome (ACS). TTS usually occurs following emotionally or physically triggering event. We report a rare case of Takotsubo syndrome following a pacemaker implantation. Methods and results A 77-year-old woman was admitted to our hospital with a third-degree atrioventricular block. She was asymptomatic with a history of hypertension, diabetes, hyperlipidaemia and extrapyramidal syndrome. Laboratory tests reported High-Sensitive cardiac Troponin T (HS-cTnT) 32.9 ng/L (ULN <14), creatine kinase muscle and brain (CK-MB) 4.1 ng/mL (ULN < 4.94) and NT-proB-type Natriuretic Peptide (NT-proBNP) 1465 pg/ml (ULN < 125). Echocardiography showed a normal left ventricular ejection fraction (EF = 58%) (Figure 2A). The patient underwent dual chamber pacemaker implantation without immediate complications. Three days later, a routine ECG showed new T wave inversions (Figure 1), in absence of symptoms. Echocardiography revealed apical akinesia, with ‘apical ballooning’ (EF 30%) (Figure 2B). Serum cardiac markers were increased (CK-MB 8.2 ng/ml, HS-cTnT 189.7 pg/ml, NT-proBNP 15 005 pg/ml. A coronary angiography excluded obstructive coronary artery disease (Figure 3). Given the impossibility of carrying out a cardiac RMI for the recent pacemaker implantation and after exclusion of other diagnoses, pacemaker implantation induced Takotsubo syndrome was suspected. Conclusions The trigger of this case of TTS was the pacemaker implantation, a relatively brief and painless procedure that, in her case could have constitute a considerable emotional and physical stress. To the best of our knowledge 13 cases of TTS after pacemaker implantation have been described to date. Of those cases, four had an asymptomatic course. Therefore, the real incidence of TTS following pacemaker implantation may be underestimated and ECG and echocardiography should always be performed after pacemaker implantation.

285 A rare case of atypical, non-triggered takotsubo recurrence

Aims Takotsubo syndrome (TTS) is an acute and reversible heart failure syndrome that, at presentation, mimics acute myocardial infarction. The most common echocardiographic manifestation is the so-called ‘apical ballooning’, but other much less common wall motion patterns have been described. The pathophysiology of the syndrome is not fully understood, but there is considerable evidence that sympathetic stimulation plays a central role. The prevalence of this syndrome is higher in post-menopausal women and in most cases, but not invariably, precipitated by an emotional or physical triggering event. A close relation between brain and circulatory system has been observed and for this reason psychiatric and neurologic disorders are often recognized as precipitating conditions. Although many risk factor persist after the acute manifestation, Takotsubo recurrences do represent an exception, especially in the absence of a clear precipitating event. Methods and results A 70-year-old woman was admitted for anginal pain associated with ischaemic electrocardiographic alterations and elevation of cardiac biomarkers. The coronary angiography with left ventriculogram and the echocardiographic findings were consistent with a diagnosis of mid-ventricular Takotsubo. Cardiac magnetic resonance confirmed the absence of an ischaemic pattern or evidence of infectious myocarditis. This case represents a recurrence of TTS, in fact two years earlier the patient was hospitalized to our division for stress cardiomyopathy with typical apical ballooning. Also in the present occasion, she had a favorable clinical course, with a complete recovery of the cardiac function at subsequent evaluations. The unicity of this case lies above all in the absence of a clear trigger event. Although, an altered mental status was present because the patient suffered from anxiety and depression on pharmacological treatment, with periods of exacerbation but not in occasion of the recurrence. Conclusions TTS is not a benign condition, with recurrence being possible even in the absence of precipitating events. Based on registry data, annual rate of Takotsubo recurrence is 1.5–1.8% and is estimated to reach 4% in life. A variable TTS pattern at recurrence is common in up to 20% of cases. Our paper reports a unique case of recurrent Takotsubo syndrome with variable patterns of ventricular involvement, with neither physical nor psychological trigger. Nevertheless, for what concerns our case, the psychiatric condition the patient suffered from, could have played a role of permanent status of sympathetic activation, that in the end elicitates the occurrence of the syndrome. A better understanding of the pathophysiology of the syndrome is needed to find evidence-based therapeutic strategies that could prevent recurrence.

Different Clinical Features between Definite and Possible Takotsubo Syndrome in a Tertiary Referral Hospital

Background: Although imaging examination to exclude coronary artery disease (CAD) is an indispensable step for a definite diagnosis of Takotsubo syndrome (TTS), this step may be overlooked in a substantial proportion of patients with secondary TTS admitted to a tertiary hospital. However, the clinical profiles and outcomes of these patients with “possible TTS” have rarely been investigated. Methods: Among 420 consecutive TTS patients with characteristic transient ventricular ballooning on repeated echocardiography, 244 patients (58.1%) who underwent an imaging study for CAD were diagnosed with “definite TTS”, whereas the remaining 176 were designated with “possible TTS”. Results: Overall, hypoxia (67.6%) and dyspnea (55.5%) were predominant presentations. The possible group was characterized by higher prevalence of male gender (46.6% vs. 35.2%, p = 0.019), secondary TTS (97.2% vs. 86.5%, p <0.001), cancer (43.2% vs. 29.1%, p = 0.003), sepsis (46.0% vs. 32.0%, p = 0.003), and non-apical ballooning pattern (30.7% vs. 21.3%, p = 0.001) with less common ST-segment elevation on electrocardiogram (18.8% vs. 34.0%, p = 0.001). The possible group showed higher frequency of mechanical ventilation (56.2% vs. 40.2%, p = 0.001), pulmonary edema (72.2% vs. 61.5%, p = 0.023) and shock management (70.5% vs. 54.1%, p = 0.001) with similar in-hospital mortality (17.2% vs. 17.0%, p = 0.964). Conclusions: In real-world clinical practice, coronary evaluation for strict diagnosis of TTS is not frequently feasible. Addition of the possible group without coronary evaluation to the clinical spectrum of TTS would be helpful for fair estimation of clinical implication of TTS.

Takotsubo cardiomyopathy associated with dydrogesterone use

Takotsubo cardiomyopathy is characterised by left ventricular apical ballooning, in the absence of coronary artery disease, and classically occurs at times of intense stress. Due to the striking preponderance of Takotsubo cardiomyopathy occurring in postmenopausal women, it has been postulated that female sex hormones may also be implicated in its pathogenesis. This case report describes the first case of Takotsubo cardiomyopathy associated with the initiation of dydrogesterone (a synthetic retroprogesterone) in a premenopausal woman.

A mimic of a STEMI in an elderly male patient at emergency department requiring no stent

An elderly male patient presented with chest pain and an initially abnormal ECG, with 1 mm ST elevation in the lateral leads. As he was pain free on arrival, he was treated locally, where a coronary angiography showed no stenosis and echocardiography showed apical ballooning which indicated Takotsubo Cardiomyopathy (TC). On further questioning, he indicated he has been worrying about his son’s financial circumstances. Patients with TC can present with a history and an ECG resembling and indistinguishable from ST-elevation Myocardial infarction or other types of Acute Coronary Syndrome (ACS).

Syndrome of Reversible Cardiogenic Shock and Left Ventricular Ballooning in Obstructive Hypertrophic Cardiomyopathy

Background Cardiogenic shock from most causes has unfavorable prognosis. Hypertrophic cardiomyopathy (HCM) can uncommonly present with apical ballooning and shock in association with sudden development of severe and unrelenting left ventricular (LV) outflow obstruction. Typical HCM phenotypic features of mild septal thickening, outflow gradients, and distinctive mitral abnormalities differentiate these patients from others with Takotsubo syndrome, who have normal mitral valves and no outflow obstruction. Methods and Results We analyzed 8 patients from our 4 HCM centers with obstructive HCM and abrupt presentation of cardiogenic shock with LV ballooning, and 6 cases reported in literature. Of 14 patients, 10 (71%) were women, aged 66±9 years, presenting with acute symptoms: LV ballooning; depressed ejection fraction (25±5%); refractory systemic hypotension; marked LV outflow tract obstruction (peak gradient, 94±28 mm Hg); and elevated troponin, but absence of atherosclerotic coronary disease. Shock was managed with intravenous administration of phenylephrine (n=6), norepinephrine (n=6), β‐blocker (n=7), and vasopressin (n=1). Mechanical circulatory support was required in 8, including intra‐aortic balloon pump (n=4), venoarterial extracorporeal membrane oxygenation (n=3), and Impella and Tandem Heart in 1 each. In refractory shock, urgent relief of obstruction by myectomy was performed in 5, and alcohol ablation in 1. All patients survived their critical illness, with full recovery of systolic function. Conclusions When cardiogenic shock and LV ballooning occur in obstructive HCM, they are marked by distinctive anatomic and physiologic features. Relief of obstruction with targeted pharmacotherapy, mechanical circulatory support, and myectomy, when necessary for refractory shock, may lead to survival and normalization of systolic function.

Tako-Tsubo syndrome. Approach to the subject and Report of 2 cases

Takotsubo syndrome, or stress cardiomyopathy, is a relatively rare transient and reversible cardiomyopathy, although its diagnosis has increased in recent years, it presents as an acute coronary syndrome (ACS) or acute heart failure, its incidence is unknown exactly in Latin America and in Cuba. We present 2 cases seen in our hospital, both 63 and 55-year-old women with typical precordial pressure pain, the first triggering psychological stress and the second physical, with electrocardiographic changes consistent with anterior infarction and cardiogenic shock, which were found in the coronary angiographic study observed normal coronary arteries and ventriculography determined apical ballooning of the left ventricle characteristic of the syndrome, with subsequent recovery and favorable clinical evolution at 6 months.