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Images in Cardiovascular Medicine Congenital Absence of Right Superior Vena Cava A “Stomach” within the Heart Ravindranath K. Shankarappa, MD, DM Ravi S. Math, MD, DM Praveen Jayan, MBBS, MD Satish Karur, MD, DM P.S. Seetharam Bhat, MS, MCh Manjunath Cholenahally Nanjappa, MD, DM A 17-year-old boy with visceroatrial situs solitus had a perimembranous ventricular septal defect scheduled for surgical closure. Preoperative echocardiography confirmed the presence of a left superior vena cava (SVC) and a substantially dilated coronary sinus. After induction of anesthesia, there was marked difficulty in advancing the guidewire into the internal jugular vein during central venous catheterization. After multiple attempts, the guidewire was advanced sufficiently to thread a central venous catheter. At surgery, the right SVC was noted to be absent; the right brachiocephalic vein drained into the left SVC. We closed the ventricular septal defect. Postoperative fluoroscopy revealed that the central venous catheter, after passing through the right internal jugular vein, had crossed the midline into the left SVC (Fig. 1). A venogram confirmed the absence of the right SVC; the right brachiocephalic vein joined the left SVC, which drained into the right atrium through the dilated coronary sinus (Fig. 2). The orientation of the coronary sinus within the heart gave the appearance of a stomach, with lesser and greater curvatures. Agitated saline solution injected into the left and right brachial vein showed contrast enhancement of the right atrium through the coronary sinus (Fig. 3). Comment Section Editor: Raymond F. Stainback, MD, Department of Adult Cardiology, Texas Heart Institute at St. Luke’s Episcopal Hospital, 6624 Fannin St., Suite 2480, Houston, TX 77030 Persistent left SVC with an absent right SVC in the presence of visceroatrial situs solitus is an extremely rare anomaly.1,2 By itself, the anomaly requires no treatment; however, its presence should prompt a search for additional cardiac malformations; for example, atrial septal defects, endocardial cushion defects, and tetralogy of Fallot.2 Rhythm disturbances such as heart block, sinus node dysfunction, and ectopic atrial rhythm have been seen.3 The condition may present difficulties during right ventricular pacemaker lead insertion, diagnostic and ablative electrophysiology proce- From: Departments of Cardiology (Drs. Jayan, Karur, Math, Nanjappa, and Shankarappa) and Cardiothoracic Surgery (Dr. Bhat), Sri Jayadeva Institute of Cardiovascular Sciences & Research, Bangalore 560069, India Address for reprints: Ravi S. Math, MD, DM, Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences & Research, Jaya Nagar 9th Block, BG Rd., Bangalore 560069, India E-mail: [email protected] © 2012 by the Texas Heart ® Institute, Houston 300 Congenital Absence of Right SVC Fig. 1 Fluoroscopic image shows the central venous catheter passing through the right internal jugular vein and then crossing the midline into the left superior vena cava. Volume 39, Number 2, 2012 A B C Fig. 2 Venograms. A) Contrast injection into the right internal jugular vein shows that the right brachiocephalic vein joins the left brachiocephalic vein and drains into the left superior vena cava. The right superior vena cava is absent. B) Contrast medium enters the coronary sinus from the left superior vena cava. C) The opacified, grossly dilated coronary sinus—resembling a stomach —drains into the right atrium. Real-time motion image is available at www.texasheart.org/ Click here for real-time motion image: Fig. 2. journal. A B Fig. 3 Contrast echocardiogram with use of agitated saline solution through the A) right and B) left brachial vein. The coronary sinus fills before the right atrium fills. Real-time motion are available www.texasheart.org/journal. Click here for images real-time motionatimage: Fig. 3A. Texas Heart Institute Journal Click here for real-time motion image: Fig. 3B. Congenital Absence of Right SVC 301 dures through the internal jugular vein, systemic venous cannulation for cardiopulmonary bypass, correction of anomalous venous connections, orthotopic heart transplantation, and endomyocardial biopsies.1,2 Because the persistent left SVC receives the entire venous drainage of the head and thorax, an abnormally large coronary sinus might indicate an absent right SVC. Through a simple injection of agitated saline solution into the right brachial vein, the diagnosis can be established if the contrast medium reaches the coronary sinus before it reaches the right atrium. Awareness of this rare anomaly can preclude surprises that might be difficult to “stomach.” 302 Congenital Absence of Right SVC References 1. Yuce M, Kizilkan N, Kus E, Davutoglu V, Sari I. Giant coronary sinus and absent right superior vena cava. Vasa 2011;40 (1):65-7. 2. Bartram U, Van Praagh S, Levine JC, Hines M, Bensky AS, Van Praagh R. Absent right superior vena cava in visceroatrial situs solitus. Am J Cardiol 1997;80(2):175-83. 3. James TN, Marshall TK, Edwards JE. De subitaneis mortibus. XX. Cardiac electrical instability in the presence of a left superior vena cava. Circulation 1976;54(4):689-97. Volume 39, Number 2, 2012