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Özlem Sarısoy, Canan Ayabakan, Kürşad Tokel, Murat Özkan, Rıza Türköz, Sait Aşlamacı
OBJECTIVE: The follow-up results of patients operated for atrioventricular septal defect (AVSD) during 1996-2016 at Başkent University are presented. METHODS: Data obtained from hospital records consists of preoperative echocardiographic and angiographic details, age and weight at surgery, operative details, Down syndrome presence, postoperative care details, early postoperative and latest echocardiographic findings and hospitalization for reintervention. RESULTS: A total of 496 patient-files were reviewed including 314 patients (63...
October 2018: Anatolian Journal of Cardiology
Robert S Stephenson, Jack Rowley-Nobel, Caroline B Jones, Rafael Guerrero, Tristan Lowe, Jichao Zhao, Henggui Zhang, Jonathan C Jarvis
Due to advances in corrective surgery, congenital heart disease has an ever growing patient population. Atrial arrhythmias are frequently observed pre- and post-surgical correction. Pharmaceutical antiarrhythmic therapy is not always effective, therefore many symptomatic patients undergo catheter ablation therapy. In patients with atrioventricular septal defects (AVSD), ablation therapy itself has mixed success; arrhythmogenic recurrences are common, and because of the anatomical displacement of the atrioventricular node, 3-degree heart block post-ablation is a real concern...
2018: Frontiers in Physiology
Donghua Xie, Junqun Fang, Zhiyu Liu, Hua Wang, Tubao Yang, Zhenqiu Sun, Aihua Wang, Lili Xiong
Congenital heart defects (CHDs) are the most common birth defects (BDs) and account for nearly one-third of all BDs. The aim of this article was to investigate the epidemiology and major subtypes of CHDs in Hunan Province, China in the last 5 years.CHD surveillance data from 2012 to 2016 were collected from 52 registered hospitals in Hunan. The prevalence rates of CHDs, incidence rates of CHDs combined with other BDs, and rates of termination of pregnancy (TOP) for CHDs among different regions, infant sexes, and maternal ages were calculated for both early fetuses (<28 weeks of gestation) and perinatal infants (PIs) (between 28 weeks of gestation and 7 days after birth)...
August 2018: Medicine (Baltimore)
Rosangela Ferese, Monica Bonetti, Federica Consoli, Valentina Guida, Anna Sarkozy, Francesca Romana Lepri, Paolo Versacci, Stefano Gambardella, Giulio Calcagni, Katia Margiotti, Francesca Piceci Sparascio, Hossein Hozhabri, Tommaso Mazza, Maria Cristina Digilio, Bruno Dallapiccola, Marco Tartaglia, Bruno Marino, Jeroen den Hertog, Alessandro De Luca
Atrioventricular septal defect (AVSD) may occur as part of a complex disorder (e.g., Down syndrome, heterotaxy), or as isolate cardiac defect. Multiple lines of evidence support a role of calcineurin/NFAT signaling in AVSD, and mutations in CRELD1, a protein functioning as a regulator of calcineurin/NFAT signaling have been reported in a small fraction of affected subjects. In this study, 22 patients with isolated AVSD and 38 with AVSD and heterotaxy were screened for NFATC1 gene mutations. Sequence analysis identified three missense variants in three individuals, including a subject with isolated AVSD [p...
October 2018: Human Mutation
Tommaso Generali, Shady El Sayed, Vinay Rao, Carlos Pardo, Stefano Congiu, Osama Jaber, Carin van Doorn
OBJECTIVE: Left atrio-ventricular valve (LAVV) regurgitation after repair of an atrio-ventricular septal defect (AVSD) may necessitate further surgery. However, redo-LAVV repair remains challenging. We sought to determine if more LAVV valves are preserved in the current era, and analyze early and longer-term results. PATIENTS: All consecutive patients with repaired AVSD who underwent redo-LAVV surgery from January 2004 to April 2017 were included. Patients with single ventricles, atrial isomerism, and complex associated anomalies were excluded...
August 2018: Journal of Cardiac Surgery
Corrado Di Mambro, Camilla Calvieri, Massimo Stefano Silvetti, Ilaria Tamburri, Salvatore Giannico, Anwar Baban, Sonia Albanese, Gianluca Brancaccio, Adriano Carotti, Fiore Salvatore Iorio, Fabrizio Drago
Atrioventricular Septal Defect (AVSD) is a rare congenital heart defect (CHD) often associated with genetic syndromes, most commonly Down syndrome (DS). Over the last four decades, surgical repair has increased survival and improved quality of life in these patients. The prevalence of bradyarrhythmias namely, atrioventricular block (AVB) and sinus node dysfunction (SND) in AVSD is partially known. 522 cases with both partial and complete AVSD (38.7% with DS), undergoing intracardiac repair from 1982 to 2016 at our institution, were reviewed from our system database...
June 13, 2018: Pediatric Cardiology
Katie E O' Sullivan, Robert Fleck, Gaia Vigano, Gerard J Fitzmaurice, Paul Cullen, Daniyal Arshad, Mohammad Abdelrahman, Alfred E Wood, Jonathan Mc Guinness, J Mark Redmond, Lars Nölke
BACKGROUND: We describe the long-term results of partial atrioventricular septal defect (AVSD) repair in a single centre encompassing a 22-year period. Described are rates of survival, reoperation and complications. METHODS: We performed a retrospective review of 556 patients undergoing AVSD repair to identify the 51 patients who underwent partial AVSD repair in Our Lady's Children's Hospital, Crumlin, Ireland, between 1993 and 2015 with long-term follow-up where available...
June 25, 2018: Irish Journal of Medical Science
Seijirow Goya, Nobuyuki Kanno, Kenji Teshima, Takanori Anndo, Takahiro Fujioka
A 4-year-old, 5.9-kg female Japanese Spitz presented with syncope and exercise intolerance. Echocardiography revealed an ostium primum atrial septal defect (ASD), a cleft mitral valve, mitral valve regurgitation (MR), and tricuspid regurgitation (TR) (velocity: 3.6 m/sec, pressure gradient: 52 mmHg), leading to a diagnosis of partial atrioventricular septal defect (AVSD) with moderate pulmonary hypertension (PH). Open-heart surgery using cardiopulmonary bypass was performed through right atriotomy. The cleft of the mitral valve was sutured with polypropylene and the AVSD was closed using an autologous pericardial patch fixed with glutaraldehyde...
July 18, 2018: Journal of Veterinary Medical Science
Tracy R Geoffrion, Kanchana Singappuli, John S K Murala
Atrioventricular septal defect (AVSD) is a common congenital cardiac surgical problem. Over the years, younger and smaller infants are having operations for this condition before irreversible cardio pulmonary changes occur. Traditionally a single or two patch techniques have been used to repair this defect. However, in the past two decades an innovative method of modified single patch technique popularized by Dr. Graham Nunn has gained worldwide popularity. This review discusses the origin, surgical principles, technique and outcomes of this method, popularly known as Nunn or Australian technique...
April 2018: Translational Pediatrics
P Syamasundar Rao, Andrea D Harris
This review discusses the management of ventricular septal defects (VSDs) and atrioventricular septal defects (AVSDs). There are several types of VSDs: perimembranous, supracristal, atrioventricular septal, and muscular. The indications for closure are moderate to large VSDs with enlarged left atrium and left ventricle or elevated pulmonary artery pressure (or both) and a pulmonary-to-systemic flow ratio greater than 2:1. Surgical closure is recommended for large perimembranous VSDs, supracristal VSDs, and VSDs with aortic valve prolapse...
2018: F1000Research
Michele Santoro, Alessio Coi, Isabella Spadoni, Fabrizio Bianchi, Anna Pierini
BACKGROUND: Down syndrome (DS) is the most common autosomal chromosomal anomaly in liveborn infants. About 40% of infants with DS have a major congenital heart defect (CHD). Among them, atrioventricular septal defects (AVSD), atrial septal defects (ASD), ventricular septal defect (VSD) and Tetralogy of Fallot (ToF) are the most common. The aim of this study was to estimate the sex difference in the occurrence of CHD in infants with DS comparing it with non-DS infants. METHOD: Live birth cases of DS diagnosed by the first year of life were extracted from the Registry of Congenital Anomalies of Tuscany (2003-2015 period)...
September 2018: European Journal of Medical Genetics
Xiaoyong Pan, Xiaohua Hu, Yu Hang Zhang, Kaiyan Feng, Shao Peng Wang, Lei Chen, Tao Huang, Yu Dong Cai
Atrioventricular septal defect (AVSD) is a clinically significant subtype of congenital heart disease (CHD) that severely influences the health of babies during birth and is associated with Down syndrome (DS). Thus, exploring the differences in functional genes in DS samples with and without AVSD is a critical way to investigate the complex association between AVSD and DS. In this study, we present a computational method to distinguish DS patients with AVSD from those without AVSD using the newly proposed self-normalizing neural network (SNN)...
April 12, 2018: Genes
Edward Buratto, Brandon Khoo, Xin Tao Ye, Michael Daley, Christian P Brizard, Yves d'Udekem, Igor E Konstantinov
BACKGROUND: Patients with atrioventricular septal defect (AVSD) may require pulmonary artery banding (PAB), either as a part of a staged univentricular palliation or to allow delayed biventricular repair in patients presenting with early heart failure. The long-term outcomes of PAB in children with AVSD have not been previously reported. METHODS: All children with AVSD who underwent PAB at a single institution were included in the study. Data were obtained from medical records and correspondence with general practitioners and cardiologists...
July 2018: Annals of Thoracic Surgery
Erkut Ozturk, Ibrahim Cansaran Tanidir, Pelin Ayyildiz, Selman Gokalp, Hasan Candas Kafali, Murat Sahin, Yakup Ergul, Sertac Haydin, Alper Guzeltas
AIM: Our aim was to evaluate the findings and the role of intraoperative epicardial echocardiography (IEE) in the management of pediatric cardiac surgery patients. METHODS: Patients evaluated with IEE between December 2015 and December 2017 were analyzed retrospectively. Demographic data, preoperative transthoracic echocardiography (TTE), and IEE reports were evaluated. RESULTS: A total of 410 patients evaluated by IEE were included in the study...
July 2018: Echocardiography
Erol Arslan, Selim Büyükkurt, Mete Sucu, Mehmet Özsürmeli, Selahattin Mısırlıoğlu, S. Cansun Demir, İ. Cüneyt Evrüke
Objective: Fetal structural malformations affect approximately 2-3% of all pregnancies. Only focusing on trisomy screening in first trimester and deferring the anatomic screening to second trimester may result with late detection of major anomalies that can be diagnosed earlier with careful examination. Material and Methods: This was a descriptive study of retrospective data that were obtained from all terminated single pregnancies due to ultrasonographic findings of major anomalies from 2011 to 2016 in our department...
August 6, 2018: Journal of the Turkish German Gynecological Association
Saima Bibi, Syed Yasir Hussain Gilani, Shawana Bibi
BACKGROUND: Congenital heart disease is a significant problem world over especially in neonates. Early diagnosis and prompt interventions in neonatal period precludes the mortality associated with this disorder. The objective of this study was to highlight the diversity of congenital cardiac defects in our region so that appropriate interventions are devised to minimize significant morbidity and mortality associated with this disorder. METHODS: This descriptive cross-sectional study was conducted at the Neonatology Unit of Department of Paediatrics, Ayub Teaching Hospital from January 2015 to December 2016...
January 2018: Journal of Ayub Medical College, Abbottabad: JAMC
Antigoni Deri, Kate English
This review article will guide the reader through the basics of echocardiographic assessment of congenital left to right shunts in both paediatric and adult age groups. After reading this article, the reader will understand the pathology and clinical presentation of atrial septal defects (ASDs), ventricular septal defects (VSDs), atrioventricular septal defects (AVSDs) and patent arterial duct. Echocardiography is the mainstay in diagnosis and follow-up assessment of patients with congenital heart disease. This article will therefore describe the echocardiographic appearances of each lesion, and point the reader towards specific features to look for echocardiographically...
March 2018: Echo Research and Practice
James M Meza, Paul J Devlin, David M Overman, David Gremmels, Gina Baffa, Meryl S Cohen, Michael D Quartermain, Christopher A Caldarone, Kamal Pourmoghadam, William M DeCampli, Cheryl T Fackoury, Luc Mertens
Quantifying unbalance, the threshold for single ventricle palliation vs biventricular repair in patients with unbalanced complete atrioventricular septal defect (AVSD), is challenging. Using a core laboratory review of baseline echocardiograms, we sought to assess the correlations among commonly used measures of unbalance and common atrioventricular valve (AVV) and ventricular sizes. A single reviewer evaluated baseline echocardiograms from an inception cohort of babies < age 1 year with complete AVSD admitted to 1 of 25 Congenital Heart Surgeon's Society institutions...
February 8, 2018: Seminars in Thoracic and Cardiovascular Surgery
Thierry Bové, Ine Strubbe, Kristof Vandekerckhove, Joseph Panzer, Katya De Groote, Daniel De Wolf, Katrien François
OBJECTIVES: Our goal was to investigate the outcome of the left atrioventricular valve (LAVV) after repair of atrioventricular septal defects (AVSDs) by analysing the mechanisms of LAVV failure. METHODS: A retrospective investigation of 202 children with AVSD, operated on between 1992 and 2016, was performed. Analysis focused on freedom from reoperation for LAVV dysfunction, with specific attention to the modes of failure. RESULTS: The population consisted of 129 (64%) patients with complete AVSD (cAVSD) and 73 (36%) patients with partial AVSD (pAVSD), corrected at a median age of 3...
July 1, 2018: Interactive Cardiovascular and Thoracic Surgery
Jason Chui, Robert H Anderson, Roberto M Lang, Wendy Tsang
With the advent of 3-dimensional echocardiography, visualization of the mitral valve has greatly improved. Recently, there has been an increase in reporting of a distinct entity called the "trileaflet mitral valve" using 3-dimensional echocardiography. It is controversial whether this is a new entity or an improved visualization of isolated mitral valve clefts or trifoliate left atrioventricular valve in the setting of an atrioventricular septal defect (AVSD) with intact septum. We present a case of a trifoliate valve, interpreting our findings based on a systematic review of previous publication on trileaflet mitral valves, isolated clefts in the mural (posterior) leaflet of the mitral valve, and trifoliate left atrioventricular valves with AVSD and intact septal structures...
February 15, 2018: American Journal of Cardiology
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