Spontaneous Coronary Artery Dissection and Fibromuscular Dysplasia: A Case Series and Genetic Links.

Spontaneous coronary artery dissection (SCAD) is a rare cause of myocardial infarction in young women. An association of fibromuscular dysplasia (FMD) with SCAD has been well established; a significant proportion of SCAD patients may have typical FMD findings in other noncoronary arteries. The current consensus recommends arterial imaging screening from head to pelvis using computed tomography angiography (CTA) or magnetic resonance angiography (MRA) in SCAD. Genetic testing for FMD should be considered in high-risk cases. We present two cases of SCAD associated with FMD and discuss the significance of genetic screening in such patients.

Association of triglyceride levels with adverse cardiovascular events in patients with ST-segment elevation myocardial infarction.

Although there is an established association between elevated triglyceride (eTG, ≥175 mg/dl) levels and adverse cardiovascular events, some studies have suggested that eTG levels may be linked to neutral or even improved clinical outcomes, particularly among patients with acute myocardial infarction. However, these studies had certain limitations, including small sample sizes, heterogeneous study populations, and inadequate statistical adjustments. To address these limitations, we conducted an analysis of 5347 patients with ST-segment elevation myocardial infarction (STEMI) between March 2003 and December 2020, using a prospective registry-based cohort from two large, regional STEMI centers. We used a triglyceride level of 175 mg/dl as the cutoff point for eTG levels. Of the study participants, 24.5% (n = 1312) had eTG levels. These patients were more likely to be younger, male, and have a higher number of cardiovascular risk factors compared to those with low TG levels. Despite these unfavorable cardiovascular risk profiles, patients with eTG levels had lower unadjusted risks of 1-year major adverse cardiac events (MACE) -a composite of myocardial infarction, stroke, and death- than those with low TG levels (8.8% vs. 11%, p = 0.034). However, after adjusting for certain clinical factors and lipid profile, eTG levels were not associated with a lower 1-year MACE (aHR: 1.10 (0.71-1.70), p = 0.7). In conclusion, a quarter of STEMI patients had eTG levels and these patients had comparable long-term cardiovascular outcomes compared to those with low TG levels after controlling for clinical factors and lipid profile.

A large hiatal hernia causing frequent premature ventricular contractions with bigeminy: A case report and review of literature.

Most patients with hiatal hernia (HH) are asymptomatic; however, common symptoms include gastroesophageal reflux disease (GERD) and heart burn. Larger hernia can cause obstruction, ischemia of the bowel, volvulus of the contents of the hernial sac, respiratory distress, and rarely, cardiac abnormalities are also noted. Most reported cardiac abnormalities associated with HH include atrial fibrillation, atrial flutter, supraventricular tachycardia, and bradycardia. We present a rare case of a large HH causing frequent premature ventricular contractions in bigeminy form that resolved with surgical correction of HH and did not recur on subsequent Holter monitoring. We highlight the potential association between HH/GERD and cardiac arrhythmias and reinforce the need to keep HH/GERD as one of the working diagnoses in a patient with cardiac arrhythmia. Learning objective: •Large hiatal hernia can cause several arrhythmias such as atrial fibrillation, atrial flutter, supraventricular tachycardia, bradycardia, and premature ventricular contractions (PVCs).•It is essential to consider hiatal hernia and gastroesophageal reflux disease as one of the differentials in the work up of cardiac arrhythmias including PVCs.

Left ventricular pseudoaneurysm without antecedent myocardial infarction.

A left ventricle pseudoaneurysm (LVPA) occurs when the left ventricle free wall rupture becomes contained by pericardium or adhesions. It is rare and has a poor prognosis. LVPA is strongly associated with myocardial infarction. Surgical management of LVPA carries a high mortality rate but is still recommended for most cases of LVPA as soon as the diagnosis is confirmed. Medical management is generally limited to asymptomatic, incidentally found lesions. We present a case of LVPA without any usual risk factors, which was successfully treated by surgery. Learning objectives: •To identify the left ventricle pseudoaneurysm (LVPA) that can present with chest pain or dyspnea, but at times can be asymptomatic•To keep a high index of suspicion for LVPA even in patients without the common risk factors such as recent myocardial infarction, cardiac surgery, or trauma•To realize that management options are individualized•To understand that despite a high surgical mortality, for large expanding LVPA, surgery is still recommended•Further research needs to be done to establish management guidelines.

Cardiac MRI in myocardial infarction without obstructive coronary artery disease (MINOCA) helps arrive at correct diagnosis - A case report and discussion.

Myocardial infarction without obstructive coronary artery disease (MINOCA) is a common condition with estimated prevalence of 5 to 15 %. It is not a benign condition and diagnosing the exact underlying etiology can be challenging, but it is important to ensure appropriate management of MINOCA patients. Cardiac magnetic resonance imaging (CMRI) can be a valuable and non-invasive test to identify the underlying etiology, as well as to risk-stratify such patients. Both the European Society of Cardiology and the American Heart Association recommend CMRI in diagnostic work up of MINOCA patients. We report a case of an 83-year-old man who presented to the emergency department with atypical chest pain but had significantly elevated cardiac troponin levels, with non-obstructive coronary artery disease on left heart catheterization. Subsequent CMRI led to the diagnosis of acute myocarditis. He was medically managed with good clinical outcomes. We discuss this case in detail and highlight the role of CMRI in MINOCA patients. As our understanding of troponin elevation and its various mechanisms continues to evolve, cardiac MRI has a significant role in diagnosis and management, as demonstrated in our case. Learning objectives: 1.Illustrate the clinical relevance and diagnosis of myocardial infarction without obstructive coronary artery disease (MINOCA).2.Explain the role of cardiac magnetic resonance imaging in diagnosis and management of MINOCA.

Quadricuspid Aortic Valve: An Introduction for Clinicians.

Quadricuspid aortic valve (QAV) is a congenital heart anomaly in which the aortic valve has four cusps of various size possibilities, as opposed to the three symmetrical cusps generally observed. This cardiac valvular abnormality is rarely identified, with an estimated incidence rate of 0.013% to 0.043%, although recent technological advancements in diagnostics have contributed to an increase in detection. Historically, it had been typically encountered during open heart surgery or postmortem; however, it is presently diagnosed primarily via ultrasound echocardiography, and could go undetected unless specifically considered. It was first reported by Babington in 1847, and since then approximately 300 cases have been published. This condition is sporadically associated with additional congenital cardiovascular defects, with coronary artery irregularities being the most common. In more than half of published QAV incidences it has led to the progressive development of aortic regurgitation (AR) usually sans aortic stenosis, particularly amongst elderly patients, often requiring surgical intervention after 50 years of age. A fifth of total instances, but two-thirds of instances with AR, warrant surgery seldom amidst complications, with reconstructive tricuspidization preferred over valve replacement.

Effect of shield placement for transient voltage mitigation due to switching surges in a 33/11 kV transformer windings.

This study presents an investigation on the effect of shield placement for mitigation of transient voltage in a 33/11 kV, 30 MVA transformer due to Standard Switching Impulse (SSI) and Oscillating Switching Impulse (OSI) surges. Generally, the winding and insulation in transformers could experience severe voltage stress due to the external impulses i.e. switching events. Hence, it is important to examine the voltage stress and identify the mitigation action i.e. shield placements in order to reduce the adverse effect to the transformer windings. First, the resistances, inductances, and capacitances (RLC) were calculated for disc type transformer in order to develop the winding RLC equivalent circuit. The SSI and OSI transient voltage waveforms were applied to the High Voltage (HV) winding whereby the transient voltages were simulated for each disc. The resulting voltage stresses were mitigated through different configurations of electrostatic shield placements. The resonant oscillations generated due to switching surges were analysed through initial voltage distribution. The analyses on the transient voltages of the transformer winding and standard error of the slope (SEb) reveal that the location of shield placement has a significant effect on the resonant switching voltages. The increment of the shield number in the windings does not guarantee optimize mitigation of the resonant switching transient voltages. It is found that the voltage stress along the windings is linear once a floating shield is placed between the HV and Low Voltage (LV) windings of the disc-type transformer under the SSI and OSI waveforms. These findings could assist the manufacturers with appropriate technical basis for mitigation of the transformer winding against the external transient switching overvoltage surges.

Extraction of winding parameters for 33/11 kV, 30 MVA transformer based on finite element method for frequency response modelling.

This paper proposes an alternative approach to extract transformer's winding parameters of resistance (R), inductance (L), capacitance (C) and conductance (G) based on Finite Element Method (FEM). The capacitance and conductance were computed based on Fast Multiple Method (FMM) and Method of Moment (MoM) through quasi-electrostatics approach. The AC resistances and inductances were computed based on MoM through quasi-magnetostatics approach. Maxwell's equations were used to compute the DC resistances and inductances. Based on the FEM computed parameters, the frequency response of the winding was obtained through the Bode plot function. The simulated frequency response by FEM model was compared with the simulated frequency response based on the Multi-conductor Transmission Line (MTL) model and the measured frequency response of a 33/11 kV, 30 MVA transformer. The statistical indices such as Root Mean Square Error (RMSE) and Absolute Sum of Logarithmic Error (ASLE) were used to analyze the performance of the proposed FEM model. It is found that the simulated frequency response by FEM model is quite close to measured frequency response at low and mid frequency regions as compared to simulated frequency response by MTL model based on RMSE and ASLE analysis.

Clinical and Demographic Characteristics of Upper Limb Dystonia.

BACKGROUND: Knowledge of characteristics in upper limb dystonia remains limited, derived primarily from small, single-site studies. OBJECTIVE: The objective of this study was to characterize demographic and clinical characteristics of upper limb dystonia from the Dystonia Coalition data set, a large, international, multicenter resource. METHODS: We evaluated clinical and demographic characteristics of 367 participants with upper limb dystonia from onset, comparing across subcategories of focal (with and without dystonia spread) versus nonfocal onset. RESULTS: Focal onset occurred in 80%; 67% remained focal without spread. Task specificity was most frequent in this subgroup, most often writer's cramp and affecting the dominant limb (83%). Focal onset with spread was more frequent in young onset (<21 years). Focal onset occurred equally in women and men; nonfocal onset affected women disproportionately. CONCLUSIONS: Upper limb dystonia distribution, focality, and task specificity relate to onset age and likelihood of regional spread. Observations may inform clinical counseling and design, execution, and interpretation of future studies. © 2020 International Parkinson and Movement Disorder Society.

Left ventricle pseudoaneurysm: Diagnosis by a new murmur.

Incomplete rupture of the ventricle free wall can occur after myocardial infarction. This occurs when an organized thrombus and the pericardium seal the ventricular perforation. This can progress to the formation of a left ventricle pseudoaneurysm (LVPA). A 70-year-old male with an antero-septal ST-elevation myocardial infarction (STEMI) underwent an emergent left heart catheterization which revealed severe three-vessel disease with occluded grafts, non-amenable to re-vascularization, and an apical thrombus. As he was high-risk for repeat coronary artery bypass graft, he was medically managed. Transthoracic echocardiogram (TTE) showed a normal left ventricle ejection fraction (LVEF), apical anterior and inferior wall akinesis, moderate sized apical thrombus, and pericardial thickening. On hospital day 7, examination revealed a new 3/6 to-and-fro murmur that was loudest at the apex. The patient was asymptomatic with normal vital signs. A repeat TTE revealed an apical wall rupture with flow into the pericardial cavity and absence of the apical thrombus. A LVPA was diagnosed and the patient was immediately referred for surgical repair. This case illustrates the potential for developing LVPA in STEMI patients and the importance of physical examination. If identified early a potential emergent situation in a previously asymptomatic patient can be averted, thereby preventing fatal consequences. .

Hypothermia as a forgotten sign of prolonged severe hypoglycaemia.

Hypothermia is often a sign of serious illness. Commonly reported aetiologies include but are not limited to sepsis, exposure to cold and endocrine disorders. Hypoglycaemia, a common occurrence, is rarely associated with hypothermia. We present a case of prolonged, severe hypothermia due to hypoglycaemia. A 58-year-old man with diabetes who presented with chest pain and was diagnosed with Non-ST elevation myocardial infarction. He was given nothing per mouth in preparation for a left heart catheterisation but received his reported insulin glargine dosage at bedtime. A few hours later, he was noted to have diaphoresis and hypoglycaemia, and his temperature steadily started dropping which was unresponsive to local warming. Once his hypoglycaemia was successfully treated with dextrose infusion, his temperature improved. An extensive workup revealed no infective or endocrine disorder.

Cardiac Lipoma: An Uncharacteristically Large Intra-Atrial Mass Causing Symptoms.

Primary tumours of the heart are often encountered in clinical practice. Different autopsy series estimate the incidence to be anywhere from 0.001% to 0.19%. Cardiac lipoma is a rare type of tumour of the heart and pericardium. It comprises approximately 10-19% of all cardiac tumours. We present a case of a large cardiac lipoma in a fifty-year-old female. She presented with sharp chest pains, palpitations, and dizziness. Acute coronary syndrome was ruled out. A transthoracic echocardiogram showed an abnormal, large, fixed right atrial mass. The mass was noted to be occupying most of the right atrium. It was excised due to its large size and persistent symptoms. On pathophysiology, the mass was definitively diagnosed to be an 80 mm × 70 mm cardiac lipoma. Postoperatively, the patient did well with resolution of her symptoms. This case provides evidence that even large, invasive, symptomatic cardiac lipomas can be successfully resected with good outcomes.

Is HPS2-THRIVE the death knell for niacin?

Niacin is a lipid-modifying therapy with proven efficacy for reducing cardiovascular events as monotherapy and when used in combination with other lipid-modifying medications impacts rates of atherosclerotic disease progression. Large outcome trials using niacin against a background of statin therapy with optimal control of atherogenic lipoprotein burden in serum were unable to demonstrate incremental benefit of niacin beyond statin therapy. We address 2 key questions: (1) Can the results from randomized clinical trials performed in stable ischemic heart disease populations (AIM-HIGH and HPS2-THRIVE) be applied to patients who sustain an acute coronary syndrome or myocardial infarction? (2) Are patients with very low baseline levels of high-density lipoprotein cholesterol (<30 mg/dL) at particularly high risk for subsequent cardiac events?

Epinephrine use results in 'stress' cardiomyopathy.

Epinephrine is a frequently used catecholamine, particularly in emergencies and during resuscitation attempts. It is not without side effects. We report a rare case of epinephrine-induced cardiomyopathy. Epinephrine was administered for bradycardia in our patient. He was treated conservatively and responded well to the treatment. Through our work we wish to highlight this adverse cardiac effect of epinephrine. We hope to increase awareness among residents and practicing physicians while using epinephrine. .

Quenched Assembly of NIR-Active Gold Nanoclusters Capped with Strongly Bound Ligands by Tuning Particle Charge via pH and Salinity.

Gold nanospheres coated with a binary monolayer of bound citrate and cysteine ligands were assembled into nanoclusters, in which the size and near-infrared (NIR) extinction were tuned by varying the pH and concentration of added NaCl. During full evaporation of an aqueous dispersion of 4.5 ± 1.8 nm Au primary particles, the nanoclusters were formed and quenched by the triblock copolymer polylactic acid (PLA)(1K)-b-poly(ethylene glycol) (PEG)(10K)-b-PLA(1K), which also provided steric stabilization. The short-ranged depletion and van der Waals attractive forces were balanced against longer ranged electrostatic repulsion to tune the nanocluster diameter and NIR extinction. Upon lowering the pH from 7 to 5 at a given salinity, the magnitude of the charge on the primary particles decreased, such that the weaker electrostatic repulsion increased the hydrodynamic diameter and, consequently, NIR extinction of the clusters. At a given pH, as the concentration of NaCl was increased, the NIR extinction decreased monotonically. Furthermore, the greater screening of the charges on the nanoclusters weakened the interactions with PLA(1K)-b-PEG(10K)-b-PLA(1K) and thus lowered the amount of adsorbed polymer on the nanocluster surface. The generalization of the concept of self-assembly of small NIR-active nanoclusters to include a strongly bound thiol and the manipulation of the morphologies and NIR extinction by variation of pH and salinity not only is of fundamental interest but also is important for optical biomedical imaging and therapy.

Anomalous origin of the left coronary artery from pulmonary artery a late presentation-Case report and review of literature.

An otherwise healthy 26-year-old female presented with sudden cardiac arrest. She was resuscitated with unsynchronized cardioversion for ventricular fibrillation. A left heart cardiac catheterization showed anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Cardiac computed tomographic angiography confirmed this abnormality. She underwent direct translocation of the left main coronary artery to the aorta, and after a stormy postoperative course was discharged home. ALCAPA is a rare congenital abnormality of the coronary system that is associated with early infant mortality and adult sudden death. The use of advanced cardiac imaging has resulted in an increase in the number of diagnosed ALCAPA cases, especially in the adult population, sometimes even in the asymptomatic. The extent of collateral circulation that develops between the right coronary artery and the left coronary artery determines the outcome; the more collateral circulation there is, the less the hypoxic damage to the ventricle. This not only informs us that people survive ALCAPA into adulthood but also highlights the importance for adult cardiologists to be aware of this interesting disease. Corrective surgery remains the treatment of choice. We present a rare case of ALCAPA, with first presentation in adulthood in the form of a malignant ventricular arrhythmia. .

Takotsubo Cardiomyopathy: Typical and Atypical Variants, A Two-Year Retrospective Cohort Study.

BACKGROUND: Typical or classical takotsubo cardiomyopathy (TCM) is associated with the characteristic abnormality of a ballooned left ventricular apex with basal segmental hyperkinesis. TCM may not present with the "classical" wall motion abnormalities but can have a variety of segmental wall motion abnormalities. The aim of our work was to assess for any unique identifying factors that can help distinguish typical and atypical variants of TCM. METHODS: We studied 11 consecutive patients between 2010 and 2012 admitted with chest pain, electrocardiographic and cardiac biomarker changes consistent with acute coronary syndrome (ACS) who underwent left heart angiography and were clinically diagnosed to have TCM. RESULTS: Our study found no specific features distinguishing typical and atypical variants of TCM. In our study, all patients were female and all had excellent outcome. One patient was in fourth decade of life, three patients in fifth and sixth decade of life, while remaining were older. One patient had diabetes mellitus, five had hypertension, four had concurrent coronary artery disease, but no patient had any family history of TCM. Nine of 11 patients had immediate clear-cut stressors. Three patients had normal ECG, two with ST segment elevation, with nine patients having only modest troponin elevations. One patient had an anomalous RCA take-off from the right coronary cusp, otherwise remaining patients had normal anatomy. One patient had only apical involvement, remaining had multiple wall motion abnormalities, and all patients had involvement of the anterior wall. Four patients had apical sparing. No inverted TCM pattern with basal akinesis with normal wall motion in the midventricular and apical regions was found among our patients. CONCLUSIONS: We report that the classification of TCM as typical versus atypical is probably not clinically meaningful. The regional wall motion abnormalities are related to catecholamine excess and to the susceptibility of that particular region to excess catecholamine. We do not know why such differences in regional susceptibility exist, and agree with the other authors that sub-classification would only add to confusion, and a delay in understanding of the disease process.

Iatrogenic Aorto-Coronary Dissection Successfully Treated With IVUS Guided Unprotected Left Main Stenting: Case Report and Review of Literature.

A 52-year-old male underwent cardiac catheterization for abnormal stress test. Trans-radial coronary angiography revealed a severe proximal left anterior descending artery (LAD) lesion. LAD angioplasty was performed with two drug-eluting stents. This resulted in dissection of the proximal LAD, the circumflex artery and the left main coronary artery (LMCA) extending back into the coronary sinus. A diagnosis of type 3 coronary dissection was made. The circumflex artery and the left coronary artery were stented, and then the LMCA was stented. Repeat intravascular ultrasound showed resolution of the dissection and TIMI-3 flow was achieved in all vessels. He underwent follow-up angiography in 1 month, which revealed patent stents with resolution of the aorto-coronary dissection. We report a rare case of iatrogenic aorto-coronary dissection that was successfully treated with unprotected left main percutaneous coronary intervention strategy alone and review the pertinent literature.