Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2022 May 31:78:103884.
doi: 10.1016/j.amsu.2022.103884. eCollection 2022 Jun.

Persistent left superior vena cava in a 29-year-old lady with Ebstein's anomaly and complete heart block. A case report and literature review

J B W Russell  1   2   3 , T R Koroma  1   2 , V Conteh  1   2   3 , J Coker  1   2 , S Conteh  1   2 , Kumar Bharat  3 , O Z Mahdi  3
Affiliations
Case Reports

Persistent left superior vena cava in a 29-year-old lady with Ebstein's anomaly and complete heart block. A case report and literature review

J B W Russell et al. Ann Med Surg (Lond). .

Abstract

Introduction: Persistent Left Superior Vena Cava (PLSVC) is a rare congenital vascular anomaly that may occur alone or in combination with complex congenital heart anomalies and dangerous arrhythmias. We report the first case in the literature of combined PLSVC, Ebstein's Anomaly (Type A) and complete atrioventricular block in an adult female, being successfully managed with permanent pacemaker implantation in Sierra Leone.

Case summary: We present an interesting case of a 29-year-old female, referred to the cardiology clinic on account of breathlessness, dizziness, and recurrent syncope. Physical examination revealed a pulse rate of 39 bpm, jugular venous pulse with occasional cannon waves, and grade 3/6 pansystolic murmur in the tricuspid valve area. An electrocardiogram confirmed complete atrioventricular block with junctional escape rhythm, while Transthoracic Echocardiogram (TTE) confirmed Ebstein's Anomaly (Type A) and moderate tricuspid regurgitation. PLSVC was discovered as an incidental intraprocedural finding. Deploying a pacemaker lead through this venous anomaly from the left side was futile. Nevertheless, we used a right sided approach that resulted in a successful permanent pacemaker implantation with optimal and stable parameters.

Conclusion: This rare case report highlights the practical challenges often encountered in the practice of cardiology during pacemaker and other cardiac device implantation. Cardiologists and critical care physicians should be acquainted with the venous anomaly of PLSVC, its variants, and procedure-associated risks, for better clinical decision making.

Keywords: Complete atrioventricular block; Ebstein anomaly; PLSVC; Permanent pacemaker.

PubMed Disclaimer

Conflict of interest statement

Authors of this article have no conflict or competing interests. Final version of the manuscript was approved by the authors.

Figures

Fig. 1
Fig. 1
Electrocardiogram (ECG) A. ECG shows complete atrioventricular block B. ECG shows ventricular pacing.
Fig. 2
Fig. 2
Transthoracic Echocardiogram A. Parasternal long axis view: red arrow is pointing to a dilated coronary sinus. White arrow is pointing to the Descending Aorta B. Apical four chamber view illustrating a downward displacement of the tricuspid valve (red arrow) relative to the mitral valve (white arrow), indicative of Type A Ebstein Anomaly. Grossly dilated right atrium (RA). (For interpretation of the references to colour in this figure legend, the reader is referred to the Web version of this article.)
Fig. 3
Fig. 3
Flouroscopic C-arm X-ray machine demonstrating the guidewire pathway and intravenous contrast A. Failure of the guidewire to cross the midline with initial bending along the left paramediastinal region B. Pathway of the guidewire along the left paramediastinal region C. Full deployment of the guidewire along the left paramediastinal region D. Domonstration of Persistent Left Superior Vena Cava after injection of constrast.
Fig. 4
Fig. 4
Angio-Computed Tomography Scan A. CT image showing Right Superior Vena Cava (RSVC) and Persistent Left Superior Vena Cava (PLSVC) B. 3D reformatted CT Imaging showing Bridging Brachiocephalic Vessel (white arrow) C. 3D reformatted CT imaging showing permanent pacemaker on the right side D. 3D reformatted CT imaging showing a normal aortogram.
See this image and copyright information in PMC

References

    1. Povoski S.P., Khabiri H. Persistent left superior vena cava: review of the literature, clinical implications, and relevance of alterations in thoracic central venous anatomy as pertaining to the general principles of central venous access device placement and venography in cancer patients. World J. Surg. Oncol. 2011;28:173. - PMC - PubMed
    1. Tak T., Crouch E., Drake G.B. Persistent left superior vena cava: incidence, significance and clinical correlates. Int. J. Cardiol. 2002;82(1):92–93. - PubMed
    1. Ruano C.A., Marinho-da-Silva A., Donato P. Congenital thoracic venous anomalies in adults: morphologic MR imaging. Curr. Probl. Diagn. Radiol. 2015;44(4):337–345. - PubMed
    1. Dearani J.A., Danielson G.K. Surgical management of Ebstein's anomaly in the adult. Semin. Thorac. Cardiovasc. Surg. 2005;17(2):148–154. - PubMed
    1. Carpentier A., Chauvaud S., Mace L., Relland J., Mihaileanu S., Marino J.P., Abry B., et al. A new reconstructive operation for Ebstein's anomaly of the tricuspid valve. J. Thorac. Cardiovasc. Surg. 1988;96:92–101. - PubMed

Publication types