Transposition of the Great Arteries: A Laterality Defect in the Group of Heterotaxy Syndromes or an Outflow Tract Malformation?

Overview

Journal Ann Pediatr Cardiol

Specialty Cardiology & Vascular Diseases

Date 2018 Oct 2

PMID 30271012

Citations 5

Authors

Rana S Al-Zahrani

Samaher H Alharbi

Rawan M A Tuwaijri

Bayan T Alzomaili

Alaa Althubaiti

Talat Mesud Yelbuz

Affiliations

Soon will be listed here.

Abstract

Background/aim: Transposition of the great arteries (TGA) is traditionally classified as a "conotruncal heart defect", implying that TGA evolves from abnormal development of the outflow tract (OFT) of the embryonic heart. However, recently published genetic data suggest that TGA may be linked to laterality gene defects rather than OFT gene defects. The aim of our study was to clarify whether there is any statistically significant link between TGA and clinically diagnosed laterality defects (heterotaxy).

Methods: Retrospective cross-sectional analysis of 533 patients diagnosed with TGA at our cardiac center over a period of 13 years (2002-2015). Hospital informatics and digital data recording systems were used for collecting patients' data and all patients were reviewed to check the echocardiograms for verification of the diagnosis, type (TGA, congenitally corrected TGA (ccTGA), and levo-position of the great arteries (LGA)), complexity of TGA, and all other variables (e.g., abdominal organ arrangement, cardiac position, presence or absence of other cardiac defects).

Results: Of 533 TGA patients, 495 (92.9%) had the usual arrangement of the internal organs, 21 (3.9%) had mirror-imagery, 7 (1.3%) had left and 10 (1.8%) had right isomerism. 444 (83.3%) patients had TGA. The number of patients who had usual visceral arrangement in each TGA type was: 418 (94.1%) in TGA, 49 (92.4%) in ccTGA, and 28 (77.7%) in LGA. 6 (1.4%) TGA patients, 4 (11.1%) patients with LGA were found to have right isomerism, while no ccTGA patient presented with this asymmetry. 4 (0.9%) TGA patients, 1 (1.9%) ccTGA patient and 2 (5.6%) patients with LGA had left isomerism. Heterotaxy (mirror-imagery, left and right isomerism) was more associated with LGA than TGA or ccTGA with a statistically significant difference ( value of 0.001).

Conclusion: In contrast to recently published genetic data, our morphological data do not disclose a significant link between TGA and heterotaxy.

Citing Articles

Heterotaxy Syndromes and Transposition: Common Genetic Basis or a Serendipitous Association?.

Sreelal T, Gupta S, Vidiyala P, Pandey N Pediatr Cardiol. 2024; 46(2):506-507.

PMID: 39230683 DOI: 10.1007/s00246-024-03638-y.

Pathogenesis and Surgical Treatment of Dextro-Transposition of the Great Arteries (D-TGA): Part II.

Zubrzycki M, Schramm R, Costard-Jackle A, Morshuis M, Gummert J, Zubrzycka M J Clin Med. 2024; 13(16).

PMID: 39200964 PMC: 11355351. DOI: 10.3390/jcm13164823.

Is Transposition of the Great Arteries Associated With Shortening of the Intrapericardial Portions of the Great Arterial Trunks? An Echocardiographic Analysis on Newborn Infants With Simple Transposition of the Great Arteries to Explore an Animal....

Omer S, Alhabshan F, Jijeh A, Caimbon N, Enriquez C, Manner J J Am Heart Assoc. 2021; 10(15):e019334.

PMID: 34278802 PMC: 8475693. DOI: 10.1161/JAHA.120.019334.

Risk factors for transposition of the great arteries in Saudi population.

Alfarhan A, Alquayt M, Alshalhoub M, Alnahdi M, Masuadi E, Alhabshan F Saudi Med J. 2020; 41(10):1054-1062.

PMID: 33026045 PMC: 7841512. DOI: 10.15537/smj.2020.10.25418.

Genetics of Transposition of Great Arteries: Between Laterality Abnormality and Outflow Tract Defect.

De Ita M, Cisneros B, Rosas-Vargas H J Cardiovasc Transl Res. 2020; 14(3):390-399.

PMID: 32734553 DOI: 10.1007/s12265-020-10064-x.

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