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complete AV canal

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https://www.readbyqxmd.com/read/30036562/mediator-complex-subunit-med12-regulates-cardiac-jelly-development-and-av-valve-formation-in-zebrafish
#1
Julia Segert, Isabelle Schneider, Ina M Berger, Wolfgang Rottbauer, Steffen Just
The molecular mechanism essential for the formation of heart valves involves complex interactions of signaling molecules and transcription factors. The Mediator Complex (MC) functions as multi-subunit machinery to orchestrate gene transcription, especially for tissue-specific fine-tuning of transcriptional processes during development, also in the heart. Here, we analyzed the role of the MC subunit Med12 during atrioventricular canal (AVC) development and endocardial cushion formation, using the Med12-deficient zebrafish mutant trapped (tpd)...
July 20, 2018: Progress in Biophysics and Molecular Biology
https://www.readbyqxmd.com/read/29336386/retrospective-study-of-complete-atrioventricular-canal-defects-anesthetic-and-perioperative-challenges
#2
Aniruddha Ramesh Janai, Wilfried Bellinghausen, Edwin Turton, Carmine Bevilacqua, Waseem Zakhary, Martin Kostelka, Farhad Bakhtiary, Joerg Hambsch, Ingo Daehnert, Florian Loeffelbein, Joerg Ender
OBJECTIVE: The objective of this study was to highlight anesthetic and perioperative management and the outcomes of infants with complete atrioventricular (AV) canal defects. DESIGN: This retrospective descriptive study included children who underwent staged and primary biventricular repair for complete AV canal defects from 1999 to 2013. SETTING: A single-center study at a university affiliated heart center. PARTICIPANTS: One hundred and fifty-seven patients with a mean age at surgery of 125 ± 56...
January 2018: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/29067078/tetralogy-of-fallot-repair-in-patients-presenting-after-infancy-a-single-surgeon-experience
#3
Tariq Waqar, Muhammad Usman Riaz, Tania Mahar
OBJECTIVE: To determine the early surgical outcomes of Tetralogy of Fallot (TOF) repair in children and young adults operated after the age of one year. METHODS: In this retrospective study, 307 cases of primary repair of Tetralogy of Fallot were done between September 2012 to February 2017, at CPE Institute of cardiology, Multan. Out of 307 operated patients, 4 (1.3%) patients had previous modified Blalock Taussig shunts, 2 (0.6%) associated ASD with TOF, 3 (0...
July 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/27343557/the-mediator-complex-subunit-med10-regulates-heart-valve-formation-in-zebrafish-by-controlling-tbx2b-mediated-has2-expression-and-cardiac-jelly-formation
#4
Steffen Just, Sofia Hirth, Ina M Berger, Mark C Fishman, Wolfgang Rottbauer
In search for novel key regulators of cardiac valve formation, we isolated the zebrafish cardiac valve mutant ping pong (png). We find that an insertional promoter mutation within the zebrafish mediator complex subunit 10 (med10) gene is leading to impaired heart valve formation. Expression of the T-box transcription factor 2b (Tbx2b), known to be essential in cardiac valve development, is severely reduced in png mutant hearts. We demonstrate here that transient reconstitution of Tbx2b expression rescues AV canal development in png mutant zebrafish...
September 2, 2016: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/26931266/incomplete-pentalogy-of-cantrell-a-case-report
#5
M A Ali, T Tazmin, T Latif, S A Haque, M A Hossain, M N Islam, R H Khan, M A Hoque
Cantrell's Pentalogy is a rare congenital malformation consists of supraumbilical abdominal wall defect, defect in the lower part of sternum, agenesis of anterior portion of diaphragm, an absence of the diaphragmatic part of the pericardium and intracardiac malformation. This case report presents a female neonate, who was born at 40 weeks of gestation weighing 2400 gm and was admitted 4 hours after delivery with the complaints of something coming out from chest. On physical examination her vital signs were within normal limit, she had a systolic murmur on heart at lower left sternal area and there was a vascular structure present on the upper part of abdomen which was pulsatile and pulsation was synchronized with cardiac pulsation...
January 2016: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/26752583/ethical-dilemmas-relating-to-the-management-of-a-newborn-with-down-syndrome-and-severe-congenital-heart-disease-in-a-resource-poor-setting
#6
Ama K Edwin, Frank Edwin, Summer J McGee
Decision-making regarding treatment for newborns with disabilities in resource-poor settings is a difficult process that can put parents and caregivers in conflict. Despite several guidelines that have helped to clarify some of the medical decision-making in Ghana, there is still no clear consensus on the specific moral criteria to be used. This article presents the case of a mother who expressed her wish that her child with Down syndrome should not have been resuscitated at birth. It explores the ethical issues at stake in both her misgivings about the resuscitation and her unwillingness to consider surgical repair of an atrioventricular (AV) canal defect...
2015: Narrative Inquiry in Bioethics
https://www.readbyqxmd.com/read/25851476/morphology-of-the-atrioventricular-valve-in-asplenia-syndrome-a-peculiar-type-of-atrioventricular-canal-defect
#7
P Francalanci, B Marino, R Boldrini, R Abella, F Iorio, C Bosman
The high risk of atrioventricular (AV) valve regurgitation is a major point of concern in the natural history of patients with atrioventricular canal defect (AVCD) and asplenia syndrome (AS). The morphology of the common AV valve in patients with AVCD and AS was therefore studied and compared with the anatomy of patients with AVCD but with atrial situs solitus. We compared the anatomic features of hearts with common AV valve (ventricular loop; AV valve alignment on ventricles; number of leaflets; number and morphology of papillary muscles and relationship of the bridging leaflets with the ventricular septum) in the hearts of 33 patients with AVCD and asplenia syndrome (Group 1) with those of 44 patients with common AV valve, complete AVCD, and atrial situs solitus (Group 2)...
May 1996: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
https://www.readbyqxmd.com/read/25838456/biventricular-repair-for-common-atrioventricular-canal-defect-with-parachute-left-atrioventricular-valve
#8
Patrick O Myers, Pedro J del Nido, Victor Bautista-Hernandez, Gerald R Marx, Sitaram M Emani, Frank A Pigula, Michele Borisuk, Christopher W Baird
OBJECTIVES: Parachute left atrioventricular (AV) valve can complicate repair of common atrioventricular canal (CAVC), and single-ventricle palliation is sometimes preferred. The goal of this study is to review our single institutional experience in biventricular repair in this patient group. METHODS: The demographic, procedural and outcome data were obtained for all children who underwent biventricular repair for complete CAVC with parachute [single left ventricular (LV) papillary muscle] or forme fruste parachute left AV valve (closely spaced LV papillary muscles) from 2001 to 2012...
February 2016: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/24958047/a-novel-technique-for-repair-of-complete-atrioventricular-canal-defect-the-central-patch-technique
#9
William I Douglas, Unnati Doshi
BACKGROUND: Two-patch, single-patch, and modified single-patch repairs are accepted techniques for repair of complete atrioventricular (AV) canal defects. We propose a novel, alternative technique: the central patch technique. METHODS: For the central patch technique, the superior and inferior bridging leaflets are attached with simple sutures to the right and left of their coaptation point. Both bridging leaflets are incised along a line above the ventricular crest, similar to a traditional single-patch technique...
July 2014: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/24659117/transjugular-melody-valve-placement-in-a-small-child-with-protein-losing-enteropathy
#10
Kiran K Mallula, Damien Kenny, Ziyad M Hijazi
The authors present a patient with situs inversus totalis, dextrocardia, and tetralogy of Fallot with complete AV canal who underwent pulmonary valve-sparing complete repair during infancy but developed progressive pulmonary outflow obstruction on serial follow-up. His right ventricular outflow tract (RVOT) was prestented as a precursor for Melody valve placement. After this, he developed protein losing enteropathy (PLE) which was unresponsive to medical management. Finally, he received transcatheter Melody valve percutaneously in the pulmonic position that lead to clinical and biochemical resolution of his PLE symptoms...
February 1, 2015: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/24204893/nitric-oxide-synthase-3-promotes-embryonic-development-of-atrioventricular-valves
#11
Yin Liu, Xiangru Lu, Fu-Li Xiang, Man Lu, Qingping Feng
Nitric oxide synthase-3 (NOS3) has recently been shown to promote endothelial-to-mesenchymal transition (EndMT) in the developing atrioventricular (AV) canal. The present study was aimed to investigate the role of NOS3 in embryonic development of AV valves. We hypothesized that NOS3 promotes embryonic development of AV valves via EndMT. To test this hypothesis, morphological and functional analysis of AV valves were performed in wild-type (WT) and NOS3(-/-) mice at postnatal day 0. Our data show that the overall size and length of mitral and tricuspid valves were decreased in NOS3(-/-) compared with WT mice...
2013: PloS One
https://www.readbyqxmd.com/read/23960629/down-s-syndrome-and-myocardial-reperfusion-injury
#12
Susheel Kumar, Richard Jonas
Down syndrome is known to be an independent risk factor for mortality after surgical repair of congenital heart anomalies. It is also associated with neurodegenerative disease and accelerated aging. The mechanism of the latter features has been attributed to abnormal handling of oxygen-free radicals as well as mitochondrial dysfunction. These properties also place the child with Down syndrome at a risk of an exaggerated myocardial ischemia/reperfusion injury. A 6 month old child with Down syndrome is reported who suffered from obvious clinical ischemia/reperfusion injury following an uncomplicated repair of complete AV canal...
January 2011: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/23871139/two-ventricle-repairs-in-the-unbalanced-atrioventricular-canal-defect-spectrum-with-midterm-follow-up
#13
John E Foker, James M Berry, Jeffrey M Vinocur, Brian A Harvey, Lee A Pyles
OBJECTIVES: Unbalanced atrioventricular (AV) canal defects include a hypoplastic ventricle (HV) and AV valve (HAVV) precluding complete 2-ventricle repairs (2VRs). Catch-up growth would solve this problem and was induced by increasing HAVV flow. The objectives were to assess reliability of HV and HAVV growth and provide 5- to 15-year 2VR follow-up. METHODS: From 1990 to 2005, 23 consecutive infants (13 females and 10 males) with echo-diagnosed unbalanced AV canal defects (n = 20) or subsets (n = 3) underwent 2VRs...
October 2013: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/23804732/repair-of-atrioventricular-canal-with-double-outlet-right-ventricle-transposition-or-truncus-arteriosus
#14
Christo I Tchervenkov, Pierre-Luc Bernier, Danny Del Duca, Samantha Hill, Noritaka Ota, Gordan Samoukovic, Hamad Al-Habib, Stephen Korkola
Atrioventricular canal and conotruncal anomalies are a heterogeneous group of lesions presenting unique challenges for surgical repair. These are the establishment of unobstructed pathways from left ventricle (LV) to aorta and from right ventricle (RV) to pulmonary artery, closure of the inlet ventricular septal defect (VSD) and atrial septal defect (ASD) ostium primum, and the avoidance of significant left and right atrioventricular valve (AV) regurgitation. Repair of complete atrioventricular canal (CAVC) with tetralogy of Fallot (TOF) has been most commonly achieved, either using a single-patch or a 2-patch technique...
April 2010: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/23804726/individualized-approach-to-repair-of-complete-atrioventricular-canal-selective-use-of-the-traditional-single-patch-technique-versus-the-australian-technique
#15
Richard A Jonas, Bassem Mora
The traditional single-patch technique for repair of complete atrioventricular (AV) canal requires surgical division of the superior and inferior common leaflets. In the neonate and young infant, subsequent resuspension of very delicate AV valve tissue on the pericardial patch can be problematic. Selective application of the modified single-patch technique as described by Nunn (Australian technique) minimizes manipulation of the AV valve leaflet tissue. Previous reports have documented that since the late 1980s, the traditional single-patch approach with leaflet resuspension is possible with a mortality of 3% or less...
April 2010: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/23804725/atrioventricular-septal-defects-lessons-learned-about-patterns-of-practice-and-outcomes-from-the-congenital-heart-surgery-database-of-the-society-of-thoracic-surgeons
#16
Jeffrey Phillip Jacobs, Marshall Lewis Jacobs, Constantine Mavroudis, Paul Jubeong Chai, Christo I Tchervenkov, Francois G Lacour-Gayet, Hal Walters, James Anthony Quintessenza
During the 4-year time interval of 2005 through 2008, the Society of Thoracic Surgeons Congenital Heart Surgery Database documented data about 2882 operations to repair atrioventricular (AV) canal defects: partial, 623 (21.5%); intermediate, 342 (11.8%);. complete, 1917 (66.3%). Mean age at complete repair (years) was as follows: partial, 6.1; intermediate, 2.9; complete, 0.6. Median age at complete repair (years) was as follows: partial, 2.6; intermediate, 0.9; complete, 0.4. Down syndrome was present in 1767 patients (61...
April 2010: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/23799731/fetal-complete-common-atrioventricular-canal-defect-spontaneous-closure-of-the-ventricular-septal-defect-in-utero-anatomic-evolution-and-postnatal-outcomes
#17
Dilachew Adebo, James St Louis, Tracy Prosen, Shanthi Sivanandam
BACKGROUND: We describe in utero anatomic evolution and postnatal outcome of complete common atrioventricular canal defect (CCAVCD). METHODS: Retrospective data on 31 fetuses with CCAVCD were analyzed. We reviewed prenatal and postnatal echocardiograms, karyotype, and postnatal outcomes. RESULTS: A total of 20 fetuses had complete data, 18 with serial fetal echocardiograms and postnatal data and 2 terminations. At initial examination, isolated CCAVCD was seen in 12 (67%) fetuses while 6 (33%) were associated with heterotaxy syndrome...
April 2013: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/21188369/complete-atrioventricular-canal-with-guarded-primum-septal-defect
#18
Susheel Kumar, Mary Donofrio, Lowell Frank, Dingchao He, Richard Jonas
Common atrioventricular canal (CAVC) is a common congenital heart lesion resulting from a defect in development of the endocardial cushions during early embryogenesis. Depending on the type of defect, CAVC can encompass a spectrum of lesions ranging from partial atrioventricular (AV) canal to complete AV canal. We describe a case of CAVC with unique anatomy in a 4-month-old male infant with Down syndrome.
April 2011: Pediatric Cardiology
https://www.readbyqxmd.com/read/20806541/factors-influencing-the-intermediate-outcome-in-patients-with-single-ventricle-physiology-after-fontan-operation
#19
M Nosal, P Valentik, A Kantorova, A S Nagi, M Sagat, R Poruban
OBJECTIVE: This study was conducted to analyze the factors affecting the intermediate outcome following the Fontan procedure in the current era. METHODS: Between January 1992 and December 2008, 189 patients underwent Fontan procedure at a median age of 3.4 years (0.4-37 years). Single left ventricle was present in 77 (40%) patients, right ventricle in 70 (37%), in 7 (3.7%) patients, the ventricular morphology was indetermined and in 35 (18.5%) a dominant systemic ventricle with smaller second ventricle was present...
2010: Bratislavské Lekárske Listy
https://www.readbyqxmd.com/read/20615778/complete-atrioventricular-canal-in-a-dog
#20
Vittorio Saponaro, Francesco Staffieri, Delia Franchini, Antonio Crovace
This report details a case of complete, type A atrioventricular (AV) canal, also called complete endocardial cushion defect, in a young dog. Complete AV canal is classified as three types: A, B or C depending on the degree of linkage between common AV valve and ventricular chambers. Despite the defect this dog, which did not undergo surgical treatment, remains in ISACHC class Ib 19 months after the initial diagnosis.
August 2010: Journal of Veterinary Cardiology: the Official Journal of the European Society of Veterinary Cardiology
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