Because the patient was to be scheduled for chemoport insertion at a later date, we decided to evaluate her vascular anatomy. A 2-dimensional transthoracic echocardiogram revealed a dilated coronary sinus. No other anatomical or functional abnormalities were detected. A magnetic resonance imaging venogram confirmed the presence of PLSVC and hypoplastic right internal jugular vein with multiple small venous channels on the right side of neck (Figure 4). It was not possible to identify the exact location of the drainage of the PLSVC; however, the dilated coronary sinus seen on the 2-dimensional transthoracic echocardiogram was suggestive of PLSVC draining into the right atrium.
The thoracic venous drainage system often is associated with various anomalies. PLSVC is one such commonly encountered anomaly. Its incidence is 0.5% among the general population and increases up to 10% in patients with congenital heart diseases.4 Incidence of congenital heart diseases is more when left superior vena cava drains into left atrium.
During embryonic development,5 the venous system has 2 superior (anterior) and 2 inferior (posterior) cardinal veins that return blood from the superior and inferior parts of the body, respectively (Figure 5). Both pairs of vein join to form the right and left common cardinal veins before entering the primitive heart. The left common cardinal vein forms the oblique vein of the left atrium and the coronary sinus. The right and left superior cardinal veins form the innominate vein. The cranial part of the right superior cardinal vein transforms into the right internal jugular vein, whereas its caudal part transforms into the right superior vena cava. By contrast, the part of the left superior cardinal vein proximal to the innominate vein forms the left internal jugular vein, whereas its distal part regresses to form the “ligament of Marshall.”
If this regression does not occur, it results in a persistent left-thoracic vascular structure like PLSVC that mostly empties into the coronary sinus. The left superior vena cava runs vertically to the left of the aortic arch and main pulmonary artery and along the left atrium. It then turns medially, pierces the pericardium, and stays in the atrioventricular groove. In 90% of cases, the PLSVC drains into the coronary sinus, causing it to dilate. In remaining 10% of the cases, the PLSVC drains into the inferior vena cava or hepatic vein or left atrium. If the CVC runs along the left mediastinal border, one should suspect the presence of PLSVC. In such a case, the CVC may be in the descending aorta, superior intercostal vein, pleura, pericardium, or mediastinum. Chest X-ray, blood gas analysis showing venous values, and a venous pressure waveform usually allow the diagnosis of PLSVC. Computed tomography scan, 3-dimensional contrast-enhanced magnetic resonance venogram, and 2-dimensional transthoracic echocardiography are additional diagnostic tools.
PLSVC draining into the right atrium via coronary sinus is associated with the risk of cardiac perforation during CVC insertion.6 Underlying PLSVC during cardiopulmonary bypass may lead to ineffective retrograde cardioplegia.7 Complications also have been reported during transvenous pacing for bradycardia and during implantation of an automated internal cardioverter defibrillator.8,9 Patients with PLSVC should be evaluated for the presence of any coexisting congenital cardiac defects like atrial septal defect, ventricular septal defect, tetralogy of Fallot, endocardial cushion defects, and cor triatriatum. PLSVC that drains into the left atrium will result in a right-to-left shunt, leading to cyanosis and risk of paradoxical embolism.4
Venous anomalies frequently are encountered in daily practice. Our case re-emphasizes the importance of using ultrasound during CVC placement. It enabled the immediate diagnosis of an absent right internal jugular vein. Because ultrasonography does not allow identification of the tip of a CVC, a chest X-ray after CVC insertion may be advisable for such purpose.
Name: Nilesh B. Sonawane, DNB, DA.
Contribution: This author helped conceive and design the study, search the literature, and prepare the manuscript.
Name: Falguni Rajendra Shah, MD, DNB, FCPS, DA.
Contribution: This author helped search the literature, edit the manuscript, and with intellectual content.
Name: Prakash Jagadish Gawankar, MD, DA.
Contribution: This author helped design the study, search the literature, edit the manuscript, and with intellectual content.
This manuscript was handled by: Hans-Joachim Priebe, MD, FRCA, FCAI.
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