Atrial Septal Defect in Cyanotic CL/Fr Mice

1998 ◽  
Vol 35 (1) ◽  
pp. 58-64
Author(s):  
Shingo Kadowaki ◽  
Michiko Sakamoto ◽  
Hiroshi Kamiishi ◽  
Takashi Tanimura
Keyword(s):  
Atrial Septal Defect ◽  
Airway Obstruction ◽  
Cleft Lip ◽  
Septal Defect ◽  
Cleft Lip And Palate ◽  
Atrial Septum ◽  
Palatal Shelf ◽  
Icr Mice ◽  
Septum Primum ◽  
Septal Development

Objective It is widely known that some newborn CL/Fr mice with cleft lip and palate (CLP) also have cyanotic symptoms, which have been thought to depend on an atrial septal defect (ASD). In a previous study, we found that cyanotic mice tended to have more severe types of CLP. We hypothesize that the mechanical airway obstruction due to a poorly developed palatal shelf and unmoved tongue in CLP(+) mice might be related to the occurrence of cyanosis. The purpose of this study was to examine the relationships between ASD and cyanosis in CLP(+) newborns. Method The newborn hearts from CLP(-), noncyanotic CLP(+), cyanotic CLP(+), CL/Fr mice and ICR mice were examined histologically, and the incidence and size of ASD was determined on neonatal day (ND) 0. In CLP(-) newborns, similar procedures were performed from ND 1 to ND 4. Furthermore, in CLP(+) newborns, development of the palatal shelf was examined. Results While all the ICR mice had a well-developed atrial septum, and the incidence of ASD was 0%, about 80% of CL/Fr mice had ASD, irrespective of the presence or absence of CLP and cyanosis. On ND 0, the septum primum was significantly shorter in cyanotic CLP(+) mice than in CLP(-) mice. It also tended to be shorter in CLP(+) mice than in CLP(-) mice. Between the cyanotics and noncyanotics, there were no significant differences in the incidences of ASD and the rate of septal development. In CLP(-) mice, the septum primum developed well later and no ASD was observed on ND 4. Cyanotic newborns had significantly less developed palatal shelves than did noncyanotics. Conclusions Cyanosis may not be related to ASD and the rate of septal development, but may be related to the occurrence of CLP in this strain. Furthermore, we confirmed that some relationship exists between the development of the palatal shelf and cyanosis. The present study supports our hypothesis concerning the cause of cyanosis in CL/Fr mice.

Septum Primum Atrial Septal Defect in an Infant with Hypoplastic Left Heart Syndrome

2014 ◽  
Vol 17 (4) ◽  
pp. 232
Author(s):  
Robert W. Loar ◽  
Harold M. Burkhart ◽  
Nathaniel W. Taggart
Keyword(s):  
Atrial Septal Defect ◽  
Hypoplastic Left Heart Syndrome ◽  
Septal Defect ◽  
Left Heart ◽  
Secundum Atrial Septal Defect ◽  
Hypoplastic Left Heart ◽  
Systemic Blood Flow ◽  
Septum Primum ◽  
Septal Development ◽  
Left Heart Syndrome

Hypoplastic left heart syndrome (HLHS) is a form of congenital heart disease characterized by severe underdevelopment of the left heart, leading to inadequate systemic blood flow. Several different atrial septal morphologies are observed in HLHS, most commonly a secundum atrial septal defect, patent foramen ovale, intact septum, and leftward displacement of the superior attachment of the septum primum. It has been postulated that atrial septal development is associated with the development of the left heart. We present a case of a newborn infant with HLHS and the unusual finding of a primum ASD.

Percutaneous Treatment of Congenital Defects of the Inter-atrial Septum in Adults – Recent Advances and Persistent Pitfalls

2009 ◽  
Vol 4 (1) ◽  
pp. 76
Author(s):  
James Slater ◽  
Mark Fisch ◽  
◽  
Keyword(s):  
Atrial Septal Defect ◽  
Adult Patient ◽  
Patent Foramen Ovale ◽  
Septal Defect ◽  
Percutaneous Treatment ◽  
Atrial Septum ◽  
Congenital Defects ◽  
Foramen Ovale ◽  
Anatomy And Physiology ◽  
Specific Tissue

William Harvey was the first scientist to describe the heart as consisting of separate right- and left-sided circulations. Our understanding of the heart’s anatomy and physiology has grown significantly since this landmark discovery in 1628. Today, we recognise not only the importance of these separate systems, but also the specific tissue that divides them. Our growing understanding of the inter-atrial septum has allowed us to identify defects within this structure and develop effective percutaneous devices for closure of these defects in the adult patient. This article discusses the formation of a patent foramen ovale (PFO) and atrial septal defect (ASD). In addition, we describe the medical illnesses caused by these defects and summarise the indications and risks related to percutaneous closure of these defects. We also report the most up-to-date transcatheter therapeutic options for closure of these common congenital defects in the adult patient.

Cobra head deformity of atrial septal occluder: blessing in disguise

2020 ◽  
pp. 1-2
Author(s):  
Uma Devi Karuru ◽  
Saurabh Kumar Gupta
Keyword(s):  
Atrial Septal Defect ◽  
Left Atrial ◽  
Septal Defect ◽  
Atrial Septum ◽  
Right Atrial ◽  
Septal Occluder Device ◽  
Occluder Device ◽  
The Right

Abstract It is not uncommon to have prolapse of the atrial septal occluder device despite accurate measurement of atrial septal defect and an appropriately chosen device. This is particularly a problem in cases with large atrial septal defect with absent aortic rim. Various techniques have been described for successful implantation of atrial septal occluder in such a scenario. The essence of all these techniques is to prevent prolapse of the left atrial disc through the defect while the right atrial disc is being deployed. In this brief report, we illustrate the use of cobra head deformity of the device to successfully deploy the device across the atrial septum.

Repair of Giant Anterior Skull Base Encephalocele Containing Intralesional Eloquent Brain: Technical Note

2021 ◽  
Author(s):  
Andrew J Kobets ◽  
Richard J Redett ◽  
Jonathan M Walsh ◽  
Joseph Lopez ◽  
Melike Guryildirim ◽  
...  
Keyword(s):  
Airway Obstruction ◽  
Skull Base ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Neural Tissue ◽  
Anterior Skull Base ◽  
Csf Rhinorrhea ◽  
Hernia Sac ◽  
Live Births ◽  
Anterior Cranial Base

Abstract BACKGROUND Encephaloceles are herniations of intracranial neural tissue and meninges through defects in the skull. Basal encephaloceles are rare anterior skull base defects incident in 1 in 35,000 live births. Sphenoethmoidal encephaloceles are even more uncommon, with an incidence of 1 in 700,000 live births. Anterior skull base encephaloceles may be life-threatening in infants, presenting as airway obstruction and respiratory compromise. They can also present with cerebrospinal fluid (CSF) rhinorrhea, purulent nasal drainage, or meningitis. OBJECTIVE To report a novel technique for repairing a giant sphenoethmoidal encephalocele containing eloquent neural tissue. METHODS A 16-mo-old girl presented with progressive airway obstruction from a giant sphenoethmoidal encephalocele that filled her oral cavity. She had multiple congenital anomalies including agenesis of the corpus callosum and cleft lip and palate. Computed tomography showed complete absence of the bony anterior cranial base, and magnetic resonance imaging demonstrated the presence of the pituitary gland and hypothalamus in the hernia sac. RESULTS We repaired the encephalocele using a combined microsurgical and endoscopic multidisciplinary approach working through transcranial, transnasal, and transpalatal corridors. The procedure was completed in a single stage, during which the midline cleft lip was also repaired. The child made an excellent neurological and aesthetic recovery with preservation of pituitary and hypothalamic function, without evidence of CSF fistula. CONCLUSION The authors describe a novel multidisciplinary technique for treating a giant sphenoethmoidal encephalocele containing eloquent brain. The cleft lip was also repaired at the same time. The ability to work through multiple corridors can enhance the safety and efficacy of an often-treacherous operative endeavor.

scholarly journals Laceration of atrial septum during balloon sizing of atrial septal defect

2007 ◽  
Vol 8 (2) ◽  
pp. 89-90 ◽  
Author(s):  
S SIVASANKARAN ◽  
S HARIKRISHNAN ◽  
N NARAYANAN ◽  
T JAGANMOHAN
Keyword(s):  
Atrial Septal Defect ◽  
Septal Defect ◽  
Atrial Septum

scholarly journals Identification and Morphogenesis of Vestibular Atrial Septal Defects

2020 ◽  
Vol 7 (3) ◽  
pp. 35
Author(s):  
Rohit S. Loomba ◽  
Justin T. Tretter ◽  
Timothy J. Mohun ◽  
Robert H. Anderson ◽  
Scott Kramer ◽  
...  
Keyword(s):  
Atrial Septal Defect ◽  
Septal Defect ◽  
Leading Edge ◽  
Atrial Septum ◽  
Mouse Embryos ◽  
University Of Florida ◽  
Interatrial Communication ◽  
Septal Defects ◽  
Inferior Portion ◽  
The University

Background: The vestibular atrial septal defect is an interatrial communication located in the antero-inferior portion of the atrial septum. Reflecting either inadequate muscularization of the vestibular spine and mesenchymal cap during development, or excessive apoptosis within the developing antero-inferior septal component, the vestibular defect represents an infrequently recognized true deficiency of the atrial septum. We reviewed necropsy specimens from three separate archives to establish the frequency of such vestibular defects and their associated cardiac findings, providing additional analysis from developing mouse hearts to illustrate their potential morphogenesis. Materials and methods: We analyzed the hearts in the Farouk S. Idriss Cardiac Registry at Ann and Robert H. Lurie Children’s Hospital in Chicago, IL, the Van Mierop Archive at the University of Florida in Gainesville, Florida, and the archive at Johns Hopkins All Children’s Heart Institute in St. Petersburg, Florida, identifying all those exhibiting a vestibular atrial septal defect, along with the associated intracardiac malformations. We then assessed potential mechanisms for the existence of such defects, based on the assessment of 450 datasets of developing mouse hearts prepared using the technique of episcopic microscopy. Results: We analyzed a total of 2100 specimens. Of these, 68 (3%) were found to have a vestibular atrial septal defect. Comparable defects were identified in 10 developing mouse embryos sacrificed at embryonic data 15.5, by which stage the antero-inferior component of the atrial septum is usually normally formed. Conclusion: The vestibular defect is a true septal defect located in the muscular antero-inferior rim of the oval fossa. Our retrospective review of autopsied hearts suggests that the defect may be more common than previously thought. Increased awareness of the location of the defect should optimize its future clinical identification. We suggest that the defect exists because of failure, during embryonic development, of union of the components that bind the leading edge of the primary atrial septum to the atrioventricular junctions, either because of inadequate muscularisation or excessive apoptosis.

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