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Disharmony between atrioventricular connections and segmental combinations: unusual variants of "crisscross" hearts
R H Anderson1, A Smith, J L Wilkinson
1Institute of Child Health, Alder Hey Children's Hospital, Liverpool, United Kingdom.
Insights
Congenital heart malformations can present with unexpected ventricular topology, challenging typical anatomical descriptions. Understanding both segment connections and individual segment topology is crucial for accurate diagnosis of complex congenital heart disease.
Area of Science:
- Cardiovascular Medicine
- Developmental Biology
- Pediatric Cardiology
Background:
- Congenital heart disease (CHD) encompasses a spectrum of structural abnormalities present at birth.
- Accurate description of cardiac morphology is essential for diagnosis, surgical planning, and understanding disease mechanisms.
- Atrioventricular (AV) connection describes the relationship between the atria and ventricles, a key determinant of cardiac topology.
Observation:
- Two cases of congenitally malformed hearts are presented, one examined post-mortem and the other studied in vivo.
- In both cases, the ventricular mass topology (left-hand or right-hand loop) was discordant with the expected pattern based on the atrioventricular connection.
- Specifically, unexpected left-sided positioning of the morphologically right ventricle was observed in relation to the ventricular topology.
Findings:
- Case 1 exhibited a concordant AV connection but a left-hand ventricular topology (l loop) with a left-sided morphologically right ventricle.
- Case 2 demonstrated a discordant AV connection but a right-hand ventricular topology (d loop) with a right-sided morphologically right ventricle.
- These findings highlight that the ventricular mass topology may not always align with the established rules based solely on the AV connection.
Implications:
- The study underscores the necessity of evaluating the topology of individual cardiac segments, not just their connections, for a comprehensive description of congenital heart malformations.
- This detailed topological analysis is critical for refining diagnostic criteria and improving the understanding of complex CHD.
- Accurate anatomical characterization aids in better predicting clinical outcomes and guiding therapeutic interventions in affected individuals.
Abstract:
Two congenitally malformed hearts are described, one studied in life and the other at autopsy, in which the topology of the ventricular mass was not as expected for the atrioventricular (AV) connection present. In Case 1, studied at autopsy, there was the usual atrial arrangement with a concordant AV connection. The morphologically right ventricle, however, was left-sided and the ventricular mass was of left-hand topology (l loop) rather than the expected right-hand pattern (d loop). In Case 2, studied during life, there was the usual atrial arrangement and a discordant AV connection. Rather than the anticipated left-hand topology (l loop), the ventricular mass was arranged in right-hand fashion (d loop) and the morphologically right ventricle was right-sided. The cases emphasize that for a full description of a congenitally malformed heart, it is often necessary to account for the topology of each segment as well as the connections (or alignment) among the segments.