Almajed MR, Almajed A, Khan N, Obri MS, Ananthasubramaniam K. Systemic right ventricle complications in levo-transposition of the great arteries: A case report and review of literature. World J Cardiol 2023; 15(10): 542-552 [PMID: 37900900 DOI: 10.4330/wjc.v15.i10.542]
Corresponding Author of This Article
Karthikeyan Ananthasubramaniam, FACC, MD, Staff Physician, Heart and Vascular Institute, Henry Ford West Bloomfield Hospital, 6777 W Maple, West Bloomfield, MI 48322, United States. kananth1@hfhs.org
Research Domain of This Article
Cardiac & Cardiovascular Systems
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Cardiol. Oct 26, 2023; 15(10): 542-552 Published online Oct 26, 2023. doi: 10.4330/wjc.v15.i10.542
Systemic right ventricle complications in levo-transposition of the great arteries: A case report and review of literature
Mohamed Ramzi Almajed, Abdulla Almajed, Naoshin Khan, Mark S Obri, Karthikeyan Ananthasubramaniam
Mohamed Ramzi Almajed, Naoshin Khan, Mark S Obri, Department of Internal Medicine, Henry Ford Hospital, Detroit, MI 48202, United States
Abdulla Almajed, College of Medicine and Medical Sciences, Arabian Gulf University, Manama 00000, Bahrain
Karthikeyan Ananthasubramaniam, Heart and Vascular Institute, Henry Ford West Bloomfield Hospital, West Bloomfield, MI 48322, United States
Author contributions: Almajed MR, Almajed A, Khan N, Obri M, Ananthasubramaniam K contributed equally to this work; All authors evaluated the case, reviewed the literature, and wrote the manuscript; All authors have read and approve of the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: Dr. Ananthasubramaniam has nothing to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Karthikeyan Ananthasubramaniam, FACC, MD, Staff Physician, Heart and Vascular Institute, Henry Ford West Bloomfield Hospital, 6777 W Maple, West Bloomfield, MI 48322, United States. kananth1@hfhs.org
Received: June 17, 2023 Peer-review started: June 17, 2023 First decision: August 10, 2023 Revised: August 23, 2023 Accepted: September 27, 2023 Article in press: September 27, 2023 Published online: October 26, 2023 Processing time: 129 Days and 1.8 Hours
Abstract
BACKGROUND
Congenitally corrected levo-transposition of the great arteries (L-TGA) is a congenital heart disease in which the ventricles and great arteries are transposed from their typical anatomy. In L-TGA, the double discordance, atrioventricular and ventriculoarterial, create an acyanotic milieu which allows patients to survive their early decades, however, progressive systemic right ventricle (sRV) dysfunction creates complications later in life. sRV dysfunction and remodeling predisposes patients to intracardiac thrombus (ICT) formation.
CASE SUMMARY
A 40-year-old male with L-TGA presented with symptoms of acute decompensated heart failure. In childhood, he had surgical repair of a ventricular septal defect. In adulthood, he developed sRV dysfunction, systemic tricuspid valve (sTV) regurgitation, and left-bundle branch block for which he underwent cardiac resynchronization therapy. Transthoracic echocardiogram showed a sRV ejection fraction of 40%, severe sTV regurgitation, and a newly identified sRV ICT. ICT was confirmed by ultrasound-enhancing agents and transesophageal echocardiography. Our patient was optimized with guideline-directed medical therapy and diuresis. Anticoagulation was achieved with a vitamin K antagonist (VKA) and he was later referred for evaluation by advanced heart failure and heart transplant services.
CONCLUSION
Anticoagulation with VKA is the mainstay of treatment in the absence of conclusive data supporting direct oral anticoagulant use in ICT in patients with congenital heart disease. This case illustrates the natural history of L-TGA and highlights the importance of surveillance and monitoring with dedicated cardiac imaging to identify complications.
Core Tip: Patients with congenital heart disease such as levo-transposition of the great arteries experience progressive cardiac dysfunction and remodeling which manifests as heart failure. This predisposes patients to the formation of intracardiac thrombus (ICT). We present a case of progressive systemic right ventricle (sRV) dysfunction resulting in an apical thrombus. Review of literature identified no cases of sRV thrombus making this one of the first reports. Guidelines do not exist for anticoagulation in patients with congenital heart disease and ICT. Therefore, clinical decisions are extrapolated from anticoagulation principles in patients without congenital heart disease. Considerations for direct oral anticoagulants in this population should be individualized and involve shared decision making.
