Open Access

ARTICLE

Classifying Cardiac Anomalies in Right and Left Isomerism: Concordant and Discordant Patterns

Lilia Oreto^1,*, Giuseppe Mandraffino², Paolo Ciliberti³, Teresa P. Santangelo⁴, Placido Romeo⁵, Antonio Celona⁵, Placido Gitto¹, Lorenzo Galletti³, Fiore S. Iorio³, Alfredo Di Pino¹, Aurelio Secinaro⁴, Paolo Guccione³, Robert H. Anderson⁶, Salvatore Agati¹

1 Mediterranean Pediatric Cardiology Center, Bambino Gesù Children’s Hospital, Via Sirina, Taormina, Italy
2 Internal Medicine Unit, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy
3 Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children’s Hospital, Roma, Italy
4 Advanced Cardiovascular Imaging Unit, Department of Imaging, Bambino Gesù Children’s Hospital, Roma, Italy
5 Radiology Department, S. Vincenzo Hospital, Taormina, Italy
6 Biosciences Institute, Newcastle University, Newcastle upon Tyne, UK

* Corresponding Author: Lilia Oreto. Email:

Congenital Heart Disease 2023, 18(1), 97-111. https://doi.org/10.32604/chd.2022.023619

Received 05 June 2022; Accepted 15 August 2022; Issue published 09 January 2023

Abstract

Aims: Evidence is emerging that, in the setting of isomerism, the atrial and bronchial arrangement are not always concordant, nor are these patterns always harmonious with the arrangement of the abdominal organs. We aimed to evaluate the concordance between these features in a cohort of patients with cardiac malformations in the setting of known isomerism, seeking to determine whether it was feasible to assess complexity on this basis, in this regard taking note of the potential value of bronchial as opposed to appendage morphology. Methods and Results: We studied 78 patients known to have isomerism of the bronchuses, 43 with right and 35 with left isomerism. Appendage anatomy could be determined in 49 cases (63%), all but one of these being concordant with bronchial anatomy. When assessing abdominal features, in only 59 cases (76%) was splenic morphology in keeping with the thoracic findings. As expected, right isomerism was associated with greater complexity of cardiac malformations, with an odds ratio of 6.53, with confidence intervals from 2.2–19.3 (p < 0.001). The odds were slightly decreased with thoraco-abdominal disharmony, when lesions shown to carry higher risk were then found in the setting of left isomerism. Conclusion: Harmony is excellent between bronchial and appendage isomerism, but less so with the arrangement of the abdominal organs. Right isomerism in our cohort, was indicative of a six-fold increase in intracardiac complexity. When discordance was found between the systems, however, the cardiac anomalies were less typical of the anticipated findings for right vs. left isomerism of the appendages.

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Graphical Abstract

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