Transient Systolic Dysfunction Research Articles

Genetic characterization of drug-induced long QT syndrome in a large prospective cohort

Abstract Introduction Drug-induced long QT syndrome (DI-LQTS) is a clinical entity with a poorly defined etiopathogenesis. Purpose The objective of the study was to determine the genetic basis of a prospective cohort of patients with DI-LQTS in the setting of a tertiary hospital center. Methods Prospective observational study with consecutive inclusion of patients with the diagnosis of DI-LQTS according to standard criteria, in a tertiary hospital, between 2018 and 2022. A genetic study was performed through massive DNA sequencing to identify variants in known genes associated with cardiopathy, arrhythmogenic and cardiomyopathy. Results 86 patients (age 65±15 years, 59% men) were included. The mean corrected QT interval (QTc) of the entire cohort was 543±56 ms. The drugs associated with QTc interval prolongation were: psychotropics 51%, antiarrhythmics 38%, anesthetics 30%, antibiotics 16%, antineoplastics 5%, others 4%. Twenty-one patients (24%) presented with ventricular arrhythmias at the time of drug-induced QTc prolongation: 10 (12%) ventricular fibrillation, 7 (8%) sustained ventricular tachycardia, 3 (3%) non-sustained ventricular tachycardia, 1 (1%) frequent ventricular premature beats. Genetic information was obtained in 83 (97%) patients. 17 pathogenic mutations associated with heart disease were identified in 15 (18%) patients: 4 (5%) patients with 5 mutations in ion channel genes (KCNE1, KCNE1B, KCNH2, RYR1, RYR2), and 12 (14%) patients with 12 mutations in genes associated with cardiomyopathy (NF1, PKD2, ABCC6, TTN, FHOD3, MYO6, COL11A1, CCDC40, CRELD1, COL4A1). Two patients had 2 pathogenic mutations each, one of them with both mutations associated with channelopathy, and another with a mutation associated with channelopathy and a second mutation related to cardiomyopathy. Only 1 patient carrying a pathogenic mutation related to cardiomyopathy had a clear phenotype, while in 3 carrier patients there was a history of transient systolic dysfunction, 2 due to tachycardiocardiopathy and 1 due to peripartum cardiomyopathy. The identification of a pathogenic mutation was not related to significant differences in QTc interval prolongation (544±63 vs 542±55 ms) or risk of ventricular arrhythmias (26% vs 24%). Conclusions The genetic basis of drug-induced long QT syndrome is essentially different from that of the congenital form of the disease. The identification of pathogenic mutations in genes associated with cardiomyopathy is relatively frequent, while those associated with ion channel genes are uncommon.

Ruptured sinus of valsalva aneurysm - a rare cause of heart failure, challenges in transcatheter closure and mid-term outcomes

Abstract Introduction The ruptured sinus of valsalva aneurysm (RSOV) portends an important cause for acute heart failure. Closure of the defect either percutaneously or surgically is of paramount importance. We report 38 cases of RSOV who presented with cardiac failure, among which 22 cases were selected and amenable for transcatheter closure.Transcatheter closure of RSOV has favorable outcomes in the short-term and mid-term follow-up. Purpose of the study To assess the short-term and midterm outcomes of transcatheter closure of RSOV. Methods All patients who presented with cardiac failure and diagnosed as RSOV by transthoracic echo, from June 2012 to Dec 2022 were included in the study.Age group was between 12 to 73 years.All the 38subjects (Males -24, Females -14) of RSOV, underwent trans esophageal echo (TEE). 22 casesere selected for device closure..The subjects were classified according to New York Heart Association (NYHA) class III - 82%, class IV- 18 %. Size of the defect was measured at the aortic end by TEE and aortogram, and upsized by 2-4 mm from the aortic end. In 21 subjects, the RSOV was entered from the aorta, and an arterio-venous loop was created, and various devices, duct occluders and atrial septal occluder and muscular ventricular septal occluder were used to close the defects. In one patient the RSOV was acquired following surgical closure of perimembranous ventricular septal defect, which was small measuring 3mm at aortic end and hence closed using 4/6 ADO II without forming the arterio-venous loop. Patients were followed up post discharge 1 week, 1 month, 3 months, 6 months, 1 year and every year till 10 years by clinical examination, ECG, and transthoracic echo. Results All 22 patients who underwent successful RSOV device closure improved to NYHA functional class II within 48 hours post procedure and to functional class I at 3 months of follow-up. 3 female patients underwent successful pregnancy and labor after RSOV device closure without any intrapartum or postpartum complications. Two patients had trivial aortic regurgitation pre-procedure remained the same status on follow up post-procedure. One patient developed) severe right coronary artery spasm, due to inadvertent RCA cannulation while crossing the defect, which cleared with nasal oxygen and intracoronary nitroglycerine. A minimal residual shunt was found in 2 patients which gradually disappeared at 6 months. One patient with a large defect (15mm), an atrial septal occluder - 20mm was deployed, developed hemolysis, and hence referred for surgery. Transient global LV systolic dysfunction was present in two patients due to afterload mismatch, whose LV function recovered after 72 hours. Conclusion Percutaneous device closure of RSOV is a feasible and an enticing alternative to surgery in selected patients, as inferred by the short and mid-term outcomes. The age group 12 to 73 years encompasses the youngest and oldest age group yet reported.PROFILE OF RSOV DEVICE CLOSURE CASESRSOV CASE SELECTION AND OUTCOMES

A Broken Heart Coexisting with Obstructive Coronary Artery Disease: Double Trouble

Background. Takotsubo is a syndrome characterized by transient regional systolic dysfunction of the left ventricle (LV). The most common clinical presentation mimics acute myocardial infarction without angiographic evidence of obstructive coronary artery disease or acute plaque rupture.1-3 The diagnosis and management became a challenge when it presented as atypical symptoms and significant obstructive coronary artery. Thus, this case report highlights the diagnosis and management of Takotsubo Cardiomyopathy with obstructive coronary artery disease.Case illustration. A 71 years old woman came to the emergency room with a chief complaint of dyspnea from one week ago and worsened in the last three days. The initial electrocardiogram showed slight ST-elevation, and thorax Rontgen showed the congestion and elongation of the aorta. Increased high-sensitive Cardiac Troponin T and The NT-Pro BNP levels were present, along with apical ballooning of the LV and reduced RV function. Physiological stress was found to be the death of her husband one week ago. Although the left ventriculography of this patient was classically depicted as the octopus trap, we did find obstructive coronary artery disease in the left anterior descending artery.
 Keywords: Takotsubo Cardiomyopathy, Uncommon presentation, Coronary artery disease, apical ballooning, acute heart failure

Description of a Patient Suspected of Myocardial Infarction With Normal Coronary Artery: A Case Report of Takotsubo Cardiomyopathy

Takotsubo cardiomyopathy (TC) is characterized by transient systolic dysfunction, presenting with electrocardiographic (ECG) changes and mimicking acute coronary syndrome (ACS). Studies have shown that 95% of patients have changes in ECG. ECG changes in TC syndrome may be similar to myocardial infarction )MI(. ECG changes can be seen in the form of ST-segment elevation or deep T-wave inversion. TC can be considered a special form of ACS. TC is not a benign entity, and conduction abnormalities can occur. The acute phase can be predicted based on decreased EF and arrhythmia. Different treatments are used in the acute phase of TC, depending on the hemodynamic condition. Our case elucidates the importance of age, female gender, symptoms, such as stress and emotion, clinical manifestations, ECG characteristics, and echocardiography in TC. This paper describes a case of TC from a hospital affiliated to Rafsanjan University of Medical Sciences. The case was a 75-year-old woman with no history of cardiovascular diseases who has been admitted to the hospital with myocardial infarction.

A Rare Case of Left Anterior Descending Coronary Artery to Pulmonary Trunk Fistula Associated with Takotsubo Cardiomyopathy.

Coronary-artery-to-pulmonary-artery fistulae represent rare vascular anomalies defined as abnormal communications between the coronary arteries and the pulmonary arterial system. Takotsubo Syndrome represents a stress-induced cardiomyopathy defined by transient regional systolic dysfunction of the left ventricle, with minimal elevation of cardiac biomarkers, without angiographic evidence of obstructive coronary artery disease. We hereby richly illustrate an unusual and rare case of a female patient with Takotsubo Cardiomyopathy and left-anterior-descending-coronary-artery-to-pulmonary-trunk fistula through multi-modality imaging evaluations, obtaining a detailed anatomical representation of the coronary arteries and the fistulous connection, which further guided the optimal treatment strategy. The patient was treated conservatively. The main teaching points of this case are the following: (1) The coronary fistula may represent just an incidental finding in a Takotsubo Cardiomyopathy clinical scenario. (2) The particularly rare association between left-anterior-descending-coronary-artery-to-pulmonary-trunk fistula and Takotsubo Cardiomyopathy presentation is mainly due to the stress-induced overstimulation of myocardial beta-1 receptors, accentuating the coronary steal phenomenon in the setting of the coronary fistula, manifesting as anginal pain, and also the stress-induced adrenergic drive causing the Takotsubo-like presentation with apical ballooning of the left ventricle.

Role of miR-204 in segmental cardiac effects of phenylephrine and pressure overload

Cardiotoxicity caused by adrenergic receptor agonists overdosing or stress-induced catecholamine release promotes cardiomyopathy, resembling Takotsubo cardiomyopathy (TC). TC is characterized by transient regional systolic dysfunction of the left ventricle. The animal models of TC and modalities for assessing regional wall motion abnormalities in animal models are lacking. We previously reported the protective role of a small noncoding microRNA-204-5p (miR-204) in cardiomyopathies, but its role in TC remains unknown. Here we compared the impact of miR-204 absence on phenylephrine (PE)-induced and transaortic constriction (TAC)-induced changes in cardiac muscle motion in the posterior and anterior apical, mid, and basal segments of the left ventricle using 2-dimensional speckle-tracking echocardiography (2-STE). Wildtype and miR-204−/− mice were subjected to cardiac stress in the form of PE for four weeks or TAC-induced pressure overload for five weeks. PE treatment increased longitudinal and radial motion in the apex of the left ventricle and shortened the peak motion time of all left ventricle segments. The TAC led to decreased longitudinal and radial motion in the left ventricle segments, and there was no difference in the peak motion time. Compared to wildtype mice, PE-induced peak cardiac muscle motion time in the anterior base of the left ventricle was significantly earlier in the miR-204−/− mice. There was no difference in TAC-induced peak cardiac muscle motion time between wildtype and miR-204−/− mice. Our findings demonstrate that PE and TAC induce regional wall motion abnormalities that 2-STE can detect. It also highlights the role of miR-204 in regulating cardiac muscle motion during catecholamine-induced cardiotoxicity.

Laboratory Aspect of Broken Heart Syndrome

Broken Heart Syndrome (BHS) is the weakness of the heart muscle due to emotional stress or physical stress called cardiomyopathy. The main etiology is a sudden release of stress hormones (catecholamines), such as norepinephrine, epinephrine, and dopamine. About 90% of BHS patients are female with average age of 67-70"‰ years, most of them are post-menopausal females. The most widely supported pathological theories are catecholamine-induced cardiotoxicity and microvascular dysfunction. The clinical condition resembles that of acute myocardial infarction, consisting of chest pain, electrocardiographic changes, elevated cardiac biomarkers, and abnormalities of heart wall motion. There is transient systolic dysfunction in the apical and/or middle segment of the left ventricle resembling acute myocardial infarction but absence of coronary artery obstructive disease. There are BHS criteria according to Mayo Clinic. Laboratory tests can be performed by examining Natriuretic Peptides, cardio myonecrosis markers (Troponin I and T, creatinine kinase, and myoglobin), and catecholamines. There is no single established biomarker for initial diagnosis of BHS that distinguishes it from STEMI. It was found that the most accurate ratio as a marker capable of differentiating BHS from STEMI in early stages was NTproBNP/TnI ratio. The InterTAK diagnostic score was used to predict the probability of BHS, differentiating it from ACS in an acute stage, prior to coronary angiography. The main differential diagnosis of BHS is ACS, besides acute myocarditis infectious. Patients with BHS should be treated as ACS until proven otherwise. The prognosis for BHS patients is generally very good.

777 TISSUE CHARACTERIZATION IN TAKOTSUBO SYNDROME

Abstract Aims Takotsubo Syndrome (TTS), also known as stress cardiomyopathy, is an important form of acute reversible myocardial damage characterized by a transient systolic dysfunction of the left ventricle, which generally occurs following intense emotional or physical stress. The availability of new imaging techniques broadened knowledge about TTS and allowed for more accurate risk stratification and their use as a potential guide to clinical management. The aims of our study was that of evaluating changes in myocardial tissue characterization on cardiac magnetic resonance imaging in patients diagnosed with TTS admitted to our center. Methods and Results From December 2018 to December 2021, 52 patients diagnosed with TTS according to the 2018 InterTAK diagnostic criteria were admitted to our ICU; only 27 patients who underwent cardiac magnetic resonance imaging were included in our study. Cardiac magnetic resonance was performed after an average of 7 days from admission to the ward, therefore not in all patients the regional wall motion abnormalities of the left ventricle found on the first echocardiogram performed were then still evident. Despite this, at the evaluation of the T2-STIR sequences, the presence of myocardial edema in the segments with previous regional dissynergies was highlighted in all patients included in the study, indicating a greater sensitivity of tissue characterization techniques. Furthermore, the analysis of T2 mapping, for the quantification of edema, also showed a significant lengthening of the average values ​​of the global T2 mapping, compared to the reference values ​​at our center [65.9 +/- 7.7 msec, vn T2 mapping = 53 ± 3 msec; mean baseline T2 mapping value: 60.4 +/- 5.5 msec, mean T2 mapping value: 65 +/- 7.1 msec, apical T2 mapping: 71 +/- 7.7 msec], with evidence of a clear base-apex gradient on 19/23 patients (82.6%) compatible with the greater involvement of the mid-apical segments typical of the classic form of "apical ballooning" TTS. Finally, the evaluation of the systolic strain also showed a significant reduction in the values ​​both in the radial component and in the circumferential and longitudinal components, especially in the mid-apical segments, even in the absence of significant regional wall motion abnormalities or with preserved left ventricular EF [mean values global radial strain (GRS) of 28.6 +/- 9.5; global circumferential strain (GCS) of 15.4 +/- 7.9; global longitudinal strain (GLS) of 12.6 +/- 3.2]. Conclusions Our study confirms that tissue characterization and functional evaluation techniques using myocardial strain with cardiac magnetic resonance appear to be more accurate, compared to the evaluation of regional wall motion abnormalities and left ventricular EF alone, in identifying pathophysiological changes and in diagnostic framework of TTS patients.

Abstract 9812: Abracadabra Alakazam: Sudden Invasion of the Pericardium by Haemophilus Influenzae

A 25 year old man presented with three days of cough, shortness of breath, and pleuritic chest pain. Initial vital signs in the ED were normal, and exam demonstrated tonsillar erythema without exudate. Labs revealed a leukocytosis of 18.9k/μL, D-dimer of 690 ng/mL, C-reactive protein of 5.7 mg/dL, and lactate elevated to 2.9 mmol/L. High-sensitivity troponin, NT-proBNP, and SARS-CoV-2 RT-PCR were all negative. Presenting electrocardiogram demonstrated PR elevation in aVR with diffuse ST-segment elevation in the inferior and anterolateral leads. Point-of-care echocardiogram demonstrated normal biventricular function without pericardial effusion. CTPA was negative for pulmonary embolism, and he was observed for presumed acute viral pericarditis. Fourteen-hours later, he became febrile to 38.3°C, tachycardic to 133 bpm, and hypotensive to 97/65 mmHg with diffuse abdominal pain. Repeat lactate was 9.0 mmol/L. This prompted an emergent CT scan which now showed a new large pericardial effusion and bilateral pleural effusions ( Panel A ). Repeat echocardiogram confirmed a large circumferential pericardial effusion with early signs of tamponade including right atrial inversion in late diastole ( Panel B ). Emergent pericardiocentesis yielded 560 mL of brown, purulent fluid ( Panel C ) with immediate improvement in hemodynamics. Bacterial gram stain and culture grew Haemophilus influenzae ( Panel D ). Immunodeficiency screening was negative. Transient severe biventricular systolic dysfunction was noted, consistent with sepsis-induced cardiomyopathy. He completed a targeted antibiotic course with partial recovery of his ejection fraction by discharge. Purulent pericarditis is rare in developed countries, and invasive H. influenzae in a young, immunocompetent adult is particularly unusual. This case illustrates the importance of early diagnosis and management of purulent pericarditis given its potential for rapid progression and high mortality.

VENTRICULAR FIBRILLATION AS PRESENTATION FOR TAKOTSUBO CARDIOMYOPATHY

VENTRICULAR FIBRILLATION AS PRESENTATION FOR TAKOTSUBO CARDIOMYOPATHY

Takotsubo syndrome triggered by emotional stress: a case report

Takotsubo syndrome is an acute cardiac syndrome characterized by transient systolic dysfunction of the left ventricle without coronary artery disease. It’s etiology has not been fully explained, physical and intense emotional stress triggers this syndrome so it is also called "broken heart syndrome" or "stress cardiomyopathy". Takotsubu syndrome has been reported that approximately 2% of patients presenting with acute coronary syndrome. In this case, we presented a 65-year-old patient who had a Takotsubo syndrome attack after emotional stress, thinking that it would contribute to the literature and increase awareness of this disease.

Takotsubo syndrome and impending thyroid storm: A case report and brief review of the literature

Stress cardiomyopathy also called Takotsubo cardiomyopathy is characterized by transient regional systolic dysfunction of the left ventricle with electrocardiographic changes and an increase in troponin levels imitating myocardial infarction, but in the absence of angiographic evidence of obstructive coronary artery disease. Although the exact causal process has not been determined, it has been mentioned in connection with thyroid diseases. We describe a woman's case that experienced severe chest pain along with electrical and analytical abnormalities. Significant ventricular dysfunction was present, and the invasive coronary angiography revealed no lesions, which was compatible with stress cardiomyopathy. Impending thyroid storm due to Graves’ disease was observed as a trigger and it is imperative to identify and treat any clinical variables that could put patients at risk for Takotsubo syndrome or have an impact on the course of their treatment.

Gut Feelings and Broken Hearts: A Case of Small Bowel Obstruction Causing Takotsubo Cardiomyopathy

Takotsubo cardiomyopathy (TCM), also known as apical ballooning syndrome or stress-induced cardiomyopathy, typically presents with features of the acute coronary syndrome. It is characterized by left ventricular apical akinesis and transient systolic dysfunction in the absence of obstructive coronary artery disease. Although its pathogenesis remains unclear, it is thought to be a catecholamine surge that is produced following intense physical or emotional stress. We present a case of TCM in a patient with small bowel obstruction (SBO), which is a rare trigger.

Abstract 13730: In-Hospital Outcomes for Takotsubo Cardiomyopathy Among Patients With Psychiatric Diagnosis

Background: Takotsubo (or stress) cardiomyopathy (TC) is a transient regional systolic dysfunction, clinically and echocardiographically resembling acute coronary syndrome (ACS) in the absence of obstructive coronary artery disease. The prevalence of psychiatric disorders is high in TC. However, it is unclear whether presence of underlying psychiatric condition impacts outcomes of TC. Methods: Hospitalizations for TC from 2012 to 2018 were identified using the National Inpatient Sample (NIS) and stratified by presence or absence of concomitant psychiatric diagnoses (major depression, dysthymia, anxiety, bipolar, substance abuse). Outcomes of interest included mortality, cardiac arrest, cardiogenic shock, intra-aortic balloon pump (IABP), mechanical ventilation, and length of stay. Differences in outcome rates were compared using the Rao-Scott chi-square test; length of stay was compared using a lognormal regression model. All analyses accounted for the NIS sampling design. Results: A total of 51,625 hospital discharges with TC were identified, of which 21,690 (42%) carried a psychiatric diagnosis. There were no statistically significant differences in mortality (1.2% vs. 1.6%; p=0.087), cardiac arrest (1.5% vs. 1.5%; p=0.853), cardiogenic shock (4.6% vs. 4.9%; p=0.427), IABP use (1.6% vs. 1.7%; p=0.695), mechanical ventilation (4.9% vs. 4.2%; p=0.113) for patients with and without psychiatric diagnosis, respectively. The length of stay among patients with psychiatric diagnosis, was marginally higher (median 3.7, IQR [3.6-3.8] days vs. 3.5 [3.4-3.6] days; p<0.01). Conclusion: Our analysis demonstrates that in-hospital mortality and complications of TC patients with and without psychiatric illness are statistically similar.

Abstract 13635: Isolated Right Ventricular Stress Cardiomyopathy: A Systematic Review of Literature

Background: Takotsubo Cardiomyopathy (TTC), also known as stress cardiomyopathy, is characterized by transient systolic and diastolic dysfunction without evidence of significant coronary artery stenosis. In 2012, TTC attributed to about 0.02% of hospitalizations in the United states, largely seen in the elderly female population. Although it primarily affects the left ventricle, there are different variant patterns documented mostly affecting younger individuals. Here we present a systematic review of right ventricle TTC. Methods: On January 7th, 20221, a systematic search without limitation in the time frame was conducted using Pubmed, Google Scholar, CINAHL, Cochrane CENTRAL, and Web of Science databases and a total of 9 cases were identified (1 case was excluded due to duplicity). Results: The mean age of the patients listed was 65.2 ± 23.35 with majority (8/9) 88% of patients being women. Commonest presentation was dyspnea (67%), followed by chest pain (22%), nonspecific discomfort in 11%, and acute heart failure in 11%. Cardiogenic shock was reported in 56% of the cases. Management and treatment included inotropic or vasopressor support in 67% of the cases and 11% requiring venoarterial extracorporeal membrane oxygenation (V-A ECMO). Improvement of right ventricular function was reported in all but 1 patient who died. Conclusion: Isolated right ventricular stress cardiomyopathy is rare but possibly underreported. Though associated with a higher incidence of cardiogenic shock on presentation than traditional stress cardiomyopathy, they do seem to spontaneously recover once out of the acute phase of the syndrome as noted in our study. While the pathogenesis of stress cardiomyopathy is still not fully understood, genetic predispositions may be involved. Current recommendations for management include conventional heart failure medications including beta blockers, ACE inhibitors and diuretics despite limited evidence.

VENTRICULAR TACHYCARDIA ELECTRICAL STORM AS A MANIFESTATION OF REVERSE VARIANT TAKOTSUBO CARDIOMYOPATHY

TOPIC: Cardiovascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Takotsubo cardiomyopathy (TTC) is a syndrome with transient systolic dysfunction in the absence of obstructive coronary arteries with the reverse variant being a rare entity (1,2). Here, we report a case of reverse TTC presenting with monomorphic ventricular tachycardia (VT) electrical storm. CASE PRESENTATION: A 76 year-old female with a history of recently diagnosed Stage IIIB lung adenocarcinoma presented to the emergency room with shortness of breath. On presentation, her vitals were: BP 134/82, pulse 120 beats/min, SPO2 93% in room air. Initial EKG showed no significant ST-T wave changes and normal QTc. Labs were significant for uptrending troponin from 0.8 to 4 ng/mL. Aspirin and heparin infusion were started for possible NSTEMI with a plan for catheterization. That night, she developed unstable, monomorphic VT (Figure 1). Consecutive synchronized cardioversions and amiodarone boluses were unsuccessful. Patient's VT terminated with repeated boluses of lidocaine followed by lidocaine infusion. Stat echocardiogram showed an ejection fraction of 25-30% with anterior and anteroseptal hypokinesis. Emergency cardiac catheterization revealed non-obstructive coronary arteries with akinesis of the basal wall and sparing of the apex suggesting reverse TTC (Figure 2). She was transferred to the critical care unit where she was transitioned to amiodarone infusion with no further VT episodes and was initiated on guideline-directed medical therapy. DISCUSSION: TTC usually occurs due to a surge of catecholamines leading to myocardial damage. The typical variant demonstrates apical ballooning with basal hypokinesis. Rarely, there may be basal hypokinesis with apical sparing, the reverse variant (2). Arrhythmias are uncommon in typical TTC and prior studies have indicated a low incidence of VT (3.2%) (3). There is sparse literature describing the incidence of VT or VT storm in the reverse variant. One of the postulated mechanisms for VT in TTC is transient QTc prolongation, however this remains controversial. (3). The incidence of arrhythmias is thought to be more prevalent during the acute phase leading to increased in-hospital mortality (3). Additionally, it remains uncertain whether prophylactic antiarrhythmics are beneficial in this population subset. Further studies are warranted to understand which variants of TTC develop critical arrhythmias. CONCLUSIONS: Reverse variant is a rare subset of TTC which can present with critical arrhythmias including VT storm. Awareness of this manifestation can help clinicians with early recognition and management of these critical arrhythmias. REFERENCE #1: Bybee KA., Prasad A. Stress-related cardiomyopathy syndromes. Circulation 2008;118(4):397–409. Doi: 10.1161/CIRCULATIONAHA.106.677625. REFERENCE #2: Templin C., Ghadri JR., Diekmann J., et al. Clinical Features and Outcomes of Takotsubo (Stress) Cardiomyopathy. New England Journal of Medicine 2015;373(10):929–38. Doi: 10.1056/NEJMoa1406761. REFERENCE #3: Pant S, Deshmukh A, Mehta K, et al. Burden of arrhythmias in patients with Takotsubo Cardiomyopathy (Apical Ballooning Syndrome). International Journal of Cardiology [Internet] 2013;170(1):64–68. Available from: https://doi.org/10.1016/j.ijcard.2013.10.041 DISCLOSURES: No relevant relationships by Anneka Hutton, source=Web Response No relevant relationships by Gagan Neupane, source=Web Response No relevant relationships by Enoemem Okpokpo, source=Web Response No relevant relationships by Bharadwaj Satyavolu, source=Web Response No relevant relationships by Raksha Sharma, source=Web Response No relevant relationships by Tracey Topacio, source=Web Response

SUSPECTED PHEOCHROMOCYTOMA PRECIPITATING TAKOTSUBO CARDIOMYOPATHY

TOPIC: Cardiovascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Takotsubo cardiomyopathy (TCM) is characterized by transient systolic dysfunction resulting in hallmark apical ballooning/hypokinesis without the presence of coronary artery disease.1 The pathophysiology entails extreme stress, which generates a sudden and massive surge of catecholamines which then markedly deranges cardiac contractility.1 One source of the catecholamine surge is pheochromocytoma: a catecholamine secreting tumor of the chromaffin cells.2 We present the case of patient who presents with both TCM and a suspected pheochromocytoma. CASE PRESENTATION: A 56-year-old female with history of both STEMI and NSTEMI presented with chest pain, nausea, vomiting, and lightheadedness. Blood pressure was 197/119 mmHg on admission. Cardiac enzymes were normal, however ECG demonstrated new lateral ST elevations in V4-V6. TTE showed apical and anterolateral akinesis with a markedly reduced EF of 18%. Nitroglycerin infusion was initiated, and patient was transferred emergently for cardiac catheterization. Patent coronary vessels were found, no intervention was performed, and medical management was pursued. Echocardiographic and catheterization findings were consistent with TCM. Further chart review showed multiple similar prior episodes plus a prior CT scan which demonstrated a 2 cm adrenal mass. Given clinical suspicion, workup for pheochromocytoma was initiated. Plasma and spot-urine metanephrines, plus spot-urine normetanephrines and 24-hour urine metanephrines were within normal limits. Plasma normetanephrines, 24-hour urine normetanephrines, epinephrine, norepinephrine, and dopamine were elevated at 731.7 pg/mL, 1538 ug/24 hours, 83 pg/mL, 4213 pg/mL, and 1020 pg/mL respectively.The patient's blood pressure was eventually controlled with oral medications. She was initiated on goal-directed medical therapy for her newly reduced EF and discharged home with endocrinology follow-up. DISCUSSION: Detection of pheochromocytoma is elusive and largely depends on elevated catecholamines in urine or blood samples via 24-hour collection. The difficulty lies in the fact that the secretion is intermittent. Measurement of VMA in urine can be done yet despite a high specificity (95%) there is a low sensitivity (64%).3 Pheochromocytoma can result in mass catecholamine release with systemic manifestations, affecting cardiac contractility. CONCLUSIONS: Our patient presented with TCM from a suspected pheochromocytoma. This diagnosis was reached via a combination of echocardiographic imaging showing apical and anterolateral akinesis, not consistent with any single coronary distribution, a clean coronary catheterization, and several lab panels demonstrating elevated levels of catecholamines/metanephrines in both the blood and urine. REFERENCE #1: Ahmad SA, Brito D, Khalid N, Ibrahim MA. Takotsubo Cardiomyopathy. In: StatPearls. StatPearls Publishing; 2020. Accessed November 13, 2020. http://www.ncbi.nlm.nih.gov/books/NBK430798/ REFERENCE #2: Y-Hassan S. Recurrent takotsubo syndrome triggered by undiagnosed pheochromocytoma. International Journal of Cardiology. 2015;187:369-371. doi:10.1016/j.ijcard.2015.03.220 REFERENCE #3: Vora KS, Shah VR. Diagnosis of pheochromocytoma. J Anaesthesiol Clin Pharmacol. 2012;28(2):274-276. doi:10.4103/0970-9185.94931 DISCLOSURES: No relevant relationships by James Gubitosa, source=Web Response No relevant relationships by Shivam Kaushik, source=Web Response

Takotsubo Syndrome: Optimizing Care with a Multidisciplinary Approach.

Symptoms of takotsubo syndrome (TTS) include acute and transient regional systolic dysfunction of the left ventricle, as well as a variety of wall-motion abnormalities. The clinical features of TTS, including initial symptoms, cardiac biomarkers, and electrocardiogram (ECG) changes, are similar to those of acute coronary syndrome, with the exception that TTS patients typically have no obstructive coronary artery disease. TTS primarily affects elderly women, and emotional or physical stress is a common cause of the disease. Exaggerated sympathetic stimulation associated with dysfunction of the limbic system has also been reported to be related to TTS occurrence. Cancer also induces emotional and physical stress. Therefore, optimization of TTS care should involve cardiac, neurological, psychiatric, and oncological approaches. The first step in optimizing TTS care is to diagnose it by cardiac means. Multimodality imaging, including ECG, echocardiogram, angiography, ventriculography, and cardiac magnetic resonance imaging, is indispensable for diagnosis, therapy management, and the evaluation of prognosis in the acute and chronic phases of TTS. The current cardiac approach during the acute phase is primarily supportive, with the goal of preventing life-threatening complications. As central nervous system diseases frequently trigger TTS, a neurological approach is also required. Appropriate psychiatric medication may reduce the risk of TTS recurrence, as not only psychiatric disorders themselves but also psychiatric medications can be the trigger for TTS. Several conditions are associated with TTS, including the novel coronavirus disease 2019. We present current knowledge of TTS in this review and describe how to optimize TTS care through a multidisciplinary approach.

Suicidal left ventricle: a case report

: We report a case of “Takotsubo cardiomyopathy” following attempted suicidal partial hanging. Takotsubo cardiomyopathy is a syndrome characterized by transient regional systolic dysfunction of the left ventricle (LV). Takotsubo cardiomyopathy was first described in 1990 in Japan. From a registry of 3,265 patients with troponin-positive ACS a prevalence of 1.2 present was reported. Takotsubo cardiomyopathy is frequently in women and mainly in older adults. The patient presented with chest pain after the suicidal attempt, electrocardiography (ECG) showed ischemic changes in the anterior leads. Echocardiography performed, which revealed that the LV showed ballooning and hypokinesia of the apical segments, and the Troponin test was positive. Coronary angiogram considering the possibility of the acute coronary syndrome was done, which showed only minimal plaguing in the left anterior descending artery. However, the LV angiogram revealed the classical features of Takotsubo cardiomyopathy. Cardiac magnetic resonance imaging (MRI) was also performed, which also confirmed the diagnosis. After three days of hospitalization, the patient was discharged with proper Psychiatric Counseling, and during the follow-up, the LV contractility had completely recovered. Takotsubo cardiomyopathy should always be considered in patients presenting with abnormal ECG during various scenarios of physical and emotional stress.

Myocardial edema, interstitial expansion and clinical correlates in takotsubo syndrome: a cardiac magnetic resonance tissue mapping study

Abstract Funding Acknowledgements Type of funding sources: None. Background takotsubo syndrome (TTS) is an acute heart failure syndrome characterized by transient systolic dysfunction, widespread myocardial edema and not trivial rate of in-hospital complications. Tissue mapping by cardiac magnetic resonance (CMR) imaging provides measure of myocardial interstitial expansion. Few studies to date comprehensively examined native T1, T2 and extracellular volume (ECV) quantification by CMR in TTS. Purpose to describe T1 and T2 mapping findings by cardiac magnetic resonance (CMR) imaging in a cohort of TTS patients and control subjects. Methods we performed CMR imaging with native T1 and T2 mapping assessment as well as ECV and late gadolinium enhancement (LGE) imaging in n = 14 TTS patients at a median of 4 (3, 7) days after the acute event. Control group consisted of n = 14 healthy individuals with no known prior acute cardiac events. Extracellular-volume (ECV) fraction estimate was derived from native and post-contrast T1 of myocardium and blood pool corrected for hematocrit as reported in literature. All mapping measurements were performed in the interventricular septum from the mid-short-axis view. Results median age of the study population was 72 years, 84% female. Typical apical ballooning was present in 72% of the patients, atypical in 28%, with median left ventricular ejection fraction (LVEF) of 45%; mid interventricular septum was involved in all patients based on the presence of wall motion abnormalities at hospital admission. Median native T1, T2 and ECV were 1078 msec vs 965 msec, 55 msec vs 47 msec and 29% vs 25% in TTS and controls respectively (p < 0.001 for all). A significant direct correlation was found between T2 and both native T1 and ECV in TTS (r = 0.759, p = 0.002 and r = 0.630, p = 0.018 respectively) but not in controls. Moreover, in TTS patients, native T2 inversely correlated with LVEF as assessed at hospital admission (r=-0.563, p = 0.037), whereas non-significant trends were observed between admission LVEF and both native T1 and ECV. No LGE was detected neither in TTS patients nor in controls. Conclusions myocardial edema, as signified by increased T2, is a prominent feature of TTS, likely driving interstitial expansion and increase of native T1 and ECV in the acute phase. Correlation of T2 with LVEF on admission suggests that CMR-based parametric assessment of myocardial edema could contribute to better characterize disease severity in TTS.