Atrioventricular Septal Defects: Pathology, Imaging, and Treatment Options
- PMID: 34196822
- DOI: 10.1007/s11886-021-01523-1
Atrioventricular Septal Defects: Pathology, Imaging, and Treatment Options
Abstract
Purpose of review: Atrioventricular septal defects (AVSD) represent a broad spectrum of congenital anomalies from simple to the most complex heart defects including some distinct types. Clinical presentation and timing of intervention differ by morphological subset and functional anatomy. Herein, we review morphological variations and characteristics that determine appropriate intervention and provide insights into functional anatomy based on detailed three-dimensional (3D) assessment of AVSDs.
Recent findings: The understanding of functional morphology of AVSDs has improved significantly with detailed 3D echocardiographic evaluation of the atrioventricular junction and valve morphology. As prenatal detection of AVSDs has increased significantly, it has become the most common fetal cardiac diagnosis enabling antenatal counseling and delivery planning. Advances in diagnosis and perioperative care have resulted in optimal outcomes. The diagnosis and management of AVSDs have improved over the years with enhanced understanding of anatomy and perioperative care resulting in optimal short and long-term outcomes.
Keywords: ACHD; Atrioventricular septal defect; Congenital heart disease; Down syndrome; Pediatric.
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References
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
-
- Anderson RH, Wessels A, Vettukattil JJ. Morphology and morphogenesis of atrioventricular septal defect with common atrioventricular junction. World J Pediatr Congenit Heart Surg. 2010;1:59–67. https://doi.org/10.1177/2150135109360813 The article comprehensively explains the basic anatomy and pathology of AVSDs with insight on the uniqueness of the atrioventricular junction.
-
- Hoffman JIE, Kaplan S, Liberthson RR. Prevalence of congenital heart disease. Am Heart J. 2004;147:425–39. https://doi.org/10.1016/j.ahj.2003.05.003 . - DOI - PubMed
-
- Reller MD, Strickland MJ, Riehle-Colarusso T, Mahle WT, Correa A. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr. 2008;153:807–13. https://doi.org/10.1016/j.jpeds.2008.05.059 . - DOI - PubMed - PMC
-
- Franklin RCG, Beland MJ, Colan SD, Walters HL, Aiello VD, Anderson RH, et al. Nomenclature for congenital and paediatric cardiac disease: the International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Iteration of the International Classification of Diseases (ICD-11). Cardiol Young. 2017;27:1872–938. https://doi.org/10.1017/S1047951117002244 . - DOI - PubMed
-
- Chaudhry A, Urbinelli L, Veldtman GR. Atrioventricular Septal Defects. In: Gatzoulis M, Webb G, Daubeney P, editors. Diagnosis and management of adult congenital heart disease. Third ed: Elsevier; 2018. pp.326-38. The article provides information on long-term complications and management of AVSDs in adulthood.
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