ABSTRACT: Persistent left superior vena cava is a common congenital anomaly. We present angiographic pictures and hemodynamic tracings from a case of superior vena cava syndrome in a patient with this anomaly. The images depict a challenging percutaneous intervention for the amelioration of the hemodynamic problem, made even more interesting as the patient was a recipient of orthotopic heart transplant.
J INVASIVE CARDIOL 2010;22:92–93
Persistent left superior vena cava (PLSVC) is rare, and yet the most commonly described thoracic venous anomaly. It is reported in 0.3% of the general population.1 A vast majority (90%) of these drain the venous blood into the right atrium via the coronary sinus.2 Because of the innocuous nature of this condition, those born with it grow into normal adults; it is not uncommon, therefore, to encounter it in patients presenting to the adult cardiac catheterization laboratory. Cardiologists treating adult patients should be alert to this anatomic variation, especially when central venous catheterization via the subclavian or internal jugular vein poses difficulties while performing procedures such as right heart catheterization or pacemaker insertion. The accompanying images are an illustrative depiction of this congenital variation, made even more interesting by a heart transplantation surgery, and provide an opportunity to review this rare yet relevant congenital anomaly. Case description. A 37-year-old patient who underwent orthotopic heart transplant over a year previously presented with superior vena cava (SVC) syndrome. The patient had a persistent left superior vena cava (PLSVC) which was anastamosed end-to-end with the donor SVC at the time of cardiac transplant surgery. Figure 1 shows simultaneous injection of contrast medium into the right internal jugular vein, left internal jugular vein and the donor SVC, and illustrates the venous drainage of the head and neck region. There was a 90% eccentric stenosis (point A) at the anastamotic site of the donor SVC and native PLSVC (Figure 2), and another less prominent stenosis of the innominate vein (point B). Pullback catheter gradients at points A and B were 20 mmHg and 5 mmHg, respectively (Figure 3). Following successful deployment of a 12 mm self-expanding nitinol stent at point B and a 9 mm polytetrafluoroethylene-covered balloon-expandable stent at point A, there was a significant decrease in the gradient across point A and obliteration of the gradient across point B (Figure 4). Jugular venous pressures decreased from 32 mmHg to 17 mmHg (Figure 5), with resultant relief in congestion in the head and neck area and the upper extremities. The relief has been maintained 6 months after the procedure, with no change in pressure gradients at the treatment sites.
1. Biffi M, Boriani G, Frabetti L, et al. Left superior vena cava persistence in patients undergoing pacemaker or cardioverter-defibrillator implantation: A 10-year experience. Chest 2001;120:139–144. 2. Schummer W, Schummer C, Frober R. Persistent left superior vena cava and central venous catheter position: Clinical impact illustrated. Surg Radiol Anat 2003;25:315–321.
_________________________________________ From the Section of Cardiology, Department of Medicine, The University of Chicago Medical Center, Chicago, Illinois. The authors report no conflicts of interest regarding the content herein. Manuscript submitted July 27, 2009, provisional acceptance given August 20, 2009, final version accepted September 8, 2009. Address for correspondence: Neeraj Jolly, MD, 5841 S. Maryland Avenue, MC 5076, Chicago, IL 60637. E-mail: email@example.com