Persistent left superior vena cava: Case report

Abstract

Persistent left superior vena cava (PLSVC) is a rare anomaly of the systemic venous circulation. We report the case of a 22-year-old female that had history of multiple repair surgeries for her esophageal atresia, as well as a right lobectomy for bronchiectasis 15 years prior. She was admitted to the surgical ward for complete resection of the right lung. A trans-thoracic echocardiography was performed as part of the pre-surgical work-up and it revealed a dilated coronary sinus which led us to suspect the presence of a PLSVC. The latter was confirmed by a simple "Bubble study" and confirmed by CT angiogram. We will discuss throughout this paper, the clinical and radiological features, as well as the embryology of this anomaly, so that the knowledge of the existence of this anatomical variant, especially if surgery or catheterization is at reach of the medical team, may lead to avoid serious complications.

Keywords: Abnormal systemic venous return; CT angiogram; Persistent superior vena cava left; TTE.

Fig. 1

Transthoracic echocardiogram, parasternal long axis view, illustrating a dilated coronary sinus (CS).

Fig. 2

Injection of agitated saline in the left arm with bubbles (yellow arrow) appearing first in the coronary sinus (A), and later into the right atrium. (B) Coronary sinus draining into an enlarged right atrium.

Fig. 3

Bubble study showing the normal connection of right superior vena cava (RSVC) by early appearance of saline contrast in the right ventricle (RV) (yellow arrow) -right arm injection (parasternal long axis view).

Fig. 4

CT angiogram of chest in axial section (A), sagittal reformatted scan (B), and coronal reformatted scan (C), showing a vascular structure following the left subclavian vein (Fig. 2A). This structure, descending along the left side of the aortic arch (Fig. 4B), consistent with PLSVC (Yellow arrow). Note the right superior vena cava in place (Fig. 2C) (Red arrow).

Fig. 5

CT angiogram of chest in the mediastinal parenchyma window, in axial section showing a collateral (orange arrow), connecting the 2 superior vena cava left (yellow arrow) at right (red arrow).

Fig. 6

Angio computed tomographic scan of chest in the parenchyma window, in axial section (A), and coronal reformatted scan (B), showing destroyed right lung with foci of dilated supra infarcted branches.

Fig. 7

Developmental anatomy of the persistent left superior vena cava as viewed from the posterior aspect of the heart. (A) Pairs of anterior and posterior cardinal veins draining into the embryonic heart via the right and left common cardinal veins. (B) Development of bridging innominate vein connecting left and right anterior cardinal veins during eighth week of gestation. (C) Regression of the right-sided superior vena cava with persistence of left-sided superior vena cava as a single superior vena cava that drains the cephalic portion of the body including upper extremities. (D) Right-sided superior vena cava connected with the persistent left superior vena cava via innominate vein in the post-natal heart. CS, coronary sinus; LACV, left anterior cardinal vein; LCCV, left common cardinal vein; LPCV, left posterior cardinal vein; LSVC, left superior vena cava; RACV, right anterior cardinal vein; RCCV, right common cardinal vein; RPCV, right posterior cardinal vein; RSVC, right superior vena cava .