![]() Although both male and female genders are affected equally, one of the studies suggests a female to male ratio of 1.3 to 1.0, especially in patients with Down syndrome. It accounts for 3% of all congenital cardiac malformations. The incidence of an atrioventricular septal defect in the general population has been reported to be 0.24 to 0. Gestational diabetes and maternal obesity have also been reported to increase the risk of non-syndromic atrioventricular septal defects. Other than its association with syndromes, gene mutations associated with the atrioventricular septal defect can also be inherited as an autosomal dominant trait. The other syndromes associated with the atrioventricular septal defect may include CHARGE, Ellis-van-Creveld, Smith-Lemli-Opitz, and 3p. Every six patients with Down syndrome have associated atrioventricular septal defect, and Down syndrome cell adhesion molecule (DSCAM) gene has been described to be associated with an atrioventricular septal defect and other congenital heart diseases in these patients. In almost all patients, the atrioventricular septal defect is caused by genetic mutations, and most of the time, it is associated with syndromes. In this review article, we will discuss the etiology, epidemiology, pathophysiology, as well as management, complications, and clinical significance of atrioventricular septal defect. Although the long term outcomes of surgical repair in an atrioventricular septal defect are influenced by the presence of associated malformations, such as ventricular hypoplasia, and down`s syndrome, the evolution of the surgical treatment of atrioventricular defects over the last few decades has significantly improved the long term survival. The incidence of atrioventricular septal defect has been estimated from 0.24 to 0.31 in 1000 live births with no significant difference in male and female gender, and it has a strong association with Down’s syndrome. Whereas the complete form of the atrioventricular septal defect (AVSD) is characterized by a common atrioventricular valve with ostium primum atrial septal defect and an unrestricted ventricular septal defect of inlet type. A partial atrioventricular septal defect is characterized by an ostium primum atrial septal defect, separate atrioventricular valves with a common junction, an inlet ventricular septal defect, and a cleft mitral valve. ![]() The atrioventricular septal defect is a congenital cardiac malformation that is characterized by a variable degree of the atrial and ventricular septal defect along with a common or partially separate atrioventricular orifice. Review the management options available for atrioventricular septal defects.Outline the evaluation of atrioventricular septal defects.Identify the etiology of atrioventricular septal defects.This activity reviews the evaluation and treatment of AVSD and highlights the role of the interprofessional team in evaluating and treating patients with this condition. In order to avoid the high morbidity and mortality associated with this condition, it must be promptly diagnosed and treated. ![]() Diagnosis of AVSD in fetal life or early neonatal period is essential in order to initiate appropriate medical treatment and to plan early surgical repair. ![]() ![]()
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