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Atrial Septal Defect - Atrioventricular Conduction Defects Syndrome

Atrial Septal Defect 7 with or without Atrioventricular Conduction Defects


Presentation

  • The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly.[books.google.de]
  • ORPHA:1479 Synonym(s): - Prevalence: Inheritance: Autosomal dominant Age of onset: No data available ICD-10: Q21.1 OMIM: 108900 UMLS: C3502353 MeSH: - GARD: - MedDRA: - The documents contained in this web site are presented for information purposes only[orpha.net]
  • Cardiomegaly and hepatomegaly may be present if CHF is present. Chest radiographs may show cardiomegaly and increased pulmonary vascularity.[utmb.edu]
  • presents anomalies in only 3% of the cases.[doctortipster.com]
  • Moderate AS may present with chest pain, dyspnea on exertion, dizziness, and syncope. Severe AS presents with weak pulses, left-sided heart failure, and chest pain and could lead to sudden death.[hawaii.edu]
Weight Gain
  • If the child is in congestive heart failure, there will be poor weight gain, the heart rate and breathing rate will be higher than normal, and the liver will be enlarged.[mottchildren.org]
  • These usually include abnormal heart sounds, congestive heart failure, intermittent cyanosis, respiratory infections, poor feeding and inadequate weight gain.[pediatriccardiacinquest.mb.ca]
  • Sometimes increased caloric density is required to achieve weight gain. If maximum medical management does not result in improved respiratory status and weight gain, pulmonary artery banding or surgical closure may be necessary.[clinicaladvisor.com]
  • When symptoms do occur, they may include Breathing problems Pounding heart Weak pulse Ashen or bluish skin color Poor feeding, slow weight gain Tiring easily Swelling of the legs or belly For partial AVSDs, if the holes between the chambers of the heart[cdc.gov]
  • Symptoms and Signs Complete atrioventricular septal defect with a large left-to-right shunt causes signs of heart failure (eg, tachypnea, dyspnea during feeding, poor weight gain, diaphoresis) by age 4 to 6 wk.[merckmanuals.com]
Disability
  • As a matter of courtesy we request that the content provider (Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities) be credited and notified in any public or private usage of this image.[cdc.gov]
  • Acta Paediatr. (1993) [ Pubmed ] Patient with partial trisomy 9q and learning disability but no pyloric stenosis. Hengstschläger, M., Prusa, A.R., Repa, C., Drahonsky, R., Deutinger, J., Pollak, A., Bernaschek, G.[wikigenes.org]
  • Journal of Intellectual Disability Research . 53 (5): 419–25. doi : 10.1111/j.1365-2788.2009.01158.x . PMID 19228275 . Cherry, C; DeBord, S; Moustapha-Nadler, N (June 2009). "Ebstein's anomaly: a complex congenital heart defect".[en.wikipedia.org]
Pathologist
  • All clinicians and scientists interested in birth defects, including pediatricians, geneticists, genetic counselors, obstetricians, and pediatric pathologists, will find this book to be an invaluable source of information.[books.google.de]
Rigor
  • By condensing much of the information presented in the first volume of the previous edition, and exercising rigorous editorial control, Drs.[books.google.de]
Renal Artery Stenosis
  • Marfan Syndrome Aortic root dilation, aortic dissection, mitral valve prolapse and regurgitation Neurofibromatosis Neurofibromata of the heart, renal artery stenosis, renal hypertension Noonan syndrome In 50% of cases: pulmonary stenosis, conduction abnormalities[pedcard.rush.edu]
Pleural Effusion
  • Post-operative pleural effusion can occur after alleviating right ventricle outflow obstruction. Aortic Stenosis AS is the obstruction of the left ventricle outflow tract.[hawaii.edu]
Gagging
  • TEE studies are invariably done in a fasting state and under topical anaesthesia of the oropharynx to reduce gag reflex. A mouth gag is needed to prevent biting and damage to the probe.[cardiophile.org]
Night Sweats
  • sweats, unexplained rashes, nail-bed hemorrhages, and malaise is important.[nursingcenter.com]

Workup

  • […] embolism resulting in a right to left shunting of blood [36] Treatment [ edit ] Patent Foramen Ovale [ edit ] Most patients with a PFO are asymptomatic and do not require any specific treatment. [37] However, those who develop a stroke require further workup[en.wikipedia.org]
Abnormal ECG
  • Though this person had undergone surgical closure of the atrial septal defect three decades back, the abnormal ECG pattern is persisting.[cardiophile.org]
  • They may be noted on routine testing to have an abnormal chest X-ray or an abnormal ECG and may have atrial fibrillation .[en.wikipedia.org]
  • Adult patients are usually recognized due to an abnormal ECG, abnormal chest radiograph, systolic murmur due to the regurgitation of the systemic AV-valve, occurrence of atrial tachycardia or an AV-block. Reduced exercise capacity might be present.[textbookofcardiology.org]
Pleural Effusion
  • Post-operative pleural effusion can occur after alleviating right ventricle outflow obstruction. Aortic Stenosis AS is the obstruction of the left ventricle outflow tract.[hawaii.edu]

Treatment

  • The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.[orpha.net]
  • The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly.[books.google.de]
  • What treatment is available? The treatments for congenital heart conditions depend on the type and severity of the condition.[bhf.org.uk]
  • Treatment of complete form of atrioventricular septal defect: Surgical treatment – It is indicated at any age.[doctortipster.com]
  • Open-heart surgery is the mainstay of treatment for children with AVSD. The repair involves placement of one or two patches to divide the common valve into right and left sides and close the holes.[mottchildren.org]

Prognosis

  • Prognosis: Cardiac failure is never seen in fetal life as well as postnatally.[sonoworld.com]
  • We suggest, however, that when ASD of the fossa ovalis type is accompanied by prolonged A-V conduction, the genetic prognosis may be drastically changed to a risk of almost 50% that the condition will recur in subsequent sibs or children of affected persons[circ.ahajournals.org]
  • […] vessels visible to the periphery of the film eventual signs of pulmonary arterial hypertension chamber enlargement right atrium right ventricle note: left atrium is normal in size unlike VSD or PDA note: aortic arch is small to normal Treatment and prognosis[radiopaedia.org]
  • If type I block is due to AV nodal disease then it is usually relatively benign and non-progressive, with a good long-term prognosis.[patient.info]
  • Treatment Treatment and prognosis of a VSD depends on the size en localisation of the defect, the pulmonary vascular resistance and possible concomitant defects.[textbookofcardiology.org]

Etiology

  • The precise etiology for the disruption of normal ventricular septation is not known, although both genetic and environmental factors have been implicated in the development of ventricular septal defects.[clinicaladvisor.com]
  • Etiology: The etiology of heart defects is heterogeneous and probably depends on the interplay of multiple genetic and environmental factors, including maternal diabetes mellitus or collagen disease, exposure to drugs such as lithium, and viral infections[sonoworld.com]
  • In those where a comprehensive evaluation is performed and an obvious etiology is not identified, they are defined as having a cryptogenic stroke.[en.wikipedia.org]
  • TEE is often used in this context while evaluating pulmonary hypertension of obscure etiology in an adult. TEE probe being very near the heart without any intervening lung tissue, can give excellent images.[cardiophile.org]
  • Splice mutations Clinical: Early childhood onset Cardiomyopathy: Familial hypertrophic, midventricular, digenic Myosin light chain kinase 2 (MYLK2) ; Chromosome 20q11.21 MYH7 Other hypertrophic cardiomyopathies Dilated cardiomyopathy General Features Etiology[neuromuscular.wustl.edu]

Epidemiology

  • The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly.[books.google.de]
  • : Australian male Genetics Mutation: Lys92Arg CALR3 protein Ca -binding chaperone Localized mainly in endoplasmic/sarcoplasmic reticulum Colocalizes with: SPTLC2 CMH 20 Nexilin (NEXN) ; Chromosome 1p31.1; Dominant Epidemiology: Chinese families Genetics[neuromuscular.wustl.edu]
  • Congenital cardiovascular malformations associated with chromosome abnormalities : an epidemiologic study. J Pediatr 1989 ; 114 : 79-90. Otero Coto E, Quero Jimenez M, Deverall PB, Blain H.[siicsalud.com]
  • The changing epidemiology of congenital heart disease . Nat Rev Cardiol 2011 ; 8 : 50 – 60 . 8. Englund , A , Jonsson , B , Zander , CS , Gustafsson , J , Annerén , G .[cambridge.org]
  • Epidemiological study of congenital heart defects in children and adolescents. Analysis of 4,538 cases. Arq Bras Cardiol 2003;80:274-278. 4. Norton ME. Teratogen Update: Fetal Effects of Indomethacin Administration During Pregnancy.[hawaii.edu]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology The pathophysiology depends on the predominant lesion (atrial vs ventricular). There is a left-to-right shunt at the atrial level due to increased relative right ventricular compliance leading to right atrial enlargement.[utmb.edu]
  • Atrioventricular Septal Defect Pathophysiology: In pathophysiological terms, atrioventricular septal defect acts as a ostium secundum interatrial septal defect , which is associated with a mitral valve defect that is accentuating pulmonary stasis, increases[doctortipster.com]
  • Each characteristic determines, in part, the pathophysiology and need for therapy. Definition VSDs are described by the anatomic location of the defect as seen from the right ventricle.[pedsinreview.aappublications.org]
  • Pathophysiology The exact pathophysiology depends on the location and severity of the defect.[textbookofcardiology.org]
  • Pathophysiology Atrial septal defects allow blood to shunt from the left atrium to the right atrium. Shunting is minimal during infancy, but typically increases with age as the right ventricle becomes more compliant.[vhlab.umn.edu]

Prevention

  • The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly.[books.google.de]
  • Medicines may be used temporarily to help with symptoms, but they don't cure the VSD or prevent permanent damage to the lung arteries.[heart.org]
  • Reference Parker SE, Mai CT, Canfield MA, Rickard R, Wang Y, Meyer RE, Anderson P, Mason CA, Collins JS, Kirby RS, & Correa A, for the National Birth Defects Prevention Network.[cdc.gov]
  • To prevent air embolism, since the left heart is open to the atmosphere through the ASD, the ascending aorta is clamped until the defect is repaired and all air has been removed from the heart.[vhlab.umn.edu]
  • Anticoagulation to prevent valve thrombosis can be difficult to maintain during pregnancy and will require frequent monitoring of anticoagulation status.[nursingcenter.com]

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