MULTIHOLE ATRIAL SEPTAL DEFECT IN AN INFANT: RARE CASE REPORT

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.

Publication Details

Journal
Zenodo (CERN European Organization for Nuclear Research)
Published
2025-08-04
DOI
https://doi.org/10.5281/zenodo.16738050
Primary Topic
Congenital Heart Disease Studies
Type
article
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article

MULTIHOLE ATRIAL SEPTAL DEFECT IN AN INFANT: RARE CASE REPORT

Zenodo (CERN European Organization for Nuclear Research)
Congenital Heart Disease Studies
article

MULTIHOLE ATRIAL SEPTAL DEFECT IN AN INFANT: RARE CASE REPORT

article en

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.

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MULTIHOLE ATRIAL SEPTAL DEFECT IN AN INFANT: RARE CASE REPORT · Zenodo (CERN European Organization for Nuclear Research) (2025) | TGRS Research Map | TGRS