Biventricular Takotsubo syndrome in a patient with coronary abnormality and end-stage renal disease

The Takotsubo syndrome is a clinical entity consisting of transient, regional left ventricular (LV) contraction abnormalities in the absence of significant epicardial coronary artery disease. Patients usually present with acute hemodynamic deterioration following an emotional or physical stress. The Takotsubo syndrome is uncommon among patients with end-stage renal disease (ESRD) and patients with congenital coronary abnormalities, such as right coronary artery (RCA) originating from left sinus of Valsalva. Here we describe a patient presenting with acute respiratory distress and anterolateral ST-T segment changes, with negative troponin-I and elevated levels of brain natriuretic peptide. Coronary angiography showed a right coronary artery arising from the left sinus of Valsalva without obstructive coronary artery disease, while ventriculography and echocardiogram showed findings compatible with apical ballooning. Magnetic resonance imaging confirmed the diagnosis of a biventricular Takotsubo cardiomyopathy.     

Platelet hyperaggregability across the coronary bed in response to rapid atrial pacing in patients with stable coronary artery disease.

BACKGROUND. Platelet aggregation is believed to contribute to the precipitation of acute ischemic syndromes. Because physical activity has been proposed as one possible trigger in converting a patient with chronic coronary artery disease to one with an acute ischemic syndrome, we examined the hypothesis that platelets become activated when coronary blood flow velocities (and thereby shear stress) increase across an atherosclerotic bed. METHODS AND RESULTS. During catheterization, 82 patients (36 with left coronary artery disease, 12 with only right coronary artery disease, and 34 with normal coronary arteries) had measurement of whole blood platelet aggregation performed on blood samples obtained simultaneously from the coronary sinus and aorta at rest, 2 minutes after onset of rapid atrial pacing, and 10 minutes after pacing was terminated. There was no arteriovenous difference in platelet aggregation under resting conditions in patients with versus those without coronary artery disease. Atrial pacing in patients with left coronary artery disease (greater than or equal to 50% stenosis in a major epicardial vessel) caused an increase in platelet aggregation in the coronary sinus blood (+64 +/- 9%, p less than 0.01) but not in arterial blood (2 +/- 8% decrease, p = NS). This increase was transient and returned nearly to baseline 10 minutes after termination of pacing. Patients with nonsignificant left coronary artery disease, those with normal coronary arteries, and patients with significant disease only in the right coronary artery (venous drainage not into the coronary sinus) did not show any changes in either the coronary sinus or arterial blood with atrial pacing. CONCLUSIONS. There is no evidence of platelet activation across a normal or an atherosclerotic coronary bed at rest. When coronary blood flow increases in the presence of significant (greater than or equal to 50%) narrowing of epicardial coronary arteries, however, platelets are activated and aggregate more easily. This mechanism may play a role in the precipitation of acute ischemic syndromes in patients with coronary artery disease.     

Spontaneous coronary artery dissection and takotsubo syndrome: An often overlooked association; review

Spontaneous coronary artery dissection (SCAD) and takotsubo syndrome (TS) are two cardiovascular syndromes with predilection for women. Both conditions may be preceded by an emotional stress or, for the affected individual, an unusual severe physical exercise. “Restitution ad integrum” occurs in most cases suffering from SCAD or TS with complete angiographic resolution of the dissected vessel and left ventricular dysfunction respectively. Recently, many cases, which were initially diagnosed as TS because of typical left ventricular ballooning pattern showed to have SCAD, have been reported; these cases were deemed to be “SCAD misdiagnosed as TS”. The left ventricular wall motion abnormality has been attributed to the ischemia caused by SCAD-affected coronary vessel especially in the left anterior descending artery (LAD) with “wrap-around course”. However, the left ventricular ballooning pattern have occurred in patients with SCAD in non-long-wrap-around LAD and SCAD in other coronary branches where coronary ischemia on its own cannot explain the left ventricular ballooning. In this review, sufficient data supporting the evidence for the possibility of coexistence of SCAD and TS is provided. Misdiagnosis of the association of the two conditions may result in mismanagement of the patient with undesirable consequences. Furthermore, the causal links between SCAD and TS is discussed.     

Fibromuscular dysplasia of the coronary and renal arteries?

Fibromuscular dysplasia (FMD) involving the coronary arteries has been described pathologically as a cause of myocardial infarction but has not been described antemortem. Unlike renal artery FMD, its clinical manifestations remain poorly characterized. We describe demographic, clinical, and coronary angiographic characteristics of seven women with acute coronary syndromes and unusual coronary anatomy who also had renal artery FMD. All subjects were female caucasians, age 42-56, who presented with prolonged chest pain and positive troponin tests. Two were smokers, two had hypertension, and one had hypercholesterolemia. None was diabetic. There were distinctive angiographic features common to all seven patients. The left anterior descending artery was involved in six, the right posterior descending artery in one. In each case, the proximal vessel appeared normal but in the middle or distal segment there was a well-demarcated abrupt transition to diffuse obliterative disease. In six of the cases, this continued distally for the remainder of the epicardial vessel. In no case was revascularization feasible. Unlike severe diffuse atherosclerotic disease, all other coronary segments were angiographically normal. Ventricular dysfunction, if present, was mild. All seven patients had typical angiographic features of renal FMD, three bilaterally. We have observed a characteristic pattern of well-demarcated obliterative coronary artery disease associated with FMD of the renal arteries. All cases presented as acute coronary syndromes in patients at relatively low risk of coronary artery disease. We propose that these appearances in the epicardial arteries, previously undescribed ante-mortem represent coronary artery fibromuscular dysplasia.     

The effects of ventricular asynchrony on myocardial perfusion

Asynchronous depolarization and contraction sequence, secondary to intraventricular conduction defects or to permanent right ventricular apical pacing, is associated with adverse effects that may be clinically evident in the failing heart. Experimental and clinical studies have suggested that asynchronous ventricular contraction deteriorates left ventricular performance and induces unfavourable left ventricular remodelling. Although such contraction does not appear to affect resting coronary artery blood flow, it increases endomyocardial pressure during diastole and decreases regional myocardial perfusion in the interventricular septum. The magnitude of these effects may correlate with the duration of the asynchrony. Despite these detrimental effects, there is no evidence that ventricular asynchrony reduces collateral myocardial blood flow, myocardial oxygen consumption or cardiac efficiency, neither in patients with normal coronary arteries, nor in patients with coronary artery disease. Furthermore, in patients with acute ischaemic syndromes, ventricular asynchrony exerts a neutral effect on the ischaemic myocardium. Cardiac resynchronization therapy improves left ventricular systolic and diastolic function without an increase in myocardial oxygen consumption or energy cost. This therapy may decrease the inhomogeneity in regional oxidative metabolism, myocardial perfusion and cardiac efficiency. Further experimental and clinical studies are needed on this area.     

Coronary Microvascular Disease in Hypertensives

Arterial hypertension can badly affect coronary circulation through several mechanisms that are not mutually exclusive, namely, coronary artery disease, left ventricular hypertrophy, and microvascular disease. Theoretical and experimental data suggest that coronary microvascular disease may exist in hypertensives, in whom it can cause both a reduction of coronary flow reserve and a shift to the right of the coronary flow autoregulation curve. To address this issue, we used dipyridamole- echocardiography test, which causes ischemic-like ST segment depression with no detectable changes in left ventricular function in different subsets of patients with microvascular disease (Syndrome X; Hypertrophic cardiomyopathy; acute heart rejection). We found that dipyridamole infusion can cause a similar pattern of response (i.e., echocardiographically silent ST segment depression) in mild - moderate essential hypertensives with normal epicardial coronary arteries, without left ventricular hypertrophy, with increased forearm minimal vascular resistances and with a reduced coronary reserve. This pattern of response identifies     

Acute coronary syndrome or apical ballooning syndrome?

Apical ballooning syndrome (ABS) is uniquely characterized by the acute onset of transient extensive kinesis of the apical and mid portions of the left ventricle without significant epicardial coronary artery stenosis, accompanied by chest symptoms and electrocardiogram changes similar to those of acute coronary syndrome. We report a case of ABS with severe coronary artery stenosis presenting as acute coronary syndrome after emotional stress. ABS should be considered a cause of left ventricular wall motion abnormalities even if a coronary arteriogram shows severe coronary artery stenosis.     

Acute myocardial infarction with diminutive right coronary artery and obstructive hypertrophic cardiomyopathy without significant coronary stenoses

Focal tissue abnormalities consistent with regional ischemia have been reported in patients with hypertrophic cardiomyopathy (HCM). Coronary microvascular dysfunction has been also reported to be present in patients with HCM despite normal epicardial coronary arteries. Moreover, it has been demonstrated that in the case of HCM and idiopathic left ventricular hypertrophy, hypoplastic coronary arteries as diminutive vessels are present and that obstructive hypertrophic cardiomyopathy is associated with enhanced thrombin generation and platelet activation. Previously, it has been described an acute myocardial infarction in a young athlete with non-obstructive hypertrophic cardiomyopathy and normal coronary arteries. We present a case of an acute myocardial infarction with diminutive right coronary artery and obstructive hypertrophic cardiomyopathy without significant coronary stenoses. To our knowledge, this is the first report of an acute myocardial infarction with diminutive right coronary artery and obstructive hypertrophic cardiomyopathy without significant coronary stenoses.     

Left main coronary artery occlusion with preserved left ventricular function: a report of three cases

Three patients with angina pectoris are reported in whom occlusion of the left main coronary artery was found at coronary arteriography. In these three patients left ventricular function was well preserved. In all three the right coronary artery was dominant and there were prominent epicardial and septal collateral vessels to the territories normally supplied by the left anterior descending and circumflex arteries. It seems reasonable to suggest that this coronary artery anatomy and collateral vessel formation accounted for the preservation of the left ventricular myocardium in these patients.     

Left main coronary artery occlusion with preserved left ventricular function: a report of three cases.

Three patients with angina pectoris are reported in whom occlusion of the left main coronary artery was found at coronary arteriography. In these three patients left ventricular function was well preserved. In all three the right coronary artery was dominant and there were prominent epicardial and septal collateral vessels to the territories normally supplied by the left anterior descending and circumflex arteries. It seems reasonable to suggest that this coronary artery anatomy and collateral vessel formation accounted for the preservation of the left ventricular myocardium in these patients.     

Transient left ventricular dysfunction under severe stress: brain-heart relationship revisited

Purpose

Transient left ventricular dysfunction in patients under emotional or physical stress, also known as tako-tsubo-like left ventricular dysfunction, has been recently been recognized as a distinct clinical entity. The aims of this review are to define this phenomenon and to explore its similarities to the left ventricular dysfunction seen in patients with acute brain injury.

Methods

MEDLINE database, bibliographies of each citation for relevant articles, and consultation with clinical experts were used to examine the clinical picture of tako-tsubo-like left ventricular dysfunction.

Results

We identified case series and a systematic review that report on patients with this syndrome. This phenomenon occurs predominantly in female patients, presenting with a variety of ST-T segment changes and mildly elevated cardiac enzymes that mimic an acute coronary syndrome. The left ventricular dysfunction, typically showing a hyperkinetic basal region and an akinetic apical half of the ventricle, occurs in the absence of obstructed epicardial coronary arteries. The ventricular dysfunction usually resolves within weeks with a generally favorable prognosis. This phenomenon has similarities to that seen in patients with acute brain injury with regard to clinical presentation, pathology, and its reversible nature.

Conclusions

Transient left ventricular dysfunction occurs in the absence of obstructive epicardial coronary artery disease. In its broadest sense, this phenomenon may encompass a range of disorders including left ventricular dysfunction after central nervous system injury.     

Transient biventricular apical ballooning: a unique presentation of the "broken heart"

Transient left ventricular (LV) apical ballooning and normal epicardial coronary arteries are the hallmarks of Takotsubo cardiomyopathy. The syndrome is often triggered by emotional or physiologic stress, and its pathogenesis is poorly understood. Current proposals focus on elevated cathecolamines in association with abnormal coronary artery endothelium, coronary microcirculation or LV geometry. Right ventricular (RV) involvement, as described in our patient, is not reported as a typical feature. Presence of RV dysfunction may affect the initial management of these patients and raises questions regarding the universal applicability of the currently proposed pathophysiologic mechanisms of this syndrome.     

Bromocriptine-induced coronary spasm caused acute coronary syndrome, which triggered its own clinical twin--Takotsubo syndrome

Bromocriptine-induced coronary spasm (BICS) causing myocardial infarction has been reported. Association between BICS and Takotsubo syndrome (TS) has not been described. We report on a 37-year-old woman presenting with a clinical picture of acute coronary syndrome 1 day after initiation of treatment with bromocriptine for ablactation 3 weeks after a full-term spontaneous vaginal delivery. Coronary angiography showed diffuse narrowing of a large diagonal branch. Left ventriculography showed widespread hypokinesia extending beyond the diagonal branch supply region. There was a slight elevation of myocardial infarction biomarkers that was disproportional to the degree of left ventricular dysfunction. Follow-up coronary angiography, intravascular ultrasound and left ventriculography showed normal coronary arteries including the diagonal branch and complete normalization of the left ventricular function. Cardiac magnetic resonance examination showed no signs of late myocardial gadolinium enhancement. The clinical picture and course of the disease was consistent with TS. Consequently, we describe for the first time a case of TS triggered by myocardial ischemia caused by BICS. Furthermore, our case and sufficient supporting data from the literature demonstrate that acute coronary syndrome is an important and frequent but up till now missed trigger factor for TS.     

Acute cardiac sympathetic disruption in the pathogenesis of the takotsubo syndrome: A systematic review of the literature to date

Takotsubo syndrome (TS), also known as broken heart syndrome and neurogenic stunned myocardium, is an acute cardiac disease entity characterized by a clinical picture mimicking that of an acute coronary syndrome. The pathogenesis of TS has not been established yet. Among the most often debated pathologic mechanisms of TS are as follows: first, multi-vessel coronary spasm; second, myocardial microvascular dysfunction; third, aborted myocardial infarction caused by transient thrombotic occlusion of a long wrap-around left anterior descending artery; fourth, left ventricular outflow tract obstruction; fifth, blood-borne catecholamine cardiac toxicity; and sixth, cardiac sympathetic disruption and norepinephrine seethe and spillover. The aim of this review is to provide a thorough analysis of the literature data coming mainly from the neurological literature and dealing with the pathogenesis of TS. Substantial evidence challenging the first five hypotheses and arguing in favor of the hypothesis that acute cardiac sympathetic eruption and norepinephrine seethe and spillover is causing TS in predisposed patients is presented.     

Takotsubo Syndrome: Clinical Manifestations,Etiology and Pathogenesis

The purpose of the review is the analysis of clinical and experimental data on the etiology and pathogenesis of takotsubo syndrome (TS). TS is characterized by contractile dysfunction, which usually affects the apical region of the heart without obstruction of coronary artery, moderate increase in myocardial necrosis markers, prolonged QTc interval (in 50% of patients), sometimes elevation of ST segment (in 19% of patients), increase N-Terminal Pro-B-Type Natriuretic Peptide level, microvascular dysfunction, sometimes spasm of the epicardial coronary arteries (in 10% of patients), myocardial edema, and life-threatening ventricular arrhythmias (in 11% of patients). Stress cardiomyopathy is a rare disease, it is observed in 0.6 - 2.5% of patients with acute coronary syndrome. The occurrence of takotsubo syndrome is 9 times higher in women, who are aged 60-70 years old, than in men. The hospital mortality among patients with TS corresponds to 3.5% - 12%. Physical or emotional stress do not precede disease in all patients with TS. Most of patients with TS have neurological or mental illnesses. The level of catecholamines is increased in patients with TS, therefore, the occurrence of TS is associated with excessive activation of the adrenergic system. The negative inotropic effect of catecholamines is associated with the activation of β₂ adrenergic receptors. An important role of the adrenergic system in the pathogenesis of TS is confirmed by studies which were performed using ¹²⁵I-metaiodobenzylguanidine (¹²⁵I -MIBG). TS causes edema and inflammation of the myocardium. The inflammatory response in TS is systemic. TS causes impaired coronary microcirculation and reduces coronary reserve. There is a reason to believe that an increase in blood viscosity may play an important role in the pathogenesis of microcirculatory dysfunction in patients with TS. Epicardial coronary artery spasm is not obligatory for the occurrence of TS. Cortisol, endothelin-1 and microRNAs are challengers for the role of TS triggers. A decrease in estrogen levels is a factor contributing to the onset of TS. The central nervous system appears to play an important role in the pathogenesis of TS.     

The heart in fatal unstable angina pectoris

Compared to patients with sudden coronary death and acute myocardial infarction, relatively little morphologic data has been reported in patients with unstable angina pectoris. This article reviews necropsy data collected from one laboratory on unstable angina pectoris. From these data, several observations are appropriate: (1) Patients with unstable angina as a group have more coronary narrowing by atherosclerotic plaque than do patients with sudden coronary death or acute or healed myocardial infarction. (2) Patients with unstable angina have a much higher frequency of severe narrowing of the left main coronary artery than do patients in other coronary subsets. (3) The coronary atherosclerotic plaques in unstable angina consist primarily of fibrous tissue, and they are more similar to those found in patients with sudden coronary death than in patients with acute myocardial infarction. (4) The frequency of acute coronary lesions (thrombi, plaque rupture, and plaque hemorrhage) is similar to that observed in patients with sudden coronary death and significantly less than that observed in acute myocardial infarction. (5) The frequency of multiluminal channels throughout the major coronary arteries is significantly higher in unstable angina compared to sudden coronary death or acute myocardial infarction. (6) The major epicardial arteries and the heart are smaller in patients with unstable angina than in patients with sudden coronary death or acute myocardial infarction. (7) The left ventricular cavity is usually of normal size in patients with unstable angina and therefore left ventricular function is usually normal.     

Thrombo‐embolic complications in takotsubo syndrome: Review and demonstration of an illustrative case

Thrombo‐embolism is one of the serious complications of takotsubo syndrome (TS) in addition to heart failure, pulmonary edema, cardiogenic shock, cardiac arrest, life‐threatening arrhythmias, left ventricular outlet tract obstruction, mitral regurgitation, cardiac rupture, and death. The most common cardio‐embolic events in TS are cerebral, renal, and peripheral embolism. Approximately, one‐third of patients with left ventricular thrombus (LVT) in TS develop embolic complications. Cardio‐embolism in TS may occur with or without the presence of detectable LVT. In the present report, the thrombo‐embolic complications in TS with the emphasis on the association of TS to both acute coronary syndrome (ACS) including coronary embolism and ischemic stroke including cerebral embolism are reviewed. This serious complication is elucidated by demonstration of the case of a 67‐year‐woman with mid‐apical TS complicated by LVT, left anterior descending artery (LAD) and left middle cerebral artery (segment M2) thrombo‐embolic occlusions. The cerebral artery thrombotic occlusion was treated successfully with endovascular thrombectomy with complete resolution of the neurological deficits. There was spontaneous recanalization of the apical LAD occlusion verified by cardiac computed tomography angiography.     

Myocardial infarction with non-obstructive coronary arteries (MINOCA): Intracoronary imaging-based diagnosis and management

Myocardial infarction with non-obstructive coronary arteries (MINOCA) is defined by clinical evidence of myocardial infarction (MI) with normal or near-normal coronary arteries on angiography. This condition is present in about 5% to 25% of patients presenting with acute coronary syndromes. MINOCA is a working diagnosis. Current guidelines and consensus recommend identification of underlying causes of MINOCA in order to optimize treatment, improve prognosis, and promote prevention of recurrent myocardial infarction. An accurate evaluation of patient history, symptoms and use of invasive and non-invasive imaging should lead to identification of epicardial or microvascular causes of MINOCA and differentiation from non-ischemic myocardial injury due to both cardiac (e.g. myocarditis) and non-cardiac disease (e.g. pulmonary embolism).In this review, we highlight the role of coronary imaging in differential diagnosis of patients presenting with MINOCA. Intravascular ultrasound and optical coherence tomography are well known technologies used in different settings from acute to chronic coronary syndromes. In MINOCA patients, coronary imaging could help to identify pathological alterations of the epicardial vessels that are not visible by coronary angiography such as plaque disruption, coronary dissection, coronary thromboembolism, coronary spasm, and coronary artery disease in patients presenting with takotsubo syndrome. In future, the widespread use of these technologies, in the right clinical context, could lead to optimization and personalization of treatment, and to better prognosis of patients presenting with MINOCA.