Anatomically corrected malposition of great arteries.

Area of Science:

• Cardiology
• Congenital Heart Disease
• Pediatric Cardiology

Background:

• The term 'anatomically corrected transposition' has been redefined as 'anatomically corrected malposition' to distinguish it from true transposition.
• Transposition is now reserved for cases where great arteries arise from morphologically inappropriate ventricles.

Purpose of the Study:

• To describe four cases of anomalous hearts with unusual great artery origins.
• To clarify the definition and anatomical entity of anatomically corrected malposition.
• To differentiate anatomically corrected malposition from classical transposition.

Main Methods:

• Morphological examination of four anomalous hearts.
• Analysis of ventriculo-arterial and atrioventricular relations.
• Comparison with classical transposition based on aortic position.

Main Results:

• All four hearts exhibited ventriculo-arterial concordance, with great arteries arising from morphologically appropriate ventricles.
• Variations included atrioventricular concordance (1 situs inversus, 3 situs solitus), right atrioventricular valve atresia, and pulmonary atresia in some cases.
• Coronary artery anomalies were present in all four cases.

Conclusions:

• Anatomically corrected malposition represents a ventriculo-arterial relation, not a discrete anomaly, and exists as an anatomical entity.
• This relation can coexist with all types of atrioventricular relations, which can be termed 'concordant' or 'discordant' anatomically corrected malposition.
• Distinguishing anatomically corrected malposition, often with a left-sided anterior aorta, from classical transposition is crucial for diagnosis and management.