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https://www.readbyqxmd.com/read/28912181/hypertrophic-cardiomyopathy-genetics-pathogenesis-clinical-manifestations-diagnosis-and-therapy
#1
REVIEW
Ali J Marian, Eugene Braunwald
Hypertrophic cardiomyopathy (HCM) is a genetic disorder that is characterized by left ventricular hypertrophy unexplained by secondary causes and a nondilated left ventricle with preserved or increased ejection fraction. It is commonly asymmetrical with the most severe hypertrophy involving the basal interventricular septum. Left ventricular outflow tract obstruction is present at rest in about one third of the patients and can be provoked in another third. The histological features of HCM include myocyte hypertrophy and disarray, as well as interstitial fibrosis...
September 15, 2017: Circulation Research
https://www.readbyqxmd.com/read/28899955/natural-history-and-clinical-predictors-of-atrial-tachycardia-in-adults-with-congenital-heart-disease
#2
Pablo Ávila, José María Oliver, Pastora Gallego, Ana González-García, María José Rodríguez-Puras, Esther Cambronero, José Ruiz-Cantador, Ana Campos, Rafael Peinado, Raquel Prieto, Fernando Sarnago, Raquel Yotti, Francisco Fernández-Avilés
BACKGROUND: Atrial tachycardias (ATs) are a significant source of morbidity in adults with congenital heart disease (CHD). This study evaluates the incidence and clinical predictors of AT in a cohort of patients with CHD. METHODS AND RESULTS: We included 3311 adults (median age at entry 22.6 years, 50.6% males) with CHD (49% simple, 39% moderate, and 12% complex) prospectively followed up in a tertiary center for 37 607 person-years. Predictors of AT were identified by multivariable Cox regression analysis accounting for left truncation...
September 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28884991/acute-cardiac-disease-in-a-patient-with-hyper-ige-syndrome
#3
A Castilano, H Watti, A Abdulbaki, K Modi, J A Bocchini, S L Bahna
We describe the case of a 24-year-old male with hyper-IgE syndrome (HIES) which was diagnosed at 4 years of age and died from a very rare cardiac complication. He had typical clinical and laboratory manifestations of HIES, including total serum IgE as high as > 100,000 IU/mL. Stem cell transplantation was not available. During the 20-year follow-up, he suffered numerous various infections of the skin and deep organs, partial lung resection, as well as multiple bone fractures. At age 24, he developed acute decompensated heart failure associated with elevated serum troponin I and brain natriuretic protein...
September 2017: European Annals of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28878587/arrhythmogenic-right-ventricular-cardiomyopathy-in-an-endurance-athlete-presenting-with-ventricular-tachycardia-and-normal-right-ventricular-function
#4
Jeffrey S Hedley, Ibhar Al Mheid, Zoubin Alikhani, Maria A Pernetz, Jonathan H Kim
Arrhythmogenic right ventricular cardiomyopathy, a genetically inherited disease that results in fibrofatty replacement of normal cardiac myocytes, has been associated with sudden cardiac death in athletes. Long-term participation in endurance exercise hastens the development of both the arrhythmic and structural arrhythmogenic right ventricular cardiomyopathy phenotypes. We describe the unusual case of a 34-year-old, symptomatic, female endurance athlete who had arrhythmogenic right ventricular cardiomyopathy in the presence of a structurally normal right ventricle...
August 2017: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28858113/extraction-of-a-dual-chamber-pacemaker-and-inserting-of-a-new-automatic-implantable-cardioverter-defibrillator-the-easy-procedure-almost-became-catastrophic-a-case-report
#5
Márcio Galindo Kiuchi, Guilherme Miglioli Lobato, Shaojie Chen
BACKGROUND: The cardiovascular illnesses are in the middle of the foremost reasons of death around the world. Deaths in Europe, from sudden cardiac death (SCD), reach nearby 700,000 individuals every year. In the United States, statistics point to the existence of nearly 1 million yearly deaths from cardiovascular sickness, of which 330,000 are the consequence of abrupt. The significance of automatic implantable cardioverter-defibrillator (ICD) has been proven in subjects with preceding myocardial infarction and stark systolic left ventricular dysfunction (secondary prevention)...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28843747/arrhythmogenic-right-ventricular-cardiomyopathy-arvc-in-a-young-female-athlete-at-36-weeks-gestation-a-case-report
#6
Hae Yoon Grace Choung, Monika Vyas, Daniel Jacoby, Brian West
A 26year old east African professional athlete presented to the obstetric clinic for a routine visit at 36 weeks gestation. She had a history of Right Ventricular Outflow Tract - Ventricular Tachycardia (RVOT-VT) with an episode of cardiac arrest in the past, and had been treated with ablation 4 years earlier. Her current visit was uneventful, her pregnancy progressing normally. Following the visit she went to a local restaurant where she suffered a cardiac arrest that was unresponsive to therapy. Chest compressions were continued from the time of her collapse until an emergency caesarian section was performed, delivering a healthy female infant...
July 25, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28840101/radiofrequency-ablation-of-ventricular-tachycardia-originating-from-a-lipomatous-hamartoma-localized-in-the-right-ventricle-cavity
#7
Jin Xu, Yingmin Chen, Xiaoying Ying, Ben He
No abstract text is available yet for this article.
August 2017: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/28838549/arrhythmias-involving-the-atrioventricular-junction
#8
REVIEW
Luigi Di Biase, Carola Gianni, Giuseppe Bagliani, Luigi Padeletti
The atrioventricular junction has a central role in electrophysiology, responsible for reentrant and automatic forms of supraventricular tachycardia. During atrioventricular nodal reentry tachycardia, the circuit involves 2 electrophysiologically separate pathways located in the vicinity of the atrioventricular node. Atrioventricular reentry tachycardia is caused by the presence of an accessory pathway located almost anywhere along the atrioventricular groove; the macroreentrant circuit involves the atrioventricular node, the accessory pathway and necessarily portions of atria and ventricles...
September 2017: Cardiac Electrophysiology Clinics
https://www.readbyqxmd.com/read/28821295/coexistence-of-congenital-left-ventricular-aneurysm-and-prominent-left-ventricular-trabeculation-in-a-patient-with-ldb3-mutation-a-case-report
#9
Shengshuai Shan, Xiaoxiao He, Lin He, Min Wang, Chengyun Liu
BACKGROUND: The coexistence of congenital left ventricular aneurysm and abnormal cardiac trabeculation with gene mutation has not been reported previously. Here, we report a case of coexisting congenital left ventricular aneurysm and prominent left ventricular trabeculation in a patient with LIM domain binding 3 gene mutation. CASE PRESENTATION: A 30-year-old Asian man showed paroxysmal sinus tachycardia and Q waves in an electrocardiogram health check. There were no specific findings in physical examinations and serological tests...
August 19, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28804588/first-reported-case-of-arrhythmogenic-right-ventricular-cardiomyopathy-in-oman
#10
Hatim Al Lawati, Humoud Al Dhuhli
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetic disorder characterized by fatty degeneration of the right ventricular myocardium with variable involvement of the left ventricle. The condition is associated with exercise-mediated ventricular tachycardia and is one of the recognized causes of sudden cardiac death in the young and in athletes. Here, we report the first confirmed case of ARVC in Oman and present its electrocardiographic, echocardiographic features, and radiological findings on gated, contrast-enhanced cardiac computed tomography...
July 2017: Oman Medical Journal
https://www.readbyqxmd.com/read/28798020/unipolar-endocardial-voltage-mapping-in-the-right-ventricle-optimal-cutoff-values-correcting-for-computed-tomography-derived-epicardial-fat-thickness-and-their-clinical-value-for-substrate-delineation
#11
Jeroen Venlet, Sebastiaan R D Piers, Gijsbert F L Kapel, Marta de Riva, Philippe F G Pauli, Rob J van der Geest, Katja Zeppenfeld
BACKGROUND: Low endocardial unipolar voltage (UV) at sites with normal bipolar voltage (BV) may indicate epicardial scar. Currently applied UV cutoff values are based on studies that lacked epicardial fat information. This study aimed to define endocardial UV cutoff values using computed tomography-derived fat information and to analyze their clinical value for right ventricular substrate delineation. METHODS AND RESULTS: Thirty-three patients (50±14 years; 79% men) underwent combined endocardial-epicardial right ventricular electroanatomical mapping and ablation of right ventricular scar-related ventricular tachycardia with computed tomographic image integration, including computed tomography-derived fat thickness...
August 2017: Circulation. Arrhythmia and Electrophysiology
https://www.readbyqxmd.com/read/28775062/de-novo-fgf12-fibroblast-growth-factor-12-functional-variation-is-potentially-associated-with-idiopathic-ventricular-tachycardia
#12
Qianqian Li, Yuanyuan Zhao, Gang Wu, Shanshan Chen, Yingchao Zhou, Sisi Li, Mengchen Zhou, Qian Fan, Jielin Pu, Kui Hong, Xiang Cheng, Qing Kenneth Wang, Xin Tu
BACKGROUND: Idiopathic ventricular tachycardia (VT) is a type of cardiac arrhythmia occurring in structurally normal hearts. The heritability of idiopathic VT remains to be clarified, and numerous genetic factors responsible for development of idiopathic VT are as yet unclear. Variations in FGF12 (fibroblast growth factor 12), which is expressed in the human ventricle and modulates the cardiac Na(+) channel NaV1.5, may play an important role in the genetic pathogenesis of VT. METHODS AND RESULTS: We tested the hypothesis that genetic variations in FGF12 are associated with VT in 2 independent Chinese cohorts and resequenced all the exons and exon-intron boundaries and the 5' and 3' untranslated regions of FGF12 in 320 unrelated participants with idiopathic VT...
August 3, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28770255/massive-accumulation-of-myofibroblasts-in%C3%A2-the-critical-isthmus-is-associated-with%C3%A2-ventricular-tachycardia-inducibility-in%C3%A2-post-infarct-swine-heart
#13
Tarvinder S Dhanjal, Nicolas Lellouche, Christopher J von Ruhland, Guillaume Abehsira, David H Edwards, Jean-Luc Dubois-Randé, Konstantinos Moschonas, Emmanuel Teiger, Alan J Williams, Christopher H George
OBJECTIVES: In this study the authors determined the extent of cellular infiltration and dispersion, and regional vascularization in electrophysiologically (EP) defined zones in post-myocardial infarction (MI) swine ventricle. BACKGROUND: The critical isthmus (CI) in post-MI re-entrant ventricular tachycardia (VT) is a target for catheter ablation. In vitro evidence suggests that myofibroblasts (MFB) within the scar border zone (BZ) may increase the susceptibility to slow conduction and VT, but whether this occurs in vivo remains unproven...
July 2017: JACC. Clinical Electrophysiology
https://www.readbyqxmd.com/read/28765748/catheter-ablation-of-peri-conduit-ventricular-tachycardia-in-a-patient-with-rastelli-procedure-for-double-outlet-right-ventricle-with-malposition-of-great-arteries
#14
Abigail Louise D Te, Fa-Po Chung, Chin Yu Lin, Atul Prabhu, Pi-Chang Lee, Shih-Ann Chen
No abstract text is available yet for this article.
July 2017: Korean Circulation Journal
https://www.readbyqxmd.com/read/28765737/local-atrial-ventricular-ratio-as-an-adjuvant-marker-for-catheter-ablation-of-atrioventricular-accessory-pathways
#15
Ki-Hun Kim, Dae-Kyeong Kim, Hyun-Ji Im, Jeong-Sook Seo, Han-Young Jin, Jae-Sik Jang, Tae-Hyun Yang, Dong-Soo Kim, So-Young Jeong, Yun Seok Song, Dong-Kie Kim, Pil-Sang Song, Sang-Hoon Seol, Doo-Il Kim
BACKGROUND AND OBJECTIVES: The earliest atrial (A)/ventricular (V) activation potential, or accessory pathway (AP) potential are commonly used as ablation targets for atrioventricular (AV) APs. However, these targets are sometimes ambiguous. SUBJECTS AND METHODS: We reviewed 119 catheter ablation cases in 112 patients diagnosed with orthodromic atrioventricular reentrant tachycardia (AVRT) or Wolff-Parkinson-White (WPW) syndrome. Local A/V amplitude potentials with the earliest activation or AP potential were measured shortly before achieving antegrade AP conduction block, ventriculoatrial block during right ventricle (RV) pacing, or AVRT termination with no AP conduction...
July 2017: Korean Circulation Journal
https://www.readbyqxmd.com/read/28744873/epicardial-ablation-of-tachyarrhythmia-in-children-experience-at-two-academic-centers
#16
Shailendra Upadhyay, Edward P Walsh, Frank Cecchin, John K Triedman, Juan Villafane, J Philip Saul
BACKGROUND: Experience with percutaneous epicardial ablation of tachyarrhythmia in pediatrics is limited. This case series addresses the feasibility, safety, and complications of the procedure in children. METHODS: A total of nine patients underwent 10 epicardial ablation procedures from 2002 to 2013 at two academic centers. Activation mapping was performed in all cases, and electroanatomic map was utilized in nine of the 10 procedures. Patients had undergone one to three failed endocardial catheter ablations in addition to medical management, and all had symptoms, a high-risk accessory pathway (AP), aborted cardiac arrest with Wolff-Parkinson-White syndrome (WPW), or ventricular dysfunction...
July 26, 2017: Pacing and Clinical Electrophysiology: PACE
https://www.readbyqxmd.com/read/28719516/echocardiography-and-passive-leg-raising-in-the-postoperative-period-a-prospective-observational-study
#17
Yasser El Hadouti, Lucía Valencia, Angel Becerra, Aurelio Rodríguez-Pérez, Jean L Vincent
BACKGROUND: Signs of hypovolaemia are frequent in the postoperative period, but not all patients need or respond to fluid administration. An increase in cardiac output (CO) after passive leg raising (PLR) has been demonstrated to be useful as a volume response predictor in non-surgical, spontaneously breathing patients. OBJECTIVE: The objective of this study was to evaluate the accuracy of transthoracic echocardiography after PLR to predict fluid responsiveness in post-surgical patients...
July 15, 2017: European Journal of Anaesthesiology
https://www.readbyqxmd.com/read/28690945/pacemaker-placement-in-persistent-left-superior-vena-cava
#18
Murtaza Sundhu, Mubbasher Syed, Sajjad Gul, Bilal Saqi, Robert Mosteller
Persistent left superior vena cava (PLSVC) is a rare disorder which is asymptomatic and hence is usually discovered while performing interventions through the left subclavian vein. We present a case of a 78-year-old male who was undergoing elective placement of a permanent pacemaker for tachycardia - bradycardia syndrome with post-conversion pauses of up to nine seconds. After achieving access through the left subclavian vein the wire kept on going on the left side of the chest instead of crossing the midline to the right side...
June 5, 2017: Curēus
https://www.readbyqxmd.com/read/28680626/years-of-palpitations-and-a-heart-rate-of-213-beats-per-minute
#19
Kevin Lee, Joseph Banta, Matthew D'Ambrosio, Apostolos Voudouris, Antonios Tsompanidis
Belhassen tachycardia is the most common idiopathic ventricular tachycardia arising from the left ventricle, classically characterized by a right bundle branch block and left axis deviation. Vigilance for Belhassen tachycardia is essential as intravenous verapamil has proven to be highly efficacious for treating symptomatic patients with this underlying arrhythmia.
July 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28638576/a-classic-case-of-arrhythmogenic-right-ventricular-cardiomyopathy-arvc-and-literature-review
#20
Htun Latt, Thein Tun Aung, Chanwit Roongsritong, David Smith
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a relatively under-recognized hereditary cardiomyopathy. It is characterized pathologically by fibro-fatty infiltration of right ventricular (RV) myocardium and clinically by consequences of RV electrical instability. Timely intervention with device therapy and pharmacotherapy may help reduce the risk of arrhythmic events or sudden cardiac death. Here, we describe a classic case of a young adult with ARVC and a brief literature review. The patient presented with exertional palpitations and ARVC was suspected after his routine electrocardiogram (EKG) revealed symmetric T wave inversions and possible epsilon waves in right precordial leads...
March 2017: Journal of Community Hospital Internal Medicine Perspectives
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