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      Persistent left superior vena cava: a case report and review of literature

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          Abstract

          Persistent left superior vena cava is rare but important congenital vascular anomaly. It results when the left superior cardinal vein caudal to the innominate vein fails to regress. It is most commonly observed in isolation but can be associated with other cardiovascular abnormalities including atrial septal defect, bicuspid aortic valve, coarctation of aorta, coronary sinus ostial atresia, and cor triatriatum. The presence of PLSVC can render access to the right side of heart challenging via the left subclavian approach, which is a common site of access utilized when placing pacemakers and Swan-Ganz catheters. Incidental notation of a dilated coronary sinus on echocardiography should raise the suspicion of PLSVC. The diagnosis should be confirmed by saline contrast echocardiography.

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          Persistent left superior vena cava: case report and literature review.

          A persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly. It is a persistent remnant of a vessel that is present as a counterpart of normal right-sided superior vena cava (SVC) in early embryological development but normally disappears later. Although it can be easily diagnosed by the characteristic chest radiographic appearance of a pulmonary artery catheter (PAC) passed through it after being inserted into the left subclavian or jugular vein, its diagnosis is usually missed by the presence of normal (right) SVC and the passage of the catheter on the right side. Its diagnosis can be confirmed by many noninvasive and invasive tests, or it is incidentally diagnosed at thoracic surgery or autopsy. If it is not associated with other congenital cardiac anomalies, it is usually asymptomatic and hemodynamically insignificant. However, PLSVC has important clinical implications in certain situations. In this article, we describe a patient with bilateral SVC (a normal right SVC and a PLSVC) identified by a PAC in the PLSVC and the pacemaker wires in the right SVC. In addition, we review the literature on prevalence, embryological development, diagnosis, and clinical implications of PLSVC.
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            Persistent left superior vena cava; survey of world literature and report of thirty additional cases.

            F Winter (1954)
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              Pathogenesis of persistent left superior vena cava with a coronary sinus connection.

              The basis for persistence of the left superior vena cava (LSVC), usually associated with cardiac malformations, is poorly understood. We examined 351 staged, serially sectioned human embryos in the Carnegie Embryological Collection and 1208 specimens with congenital cardiovascular malformations in the Pathology Collection of the Johns Hopkins Hospital. A standardized questionnaire was answered for each embryo and autopsy case and a computer program was employed to tabulate concurrent anatomic features. In the normal embryos a symmetric venous system appeared with the heart tube at Carnegie stage 9; the sinoatrial junction translocated to the right and the relationship of the coronary sinus to the LSVC was established by stage 12. The LSVC was patent through stage 20 and subsequently underwent luminal obliteration by compression between the left atrium and the hilum of the left lung. Among the 1208 hearts with a congenital abnormality, 104 (9%) had a persistent LSVC with a coronary sinus connection. Statistically, significantly more frequent associations were found between persistent LSVC and atrioventricular canal defects, cor triatriatum, and mitral atresia and a significantly less frequent association was observed between persistent LSVC and atrial septal defect or patent foramen ovale as a primary defect. The normally late embryonic obliteration of the LSVC suggests that its persistence would be secondary to reduce cardiac compression or to blood flow redistribution at an early stage, and the malformations associated with persistent LSVC support that view. Identification of a persistent left superior vena cava with coronary sinus connection should suggest an associated malformation, especially atrioventricular canal, cor triatriatum, or mitral atresia.
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                Author and article information

                Journal
                Cardiovasc Ultrasound
                Cardiovascular Ultrasound
                BioMed Central
                1476-7120
                2008
                10 October 2008
                : 6
                : 50
                Affiliations
                [1 ]Department of Medicine, Boston University School of Medicine, Boston, MA, USA
                [2 ]Department of Medicine, Section of Cardiology, Boston University School of Medicine, Boston, MA, USA
                [3 ]Department of Radiology, Boston University School of Medicine, Boston, MA, USA
                [4 ]Division of Cardiology, Michigan State University, East Lansing, MI, USA
                [5 ]Maulana Azad Medical College, New Delhi, India
                Article
                1476-7120-6-50
                10.1186/1476-7120-6-50
                2576163
                18847480
                a5fa1bea-cf18-4253-bb44-8a635755fb35
                Copyright © 2008 Goyal et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 1 October 2008
                : 10 October 2008
                Categories
                Case Report

                Cardiovascular Medicine
                Cardiovascular Medicine

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