Imaging characteristics and associations of left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers on multidetector CT

Abstract

Aim: To study the imaging characteristics and the associations of left atrioventricular valve atresia with patent aortic root and lateralized atrial chambers on multidetector computed tomography (CT) angiography. Materials and Methods:We retrospectively evaluated all the CT angiography studies performed for the evaluation of various congenital heart diseases at our center from January 2014 to December 2021. Cases with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers were identified.The types of cardiovascular involvement as identifiable on CT angiography were studied and compiled.Results: A total of 38 patients with left atrioventricular valve atresia and patent aortic root were included (median age: 5 months, males: 31). A total of 89.5% had usual atrial arrangement and 10.5% had mirror imagery of atria. Normal drainage of right and left superior caval veins and inferior caval vein into systemic venous atrium was seen in 77.8%, 61.5%, and 86.6%, respectively. Anomalous pulmonary venous drainage was seen in 3 (7.9%) patients. Atrial and ventricular septal defects were the most common associations. Imperforate left atrioventricular membrane was seen in 9 (23.7%) patients and absent left atrioventricular connection in 27 (71.1%).Discordant right atrioventricular connection was seen in 5 (13.1%) patients. While a double outlet right ventricle (76.3%) was the most common ventriculoarterial connection, discordant ventriculo-arterial connection (transposition) in 4 (10.5%).Most patients had good sized pulmonary arteries. Patent arterial duct and right aortic arch were seen in 29% and 21%, respectively. Aberrant right subclavian artery was the most common arch vessel anomaly. Coronary anomalies were seen in 7 patients (19%); single coronary artery being the most common. Conclusions: Majority of the patients with left atrioventricular valve atresia and patent aortic root with lateralized atrial chambers have usual atrial arrangement, normal right atrioventricular connections and a double outlet configuration of the right ventricle. Absent left atrioventricular connection is a more common than imperforate left atrioventricular valve membrane. Atrial and ventricular septal

Understanding the heterogeneity of “mitral atresia” with patent aortic root

“…The third advantage of computerized tomographic investigation is that it reveals the details not only of the cardiac structures but also the arrangement of the remaining thoracic and abdominal organs. In the investigations now published in this issue of the journal, for example, the Indian investigators were able to demonstrate disharmony between the arrangement of the atrial, thoracic, and abdominal arrangements in three of their patients with lateralized atrial chambers, and in two individuals with isomeric atrial appendages 3,4 . Significantly, the disharmony in those with isomeric appendages was between the lungs, bronchuses, and abdominal organs, but still, the bronchial arrangement provides an accurate guide to the type of isomerism present.…”

“…They recognized that the problems existing in providing a simple classification for these lesions are greatly increased in the setting of isomeric, rather than lateralized, atrial appendages. 3,4 Hence, they have produced two excellent manuscripts, rather than seeking to combine the overall findings. Their reports now serve to show how analysis of computed tomographic datasets is rapidly establishing itself as the new "gold standard" for the description of the anatomy of the congenitally malformed heart.…”

“…In the investigations now published in this issue of the journal, for example, the Indian investigators were able to demonstrate disharmony between the arrangement of the atrial, thoracic, and abdominal arrangements in three of their patients with lateralized atrial chambers, and in two individuals with isomeric atrial appendages. 3,4 Significantly, the disharmony in those with isomeric appendages was between the lungs, bronchuses, and abdominal organs, but still, the bronchial arrangement provides an accurate guide to the type of isomerism present. Even more significantly, when using their tomographic datasets, it proved possible in all instances to identify the presence of isomeric atrial appendages on the basis of the extent of their pectinated walls relative to the vestibules of the atrioventricular junctions.…”

“…The investigators working in New Delhi encountered an additional problem. They recognized that the problems existing in providing a simple classification for these lesions are greatly increased in the setting of isomeric, rather than lateralized, atrial appendages 3,4 . Hence, they have produced two excellent manuscripts, rather than seeking to combine the overall findings.…”