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Maximilian D Hien, Fernando Benito Castro, Philippe Fournier, Anne Filleron, Tu-Anh Tran
OBJECTIVES: We report on a rare but severe complication of adenosine use in a child with reentry tachycardia. METHODS AND RESULTS: Treatment with adenosine, which is the standard medical therapy of atrioventricular reentry tachycardia, led to the development of an irregular wide complex tachycardia, caused by rapid ventricular response to atrial fibrillation. The girl was finally stabilized with electrical cardioversion. We analyze the pathomechanism and discuss possible treatment options...
October 8, 2016: Pediatric Emergency Care
Torsten Konrad, Sebastian Sonnenschein, Frank Patrick Schmidt, Hanke Mollnau, Karsten Bock, Blanca Quesada Ocete, Thomas Münzel, Cathrin Theis, Thomas Rostock
AIMS: Different cardiac arrhythmias have been suggested to be associated with Danon disease, e.g. Wolff-Parkinson-White syndrome. However, a systematic electrophysiological investigation of patients with Danon disease is lacking thus far. METHODS AND RESULTS: Seven patients with Danon disease (4 males, 35.8 ± 10.8 years; 3 females, 51.3 ± 19.9 years) from 3 different families were studied. In all patients, the presence of Danon disease was confirmed by western blot of biopsy material or genetic testing...
October 14, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
Rajashree Madabushi, Anil Agarwal, Saipriya Tewari, Sujeet K S Gautam, Sandeep Khuba
Wolff Parkinson White (WPW) syndrome is a condition in which there is an aberrant conduction pathway between the atria and ventricles, resulting in tachycardia. A 42-year-old patient, who was treated for WPW syndrome previously, presented with chronic somatic pain. With her cardiac condition in mind, she was thoroughly worked up for a recurrence of disease. As part of routine screening of all patients at our pain clinic, she was found to have severe depression as per the Patient Health Questionnaire-9 (PHQ-9) criteria...
October 2016: Korean Journal of Pain
Maura Sammon, Alveena Dawood, Scott Beaudoin, Richard A Harrigan
BACKGROUND: One of the principal tasks of an emergency physician is identifying potentially life-threatening conditions in the undifferentiated patient; cardiac dysrhythmia is an example of such a condition. A systematic approach to a patient with atypical dysrhythmia enables proper identification of such-life threatening conditions. CASE REPORT: We describe a 31-year-old man presenting to the emergency department with an undifferentiated dysrhythmia after naloxone reversal of an opiate overdose...
October 7, 2016: Journal of Emergency Medicine
Carlos Manuel Teixeira, Telmo António Pereira, Ana Margarida Lebreiro, Sérgio Alexandre Carvalho
Background: There are currently several electrocardiographic algorithms to locate the accessory pathway (AP) in patients with Wolff-Parkinson-White (WPW) syndrome. Objective: To compare the ability of electrocardiographic algorithms in identifying the location of the AP in patients with WPW pattern referred for ablation. Methods: Observational, cross-sectional, retrospective study with 111 patients with WPW syndrome referred for AP ablation...
September 12, 2016: Arquivos Brasileiros de Cardiologia
Xiaodong Yang, John Mudgett, Ghina Bou-About, Marie-France Champy, Hugues Jacobs, Laurent Monassier, Guillaume Pavlovic, Tania Sorg, Yann Herault, Benoit Petit-Demouliere, Ku Lu, Wen Feng, Hongwu Wang, Li-Jun Ma, Roger Askew, Mark D Erion, David E Kelley, Robert W Myers, Cai Li, Hong-Ping Guan
Mutations of AMPKγ2 subunit, N488I (AMPKγ2NI) and R531G (AMPKγ2RG), are associated with Wolff-Parkinson-White (WPW) syndrome, a cardiac disorder characterized by ventricular pre-excitation in humans. Cardiac-specific transgenic overexpression of human AMPKγ2NI or AMPKγ2RG leads to constitutive AMPK activation and the WPW phenotype in mice. However, overexpression of these mutant proteins also caused profound, non-physiological increase in cardiac glycogen, which might abnormally alter the true phenotype...
September 12, 2016: Journal of Biological Chemistry
Chang Xie, Ya-Ping Zhang, Lu Song, Jie Luo, Wei Qi, Jialu Hu, Danbo Lu, Zhen Yang, Jian Zhang, Jian Xiao, Bin Zhou, Jiu-Lin Du, Naihe Jing, Yong Liu, Yan Wang, Bo-Liang Li, Bao-Liang Song, Yan Yan
PRKAG2 cardiac syndrome is an autosomal dominant inherited disease resulted from mutations in the PRKAG2 gene that encodes γ2 regulatory subunit of AMP-activated protein kinase. Affected patients usually develop ventricular tachyarrhythmia and experience progressive heart failure that is refractory to medical treatment and requires cardiac transplantation. In this study, we identify a H530R mutation in PRKAG2 from patients with familial Wolff-Parkinson-White syndrome. By generating H530R PRKAG2 transgenic and knock-in mice, we show that both models recapitulate human symptoms including cardiac hypertrophy and glycogen storage, confirming that the H530R mutation is causally related to PRKAG2 cardiac syndrome...
October 2016: Cell Research
Turker Sengul, Ayten Saracoglu, Sibel Sener, Olgac Bezen
Wolff-Parkinson-White (WPW) syndrome is a rare pre-excitation syndrome which develops when atrioventricular conduction occurs through a pathologic accessory pathway known as the bundle of Kent instead of atrioventricular node, hence resulting in tachycardia. Patients with WPW syndrome may experience various symptoms arising from mild-to-moderate chest disease, palpitations, hypotension, and severe cardiopulmonary dysfunction. These patients are most often symptomatic because of cardiac arrhythmias. In this case report, we present an uneventful anesthetic management of a pregnant patient with WPW syndrome undergoing cesarean delivery...
September 2016: Journal of Clinical Anesthesia
Irene Bottillo, Carla Giordano, Bruna Cerbelli, Daniela D'Angelantonio, Martina Lipari, Taisia Polidori, Silvia Majore, Enrico Bertini, Adele D'Amico, Diana Giannarelli, Carmelilia De Bernardo, Laura Masuelli, Francesco Musumeci, Andrea Avella, Federica Re, Elisabetta Zachara, Giulia d'Amati, Paola Grammatico
BACKGROUND: Danon disease (DD) is a rare disorder characterized by cardiomyopathy, intellectual disability, and proximal myopathy. It is caused by mutations in the LAMP2 gene on X chromosome. Female patients most often present with late-onset cardiomyopathy and slow disease progression, but early-onset cases with unfavorable prognosis have been reported. CASE REPORT: We describe the clinical, pathological, and molecular features of a novel LAMP2 c.453delT mutation in a female patient with severe hypertrophic cardiomyopathy, Wolff Parkinson White (WPW) syndrome and rapid progression to heart failure, requiring heart transplant...
September 2016: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
Lijun Fu, Sushan Luo, Shuang Cai, Wenjing Hong, Ying Guo, Jinjin Wu, Tingliang Liu, Chongbo Zhao, Fen Li, Huimin Huang, Meirong Huang, Jian Wang
Danon disease is an X-linked disorder with the clinical triad of cardiomyopathy, skeletal myopathy, and mental retardation. Early diagnosis of this disease remains a challenge, especially in the pediatric population. In this study, we developed a targeted panel-based next generation sequencing pipeline to identify mutations by sequencing of selected candidate genes in 136 pediatric patients with either hypertrophic cardiomyopathy (HC) or idiopathic dilated cardiomyopathy (IDC). This led to the identification of lysosome-associated membrane protein 2 (LAMP2) mutations in 4 of the 64 (6%) probands with HC, including 3 novel nonsense mutations (p...
September 15, 2016: American Journal of Cardiology
Abdel J Fuenmayor, Francisco J Rodríguez, María Del Carmen Gutiérrez
No abstract text is available yet for this article.
October 15, 2016: International Journal of Cardiology
Enes Elvin Gül, Celal Akdeniz, Volkan Tuzcu
The atriofascicular accessory pathway (AP), known as the Mahaim pathway, is a rare form of pre-excitation, comprising less than 3% of all APs. Mahaim AP is characterized by decremental, anterograde-only conduction, and antidromic tachycardia with left bundle branch morphology. Prevalence of Mahaim AP in Ebstein's anomaly is significantly high. In addition, combination of Wolff-Parkinson-White (WPW) syndrome and Mahaim AP in patients with Ebstein's anomaly has been reported. Presently described is the coexistence of Mahaim AP and manifest WPW syndrome in a patient with Ebstein's anomaly, who was successfully ablated without fluoroscopy...
July 2016: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
Kelly Kesler, Shadi Lahham
No abstract text is available yet for this article.
July 2016: Western Journal of Emergency Medicine
Lawrence Ek Gray, Peter Vuillermin
No abstract text is available yet for this article.
June 2016: Journal of Paediatrics and Child Health
Sylvestre Maréchaux
No abstract text is available yet for this article.
June 2016: Circulation. Cardiovascular Imaging
Tomoko Ishizu, Yoshihiro Seo, Miyako Igarashi, Yukio Sekiguchi, Tomoko Machino-Ohtsuka, Kojiro Ogawa, Kenji Kuroki, Masahiro Yamamoto, Akihiko Nogami, Yasushi Kawakami, Kazutaka Aonuma
BACKGROUND: We have developed a noninvasive isochrone activation imaging (AI) system with 3-dimensional (3D) speckle tracking echocardiography (STE), which allows visualization of the wavefront image of mechanical propagation of the accessory pathway (ACP) in Wolff-Parkinson-White syndrome. METHODS AND RESULTS: Patients with manifest Wolff-Parkinson-White syndrome were imaged in 3D-STE AI mode, which quantified the time from QRS onset to regional endocardial deformation...
June 2016: Circulation. Cardiovascular Imaging
Hideki Uemura
BACKGROUND: Atrial arrhythmias are frequently described in congenital heart disease. OBJECTIVES: To provide a surgical perspective of anti-arrhythmic procedures and strategic approaches. METHODS: Discussion of the history of anti-arrhythmic treatments in congenital heart disease. RESULTS: Before the advent of the Maze procedure (first published in 1991), surgery mainly focused on patients with Wolff-Parkinson-White syndrome and also on arrhythmias in Ebstein's malformation...
June 2016: Herzschrittmachertherapie & Elektrophysiologie
Kazuma Sugie, Hiroyuki Yoshizawa, Kenji Onoue, Yoko Nakanishi, Nobuyuki Eura, Megumu Ogawa, Tomoya Nakano, Yasuhiro Sakaguchi, Yukiko K Hayashi, Toshifumi Kishimoto, Midori Shima, Yoshihiko Saito, Ichizo Nishino, Satoshi Ueno
Danon disease, primary lysosome-associated membrane protein-2 (LAMP-2) deficiency, is characterized clinically by cardiomyopathy, myopathy and intellectual disability in boys. Because Danon disease is inherited in an X-linked dominant fashion, males are more severely affected than females, who usually have only cardiomyopathy without myopathy or intellectual disability; moreover, the onset of symptoms in females is usually in adulthood. We describe a girl with Danon disease who presented with hypertrophic cardiomyopathy and Wolff-Parkinson-White (WPW) syndrome at 12 years of age...
May 5, 2016: Neuropathology: Official Journal of the Japanese Society of Neuropathology
Ahmet Taha Alper, Adnan Kaya, Ahmet İlker Tekkesin, Ahmet Öz
Fabry disease is the second most common inherited (X-linked recessive) lysosomal storage disease associated with multiple organ involvement. Cardiac involvement of Fabry disease varies. Successful radiofrequency ablation of a Fabry disease patient with Wolff-Parkinson-White syndrome and left ventricular noncompaction is described in the present report.
April 2016: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
Pravalika Deviseti, Vinayak S Pujari
Wolff-Parkinson-White (WPW) syndrome is an uncommon cardiac condition where there is an abnormal band of atrial tissue connecting atria and ventricles which can electrically bypass atrioventricular node. The anaesthetic management in these patients is challenging as life threatening complications can occur perioperatively like paroxysmal supraventricular tachycardia and atrial fibrillation. Also, regional anaesthetic technique like subarachnoid block is a safe and cost effective alternative to general anaesthesia as it avoids polypharmacy...
February 2016: Journal of Clinical and Diagnostic Research: JCDR
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