complete AV canal

The hypoglossal canal (HC) is an unusual location of the posterior fossa dural arteriovenous fistula (AVF), which usually occurs in the transverse or sigmoid sinus. Herein, we report a case of HC dural AVF successfully treated with transvenous coil embolization using detachable coils in a 68-year-old woman who presented with headache and left pulsatile tinnitus for 2 months. Brain magnetic resonance imaging (MRI) and cerebral angiography revealed left HC dural AVF. The pulsatile bruit disappeared immediately after the procedure...

Dural arteriovenous fistulas (DAVFs) are acquired pathological arteriovenous connections involving vessels that usually supply the meninges. A DAVF in the region of the hypoglossal canal is a rare form of fistula that involves the anterior condylar confluence or anterior condylar vein. We report a case of hypoglossal canal DAVF that was successfully embolized transvenously through a superior ophthalmic vein (SOV) approach. After failed attempts through jugular access, our patient was treated by a unique percutaneous direct puncture approach through the SOV, achieving complete obliteration of the fistula...

OBJECTIVES: There are limited data available on the height of the ventricular component of the septal deficiency (VSD) in patients undergoing complete atrioventricular septal defect (CAVSD) repair. VSD height may influence optimal choice of repair strategy with potential consequences for long-term outcomes. We aimed to measure VSD height using 2-dimensional echocardiography and review its association with postoperative outcomes. METHODS: We retrospectively reviewed the preoperative echocardiograms of 45 consecutive patients who underwent CAVSD repair between May 2010 and December 2015 at a single centre...

BACKGROUND: Congenital heart disease (CHD) is one of the most common birth defects. Copy number variations (CNVs) have been proved to be important genetic factors that contribute to CHD. Here we screened genome-wide CNVs in Chinese children with complete atrioventricular canal (CAVC) and single ventricle (SV), since there were scarce researches dedicated to these two types of CHD. METHODS: We screened CNVs in 262 sporadic CAVC cases and 259 sporadic SV cases respectively, using a customized SNP array...

OBJECTIVE: To determine whether children with Down syndrome (DS) receive higher doses of opioid medications compared with children without DS for repair of complete atrioventricular canal (CAVC). DESIGN: A retrospective chart review of children with and without DS who underwent primary repair of CAVC. The exclusion criteria included unbalanced CAVC and patients undergoing biventricular staging procedures. The primary outcome was oral morphine equivalents (OME) received in the first 24 hours after surgery...

Tetrasomy 21 is a rare occurrence. Only 14 cases have been reported in the literature, 8 of which are partial tetrasomy cases and 6 which are complete tetrasomy cases. Of the incidences, no proband with true complete tetrasomy 21 has survived the neonatal period. We report complete mosaic tetrasomy 21 in a female infant with the typical Down syndrome phenotype, including Hirschsprung's disease and atrioventricular (AV) canal defect. This is in contrast to cases of partial tetrasomy 21, which often have an atypical trisomy 21 presentation and multiple nonspecific traits, including short stature, microcephaly, and developmental delays...

BACKGROUND: During amphibian metamorphosis, the crucial moment lies in the rearrangement of the heart, reflecting the changes in circulatory demands. However, little is known about the exact shifts linked with this rearrangement. Here, we demonstrate such myocardial changes in axolotl (Ambystoma mexicanum) from the morphological and physiological point of view. RESULTS: Micro-CT and histological analysis showed changes in ventricular trabeculae organization, completion of the atrial septum and its connection to the atrioventricular valve...

BACKGROUND: The optimal timing, surgical technique, and the influence of Trisomy 21 on the outcome of surgical repair of Complete Atrioventricular Canal Defect remains uncertain. We reviewed our experience in the repair of CAVC to identify the influence of these factors on operative outcomes. METHODS: A prospective study included 70 patients, who underwent repair of CAVC at our institute between July, 2016 and October, 2019. Primary endpoint was mortality and the secondary endpoint was a degree of left atrioventricular valve regurgitation...

BACKGROUND: The symptoms of dural arteriovenous fistula (dAVF) vary according to the location of the fistula and the pattern of venous drainage. Here, we report the case of a patient with a dAVF-induced glossopharyngeal neuralgia. CASE DESCRIPTION: We report a case of a patient with right glossopharyngeal neuralgia caused by a posterior condylar canal (PCC) dAVF. The glossopharyngeal neuralgia was accompanied by persistent tinnitus and repetitive right side otalgia, as well as ipsilateral shoulder and throat pain, lasting for about 30 s...

In this review, we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) outcomes for COVID-19 infection in adults with congenital heart disease which showed no increased mortality compared to the general population, (2) hepatorenal dysfunction before transplantation in patients with Fontan is associated with increased mortality, (3) abnormal Von Willebrand factor metabolism and angiopoietin signaling may contribute to pulmonary AVM formation in children with a Glenn circulation, (4) low cardiac output after the Norwood procedure which improves with higher hemoglobin and with milrionone, (5) a comparison of staged versus complete repair in neonatal tetralogy of Fallot reveiling the pros and cons of each strategy, (6) the long-term outcomes of early repair of complete atrioventricular canal show no difference in outcomes in patients who were repaired below 3 months of life...

The authors describe a case of filum terminale arteriovenous fistula (FTAVF) in association with a large L2-L3 disc sequestration and diffuse lumbar arachnoiditis. A 64-year-old male manifested with chronic back pain and gait difficulty. Magnetic resonance imaging (MRI) of the thoracic and lumbosacral spine revealed spinal cord congestion extending from the conus medullaris to the level of T9. There was a large disc sequestration came from L2-L3 disc herniation. In addition, thickening, clumping, and enhancement of the entire cauda equina were noted, probably representing arachnoiditis...

OBJECTIVES: The angle between the inter-ventricular septum and the ascending aorta can be measured during a sonographic fetal survey while viewing the left ventricular outflow tract (LVOT angle). Our aim was to compare the LVOT angle between fetuses with and without conotruncal cardiac anomaliesrmations. METHODS: In this prospective observational study, we compared the LVOT angle between normal fetuses, at different gestational age, and fetuses with cardiac malformations...

INTRODUCTION: Pediatric patients who receive pacemakers may have a lifetime of repeat exposure to fluoroscopy. His bundle pacing may provide better long-term pacing for these patients. To minimize fluoroscopy and for improved localization of the His bundle, we utilized three-dimensional mapping to guide His bundle pacing and we follow-up short-term results. METHODS: An Octapolar Livewire catheter (Abbott, Minneapolis, USA) was used for mapping and location of the His bundle while 3-dimensional mapping via the EnSite Precision system (Abbott Medical, Abbott Park, IL) was utilized to create a 3-dimensional geometry...

Complete atrioventricular canal defect is a CHD associated with intra-cardiac shunting of blood, which can lead to irreversible changes in pulmonary vascular resistance and pulmonary artery hypertension if unrepaired. Patients with Trisomy 21 are at risk for early development of pulmonary artery hypertension if left uncorrected.1,2. OBJECTIVES: The purpose of this study is to describe the evolution of pulmonary artery hypertension after repair of complete atrioventricular canal defect and to determine the time to normalisation of pulmonary artery pressure in both patients with and without Trisomy 21...

Adult congenital heart disease patients may undergo numerous fluoroscopically guided procedures including pacemaker implantation during their lifetime. One alternative to traditional pacemaker setup which may improve long-term pacing outcomes is His bundle pacing. Given the altered His-bundle location, and given increased radiation exposure over a lifetime, we used 3-dimensional mapping to locate the His and to minimize fluoroscopy for placement of a His-bundle pacemaker system in a 31-year old patient with atrioventricular canal defect and complete heart block with 100% RV pacing and epicardial lead fracture...

Cardiovascular development is a complex process that starts with the formation of symmetrically located precardiac mesodermal precursors soon after gastrulation and is completed with the formation of a four-chambered heart with distinct inlet and outlet connections. Multiple transcriptional inputs are required to provide adequate regional identity to the forming atrial and ventricular chambers as well as their flanking regions; i.e., inflow tract, atrioventricular canal, and outflow tract. In this context, regional chamber identity is widely governed by regional activation of distinct T-box family members...

OBJECTIVE: To assess the feasibility and outcomes of biventricular conversion following takedown of Fontan circulation. METHODS: Retrospective analysis of patients who had takedown of Fontan circulation and conversion to biventricular circulation at a single center from September 2007 to April 2020. Failing Fontan physiology was defined as Fontan circulation pressure >15 mm Hg and/or the presence of associated complications. RESULTS: Biventricular conversion was performed in 23 patients at a median age of 10...

OBJECTIVES: Although neonates and infants undergoing cardiac surgery on cardiopulmonary bypass (CPB) are at high risk of developing perioperative morbidity and mortality, including lung injury, the intraoperative profile of lung injury in this cohort is not well-described. Given that the postoperative course of patients in the pediatric cardiac surgical arena has become increasingly expedited, the objective of this study was to characterize the profiles of postoperative mechanical ventilatory support in neonates and infants undergoing cardiac surgery on CPB and to examine the characteristics of lung mechanics and lung injury in this patient population who are potentially amendable to early postoperative recovery in a single tertiary pediatric institution...

OBJECTIVES: Previous studies investigating risk factors associated with reoperation or mortality after repair of complete atrioventricular septal defect (CAVSD) often have not included sizeable cohorts undergoing modified single-patch repair. Both double patch and modified single-patch techniques have been widely used in Australia since the 1990s. Using a large multi-institutional cohort, we aimed to identify risk factors associated with reoperation or mortality following CAVSD repair...

BACKGROUND: Complete atrioventricular septal defects (CAVSD) include a variable spectrum of congenital malformations with different forms of clinical findings. We examined early and midterm outcomes, the need for reoperation, postoperative residual AV valve regurgitation, and other risk factors after various CAVSD repairs. METHODS: Between 2014 and 2018, we have performed 89 isolated CAVSD repairs. We retrospectively reviewed the patients' medical records. Patients were divided into three groups according to their repair techniques modified one patch repair (MP) (n = 16); two patch repair (TP) (n = 49); and left anterior leaflet augmentation (ALA) technique (n = 24)...