complete AV canal

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)...

Congenital heart block is a rare and lethal condition in paediatric population associated with maternal connective tissue disorders and rarely with structural cardiac disease like atrioventricular canal defects with or without left isomerism and congenitally corrected transposition of great arteries. Pacing in neonate if indicated is generally accomplished by epicardial pacing systems. However, in cases of significant bradycardia and haemodynamic instability, temporary pacemaker implantation via transvenous approach remains as a suitable option...

Spinal extradural arteriovenous fistulas are rare vascular lesion which are defined as abnormal direct connection between an artery or arteries and the extradural venous plexus within the spinal canal and/or intravertebral foramen. These lesions with exclusive extradural venous drainage are even rarer. Because of the limited cases the natural history, demography and treatment understanding of these are limited. Endovascular treatment remains the mainstay of treatment for these lesions. This case and its management are thus presented for its rarity...

Advances in virtual reality have made it possible for clinicians and trainees to interact with 3D renderings of hearts with congenital heart disease in 3D stereoscopic vision. No study to date has assessed whether this technology improved instruction compared to standard 2D interfaces. The purpose of this study was to assess whether stereoscopic virtual reality improves congenital heart disease anatomy education. Subjects in a prospective, blinded, randomized trial completed a pre-test assessing factual and visuospatial knowledge of common atrioventricular canal and were randomized to an intervention or control group based on their score...

Pulmonary vein stenosis (PVS) is a rare, frequently lethal disease with heterogeneous phenotypes and an unclear etiology. Limited studies have reported associations between PVS and congenital heart disease (CHD), chronic lung disease (CLD), and/or prematurity; however, to date, there have been no studies that report detailed clinical syndromic phenotypes and the potential role of genetics in PVS. An existing registry of multivessel PVS patients seen at Boston Children's Hospital (BCH) was queried between August 2006 and January 2017 for all existing genetic testing data on these patients...

We demonstrate the repair in an infant of tetralogy of Fallot with complete atrioventricular canal defect using a 2-patch technique with transannular reconstruction of the right ventricular outflow tract due to a diminutive pulmonary valve annulus. This approach is reproducible and particularly valuable to surgeons who routinely use a 2-patch technique to repair an isolated complete atrioventricular canal defect.

BACKGROUND: Atrioventricular canal defect is a rare congenital disorder of the heart and describes the presence of an atrial septal defect, a variable presentation of ventricular septal alterations including ventricular septal defect malformations in the mitral and tricuspid valves. The defect has been described in human beings, dogs, cats, pigs, and horses. CASE PRESENTATION: This paper describes the case of a complete atrioventricular canal defect in a four-year-old intact male pet ferret (Mustela putorius furo), which was presented due to posterior weakness, ataxia, and decreased appetite...

AV canal defects (AVCD) are caused by maldevelopment of the endocardial cushions and typically include a primum atrial septal defect (ASD), an inlet ventricular septal defect (VSD), and a common atrioventricular valve. The variations in deformities provide the basis for the many terms used in the anatomical classifications: partial, transitional, intermediate, and complete common AVCD (balanced or unbalanced). The balanced complete common AVCDs are classified as Rastelli A, B, C depending on the anomaly of the anterior bridging leaflet division and attachments...

BACKGROUND: Repair of complete atrioventricular canal (CAVC) is often complicated by atrioventricular valve regurgitation, particularly of the left-sided valve. Understanding the 3-dimensional (3D) structure of the atrioventricular canal annulus before repair may help to inform optimized repair. However, the 3D shape and movement of the CAVC annulus has been neither quantified nor rigorously compared with a normal mitral valve annulus. METHODS: The complete annuli of 43 patients with CAVC were modeled in 4 cardiac phases using transthoracic 3D echocardiograms and custom code...

OBJECTIVE: To evaluate long-term transplant-free survival and causes of death in the trisomy 21 (T21) population after surgery for congenital heart disease (CHD) in comparison with patients who are euploidic. STUDY DESIGN: This is a retrospective cohort study from the Pediatric Cardiac Care Consortium, enriched with prospectively collected data from the National Death Index and the Organ Procurement and Transplantation Network for patients with sufficient direct identifiers...

Hypoglossal canal dural arteriovenous fistulas (HC-DAVF) involve the anterior condylar vein (ACV) and anterior condylar confluence (ACC). They often present with tinnitus, bruit, and hypoglossal nerve palsy. The most common treatment in HC-DAVFs is transvenous embolization using coils and the most direct transvenous route is the trans-internal jugular vein access. When this approach is not feasible, a treatment attempt is possible through alternative routes. We report 2 patients with DAVFs involving the anterior condylar confluence...

This study examined the prevalence and distribution of elevated systolic pulmonary arterial pressure, measured by echocardiography, in young patients with down syndrome associated or not with congenital heart disease and surgical correction during childhood. Pulmonary artery systolic pressure, computed by regurgitant tricuspid flow velocity evaluation, is the most frequently used parameter for the screening of pulmonary hypertension. Down syndrome and congenital heart disease often coexist and the probability to detect elevated systolic pulmonary arterial pressure in this setting is high...

BACKGROUND: An elongated styloid process can less frequently lead to symptomatic compression of the internal jugular vein (IJV). We present the first case of dural arteriovenous fistula (DAVF) in association with compressed IJV by an elongated styloid process. CASE DESCRIPTION: A 77-year-old woman presented with pulsating tinnitus. She was diagnosed with DAVF at the right hypoglossal canal and underwent transvenous embolization (TVE). The shunt flow was reduced and the symptom disappeared after TVE...

The association of atretic right superior vena cava with persistent left superior vena cava draining directly into left atrium with absent coronary sinus in atrioventricular canal defect is virtually unknown in adults with no case reported so far. Though atretic right superior vena cava with persistent left superior vena cava is an extremely rare venous anomaly seen in congenital heart disease, it has important clinical implications in cardiac surgery and interventional cardiology. Atrial arrhythmias and right bundle branch block are common with advancing age in partial atrioventricualr canal defect but complete heart block has scarcely been reported in the medical literature...

The association of complete atrioventricular canal with transposition of the great arteries is rare, with a prevalence of less than 3-5%. We present an 18-month-old patient with a complete atrioventricular canal, side-by-side transposition of the great arteries, and anomalous coronary anatomy, managed initially with pulmonary banding and then by arterial switch with complete atrioventricular canal repair at early infancy.

BACKGROUND: Spinal dural arteriovenous fistulas (DAVFs) and spinal arteriovenous malformations (AVMs) are relatively rare. Moreover, multiple spinal cord vascular lesions in an individual, such as double DAVFs and DAVF with AVM, are extremely rare. To date, there are only several reported cases of concomitant spinal DAVFs and AVMs. CASE DESCRIPTION: A 25-year-old woman presented with pain of the right lower limb persisting for 1 month. Spinal magnetic resonance imaging showed prominent signal flow voids in the lumbosacral canal...

In order to evaluate physicians' willingness to seek legal action to mandate surgery when parents refuse surgery for various congenital heart lesions, we surveyed pediatric cardiologists and cardiovascular surgeons at 4 children's hospitals. We asked whether physicians would support parental refusal of surgery for specific heart defects and, if not, whether they would seek legal action to mandate surgery. We then analyzed associations between physicians' willingness to mandate surgery and national operative mortality rates for each lesion...

BACKGROUND: Spinal arteriovenous fistula (AVF) may rarely associate with spinal dysraphism, that is, tethered spinal cord and spinal intradural lipoma. Spinal extradural angiolipoma coexisting with spinal AVF has not been reported in the literature. We reported an extremely rare case of sacral angiolipoma associated with tight filum terminale and sacral spina bifida coexisting with spinal AVF within this tumor. CASE DESCRIPTION: A 55-year-old women presented with progressive myelopathy for 10 months...

PURPOSE: To analyze the safety and efficacy of primary endovascular treatment (EVT) for acute ischemic stroke (AIS) in patients younger than 18 years of age. METHODS: Review of 4 patients < 18 years of age with AIS, prospectively enrolled in an electronic database registry for acute ischemic stroke patients who underwent thrombectomy at tertiary centers, from January 2011 to February 2017. Clinical and imaging data were analyzed. RESULTS: All patients were female...

We sought to examine the role of preoperative and 2nd postoperative day albumin levels as predictors for postoperative course in infants undergoing repair of congenital heart disease. This retrospective, single-center, observational study comprised consecutive infants younger than 1 year who had undergone repair of tetralogy of Fallot, ventricular septal defect, complete atrioventricular canal or transposition of the great arteries over a 25 months period. We correlated preoperative and postoperative day (POD) #2 albumin level to vaso-inotropic score (VIS) and intensive care unit (ICU) length of stay (LOS) as markers for degree and duration of postoperative cardiac support...