[Anatomically corrected levo-malposition of the great arteries]

Area of Science:

• Cardiology
• Pediatric Cardiology
• Congenital Heart Disease

Background:

• Anatomically corrected malposition of the great arteries (ACMGA) is a rare congenital heart defect characterized by atrioventricular and ventriculoarterial concordance.
• Unlike corrected transposition, ACMGA involves normally related atrioventricular connections but abnormally arising great arteries from their correct ventricles.

Observation:

• This case report presents a rare instance of laevomalposition of the great arteries with dextrocardia and situs solitus in a young infant.
• The condition was associated with a ventricular septal defect and bilateral abnormal conal myocardium, leading to subpulmonary obstruction.

Findings:

• The study details the specific anatomical arrangement in ACMGA, where the pulmonary trunk arises from the morphologically right ventricle and the aorta from the morphologically left ventricle.
• The abnormal great artery relationship is attributed to the interposition of bilateral abnormal conal myocardium, resulting in the absence of aortic valve-mitral valve fibrous continuity.

Implications:

• This case highlights the complex spectrum of congenital heart defects and the importance of precise anatomical diagnosis.
• Understanding the embryological basis of ACMGA, including abnormal conal development, is crucial for accurate diagnosis and management of affected infants.
• Further research into the genetic and developmental factors underlying ACMGA may improve diagnostic and therapeutic strategies for this rare condition.