{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,28]],"date-time":"2026-01-28T06:16:24Z","timestamp":1769580984071,"version":"3.49.0"},"reference-count":77,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T00:00:00Z","timestamp":1761868800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCDD"],"abstract":"<jats:p>Heterotaxy syndrome is characterized by a tendency for bilaterally symmetric arrangement (isomerism) of inner organs. It is frequently associated with complex congenital heart defects (CHDs). In \u201cheterotaxic\u201d hearts, the tendency for isomerism is confined to the atria. The ventricular segment always shows asymmetric arrangements (D-hand or L-hand topology). This study aimed to determine the statistical distribution of ventricular topology among patients with CHDs associated with heterotaxy and to identify possible associations between ventricular topology and cardiovascular disorders and survival. It is a retrospective cross-sectional study on 192 patients treated at a single center between 2000 and 2023. Our cohort had 115 patients of left atrial isomerism (LAI) and 77 of right atrial isomerism (RAI). The whole cohort (n = 192) showed a bias towards ventricular D-hand topology (67%), which was statistically significant in LAI (74%). In contrast, RAI showed an almost equal distribution (57% D-hand, 43% L-hand). No significant associations were found between ventricular topology and major CHDs or mortality. Significant associations were observed between ventricular topology and cardiac apex position, direction of p-wave axis, and aortic arch sidedness. We conclude that, in the setting of heterotaxy, especially RAI, ventricular topology and aortic arch sidedness both behave as binary anatomical variables showing a tendency for randomized occurrence. This tendency for statistically symmetric distribution is interpreted as reflecting the syndrome-specific tendency for bilateral symmetry.<\/jats:p>","DOI":"10.3390\/jcdd12110430","type":"journal-article","created":{"date-parts":[[2025,11,3]],"date-time":"2025-11-03T16:18:42Z","timestamp":1762186722000},"page":"430","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Ventricular Topology in Congenital Heart Defects Associated with Heterotaxy: Can We Find Patterns Reflecting the Syndrome-Specific Tendency for Visceral Symmetry?"],"prefix":"10.3390","volume":"12","author":[{"given":"Takhfif","family":"Othman","sequence":"first","affiliation":[{"name":"King Abdulaziz Cardiac Center, Ministry of National Guard Health Affairs, Riyadh 11426, Saudi Arabia"},{"name":"King Abdullah International Medical Research Center (KAIMRC), Riyadh 11426, Saudi Arabia"},{"name":"Department of Cardiac Sciences, King Saud Bin Abdulaziz University for Health Sciences, Riyadh 11426, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5943-7034","authenticated-orcid":false,"given":"Abdulsalam Mohammad Adnan","family":"Alsaiad","sequence":"additional","affiliation":[{"name":"King Abdulaziz Cardiac Center, Ministry of National Guard Health Affairs, Riyadh 11426, Saudi Arabia"},{"name":"King Abdullah International Medical Research Center (KAIMRC), Riyadh 11426, Saudi Arabia"},{"name":"Department of Cardiac Sciences, King Saud Bin Abdulaziz University for Health Sciences, Riyadh 11426, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7841-1593","authenticated-orcid":false,"given":"Abdulraouf M. Z.","family":"Jijeh","sequence":"additional","affiliation":[{"name":"King Abdulaziz Cardiac Center, Ministry of National Guard Health Affairs, Riyadh 11426, Saudi Arabia"},{"name":"King Abdullah International Medical Research Center (KAIMRC), Riyadh 11426, Saudi Arabia"},{"name":"Department of Cardiac Sciences, King Saud Bin Abdulaziz University for Health Sciences, Riyadh 11426, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J\u00f6rg","family":"M\u00e4nner","sequence":"additional","affiliation":[{"name":"Institute for Anatomy and Cell Biology, UMG, Georg-August-University of G\u00f6ttingen, D-37075 G\u00f6ttingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2318-2981","authenticated-orcid":false,"given":"Talat Mesud","family":"Yelbuz","sequence":"additional","affiliation":[{"name":"King Abdulaziz Cardiac Center, Ministry of National Guard Health Affairs, Riyadh 11426, Saudi Arabia"},{"name":"King Abdullah International Medical Research Center (KAIMRC), Riyadh 11426, Saudi Arabia"},{"name":"Department of Cardiac Sciences, King Saud Bin Abdulaziz University for Health Sciences, Riyadh 11426, Saudi Arabia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,10,31]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.ydbio.2005.07.038","article-title":"Left\u2013right asymmetry and congenital cardiac defects: Getting to the heart of the matter in vertebrate left\u2013right axis determination","volume":"288","author":"Ramsdell","year":"2005","journal-title":"Dev. 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