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congenital heart block

Andrés Alonso-García, Felipe Atienza, Pablo Ávila, Clara Ugueto, Miriam Centeno, Reyes Álvarez, Tomás Datino, Esteban González-Torrecilla, Evaristo Castellanos, Gerard Loughlin, Constancio Medrano, Ángel Arenal, Francisco Fernández-Avilés
INTRODUCTION AND OBJECTIVES: Catheter ablation has become the treatment of choice in an increasing number of arrhythmias in children and adolescents. There is still limited evidence of its use at a national level in Spain. The aim was to describe the characteristics and results of a modern monocentric series form a referral tertiary care centre. METHODS: Retrospective register of invasive procedures between 2004 and 2016 performed in patients under 17 years and recorded clinical characteristic, ablation methodology and acute and chronic results of the procedure...
February 23, 2018: Revista Española de Cardiología
Taner Kasar, Pelin Ayyildiz, Gulhan Tunca Sahin, Erkut Ozturk, Selman Gokalp, Sertac Haydin, Alper Guzeltas, Yakup Ergul
BACKGROUND: We aimed to evaluate rhythm abnormalities in cases of congenitally corrected transposition of the great arteries (ccTGA) and associated treatment strategies. PATIENTS AND METHODS: This retrospective cohort study included 65 pediatric patients with ccTGA who were admitted to the clinic between 2009 and 2017. The patients were divided into two groups, and surgical data, Holter electrocardiographic (ECG) recordings, ECG recordings, electrophysiological data, and device implantation data on the two groups were compared...
February 24, 2018: Congenital Heart Disease
Peter Brocklehurst, David Field, Keith Greene, Edmund Juszczak, Sara Kenyon, Louise Linsell, Chris Mabey, Mary Newburn, Rachel Plachcinski, Maria Quigley, Philip Steer, Liz Schroeder, Oliver Rivero-Arias
BACKGROUND: Continuous electronic fetal monitoring (EFM) in labour is widely used and computerised interpretation has the potential to increase its utility. OBJECTIVES: This trial aimed to find out whether or not the addition of decision support software to assist in the interpretation of the cardiotocograph (CTG) reduced the number of poor neonatal outcomes, and whether or not it was cost-effective. DESIGN: Two-arm individually randomised controlled trial...
February 2018: Health Technology Assessment: HTA
Xing Liu, Jianmei Zheng, Zhongcai Fan, Li Rao
RATIONALE: Brugada syndrome (BrS) is a cardiac ion channel disease that is caused by an autosomal dominant genetic abnormality. A ventricular septal defect is a common congenital heart disease, in which genetic defects play a significant role. PATIENT CONCERNS: We report an extremely rare case of a 42-year-old male with congenital heart disease, who suffered recurrent syncope and gastrointestinal bleeding. His electrocardiogram showed an unusual right bundle branch block-like pattern and ST-segment elevation in leads V1-V3...
November 2017: Medicine (Baltimore)
Manoj Kumar Sahu, Anupam Das, Bharat Siddharth, Sachin Talwar, Sarvesh Pal Singh, Atul Abraham, Arin Choudhury
BACKGROUND: Postoperative arrhythmias are a known complication after cardiac surgical repairs for congenital heart disease. METHODS: Data were reviewed pertaining to incidence, diagnosis, potential risk factors, and management of postoperative arrhythmias in 369 consecutive patients under 18 years of age, undergoing elective open heart surgery. All children were admitted to the intensive care unit and continuous electrocardiographic monitoring was performed. Patient factors such as Aristotle Basic Complexity Score, total surgical duration, hypotension, tachycardia, serum lactate level, and inotropic score were analyzed...
January 2018: World Journal for Pediatric & Congenital Heart Surgery
Elena Mattia, Ariela Hoxha, Marta Tonello, Maria Favaro, Teresa Del Ross, Antonia Calligaro, Anna Ghirardello, Amelia Ruffatti
BACKGROUND: Anti-p200 antibodies have been receiving growing interest in view of findings associating their presence to risk of fetal autoimmune congenital heart block (CHB). The study compares and evaluates the performance of two assays currently being used for their detection. METHODS: One hundred and sixteen pregnant women positive for anti-SSA/Ro52 antibodies were considered as the study population. Fifty women negative for anti-SSA/Ro52 antibodies were considered as the control population...
January 5, 2018: Clinical Chemistry and Laboratory Medicine: CCLM
Gemma Penford, Daniel Quandt, Chetan Mehta, Vinay Bhole, Rami Dhillon, Anna Seale, Oliver Stumper
BACKGROUND: Gore-Tex® grafts are integral in the management of congenital heart disease. Issues of graft stenosis or somatic outgrowth may precipitate high-risk early surgery, and catheter intervention is a relatively under-reported management option. OBJECTIVES: To assess efficacy, safety, and outcomes of stenting and overdilating small Gore-Tex® vascular grafts with the aim of optimizing surgical timing. METHODS: Retrospective analysis of single-center, 13-year experience of 93 graft stenting interventions or reintervention in 80 patients, with the aim of relieving stenosis ± overdilation, to depose surgical graft revision or optimize surgical timing and candidacy...
December 20, 2017: Catheterization and Cardiovascular Interventions
Ai Kojima, Fumiaki Shikata, Toru Okamura, Takashi Higaki, Seiko Ohno, Minoru Horie, Shunji Uchita, Yujiro Kawanishi, Kenji Namiguchi, Takumi Yasugi, Hironori Izutani
BACKGROUND: Congenital long QT syndrome (LQTS) can cause ventricular arrhythmic events with syncope and sudden death resulting from malignant torsades de pointes (TdP) followed by ventricular fibrillations (VFs). However, the syndrome is often overlooked prior to the development of arrhythmic events in patients with congenital heart diseases demonstrating right bundle branch block on electrocardiogram (ECG). We present a case of an adult patient with congenital heart disease who developed VFs postoperatively, potentially due to his mutation in a LQTS related gene, which was not identified on preoperative assessment due to incomplete evaluation of his family history...
December 19, 2017: Journal of Cardiothoracic Surgery
Danielle E Harake, Kevin M Shannon, Jamil A Aboulhosn, Jeremy P Moore
INTRODUCTION: The bidirectional Glenn operation for congenital heart disease produces anatomical constraints to conventional transvenous pacemaker implantation. An iliac approach, although not previously described in this population, is potentially a preferable alternative to a thoracotomy for epicardial pacing. METHODS AND RESULTS: A single-center retrospective review was performed for all patients that underwent transvenous pacemaker implantation following the bidirectional Glenn operation with partial biventricular repair...
December 14, 2017: Journal of Cardiovascular Electrophysiology
Rebecca Fischer-Betz, Christof Specker
Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease with a high prevalence in females of childbearing age. Pregnancy in SLE nowadays has favorable outcomes for the majority of women. However, flares of disease activity, preeclampsia, fetal loss, and preterm birth are well-known risks in such pregnancies. Anti-SS-A(Ro)/SS-B(La) antibodies put fetuses at risk for congenital heart block and neonatal lupus. Several risk factors for adverse pregnancy outcomes have been identified. Women with antiphospholipid antibodies or antiphospholipid syndrome and lupus nephritis represent a group with high risk for obstetric complications...
June 2017: Best Practice & Research. Clinical Rheumatology
Jürgen Hörer, Régine Roussin, Emanuel LeBret, Mohamed Ly, Jarrah Abdullah, Rafaella Marzullo, Jelena Pabst von Ohain, Emre Belli
OBJECTIVES: Adults with congenital heart disease in need of heart surgery frequently present with significant comorbidity. Furthermore, additional technical difficulties often related to redo operations increase the risk for postoperative mortality and morbidity. Hence, next to the type of the procedure, additional procedure-dependent and procedure-independent factors have to be considered for risk evaluation. The recently proposed grown-ups with congenital heart disease (GUCH) mortality and morbidity scores account for these additional risk factors...
December 1, 2017: Heart: Official Journal of the British Cardiac Society
Christophe Garweg, Joris Ector, Gabor Voros, Adèle Greyling, Bert Vandenberk, Stefaan Foulon, Rik Willems
AIM: Leadless cardiac pacemaker has been developed to reduce complications related to cardiac pacing and is considered as an alternative to conventional pacemaker although safety and efficacy data in clinical practice are limited. The purpose of this study was to investigate the safety and efficacy profile of Micra Transcatheter Pacing System (TPS) used in daily clinical activity with a focus on challenging cases for conventional pacing. METHODS: A total of 66 patients (46 men, 79...
November 30, 2017: Acta Cardiologica
Iuliu Scurtu, Flaviu Tabaran, Mircea Mircean, Gavril Giurgiu, Andras Nagy, Cornel Catoi, Dan G Ohad
BACKGROUND: Double chambered right ventricle (DCRV) is a congenital heart anomaly where the right ventricle is divided into two chambers. We describe, for the first time, an unusual combination of DCRV combined with some other congenital heart defects. CASE PRESENTATION: A 1.2-year-old Golden Retriever was presented with lethargy, exercise intolerance and ascites. Physical examination revealed an irregularly irregular pulse and a grade V/VI, systolic, right cranial murmur...
November 29, 2017: BMC Veterinary Research
Kai-Yu Zhou, Yi-Min Hua
OBJECTIVE: Congenital heart block (CHB) is a rare but life-threatening disorder. More than half of CHB cases are associated with maternal autoimmune, which are termed as autoimmune-associated CHB. This review summarized the recent research findings in understanding autoimmune-associated CHB, discussed the current diagnostic approaches and management strategies, and summarized the problems and future directions for this disorder. DATA SOURCES: We retrieved the articles published in English from the PubMed database up to January 2017, using the keywords including "Autoimmune-associated", "Autoimmune-mediated", and "Congenital heart block"...
December 5, 2017: Chinese Medical Journal
Claudia Renaud, Edgar Jaeggi, Varsha Thakur
Fetal hydrops is a serious complication of immune-mediated congenital complete atrioventricular block. We present the case of a fetus with severe hydrops and profound bradycardia and an unusual favourable outcome. This case enhances the importance of considering the contribution of ventricular ectopic beats to the cardiac output when counselling and predicting outcome of complete heart block.
January 2018: Cardiology in the Young
Hannah C Ainsworth, Miranda C Marion, Tiziana Bertero, Antonio Brucato, Rolando Cimaz, Nathalie Costedoat-Chalumeau, Micaela Fredi, Patrick Gaffney, Jennifer Kelly, Kateri Levesque, Alice Maltret, Nathalie Morel, Veronique Ramoni, Amelia Ruffatti, Carl D Langefeld, Jill P Buyon, Robert M Clancy
OBJECTIVE: Fetal exposure to maternal anti-SSA/Ro antibodies is necessary but not sufficient for the development of autoimmune congenital heart block (CHB), suggesting that other factors, such as fetal genetic predisposition, are important. Given the previously described association between major histocompatibility complex alleles and CHB risk, we undertook the present study to test the hypothesis that a variant form of HLA-C Asn80Lys, which binds with high affinity to an inhibitory killer cell immunoglobulin-like receptor (KIR) and thus renders natural killer (NK) cells incapable of restricting inflammation, contributes to the development of CHB...
November 2017: Arthritis & Rheumatology
Fuanglada Tongprasert, Rekwan Sittiwangkul, Thitikarn Lerthiranwong, Theera Tongsong
Maternal autoantibodies to Ro/SSA are often linked to congenital heart block and rarely associated with structural defects. We describe the case of a fetus with anti-Ro-mediated second-degree block at 19 weeks, which progressed to a complete block, fibroelastosis, atrioventricular valve insufficiency, and semilunar valve stenosis/insufficiency at 20, 22, 24, and 26 weeks, respectively, although the fetus received transplacental anti-arrhythmic drugs. The 2150-g fetus was vaginally delivered at 35 weeks. An external pacemaker was inserted immediately after birth and replaced with permanent pacemaker at the age of 3 months...
October 12, 2017: Journal of Clinical Ultrasound: JCU
Ashwin Kodliwadmath
Congenitally corrected transposition of great vessels (CCTGV) is a rare congenital heart disease (CHD) accounting for <1% of CHDs. CCTGV with infundibular pulmonary stenosis (PS) with ventricular septal defect (VSD) is part of Fallot's physiology. It is known to be associated with bradyarrhythmias like atrioventricular (AV) blocks, and acquired complete AV block occurs at a rate of 2% per year. Patients can have left-sided accessory pathways, which may cause atrioventricular reentrant tachycardia (AVRT)...
2017: International Medical Case Reports Journal
Haitham A Badran, M A Abdelhamid, Mazen T Ibrahim, Ayman M Abdelmoteleb, John K Zarif
BACKGROUND: Cardiac resynchronization therapy (CRT) is an established treatment for patients with advanced heart failure that results in improvement of left ventricle (LV) systolic function and LV reverse remodeling. This may have a positive effect on the size and the function of the left atrium (LA). We assessed the LA function, dimensions, and volumes before and after CRT implantation. METHODS: A total of 37 patients with mean age of 55.3 ± 9.64 years including 11 (29...
October 2017: Journal of the Saudi Heart Association
Laura G Bracaglia, Michael Messina, Shira Winston, Che-Ying Kuo, Max Lerman, John P Fisher
Vascular grafts that can support total replacement and maintenance by the body of the injured vessel would improve outcomes of major surgical reconstructions. Building scaffolds using components of the native vessel can encourage biological recognition by native cells as well as mimic mechanical characteristics of the native vessel. Evidence is emerging that incorporating predetermined building-blocks into a tissue engineering scaffold may oversimplify the environment and ignore critical structures and binding sites essential to development at the implant...
October 16, 2017: Biomacromolecules
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