Systemic Right Ventricle Complications in Levo-transposition of the Great Arteries: A Case Report and Review of Literature

Overview

Journal World J Cardiol

Publisher Baishideng Publishing Group

Specialty Cardiology & Vascular Diseases

Date 2023 Oct 30

PMID 37900900

Authors

Mohamed Ramzi Almajed

Abdulla Almajed

Naoshin Khan

Mark S Obri

Karthikeyan Ananthasubramaniam

Affiliations

Soon will be listed here.

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.

References

Yang H, Bouma B, Dimopoulos K, Khairy P, Ladouceur M, Niwa K . Non-vitamin K antagonist oral anticoagulants (NOACs) for thromboembolic prevention, are they safe in congenital heart disease? Results of a worldwide study. Int J Cardiol. 2019; 299:123-130. DOI: 10.1016/j.ijcard.2019.06.014. View

Spigel Z, Binsalamah Z, Caldarone C . Congenitally Corrected Transposition of the Great Arteries: Anatomic, Physiologic Repair, and Palliation. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2019; 22:32-42. DOI: 10.1053/j.pcsu.2019.02.008. View

Yokota Y, Kawanishi H, Hayakawa M, KUMAKI T, Takarada A, Nakanishi O . Cardiac thrombus in dilated cardiomyopathy. Relationship between left ventricular pathophysiology and left ventricular thrombus. Jpn Heart J. 1989; 30(1):1-11. DOI: 10.1536/ihj.30.1. View

Fleddermann A, Hayes C, Magalski A, Main M . Efficacy of Direct Acting Oral Anticoagulants in Treatment of Left Ventricular Thrombus. Am J Cardiol. 2019; 124(3):367-372. DOI: 10.1016/j.amjcard.2019.05.009. View

Wallis G, Debich-Spicer D, Anderson R . Congenitally corrected transposition. Orphanet J Rare Dis. 2011; 6:22. PMC: 3116458. DOI: 10.1186/1750-1172-6-22. View

Lattuca B, Bouziri N, Kerneis M, Portal J, Zhou J, Hauguel-Moreau M . Antithrombotic Therapy for Patients With Left Ventricular Mural Thrombus. J Am Coll Cardiol. 2020; 75(14):1676-1685. DOI: 10.1016/j.jacc.2020.01.057. View

OGara P, Kushner F, Ascheim D, Casey Jr D, Chung M, de Lemos J . 2013 ACCF/AHA guideline for the management of ST-elevation myocardial infarction: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2012; 61(4):e78-e140. DOI: 10.1016/j.jacc.2012.11.019. View

Hiramatsu T, Matsumura G, Konuma T, Yamazaki K, Kurosawa H, Imai Y . Long-term prognosis of double-switch operation for congenitally corrected transposition of the great arteries. Eur J Cardiothorac Surg. 2012; 42(6):1004-8. DOI: 10.1093/ejcts/ezs118. View

Pujol C, Mussigmann M, Schiele S, Nagdyman N, Niesert A, Kaemmerer H . Direct oral anticoagulants in adults with congenital heart disease - a single centre study. Int J Cardiol. 2019; 300:127-131. DOI: 10.1016/j.ijcard.2019.09.077. View

10.

Abdelnabi M, Saleh Y, Fareed A, Nossikof A, Wang L, Morsi M . Comparative Study of Oral Anticoagulation in Left Ventricular Thrombi (No-LVT Trial). J Am Coll Cardiol. 2021; 77(12):1590-1592. DOI: 10.1016/j.jacc.2021.01.049. View

11.

Ibanez B, James S, Agewall S, Antunes M, Bucciarelli-Ducci C, Bueno H . 2017 ESC Guidelines for the management of acute myocardial infarction in patients presenting with ST-segment elevation. Rev Esp Cardiol (Engl Ed). 2017; 70(12):1082. DOI: 10.1016/j.rec.2017.11.010. View

12.

Samanek M, VORISKOVA M . Congenital heart disease among 815,569 children born between 1980 and 1990 and their 15-year survival: a prospective Bohemia survival study. Pediatr Cardiol. 1999; 20(6):411-7. DOI: 10.1007/s002469900502. View

13.

Scognamiglio G, Fusco F, Hankel T, Bouma B, Greutmann M, Khairy P . Safety and efficacy of non-vitamin K antagonist oral anticoagulants for prevention of thromboembolism in adults with systemic right ventricle: Results from the NOTE international registry. Int J Cardiol. 2020; 322:129-134. DOI: 10.1016/j.ijcard.2020.08.034. View

14.

Cruz Rodriguez J, Okajima K, Greenberg B . Management of left ventricular thrombus: a narrative review. Ann Transl Med. 2021; 9(6):520. PMC: 8039643. DOI: 10.21037/atm-20-7839. View

15.

Camaj A, Fuster V, Giustino G, Bienstock S, Sternheim D, Mehran R . Left Ventricular Thrombus Following Acute Myocardial Infarction: JACC State-of-the-Art Review. J Am Coll Cardiol. 2022; 79(10):1010-1022. DOI: 10.1016/j.jacc.2022.01.011. View

16.

Garg P, van der Geest R, Swoboda P, Crandon S, Fent G, Foley J . Left ventricular thrombus formation in myocardial infarction is associated with altered left ventricular blood flow energetics. Eur Heart J Cardiovasc Imaging. 2018; 20(1):108-117. PMC: 6302263. DOI: 10.1093/ehjci/jey121. View

17.

Alcalai R, Butnaru A, Moravsky G, Yagel O, Rashad R, Ibrahimli M . Apixaban vs. warfarin in patients with left ventricular thrombus: a prospective multicentre randomized clinical trial‡. Eur Heart J Cardiovasc Pharmacother. 2021; 8(7):660-667. DOI: 10.1093/ehjcvp/pvab057. View

18.

Bautista-Hernandez V, Marx G, Gauvreau K, Mayer Jr J, Cecchin F, Del Nido P . Determinants of left ventricular dysfunction after anatomic repair of congenitally corrected transposition of the great arteries. Ann Thorac Surg. 2006; 82(6):2059-65. DOI: 10.1016/j.athoracsur.2006.06.045. View

19.

Robinson A, Trankle C, Eubanks G, Schumann C, Thompson P, Wallace R . Off-label Use of Direct Oral Anticoagulants Compared With Warfarin for Left Ventricular Thrombi. JAMA Cardiol. 2020; 5(6):685-692. PMC: 7177639. DOI: 10.1001/jamacardio.2020.0652. View

20.

Meltzer R, Visser C, Fuster V . Intracardiac thrombi and systemic embolization. Ann Intern Med. 1986; 104(5):689-98. DOI: 10.7326/0003-4819-104-5-689. View