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A criss-cross heart. Detailed anatomic description and discussion of morphogenesis
Insights
Rare criss-cross atrioventricular connections in hearts present unique anatomical challenges. This case study explores a rare heart anomaly combining features of transposition of the great arteries and atrioventricular discordance.
Area of Science:
- Cardiology
- Developmental Biology
- Anatomical Pathology
Background:
- Congenital heart defects involving complex spatial arrangements of cardiac chambers and great arteries are challenging to diagnose.
- Atrioventricular connections and valve relationships are critical determinants of cardiac function.
Observation:
- A rare case of criss-cross atrioventricular connections is presented, exhibiting features of transposition of the great arteries.
- The anatomical configuration resulted in atrioventricular discordance, mimicking congenitally corrected transposition hemodynamically.
Findings:
- The study proposes hypotheses for the unusual anatomy, including significant counterclockwise rotation of the ventricular septum or an early embryogenic anomalous communication.
- These hypotheses aim to explain the crossed atrioventricular connections and altered great artery valve positions.
Implications:
- Understanding such rare anomalies enhances diagnostic capabilities for complex congenital heart disease.
- Further research into embryogenesis can elucidate mechanisms behind these intricate cardiac malformations.
Abstract:
Hearts with criss-cross atrioventricular connections are rare. Reports in the literature describe great anatomic variations with regard to the atrioventricular connections, spatial relationships of the ventricles, and connections and spatial relationships of the great arteries. In the example described in this report, the basic anatomic picture was that of transposition of the great arteries, but the criss-cross atrioventricular valves had resulted in atrioventricular discordance; hemodynamically, therefore, the situation resembled congenitally corrected transposition. One hypothesis for this anatomic configuration is that pronounced counterclockwise rotation (as viewed from below) brought the ventricular septum into a frontal plane and altered the respective positions of the pulmonary and aortic valves from those usually seen in congenitally corrected transposition and thereby resulted in crossed atrioventricular connections. An alternative hypothesis is that in this case one of the atrioventricular valves (the anteriorly positioned valve) may represent an anomalous communication that developed early in embryogenesis and connected the left atrial appendage to the right ventricular infundibulum.