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Atrioventricular Septal Defect

AVSD


Presentation

  • We present an unusual case of a Down's syndrome patient whose septal defect first presented at 20 years of age.[ncbi.nlm.nih.gov]
  • The present patient showed very late onset pulmonary hypertension, which may be a critical parameter for survival.[ncbi.nlm.nih.gov]
  • After surgical correction of the shunt and the associated mitral incompetence, the patient experienced a good outcome.Echocardiographic and intraoperative findings are presented, along with a plausible explanation for the mechanism and presentation of[ncbi.nlm.nih.gov]
  • We present a case of this association treated with the previously unreported application of the cone reconstruction (da Silva repair) as a secondary strategy.[ncbi.nlm.nih.gov]
  • We present a review of rare intermediate type atrioventricular septal defect in the elderly, and we discuss the surgical issues in this case.[ncbi.nlm.nih.gov]
Large Ears
  • We report on a patient with a unique constellation of anomalies comprising trigonomicrocephaly, asymmetric severe micrognathia, large ears, atrioventricular septal defect, vertebral anomalies, bilateral cutaneous syndactyly of fingers and toes, unilateral[ncbi.nlm.nih.gov]
Cryptorchidism
  • […] report on a patient with a unique constellation of anomalies comprising trigonomicrocephaly, asymmetric severe micrognathia, large ears, atrioventricular septal defect, vertebral anomalies, bilateral cutaneous syndactyly of fingers and toes, unilateral cryptorchidism[ncbi.nlm.nih.gov]

Workup

ST Elevation
  • She exhibited chest pain with ST elevation, and coronary computed tomography showed that the LMCA was compressed between the dilated pulmonary artery and aorta. After her hyperthyroidism was treated, her chest pain resolved.[ncbi.nlm.nih.gov]
Torsades De Pointes
  • Bradycardia secondary to heart block can lead to abnormal QT prolongation and precipitate Torsades de Pointes and arrest. Here, a case report of these events is described, together with a brief literature review.[ncbi.nlm.nih.gov]

Treatment

  • We discuss her successful surgical treatment in terms of the available medical literature.[ncbi.nlm.nih.gov]
  • Two-patch technique through a transatrial approach alone is a good treatment choice for patients. Management of right ventricular outflow tract (RVOT) is similar to that of isolated TOF.[ncbi.nlm.nih.gov]
  • Three patients are still on vasodilator treatment 1, 2.6 and 6years after surgery, other two were lost to follow-up.[ncbi.nlm.nih.gov]
  • BACKGROUND: Primary repair is the preferred strategy for surgical treatment of complete atrioventricular septal defect combined with the tetralogy of Fallot. However, a staged approach may be preferable for very small or cyanotic infants.[ncbi.nlm.nih.gov]
  • Hence, treatment was conservative. At autopsy, a huge (3 cm diameter) ostium primum atrial septal defect (ASD) was found, together with deformation of the right and left atrioventricular valves and extreme dilatation of the heart.[ncbi.nlm.nih.gov]

Prognosis

  • Abstract Complete atrioventricular septal defect (CAVSD) is a fetal cardiac malformation (5% of all cardiac malformations) that can be detected prenatally with a reserved prognosis.[ncbi.nlm.nih.gov]
  • Single-stage repair of CAVSD and CoA appears to offer a good prognosis provided that valve morphology is amenable to repair.[ncbi.nlm.nih.gov]
  • Postnatal/Prognosis? Management of AVSD generally involves surgery. Outcomes vary widely based upon the presence of other abnormalities or genetic syndromes.[prenatalpediatrics.org]
  • Decreasing operative mortality has significantly altered the prognosis of these patients with and without Down’s syndrome.[heart.bmj.com]
  • Prenatal consultations with pediatric cardiology, pediatric cardiothoracic surgery, and neonatology specialists are recommended to discuss the prognosis and management after birth.[columbiaobgyn.org]

Etiology

  • […] complications/etiology; reoperation; time factors; treatment outcome[ncbi.nlm.nih.gov]
  • PURPOSE: The genetic etiology of atrioventricular septal defect (AVSD) is unknown in 40% cases. Conventional sequencing and arrays have identified the etiology in only a minority of nonsyndromic individuals with AVSD.[ncbi.nlm.nih.gov]
  • Hypoplastic Left Heart Syndrome/physiopathology* Hypoplastic Left Heart Syndrome/surgery Infant Infant, Newborn Severity of Illness Index Ventricular Dysfunction, Left/etiology Ventricular Dysfunction, Left/physiopathology* Ventricular Dysfunction, Left[ncbi.nlm.nih.gov]
  • KEYWORDS: Coronary disease/etiology; heart defects, congenital/complications/surgery; heart septal defects/physiopathology/surgery; longevity; pregnancy complications, cardiovascular; pulmonary valve stenosis/physiopathology; risk factors; treatment outcome[ncbi.nlm.nih.gov]
  • Types atrial septal defects (ASDs) ventricular septal defects Etiology autosomal dominant AVSD Locus AVSD1 (1p31-p21) Locus AVSD2 (3p25): CRELD1 coding for a cell adhesion molecule previously known as "cirrin" ( 12632326 ) Locus GJA2 (6q21-q23.2): GJA2[humpath.com]

Epidemiology

  • KEYWORDS: Abnormalities, multiple/genetics; Down syndrome/complications; cardiomyopathy, hypertrophic/complications/congenital; fatal outcome; heart defects, congenital/diagnosis/epidemiology; heart septal defects, ventricular/embryology/physiopathology[ncbi.nlm.nih.gov]
  • Epidemiology and cause-specific outcome of hypertrophic cardiomyopathy in children: findings from the Pediatric Cardiomyopathy Registry. Circulation. 2007; 115 (6):773–81. [ PubMed ] 15. Clark CE, Henry WL, Epstein SE.[ncbi.nlm.nih.gov]
  • Epidemiology: AVSD is relatively uncommon, occurring in about 4%-5% of all forms of congenital heart disease.[clinicaladvisor.com]
  • The Epidemiology of Congenital Heart Disease, The Baltimore-Washington Infant Heart Study (1981-1989). Perspectives in Pediatric Cardiology. Mount Kisco, NY: Futura Publishing Co; 1993. Vol 4: Freeman SB, Taft LF, Dooley KJ, et al.[emedicine.medscape.com]
  • Atrial and ventricular septal defects - epidemiology and spontaneous closure. J Matern Fetal Neonatal Med. 2006; 19 :271–6. [ PubMed ] [ Google Scholar ] 17. Samanek M, Goetzova J, Benesova D.[ncbi.nlm.nih.gov]
Sex distribution
Age distribution

Pathophysiology

  • The pathophysiology of atrioventricular septal defect is variable, and ranges from mild left to right shunting similar to a simple atrial septal defect to complex single-ventricle heart disease.[ncbi.nlm.nih.gov]
  • ., M.D. [1] Overview Pathophysiology There are many underlying mechanisms resulting in atrioventricular septal defect depends largely on the nature of interaction of blood flow between the right and left sides of the heart.[wikidoc.org]
  • 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]
  • The aim of this chapter is to present a model of how noninvasive multimodal imaging can improve the understanding of pathophysiology and increase the accuracy of the diagnosis of the specific congenital heart disease.[accessmedicine.mhmedical.com]
  • Pathophysiology If there is a defect in the septum, it is possible for blood to travel from the left side of the heart to the right side of the heart, or the other way around.[savetipedijatra.com]

Prevention

  • ., La Ciotat Cedex, France) was placed beneath the atrioventricular valve to prevent rupture of the mitral aneurysm and support the anterior mitral leaflet by creating a new annulus.[ncbi.nlm.nih.gov]
  • The performance of preventive gesture during repair seemed to be unjustified. The surgical management of this reoperation consists of a simple and safe procedure. The Author(s) 2015.[ncbi.nlm.nih.gov]
  • This may involve: A yearly exam with a heart specialist Regular tests of heart function Changes to treatment plan Prevention There is no way to prevent AVSD since the cause is unknown. References Atrioventricular (AV) canal defect.[lahey.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]
  • This consists of a cleft or notch in the "flaps" or leaflets of the valve, preventing complete separation of the left ventricle from the left atrium. 2) An Atrial Septal Defect (ASD), or hole in the septum (muscle wall) that separates the right and left[chla.congenital.org]

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