Atrioventricular septal defect: From embryonic development to long-term follow-up

Emmeline E Calkoen; Mark G Hazekamp; Nico A Blom; Bernadette B L J Elders; Adriana C Gittenberger-de Groot; Monique C Haak; Margot M Bartelings; Arno A W Roest; Monique R M Jongbloed

doi:10.1016/j.ijcard.2015.09.081

Atrioventricular septal defect: From embryonic development to long-term follow-up

Int J Cardiol. 2016 Jan 1:202:784-95. doi: 10.1016/j.ijcard.2015.09.081. Epub 2015 Sep 26.

Authors

Emmeline E Calkoen¹, Mark G Hazekamp², Nico A Blom³, Bernadette B L J Elders³, Adriana C Gittenberger-de Groot⁴, Monique C Haak⁵, Margot M Bartelings⁶, Arno A W Roest³, Monique R M Jongbloed⁷

Affiliations

¹ Departments of Paediatric Cardiology and Anatomy & Embryology, Leiden University Medical Center, Leiden, The Netherlands.
² Department of Thoracic surgery, Leiden University Medical Center, Leiden, The Netherlands.
³ Departments of Paediatric Cardiology, Leiden University Medical Center, Leiden, The Netherlands.
⁴ Departments of Cardiology and Anatomy & Embryology, Leiden University Medical Center, Leiden, The Netherlands.
⁵ Department of Obstetrics, Leiden University Medical Center, Leiden, The Netherlands.
⁶ Department of Anatomy & Embryology, Leiden University Medical Center, Leiden, The Netherlands.
⁷ Departments of Cardiology and Anatomy & Embryology, Leiden University Medical Center, Leiden, The Netherlands. Electronic address: m.r.m.jongbloed@lumc.nl.

PMID: 26476030
DOI: 10.1016/j.ijcard.2015.09.081

Abstract

Atrioventricular septal defect (AVSD) covers a spectrum of heart anomalies with a common atrioventricular connection and has an incidence of 4-5.3 per 10.000 live births. About half of the AVSDs occur in patient with Down syndrome. This review provides a bench to bedside overview of AVSD. Developmental aspects, nomenclature, anatomy, and classification of AVSD are discussed. Furthermore an overview of genetic and maternal risk factors for AVSD is provided, and available literature on (fetal) diagnosis, surgical techniques and follow-up is presented. Special attention is given to differences in developmental, anatomical and prognostic factors of AVSD between non-syndromic and Down syndrome patients.

Keywords: Atrioventricular septal defect; Congenital heart disease; Endocardial cushion defect.

Publication types

Research Support, Non-U.S. Gov't
Review

MeSH terms

Female
Follow-Up Studies
Global Health
Heart Septal Defects / embryology*
Heart Septal Defects / epidemiology*
Humans
Incidence
Pregnancy
Risk Factors
Time Factors

Supplementary concepts

Atrioventricular Septal Defect