Download Congenital Absence of Right Superior Vena Cava

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