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Akimichi Shibata, Hiroki Mori, Kazuki Kodo, Toshio Nakanishi, Hiroyuki Yamagishi
BACKGROUND: Recent progress in surgical and intensive care has improved the prognosis of congenital heart disease (CHD) associated with heterotaxy syndrome. Less is known, however, about pulmonary vascular complications in these patients. Methods and Results: We reviewed medical records of 236 patients who were diagnosed with polysplenia syndrome at 2 institutions for pediatric cardiology in Japan from 1978 to 2015. We selected and compared the clinical records of 16 patients with polysplenia who had incomplete atrioventricular septal defect (AVSD) as the polysplenia group, and 22 age-matched patients with incomplete AVSD without any syndromes including polysplenia as the control group...
March 5, 2019: Circulation Journal: Official Journal of the Japanese Circulation Society
Rui Meng Yang, Jiong Tao, Ming Zhan, Hao Yuan, Hai Hong Wang, Sai Juan Chen, Chen Zhu, Hugues de Thé, Jun Zhou, Yin Guo, Jun Zhu
TAMM41, located within the congenital heart diseases (CHD) sensitive region of 3p25 deletion syndrome, is a mitochondrial membrane maintenance protein critical for yeast survival, but its function in higher vertebrates remains unknown. Via in vivo zebrafish model, we found that tamm41 is highly expressed in the developing heart and deficiency of which led to heart valve abnormalities. Molecular mechanistic studies revealed that TAMM41 interacts and modulates the PINK1-PARK2 dependent mitophagy pathway, thereby implicating TAMM41 in heart valve development during zebrafish embryonic cardiogenesis...
March 1, 2019: Cell Death and Differentiation
Anastasia Schleiger, Oliver Miera, Björn Peters, Katharina R L Schmitt, Peter Kramer, Jelena Buracionok, Peter Murin, Mi-Young Cho, Joachim Photiadis, Felix Berger, Stanislav Ovroutski
OBJECTIVES: We analysed our 29-year experience of surgical repair of atrioventricular septal defect (AVSD) to define risk factors for mortality and reoperation. METHODS: Between 1988 and 2017, 508 patients received AVSD repair in our institution; 359 patients underwent surgery for complete AVSD, 76 for intermediate AVSD and 73 for partial AVSD. The median age of the patients was 6.1 months (interquartile range 10.3 months), and the median weight was 5.6 kg (interquartile range 3...
December 22, 2018: Interactive Cardiovascular and Thoracic Surgery
E Blondiaux, G Autret, F Dhombres, M Gonzales, E Audureau, O Clément, J-M Jouannic, L Houyel
PURPOSE: The purpose of this study was to compare non-invasive high-spatial-resolution postmortem cardiac magnetic resonance imaging (MRI) and autopsy findings for evaluating the septal insertion of atrioventricular valves in fetuses. MATERIALS AND METHODS: Five fetal heart specimens including two normal hearts, one heart with complete atrioventricular septal defect (AVSD) and two hearts with linear insertion of atrioventricular valves (LIAVV; gestational age 17 to 34 weeks) were studied with cardiac MRI using a 4...
December 7, 2018: Diagnostic and Interventional Imaging
Meriem Mostefa Kara, Lucile Houyel, Damien Bonnet
The aim of this study was to analyse the anatomy of the ventricular septal defect (VSD) in heart specimens with interruption of the aortic arch (IAA) in order to explore the hypothesis of different embryologic mechanisms for the different anatomic types of IAA. We examined 42 human heart specimens, 25 with IAA as the main disease with concordant atrioventricular and ventriculo-arterial connections and two distinct great arteries, and 17 hearts with IAA associated with other malformations [six common arterial trunk (CAT), five double-outlet right ventricle (DORV), three transposition of the great arteries (TGA), three atrioventricular septal defect (AVSD)]...
February 2019: Journal of Anatomy
Maciej Słodki, Małgorzata Soroka, Giuseppe Rizzo, Maria Respondek-Liberska
INTRODUCTION: Prenatal atrioventricular septal defect (AVSD) on frequent occasions coexists with other cardiac or extracardiac abnormalities or malformation which may change the prognosis and the management with the fetus and the newborn. The aim of the research was to assess the prognosis and the outcome of prenatally diagnosed AVSD based on the classification which also includes coexisting extracardiac abnormalities and malformations as well as its influence on the prenatal consultation...
December 4, 2018: Journal of Maternal-fetal & Neonatal Medicine
Anja Lehner, Florian E Herrmann, Julinda Mehilli, Nikolaus A Haas
Transcatheter aortic valve replacement is mostly performed in elderly patients with severely calcified aortic valves. There are few reports about its use for pure aortic regurgitation, few reports about its use in adolescent patients and to the best of our knowledge no report about the use of an Edwards Sapien valve in the aortic position in an underage patient after surgically corrected congenital heart disease (CHD). Decompensation of a complex CHD can be difficult to manage and may require unusual solutions...
November 20, 2018: Catheterization and Cardiovascular Interventions
Minoti Bhakta, Mahesh S Padanad, John P Harris, Christina Lubczyk, James F Amatruda, Nikhil V Munshi
BACKGROUND: Many human gene mutations have been linked to congenital heart disease (CHD), yet CHD remains a major health issue worldwide due in part to an incomplete understanding of the molecular basis for cardiac malformation. RESULTS: Here we identify the orthologous mouse Pou6f1 and zebrafish pouC as POU homeodomain transcription factors enriched in the developing heart. We find that pouC is a multi-functional transcriptional regulator containing separable activation, repression, protein-protein interaction, and DNA binding domains...
November 16, 2018: Developmental Dynamics: An Official Publication of the American Association of Anatomists
Mi Hee Lim, Si Chan Sung, Hyung Tae Kim, Kwang Ho Choi, Hyoung Doo Lee, Geena Kim
We report a case of successful repair of truncus arteriosus (TA) associated with complete atrioventricular septal defect (c-AVSD) using a staged approach. TA associated with c-AVSD is a very rare congenital cardiac anomaly. No report of successful staged repair in South Korea has yet been published. We performed bilateral pulmonary artery banding when the patient was 33 days old, and total correction using an extracardiac conduit was performed at the age of 18 months. The patient recovered uneventfully and is doing well...
October 2018: Korean Journal of Thoracic and Cardiovascular Surgery
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...
December 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
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