Takotsubo-like Cardiomyopathy Research Articles

Cardiovascular Complications in Pheochromocytoma and Paraganglioma: Does Phenotype Matter?

Adrenaline-producing tumors are mostly characterized by a sudden release of catecholamines with episodic symptoms. Noradrenergic ones are usually less symptomatic and characterized by a continuous overproduction of catecholamines that are released into the bloodstream. Their effects on the cardiovascular system can thus be different. The aim of this study was to determine the prevalence of cardiovascular complications by catecholamine phenotype. We retrospectively analyzed data on the prevalence of cardiovascular events in 341 consecutive patients with pheochromocytoma and paraganglioma treated from 1995 to 2023. Biochemical catecholamine phenotype was determined based on plasma or urinary catecholamines and metanephrines. According to the phenotype, 153 patients had noradrenergic pheochromocytoma and paraganglioma and 188 had adrenergic pheochromocytoma and paraganglioma. In the whole sample, the incidence of serious cardiovascular complications was 28% (95 patients), with no difference between the phenotypes or sexes. The noradrenergic phenotype had significantly more atherosclerotic complications (composite end point of type 1 myocardial infarction and symptomatic peripheral artery disease; odds ratio, 3.58 [95% CI, 1.59-8.83]; P=0.003), while the adrenergic phenotype more often had type 2 myocardial infarction and takotsubo-like cardiomyopathy (OR, 0.24 [95% CI, 0.09-0.57]; P=0.002). These changes remained even after adjustment for conventional risk factors of atherosclerosis. We found a 28% incidence of cardiovascular complications in a consecutive group of patients with pheochromocytoma and paraganglioma. Patients presenting with a noradrenergic phenotype have a higher incidence of atherosclerotic complications, while the adrenergic phenotype is associated with a higher incidence of acute myocardial damage due to takotsubo-like cardiomyopathy.

Multi-Omics Analysis Reveals the Role of Sigma-1 Receptor in a Takotsubo-like Cardiomyopathy Model.

Takotsubo syndrome (TTS) is a stress-induced cardiomyopathy that presents with sudden onset of chest pain and dyspneic and cardiac dysfunction as a result of extreme physical or emotional stress. The sigma-1 receptor (Sigmar1) is a ligand-dependent molecular chaperone that is postulated to be involved in various processes related to cardiovascular disease. However, the role of Sigmar1 in TTS remains unresolved. In this study, we established a mouse model of TTS using wild-type and Sigmar1 knockout mice to investigate the involvement of Sigmar1 in TTS development. Our results revealed that Sigmar1 knockout exacerbated cardiac dysfunction, with a noticeable decrease in ejection fraction (EF) and fractional shortening (FS) compared to the wild-type model. In terms of the gut microbiome, we observed regulation of Firmicutes and Bacteroidetes ratios; suppression of probiotic Lactobacillus growth; and a rise in pathogenic bacterial species, such as Colidextribacter. Metabolomic and transcriptomic analyses further suggested that Sigmar1 plays a role in regulating tryptophan metabolism and several signaling pathways, including MAPK, HIF-1, calcium signaling, and apoptosis pathways, which may be crucial in TTS pathogenesis. These findings offer valuable insight into the function of Sigmar1 in TTS, and this receptor may represent a promising therapeutic target for TTS.

SAT333 A Case Of Pheochromocytoma Initially Presenting As Takotsubo-like Cardiomyopathy

Abstract Disclosure: J.S. Figueroa: None. S.W. Holland: None. J. Cheng: None. M.C. Sullivan: None. Background: Takotsubo-like cardiomyopathy (TCM) is a known but rarely seen clinical complication associated with pheochromocytoma that is important to recognize. Case Presentation: A 68 year old woman with a history of hypertension on verapamil, lisinopril-hydrochlorothiazide, clonidine, hyperlipidemia, asthma, and chronic kidney disease presents for nausea and vomiting associated with pressure like chest pain. Cardiac work up revealed elevated troponins and T wave inversion in the anterolateral leads concerning for non-ST segment elevation myocardial infarction (NSTEMI). CT Abdomen study revealed two right sided adrenal nodules 2.4 x 1.8 cm and 1.9 x1.1 cm. Coronary angiography was normal but study was concerning for TCM. She developed new onset atrial fibrillation and was treated with calcium channel blocker and beta blocker. Transthoracic echocardiography showed apical hypokinesis and a mildly decreased ejection fraction of 41%. She was discharged home on beta blocker and losartan therapy. The patient presented 5 days later with similar symptoms and a maximum elevated blood pressure 170/110 mm Hg. Labs revealed elevated plasma free metanephrines level of 505 pg/ml (ref range ≤57 pg/ml) with normal renin, aldosterone, and cortisol levels. CT adrenal protocol study showing pre-contrast density of largest nodule 39 Hounsfield units (HU), postcontrast density 81 HU, and washout density 56 HU with washout at 59.5%. The smaller nodule had pre-contrast density of 31 HU, postcontrast of 65 HU, washout density of 61 HU with washout at 11.8%. Endocrine and Endocrine surgery were consulted at this time. Alpha blocker tamsulosin was initiated and beta blocker therapy was discontinued. Patient underwent right adrenalectomy with subsequent improvement of her ejection fraction two months later. Histology was concerning for malignancy for which she is currently being followed. Conclusion: This case highlights TCM as a presenting sign for a pheochromocytoma. A study by Agarwal et al. found that a minority of patients who had TCM due to pheochromocytoma developed symptoms typical of the tumor such as headache, hypertension, palpitations, and diaphoresis. The study also found that complications such as cardiogenic shock and heart failure were higher in pheochromocytoma related TCM.¹ Consider catecholamine producing tumors as a potential cause of TCM where appropriate alpha and beta blockade may help to reduce symptoms. Earlier recognition of a potential pheochromocytoma would also have discouraged the use of a beta blocker initially until pheochromocytoma was ruled out. An individualized, multidisciplinary approach helps lead to a timely diagnosis and appropriate treatment decisions for these rare tumors. References (1) Agarwal, V.,Kant, G.,Hans N., Messerli F.H. Takotsubo-like Cardiomyopathy in Pheochromocytoma. International Journal of Cardiology 2011; Vol 153,3,241-248 Presentation: Saturday, June 17, 2023

Pomegranate Peel Extract Attenuates Isoprenaline-Induced Takotsubo-like Myocardial Injury in Rats.

Takotsubo syndrome (TTS) is an acute heart failure syndrome characterised by catecholamine-induced oxidative tissue damage. Punica granatum, a fruit-bearing tree, is known to have high polyphenolic content and has been proven to be a potent antioxidant. This study aimed to investigate the effects of pomegranate peel extract (PoPEx) pre-treatment on isoprenaline-induced takotsubo-like myocardial injury in rats. Male Wistar rats were randomised into four groups. Animals in the PoPEx(P) and PoPEx + isoprenaline group (P + I) were pre-treated for 7 days with 100 mg/kg/day of PoPEx. On the sixth and the seventh day, TTS-like syndrome was induced in rats from the isoprenaline(I) and P + I groups by administering 85 mg/kg/day of isoprenaline. PoPEx pre-treatment led to the elevation of superoxide dismutase and catalase (p < 0.05), reduced glutathione (p < 0.001) levels, decreased the thiobarbituric acid reactive substances (p < 0.001), H2O2, O2- (p < 0.05), and NO2- (p < 0.001), in the P + I group, when compared to the I group. In addition, a significant reduction in the levels of cardiac damage markers, as well as a reduction in the extent of cardiac damage, was found. In conclusion, PoPEx pre-treatment significantly attenuated the isoprenaline-induced myocardial damage, primarily via the preservation of endogenous antioxidant capacity in the rat model of takotsubo-like cardiomyopathy.

Perioperative outcomes of pheochromocytoma/paraganglioma surgery preceded by Takotsubo-like cardiomyopathy

BackgroundPheochromocytomas and paragangliomas can induce severe cardiovascular manifestations such as Takotsubo-like cardiomyopathy. What the perioperative outcomes are of patients presenting with pheochromocytomas/paragangliomas preceded by Takotsubo-like cardiomyopathy remains an unresolved question. MethodsFrom 2006 to 2019, all patients who underwent surgery for pheochromocytomas/paragangliomas preceded by Takotsubo-like cardiomyopathy were included from 3 high-volume centers, with specific attention to perioperative hemodynamic instability and postoperative outcomes. ResultsOverall, 37 patients were included, with a median age of 45 years. Patients were operated on 2 months (1–4) after a Takotsubo-like cardiomyopathy episode; 33 (89%) had a laparoscopic approach. All those who underwent surgery presented in a hemodynamically stable situation. All except 1 of the pheochromocytomas/paragangliomas patients had at least 1 antihypertensive treatment at the time of surgery. The median preoperative systolic blood pressure in the Takotsubo-like cardiomyopathy group was 120 mm Hg (95–132). Overall, 27/34 (79%) of patients required vasoactive drugs during surgery with nicardipine (n = 22), esmolol (n = 12), and/or norepinephrine (n = 8). No patient presented a catecholamine-induced life-threatening complication such as hypertensive crisis, cardiac arrhythmias, pulmonary edema, cardiac ischemia, or Takotsubo-like cardiomyopathy in the perioperative period. Severe morbi-mortality was nil. The systematic review identified 5 studies including 38 pheochromocytomas/paragangliomas patients with at least 1 episode of acute heart failure considered as Takotsubo-like cardiomyopathy before surgery, of which 28 patients had delayed surgery with 1 postoperative death. ConclusionHemodynamically stabilized patients with pheochromocytomas/paragangliomas preceded by Takotsubo-like cardiomyopathy can be safely scheduled for an elective pheochromocytomas/paragangliomas surgery, with similar intra and postoperative outcomes as those without Takotsubo-like cardiomyopathy.

Cardiac macrophages regulate isoproterenol-induced Takotsubo-like cardiomyopathy.

Takotsubo syndrome (TTS) is an acute, stress-induced cardiomyopathy that occurs predominantly in women after extreme physical and/or emotional stress. To date, our understanding of the molecular basis for TTS remains unknown and, consequently, specific therapies are lacking. Myocardial infiltration of monocytes and macrophages in TTS has been documented in clinical studies. However, the functional importance of these findings remains poorly understood. Here, we show that a single high dose of isoproterenol (ISO) in mice induced a TTS-like cardiomyopathy phenotype characterized by female predominance, severe cardiac dysfunction, and robust myocardial infiltration of macrophages. Single-cell RNA-Seq studies of myocardial immune cells revealed that TTS-like cardiomyopathy is associated with complex activation of innate and adaptive immune cells in the heart, and macrophages were identified as the dominant immune cells. Global macrophage depletion (via clodronate liposome administration) or blockade of macrophage infiltration (via a CCR2 antagonist or in CCR2-KO mice) resulted in recovery of cardiac dysfunction in ISO-challenged mice. In addition, damping myeloid cell activation by HIF1α deficiency or exposure to the immunomodulatory agent bortezomib ameliorated ISO-induced cardiac dysfunction. Collectively, our findings identify macrophages as a critical regulator of TTS pathogenesis that can be targeted for therapeutic gain.

Surgical adrenalectomy during rescue Extracorporeal Life Support for pheochromocytoma induced cardiogenic shock: a case report

Introduction : Pheochromocytoma, a rare catecholamine-producing tumor, has been described to provoke stress-induced Takotsubo-like cardiomyopathy and even severe refractory cardiogenic shock. In this case report, venoarterial extracorporeal membrane oxygenation (VA-ECMO) was used for hemodynamic stabilization and was continued during the resection of a large neuroendocrine tumor. Description : A 69-year old male, recently diagnosed with a pheochromocytoma, was referred to our center because of severe cardiogenic shock after induction of anesthesia for resection of the mass. Despite adequate alpha-and beta-adrenergic blockade for one month, he developed malignant hypertension with subsequently hemodynamic collapse. After successful cardiopulmonary resusci-tation he developed pulmonary oedema with severe hypoxemia and persistent hemodynamic lability. On arrival in our hospital, echocardiography revealed significant left ventricular impairment. Decision was made to commence him on VA-ECMO for a refractory cardiogenic shock with severe pulmonary oedema. Because of persistent blood pressure swings despite VA-ECMO and beta-adrenergic blockade, we decided to remove the tumor on mechanical circulatory support by an open surgical approach the next day. After clamping of the adrenal circulation, the patient experienced profound hypotension requiring high doses of epinephrine and a methylene blue infusion. Surgical resection was successful but complicated by a postoperative bleeding for which the patient underwent an emergent endovascular embolization of an adrenal artery. Over the next days, there was progressive cardiac recovery and the patient was weaned off VA-ECMO on the fourth postoperative day. The patient was discharged from the intensive care 27 days after admission and left the hospital on day 30. Discussion: VA-ECMO seems to be a feasible last resort therapy in refractory cardiogenic shock induced by pheochromocytoma crisis as suggested by a high survival rate in literature. Despite its support benefit during this type of shock, surgical removal of the tumor is rarely done with mechanical circulatory support and heparinization.

Acute cardiac complications and subclinical myocardial injuries associated with pheochromocytoma and paraganglioma

BackgroundCatecholamine excess arising from pheochromocytomas and paragangliomas (PPGLs) can cause a wide spectrum of cardiac manifestations, including acute cardiac complications (ACCs) and subclinical myocardial injuries (SMIs). In this study, we aimed to conduct a comprehensive analysis of ACCs and SMIs in a large cohort of patients with PPGLs.MethodsWe retrospectively analyzed the clinical data of consecutive patients with PPGLs admitted between January 2013 and July 2020 (n = 189). The prevalence of ACCs and SMIs and characteristics of patients identified with ACCs and SMIs were investigated. Moreover, comparisons were performed between patients with and without ACCs.ResultsFourteen patients (7.4%) fulfilled the criteria for ACCs, including nine (4.8%) who presented with Takotsubo-like cardiomyopathy, four (2.1%) with heart failure with preserved ejection fraction, and finally one (0.5%) with catecholamine-induced cardiomyopathy. Compared to those without ACCs (n = 175), patients with ACCs had a higher prevalence of epinephrine-producing PPGLs (81.8% vs 33.9%, P = 0.006) and were more likely to show invasive behavior (61.5% vs 27.3%, P = 0.022) or hemorrhage/necrosis (53.9% vs 17.4%, P = 0.005) on histology. The apical sparing pattern (5/7, 71.4%) was the dominant impairment pattern of longitudinal strain (LS) for patients displaying Takotsubo-like cardiomyopathy. In patients without cardiac symptoms, a fairly high proportion (21/77, 27.3%) of patients who underwent screening for troponin and/or natriuretic peptide and/or echocardiography had SMIs.ConclusionsOne in every fourteen PPGL patients presented with ACCs, and in the patients with Takotsubo-like cardiomyopathy, the apical sparing pattern was the primary impairment pattern of LS. Additionally, nearly one-third of patients without symptoms had SMIs. The diagnosis of PPGLs should be considered in patients with acute reversible cardiomyopathy, especially in those exhibiting an apical sparing pattern of LS.

Mechanisms and Management of Immune Checkpoint Inhibitor-Related Cardiac Adverse Events.

Onco-cardiology recently emerged as a novel discipline to provide effective cardioprotective care against cancer therapeutics-related cardiac adverse events (CAEs) and support the continuity of optimal cancer treatment. Immune checkpoint inhibitors (ICIs) have revolutionized cancer therapy and dramatically improved outcomes in patients with advanced or refractory cancers. However, ICIs intrinsically stimulate systemic immune responses and can potentially induce a spectrum of immune-related adverse events (irAEs), which can affect any organs of the body. The manifestation of cardiac irAEs includes myocarditis, arrhythmias and conduction abnormalities, and pericardial diseases. Takotsubo-like cardiomyopathy is also included as a manifestation of ICI-related CAEs, but the pathophysiological relevance is unclear. Although the incidence is rare, ICI-related CAEs are life-threatening and potentially fatal. Elucidating pathophysiology and establishing management measures of ICI-related CAEs are one of the most urgent challenges in the field of onco-cardiology.

SAT-225 A Rare Cause of Cardiogenic Shock

Background: Rare cases of pheochromocytoma are associated with stress induced Takotsubo cardiomyopathy, occurring in approximately 3% of secreting pheoochromocytoma and paraganglioma (PPGL). Case: A 39-year-old female with a history of hypertension and anxiety disorder, re-presented to the ED within 24 hours with headache, vomiting and severe hypertension (BP 205/70 mmHg). She rapidly developed acute pulmonary oedema and cardiogenic shock. ECG revealed dynamic ST-segment abnormalities and troponin rise peaked at 38,000 ng/L (N < 16ng/L). The coronary angiography excluded any coronary artery lesion. Echocardiography revealed severe global hypokinesis with an ejection fraction of 5% and evidence of apical ballooning. A bedside abdominal ultrasound revealed 7cm left adrenal mass confirmed by computed tomography (CT). Investigations revealed a metanephrine level of > 13,760 pmol/L (N < 900 pmol/L) and normetanephrine of > 60,780 pmol/L (N < 500 pmol/L). Her clinical status deteriorated despite on diuretics, inotropes and mechanical ventilation. Extra-corporeal membrane oxygenation (ECMO) was implanted. Alpha adrenergic blockades were initiated followed by beta blockers, during which control of blood pressure was achieved. Her haemodynamic status improved and ECMO removed 8 days post-implantation. Two weeks after discharge from hospital, she underwent uncomplicated open adrenalectomy; histologic examination of the mass confirmed the pheochromocytoma diagnosis. Reference 1. Gagnon N, Mansour S, Bitton Y et al. Takotsubo-like cardiomyopathy in a large cohort of patients with pheoochromocytoma and paraganglioma. Endocrine Practice.2017;10: 1178-1192

Beneficial Effect of TMAO in Spontaneously Hypertensive Heart Failure Rats is Associated with Diuretic and Hypotensive Actions.

There is an ongoing debate weather trimethylamine‐oxide (TMAO), a molecule present in seafood and a derivate of microbiota metabolism, is beneficial or harmful for the circulatory system. Numerous biophysical studies show that TMAO protects proteins exposed to osmotic and hydrostatic stress (HS). We hypothesized that TMAO may benefit the circulatory system by protecting cardiac proteins exposed to HS produced by the contracting heart.Male, 6‐week‐old, Sprague‐Dawley (SD, n=40) and Spontaneously‐Hypertensive‐Heart‐Failure (SHHF, n=18) rats were randomly divided into either control (Water) or TMAO treatment. After 50 weeks, half of SD were given Isoprenaline (s.c.) to increase arterial blood pressure (BP) and produce a mild, Takotsubo‐like cardiomyopathy (ISO). In vitro, cardiac lactic dehydrogenase (LDH) with or without TMAO was exposed to HS (changes in hydrostatic pressure similar, and higher than those present in the heart) for up to 24hrs and was evaluated using fluorescence correlation spectroscopy.After 58 weeks of the treatment survival was 100% in SD‐Water, SD‐TMAO, ISO‐TMAO and 90% in ISO‐WATER. In SHHF‐Water survival was 66% vs 100% in SHHF‐TMAO (p=0.065). In general, TMAO‐treated rats showed higher natriuresis and diuresis. In comparison to SHHF‐Water, SHHF‐TMAO showed significantly lower plasma NT‐proBNP, lower diastolic BP, lower liver angiotensinogen expression and a trend towards lower cardiac fibrosis. In vitro, exposure of LDH to HS with or without TMAO did not affect the protein structure. TMAO does not exert negative effects. In contrast, TMAO reduces mortality in HF rats that is associated with natriuretic, diuretic and hypotensive effects. HS produced by the contracting heart is neutral for cardiac LDH protein structure.Support or Funding InformationSupported by National Science Centre, Poland grant no 2018/31/B/NZ5/00038.

Endovascular Therapy for Aneurysmal Subarachnoid Hemorrhage Complicated by Neurogenic Pulmonary Edema and Takotsubo-Like Cardiomyopathy: A Report of Ten Cases.

Objective:Patients sustaining aneurysmal subarachnoid hemorrhage (aSAH) can be further complicated by neurogenic pulmonary edema (NPE) and Takotsubo-like cardiomyopathy (TCM) with dismal outcomes. The present study aimed to validate the efficacy of endovascular therapy for patients with aSAH complicated by NPE and TCM.Materials and Methods:Patients who were diagnosed with aSAH complicated by NPE and TCM and treated by endovascular therapy were retrospectively evaluated.Results:In the past 5 years, a total of ten female patients with aSAH were also diagnosed with NPE and TCM. Six of the ten were cases with high-grade aSAH (Hunt and Hess Grades IV and V), whereas four were low-grade aSAH (Grades II and III). The locations of ruptured aneurysms were the internal carotid-posterior communicating artery junctional site in five patients, the anterior communicating artery in two, the vertebral artery in two, and the middle cerebral artery in one. These aneurysms were successfully embolized by endovascular therapy without any procedure-associated complications. The clinical outcome measure at 6 months after discharge on the Modified Rankin Scale was found to be 0 in four patients, 1 in two, 3 in one, and 5 in three.Conclusions:Endovascular therapy can be a feasible, alternative measure for the treatment of patients with high-grade aSAH who also have NPE and TCM.

Management of spontaneous coronary artery dissection complicated by cardiogenic shock using mechanical circulatory support with the Impella device.

The aim is to evaluate the feasibility of using mechanical circulatory support, specifically the Impella device, in spontaneous coronary artery dissection (SCAD) patients with cardiogenic shock. The therapeutic options for managing SCAD complicated by cardiogenic shock are limited. Risky revascularization procedures are often necessary. This was a multicenter case series in the United States. Approximately 20 cases of Impella implantation in patients with SCAD are known. The implanting physician for each of these cases was contacted and de-identified records were requested. The records were analyzed for Impella indications, outcomes, and complications. Records from four cases were received. All patients survived to hospital discharge and no major complications were observed. In two cases, cardiogenic shock developed in the absence of ongoing ischemia, suggesting a Takotsubo-like cardiomyopathy. In these cases, the Impella provided hemodynamic support until the patient's cardiac function recovered. Although a small case series, given the scarcity of SCAD cases complicated by cardiogenic shock and the limited therapies available to treat these patients, these data are of clinical value in highlighting the feasibility of Impella use in SCAD. The Impella can be valuable for procedural support and in cardiogenic shock, especially in cases without evidence of ongoing ischemia.

Characterization of the Myocardial Inflammatory Response in Acute Stress-Induced (Takotsubo) Cardiomyopathy

Takotsubo cardiomyopathy is an acute stress-induced heart failure syndrome for which the exact pathogenic mechanisms are unclear, and consequently, no specific treatment exists. In an experimental model of stress-induced takotsubo-like cardiomyopathy, the authors describe the temporal course of a chronic inflammatory response post-induction, with an initial early influx of neutrophils into myocardial tissue followed by macrophages that are typical of a proinflammatory M1 phenotype, and a nonsignificant increase in systemic inflammatory cytokines. Post-mortem myocardium from the more complex clinical takotsubo patients share features of the study's experimental model. These findings suggest modulators of inflammation could be apotential therapeutic option.

Mechanical circulatory devices in acute heart failure.

Temporary circulatory support (TCS) with short-term mechanical circulatory support (MCS) devices is increasingly used as a salvage therapy for patients with refractory cardiogenic shock. This article provides an overview of current devices, their indications and management, and discusses results of recent case series and trials. Percutaneous active MCS devices (Impella, TandemHeart…) and venoarterial extracorporeal membrane oxygenation (VA-ECMO) are utilized as a bridge to 'decision' that includes weaning after cardiac function recovery, transplantation, long-term MCS and withdrawal in case of futility. VA-ECMO is considered the first-line TCS since it allows rapid improvement in oxygenation, is less expensive, and is also suitable for patients with biventricular failure. Combining Impella or intra-aortic balloon pump support with ECMO might decrease left ventricular pressure and improve outcomes. Sepsis-associated cardiomyopathy, massive pulmonary embolism, arrhythmic storm and Takotsubo-like cardiomyopathy are among emerging indications for TCS. TCS have become the cornerstone of the management of patients with cardiogenic shock, although the evidence supporting their efficacy is limited. VA-ECMO is considered the first-line option, with a growing number of accepted and emerging indications. Randomized clinical trials are now needed to determine the respective place of different MCS devices in cardiogenic shock treatment strategies.

Severe pheochromocytoma crisis, Type B, resulting in Takotsubo-like cardiomyopathy and fulminant refractory cardiogenic shock, successfully treated with extracorporeal membrane oxygenation (ECMO) but with fatal neurological sequelae for the patient.

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Abstract #112 Careful with That Needle! Takotsubolike Cardiomyopathy Presenting with Apparent Stemi Following Biopsy of a Pheochromocytoma

Abstract #112 Careful with That Needle! Takotsubolike Cardiomyopathy Presenting with Apparent Stemi Following Biopsy of a Pheochromocytoma

Frequency of Inverted Electrocardiographic T Waves (Cerebral T Waves) in Patients With Acute Strokes and Their Relation to Left Ventricular Wall Motion Abnormalities

Transient, symmetric, and deep inverted electrocardiogram (ECG) T waves in the setting of stroke, commonly referred to as cerebral T waves, are rare, and the underlying mechanism is unclear. Our study aimed to test the hypothesis that cerebral T waves are associated with transient cardiac dysfunction. This retrospective study included 800 patients admitted with the primary diagnosis of hemorrhagic or ischemic stroke. ECGs were examined for cerebral T waves, defined as T-wave inversion of ≥5 mm depth in ≥4 contiguous precordial leads. Echocardiograms of those meeting these criteria were examined for the presence of left ventricular (LV) wall motion abnormalities. Follow-up evaluation included both ECG and echocardiogram. Of the 800 patients, 17 had cerebral T waves on ECG (2.1%). All 17 patients had ischemic strokes, of which 11 were in the middle cerebral artery distribution (65%), and 2 were cerebellar (12%), whereas the remaining 4 involved other locations. Follow-up ECG showed resolution of the T-wave changes in all 17 patients. Of these patients, 14 (82%) had normal wall motion, and 3 had transient wall motion abnormalities (18%). Two of these patients had Takotsubo-like cardiomyopathy with apical ballooning, and the third had globally reduced LV function. Coronary angiography showed no significant disease to explain the LV dysfunction. In summary, in our cohort of patients with acute stroke, cerebral T waves were rare and occurred only in ischemic stroke. Eighteen percent of patients with cerebral T waves had significant transient wall motion abnormalities. Patients with stroke with cerebral T waves, especially in those with ischemic strokes, should be assessed for cardiac dysfunction.

Takotsubo-like Cardiomyopathy in a Large Cohort of Patients with Pheochromocytoma and Paraganglioma

Pheochromocytoma (PHEO) and paraganglioma (PGL) (PPGL) may cause acute Takotsubo-like catecholamine cardiomyopathy (TLC). The objective of this study was to determine the prevalence and clinical presentation of TLC in a large cohort of patients with PPGL. We reviewed retrospectively the records of consecutive patients with PPGL investigated in our center from 1995 to 2016. We collected clinical and paraclinical data of patients that had TLC in this cohort. We performed a literature review of cases of Takotsubo cardiomyopathy related to PPGL described between 1990 and 2015. Our cohort included 275 patients with PPGL. Acute TLC was found in 4 of 152 (2.6%) patients with secreting PPGL. There was no event recorded in 123 patients with unknown presurgical secretion (n = 51) or nonsecreting PPGL (n = 72). Four patients (44 to 79 years old) fulfilled the criteria for TLC, including 2 PHEO and 2 PGL patients. A precipitating stressor event was identified in 3 cases including surgery (n = 2) and upper respiratory tract infection. In all cases, the diagnosis of PPGL came after the cardiac event and following the investigation of a lesion incidentally found at imaging. Moreover, we identified in the literature 59 cases described in the last 25 years and analyzed this cohort together with our 4 new cases. Acute TLC may be found in up to 3% of patients with secreting PPGL. Considering that the diagnosis of PPGL was performed following incidental finding of radiologic mass, the real prevalence of PPGL in TTC remains to be determined. ECG = electrocardiogram; LVEF = left ventricular ejection fraction; MIBG = metaiodobenzylguanidine; PGL = paraganglioma; PHEO = pheochromocytoma; PPGL = pheochromocytoma and paraganglioma; TLC = Takotsubo-like cardiomyopathy; TTC = Takotsubo cardiomyopathy; ULN = upper limit of normal.

Acute chest pain in an anxious woman

A 63 years old woman with past medical history of hypertension, osteoporosis, dyslipidaemia and anxiety, was admitted in the emergency room with acute chest pain after a family discussion, she presented oppressive retrosternal pain 7/10 of intensity, that irradiated to the left shoulder and the left arm, she was diaphoretic, her vital signs were normal and the physical examination was unremarkable, there were no abnormalities in the electrocardiogram and the blood tests showed normal haemogram as well the renal, liver and thyroid function, the troponin I was 6.9 μg/ml (normal value 0,04 μg/ml), a diagnosis of Non-ST Segment Elevation Myocardial Infarction (NSTEMI) was admitted, and an angiography was scheduled. In the meanwhile the chest pain relieved with nitroglycerine administration and anti-ischaemic treatment and the patient stayed haemodynamically stable. What is the diagnosis? Fig. 1 shows the ventriculography pattern of this patient that is typically known as Tako tsubo disease (TTD) or apical ballooning with a static apex and compensatory hipercontractility of the basal segments, without coronary abnormalities. The emotional stress has been related to this entity, causing a coronary vasospasm and the subsequent myocardial ischemia, due to these physiopathological facts, the clinical symptoms and laboratory findings of this illness look like the acute coronary syndromes. Some author had proposed the association between neoplasic diseases and TTD, mainly in patients that presented this condition without any stressing stimuli, they suggest that the catecholamines liberation by some tumours may be involved in the pathogenesis of those cases [[1]Sattler K. El-Battrawy I. Lang S. Zhou X. Schramm K. Tülümen E. et al.Prevalence of cancer in Takotsubo cardiomyopathy: short and long-term outcome.Int. J. Cardiol. 2017; https://doi.org/10.1016/j.ijcard.2017.02.093Abstract Full Text Full Text PDF Scopus (36) Google Scholar]. The prognosis of this condition is generally benign, although it may be complicated with malignant arrhythmias, cardiac rupture and cardiac tamponade [[1]Sattler K. El-Battrawy I. Lang S. Zhou X. Schramm K. Tülümen E. et al.Prevalence of cancer in Takotsubo cardiomyopathy: short and long-term outcome.Int. J. Cardiol. 2017; https://doi.org/10.1016/j.ijcard.2017.02.093Abstract Full Text Full Text PDF Scopus (36) Google Scholar]. Although the clinical and radiologic findings are typical, this illness only can be confirmed with after the myocardial function normalization in the follow up [[2]Giusca Sorin Eisele Tom Nunninger Peter Benedikt Münz G.K. Aborted sudden cardiac death in a female patient presenting with Takotsubo-like cardiomyopathy due to epicardial coronary vasospasm.Case Reports Cardiol. 2017; https://doi.org/10.1155/2017/7875240Crossref PubMed Google Scholar]. In this case the patient had completely recovered the cardiac function after 3 months of follow up under medical treatment with beta-blockers, ACE inhibitors as suggested in the literature [[2]Giusca Sorin Eisele Tom Nunninger Peter Benedikt Münz G.K. Aborted sudden cardiac death in a female patient presenting with Takotsubo-like cardiomyopathy due to epicardial coronary vasospasm.Case Reports Cardiol. 2017; https://doi.org/10.1155/2017/7875240Crossref PubMed Google Scholar]. Despite the available evidence many doubts remain about the appropriated follow up and prognosis of these patients [[3]Drakopoulou M.I. Gatzoulis M.A. Is Takotsubo syndrome a benign condition?.Hell. J. Cardiol. 2017; https://doi.org/10.1016/j.hjc.2017.03.009Crossref PubMed Scopus (2) Google Scholar]. The authors want to highlight this so called “benign” entity that can lead to fatal complications.