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Complete Atrioventricular Canal Defect with Multiple Atrial Septal Defects: Rare case report and review of literature


Sr No:
Page No: 15-21
Language: English
Authors: Akhil Mehrotra*, Mohammad Shaban, Anjali Chouhan, Faiz Illahi Siddiqui
Received: 2025-07-10
Accepted: 2025-07-28
Published Date: 2025-08-01
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Abstract:
Complete atrioventricular canal defect (CAVCD), also referred to as complete atrioventricular septal defect, is characterized by an ostium primum atrial septal defect, a common atrioventricular valve and a variable deficiency of the ventricular septum inflow. CAVCD is an uncommon congenital heart disease, accounting for about 3% of cardiac malformations. CAVCD occurs in two out of every 10,000 live births. Both sexes are equally affected and there is a striking association with Down syndrome. Depending on the morphology of the superior leaflet of the common atrioventricular valve, 3 types of CAVCD have been delineated (type A, B and C, according to Rastelli's classification). CAVCD results in a significant interatrial and interventricular systemic-to-pulmonary shunt, thus inducing right ventricular pressure and volume overload and pulmonary hypertension. It becomes symptomatic in infancy due to congestive heart failure and failure to thrive. We are reporting a rare case of CAVCD with multiple atrial septal defects (MASD) in a two month old female infant detected by color doppler transthoracic echocardiography (TTE). To our knowledge this is a singular case report of CAVCD in association with MASD.
Keywords: Complete atrioventricular canal defect, CAVCD, Multihole atrial septal defect, Rastelli classification, Cyanotic congenital heart disease.

Journal: IRASS Journal of Multidisciplinary Studies
ISSN(Online): 3049-0073
Publisher: IRASS Publisher
Frequency: Monthly
Language: English

Complete Atrioventricular Canal Defect with Multiple Atrial Septal Defects: Rare case report and review of literature