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cardiac syndrome x

Farzad Masoudkabir, Ali Vasheghani-Farahani, Elham Hakki, Hamidreza Poorhosseini, Saeed Sadeghian, Seyed Hesameddin Abbasi, Shahram Bahmanyar, Seyed Ebrahim Kassaian
A major diagnostic challenge for cardiologists is to distinguish cardiac syndrome X (CSX) from obstructive coronary artery disease in women with typical angina and a positive exercise tolerance test (ETT). We performed this study to develop a scoring system that more accurately predicts CSX in this patient population. Data on 976 women with typical angina and a positive ETT who underwent coronary angiography at our center were randomly divided into derivation and validation datasets. We developed a backward stepwise logistic regression model that predicted the presence of CSX, and a scoring system was derived from it...
February 2018: Texas Heart Institute Journal
Nagy Mekhail, Ogi Visnjevac, Gerges Azer, Diana Sue Mehanny, Priya Agrawal, Victor Foorsov
To assess the efficacy of spinal cord stimulation (SCS) for each indication, one must critically assess each specific clinical outcome to identify outcomes that benefit from SCS therapy. To date, a comprehensive review of clinically relevant outcome-specific evidence regarding SCS has not been published. We aimed to assess all randomized controlled trials from the world literature for the purpose of evaluating the clinical outcome-specific efficacy of SCS for the following outcomes: perceived pain relief or change pain score, quality of life, functional status, psychological impact, analgesic medication utilization, patient satisfaction, and health care cost and utilization...
February 23, 2018: Regional Anesthesia and Pain Medicine
Z Liu, X Yang, Z Tian, J Qian, Q Wang, J Zhao, C Huang, Y Liu, X Guo, H Wang, J Lai, M Li, Y Zhao, X Zeng
Pulmonary arterial hypertension (PAH) is a rare and severe complication of primary Sjögren's syndrome (pSS). Cohort studies indicate that the underlying diseases of PAH associated with connective tissue disease (CTD-PAH) in Asian countries are different from that in the United States and in Europe. This study investigated the clinical characteristics, survival, and prognostic factors of pSS-PAH in Chinese patients. We enrolled 29 patients with pSS-PAH who visited our referral center during August 2007 and May 2015...
January 1, 2018: Lupus
Lingling Zhu, Limei Xie
INTRODUCTION: Joubert syndrome (JS) is a rare autosomal recessive inherited disease belonging to ciliopathy with the causative mutation of genes. Except for X-linked inheritance, the high recurrence rate of a family is about 25%. After birth, it may cause a series of neurological symptoms, even with retina, kidney, liver, and other organ abnormalities, which is defined as Joubert syndrome and related disorders (JSRD). Molecular genetics research contributes to disease prediction and genetic counseling...
December 2017: Medicine (Baltimore)
Leif-Christopher Engel, Ulf Landmesser, Kevin Gigengack, Thomas Wurster, Constantina Manes, Georg Girke, Milosz Jaguszewski, Carsten Skurk, David M Leistner, Alexander Lauten, Andreas Schuster, Bernd Hamm, Rene M Botnar, Marcus R Makowski, Boris Bigalke
OBJECTIVES: This study sought to investigate the potential of the noninvasive albumin-binding probe gadofosveset-enhanced cardiac magnetic resonance (GE-CMR) for detection of coronary plaques that can cause acute coronary syndromes (ACS). BACKGROUND: ACS are frequently caused by rupture or erosion of coronary plaques that initially do not cause hemodynamically significant stenosis and are therefore not detected by invasive x-ray coronary angiography (XCA). METHODS: A total of 25 patients with ACS or symptoms of stable coronary artery disease underwent GE-CMR, clinically indicated XCA, and optical coherence tomography (OCT) within 24 h...
January 12, 2018: JACC. Cardiovascular Imaging
Roopa Kanakatti Shankar, Philippe F Backeljauw
Turner syndrome (TS) is characterized by partial or complete loss of the second X-chromosome in phenotypic females resulting in a constellation of clinical findings that may include lymphedema, cardiac anomalies, short stature, primary ovarian failure and neurocognitive difficulties. Optimizing health care delivery is important to enable these individuals achieve their full potential. We review the current best practice management recommendations for individuals with TS focusing on the latest consensus opinion in regard to genetic diagnosis, treatment of short stature, estrogen supplementation, addressing psychosocial issues, as well screening for other comorbidities...
January 2018: Therapeutic Advances in Endocrinology and Metabolism
Ravi Vaidyanathan, Hanora Van Ert, Kazi T Haq, Stefano Morotti, Samuel Esch, Elise C McCune, Eleonora Grandi, Lee L Eckhardt
BACKGROUND: In human cardiac ventricle, IK1 is mainly comprised Kir2.1, but Kir2.2 and Kir2.3 heterotetramers occur and modulate IK1. Long-QT syndrome-9-associated CAV3 mutations cause decreased Kir2.1 current density, but Kir2.x heterotetramers have not been studied. Here, we determine the effect of long-QT syndrome-9-CAV3 mutation F97C on Kir2.x homo- and heterotetramers and model-associated arrhythmia mechanisms. METHODS AND RESULTS: Super-resolution microscopy, co-immunoprecipitation, cellular electrophysiology, on-cell Western blotting, and simulation of Purkinje and ventricular myocyte mathematical models were used...
January 2018: Circulation. Arrhythmia and Electrophysiology
Muhammad Naveed, Imran Shair Mohammad, Li Xue, Sara Khan, Wang Gang, Yanfang Cao, Yijie Cheng, Xingxing Cui, Chen DingDing, Yu Feng, Wang Zhijie, Zhou Xiaohui
Complicated pathophysiological syndrome associated with irregular functioning of the heart leading to insufficient blood supply to the organs is linked to congestive heart failure (CHF) which is the leading cause of death in developed countries. Numerous factors can add to heart failure (HF) pathogenesis, including myocardial infarction (MI), genetic factors, coronary artery disease (CAD), ischemia or hypertension. Presently, most of the therapies against CHF cause modest symptom relief but incapable of giving significant recovery for long-term survival outcomes...
March 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
Benjamin Abels, Steffen Pfeiffer, Jana Stix, Johannes Schwab
We present a case of an 85-year-old patient who underwent clinical work-up for chronic heart failure, acute coronary syndrome, and pulmonary embolism, until she was diagnosed with a cardiac mass that was histologically identified as sarcoma. The aim of this educational case report is to raise awareness of cardiac masses and to point out diagnostic hints towards a cardiac tumor on chest X-ray, coronary angiography, echocardiography, and chest CT. Moreover, the vital role of cardiac magnetic resonance for the diagnosis of a cardiac mass is highlighted...
November 2017: Journal of Radiology Case Reports
Karin H Humphries, May K Lee, Mona Izadnegahdar, Min Gao, Daniel T Holmes, Frank X Scheuermeyer, Martha Mackay, Andre Mattman, Eric Grafstein
OBJECTIVE: While sex differences in the treatment and outcomes of subjects with acute coronary syndromes are well documented, little is known about the impact of cardiac troponin (cTn) levels obtained in the emergency department (ED) on the observed sex differences. We sought to determine whether cTn levels by chest pain features modify sex differences in diagnosis, treatment and outcomes in patients presenting with chest pain suggestive of ischemia. METHODS: All adults presenting to two hospitals in Vancouver, Canada between May 2008 and Mar 2013 with ischemic chest pain and with cTn testing were included in the study...
December 23, 2017: Academic Emergency Medicine: Official Journal of the Society for Academic Emergency Medicine
Catherine L Mercer, Gaia Andreoletti, Aisling Carroll, Anthony P Salmon, I Karen Temple, Sarah Ennis
BACKGROUND: Familial Ebstein anomaly is a rare form of congenital heart disease. We report 7 individuals among 2 generations of 1 family with Ebstein anomaly. This family was first reported in 1991 by Balaji et al in which family members were also reported to have a mild skeletal phenotype. The most likely mechanism of inheritance was concluded to be autosomal dominant. We sought to identify the genetic pathogenesis in this family using a next generation sequencing approach. METHODS AND RESULTS: Whole exome sequencing was performed in 2 cousins in this family using the Agilent SureSelect Human all Exon 51 Mb version 5 capture kit...
December 2017: Circulation. Cardiovascular Genetics
Kahraman Yakut, İlkay Erdoğan, Birgül Varan, İlyas Atar
BACKGROUND: Brugada syndrome is a disease characterized by a specific electrocardiographic pattern and an increased risk of sudden cardiac death. We present this case with the updated literature to emphasise the need to consider the diagnosis of Brugada syndrome in patients admitted to the emergency ward with sudden cardiac arrest. CASE REPORT: A 16-year-old female patient was admitted to the emergency ward with complaints of weakness and abdominal pain, and she had four cardiac arrests during her evaluation period...
December 1, 2017: Balkan Medical Journal
Tse-Min Lu, Tzong-Shyuan Lee, Shing-Jong Lin, Wan-Leong Chan, Chiao-Po Hsu
BACKGROUND: The pathophysiology of cardiac syndrome X is multifactorial and endothelial dysfunction has been implicated as important contributing factor. Asymmetric dimethylarginine (ADMA), characterized as a circulating endogenous inhibitor of nitric oxide synthase, may have been implicated as an important contributing factor for the development of endothelial dysfunction. In this study, we aim to assess the predictive power of ADMA for long-term prognosis in patients with cardiac syndrome X...
2017: PloS One
X T Wang, D W Liu, H M Zhang, Y Long, X D Guan, H B Qiu, K J Yu, J Yan, H Zhao, Y Q Tang, X Ding, X C Ma, W Du, Y Kang, B Tang, Y H Ai, H W He, D C Chen, H Chen, W Z Chai, X Zhou, N Cui, H Wang, X Rui, Z J Hu, J G Li, Y Xu, Y Yang, B Ouyan, H Y Lin, Y M Li, X Y Wan, R L Yang, Y Z Qin, Y G Chao, Z Y Xie, R H Sun, Z Y He, D F Wang, Q Q Huang, D P Jiang, X Y Cao, R G Yu, X Wang, X K Chen, J F Wu, L N Zhang, M G Yin, L X Liu, S W Li, Z J Chen, Z Luo
To establish the experts consensus on the right heart function management in critically ill patients. The panel of consensus was composed of 30 experts in critical care medicine who are all members of Critical Hemodynamic Therapy Collaboration Group (CHTC Group). Each statement was assessed based on the GRADE (Grading of Recommendations Assessment, Development, and Evaluation) principle. Then the Delphi method was adopted by 52 experts to reassess all the statements. (1) Right heart function is prone to be affected in critically illness, which will result in a auto-exaggerated vicious cycle...
December 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
Erkan Yildirim, Uygar Cagdas Yuksel, Murat Celik, Baris Bugan, Mutlu Gungor, Yalcin Gokoglan, Mustafa Koklu, Suat Gormel, Salim Yasar, Cem Barcin
Objective The vessels involved in the microcirculation are too small to be visualized by conventional angiography and no tools are currently available that can directly evaluate the coronary microcirculation. This study evaluated the coronary clearance frame count (CCFC) in patients with cardiac syndrome X (CSX). Methods The retrospective study enrolled patients with angina, who had a positive nuclear imaging test and normal coronary angiography; and a control group consisting of patients who underwent an angiogram to exclude coronary artery disease...
January 1, 2017: Journal of International Medical Research
Mariana Moysés-Oliveira, Giuliana Giannuzzi, Richard J Fish, Jill A Rosenfeld, Florence Petit, Maria de Fatima Soares, Leslie Domenici Kulikowski, Adriana Di-Battista, Malú Zamariolli, Fan Xia, Thomas Liehr, Nadezda Kosyakova, Gianna Carvalheira, Michael Parker, Eleanor G Seaby, Sarah Ennis, Rodney D Gilbert, R Tanner Hagelstrom, Maria L Cremona, Wenhui L Li, Alka Malhotra, Anjana Chandrasekhar, Denise L Perry, Ryan J Taft, Julie McCarrier, Donald G Basel, Joris Andrieux, Taiza Stumpp, Fernanda Antunes, Gustavo José Pereira, Marguerite Neerman-Arbez, Vera Ayres Meloni, Margaret Drummond-Borg, Maria Isabel Melaragno, Alexandre Reymond
We report five individuals with loss-of-function of the X-linked AMMECR1: a girl with a balanced X-autosome translocation and inactivation of the normal X-chromosome; two boys with maternally inherited and de novo nonsense variants; and two half-brothers with maternally inherited microdeletion variants. They present with short stature, cardiac and skeletal abnormalities, and hearing loss. Variants of unknown significance in AMMECR1 in four male patients from two families with partially overlapping phenotypes were previously reported...
February 2018: Human Mutation
L Chen, X H Ma, L Zhao, R Bai, S N Li, L Wang, H Chen, Z M Fan, M W Lou, Y D Niu
Objective: To retrospectively analyze the potential correlation between cardiac magnetic resonance (CMR) imaging and clinical features and idiopathic arrhythmia in patients with straight back syndrome (SBS). Methods: Patients receiving CMR imaging examination from April 2015 to March 2016 at our department (n=1 432) were screened, 76 patients met the diagnosis criteria of flat chest (anteroposterior diameter/transthoracic diameter (APD/TTD) ratio<0.37 at the T8 vertebra). After excluding 33 patients with structural heart disease, 43 SBS patients were divided into two groups: SBS without obvious morphological change in the heart (group A, n=19) and SBS with morphological change of the heart (group B, n=24)...
November 24, 2017: Zhonghua Xin Xue Guan Bing za Zhi
Alan Sugrue, Ram K Rohatgi, Peter A Noseworthy, Vaclav Kremen, J Martijn Bos, Bo Qiang, Yehu Sapir, Zachi I Attia, Christopher G Scott, Peter Brady, Samuel J Asirvatham, Paul A Friedman, Michael J Ackerman
BACKGROUND: Although the hallmark of long-QT syndrome (LQTS) is abnormal cardiac repolarization, there are varying degrees of phenotypic expression and arrhythmic risk. Our aim was to evaluate the performance of a morphological T-wave analysis program in defining breakthrough LQTS arrhythmic risk beyond the QTc value. METHODS AND RESULTS: We analyzed 407 genetically confirmed patients with LQT1 (n=246; 43% men) and LQT2 (n=161; 41% men) over the mean follow-up period of 6...
November 2017: Circulation. Arrhythmia and Electrophysiology
S Wen, W Sun, Z Li, X Zhuang, G Zhao, C Xie, M Zheng, J Jing, P Xiao, M Wang, J Han, J Ren, H Liu, H Lu, N Jin
Porcine circovirus type 3 (PCV3) is a novel circovirus that was firstly detected in the USA. PCV3 is associated with porcine dermatitis and nephropathy syndrome (PDNS), reproductive failure and cardiac and multisystemic inflammation. Latterly, PCV3 was detected in Guangxi, China. Forty-one of 108 (37.96%) samples and nine of 47 (19.14%) samples were PCV3 positive in pig farms and pig slaughter houses, respectively. Three PCV3 strains were sequenced and designated PCV3-China/GX2016-1, PCV3-China/GX2016-2 and PCV3-China/GX2016-3...
November 8, 2017: Transboundary and Emerging Diseases
Michele Dibattista, Simona Lobasso, Sebastiano Stramaglia, Angela Corcelli
Barth syndrome is a rare X-linked disease affecting less than 200 individuals worldwide. Several comorbidities have been associated with the pathology and, among those, cardiac myopathy and neutropenia are the most life threatening. The appropriate nutritive support is important to sustain the everyday life of Barth syndrome patients given the chronic fatigue they experience. Since they often prefer salty and fried food, and avoid vegetables and fruits, their eating habit and food preferences do not always provide the proper amount of vitamins and amino acids...
2017: PloS One
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