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374
JACC Vol. 5. No.2
February 19X5:374-X
Persistent Left Superior Vena Cava and Right Superior Vena Cava
Drainage Into the Left Atrium Without Arterial Hypoxemia
EDGAR C. SCHICK, JR., MD, FACC, JOHN LEKAKIS, MD, JAMES A. ROTHENDLER, MD, FACC,
THOMAS J. RYAN, MD, FACC
Boston, Massachusetts
A 46 year old patient who presented with an acute myovecardial infarction was discovered to have a systemic ve·
nous communication with the left heart during attempted insertion ofa pulmonary flotation catheter. There
was no evidence of cyanosis or systemic arterial desaturation. A right superior vena cava that emptied into
the right superior pulmonary vein and a persistent left
Anomalous systemic venous drainage into the left atrium is
uncommon and most often related to persistence of the left
superior vena cava (I). Tabulation of the four largest published studies of persistent left superior vena cava (2-5),
an anomaly estimated to occur in 3 to 4% of patients with
congenital heart disease, yields a 7% prevalence of left atrial
termination among a total of 229 cases. Rarer still are reports
in which a persistent right superior vena cava empties into
the left atrium; to date, only 10 such cases have been reported in the English language (6-15). Although these
anomalies are unusual and most often discovered very early
in life because of associated cyanosis, our case illustrates
the potential for a complex venous abnormality to elude
detection, present problems in patient management and,
when uncorrected, carry a risk of serious complications.
Case Report
Clinical presentation. A 46 year old white man was
admitted to University Hospital on September 20, 1978 with
chest pain and electrocardiographic evidence of acute inferior myocardial infarction. There was no history of previous cardiac problems. Notable findings on physical examination included the absence of cyanosis or clubbing and
From the Evans Memorial Department of Clinical Research and DeMedicine, University Hospital. Boston University Medical
partment of Medicine.
Boston, Massachusetts. Manuscript received April II, 1984; reCenter. Boston.
22, 1984, accepted September 20.
20, 1984.
vised manuscript received August 22.
Jr., MD.
MD, Cardiology Section.
Section,
Address for reprints: Edgar C. Schick, Jr..
Street, Boston.
Boston, Massachusetts 02118.
University Hospital, 75 E. Newton Street.
~i19X5
~)J
9X5
by the American College of Cardiology
superior vena cava draining into the coronary sinus were
confirmed pathologically after death related to a brain
abscess. The embryology, physiology- and noninvasive
diagnostic approach to this unique venous anomaly are
discussed.
(J Am Coil CardioI1985;5:374-8)
the presence of bibasilar rales and an S,
SJ gallop. The hemoglobin was 15.2 gldl, the hematocrit 45.6%.
Diagnostic evaluation. A pulmonary flotation catheter
was passed percutaneously through the left subclavian vein
to facilitate management of heart failure. On the chest XA), the catheter entered the pulmonary artery
ray film (Fig. IIA),
from a left superior vena cava by ~ay of a posterior route,
apparently through the coronary sinus. When this catheter
subsequently dislodged from the pulmonary artery, an attempt was made to position a second catheter from the right
median basilic approach, but systemic pressures and oxygen
saturation were obtained. A chest X-ray film obtained before
removal confirmed the catheter's course through the left
ventricle into the aorta (Fig. I B).
Two-dimensional echocardiography
echocardio[?raphy was performed to
further elucidate the nature of the venous anomaly. In the
parasternal long-axis view, in addition to inferior wall hypokinesia, an enlarged coronary sinus was identified as a
discrete circular structure dorsal and slightly cephalad to the
posterior mitral valve leaflet. After injection of agitated
indocyanine green dye into a left antecubital vein, there was
opacification of the right atrium without evidence of right
to left shunt. However, an injection into the right cephalic
vein resulted in prompt visualization of the left side of the
heart and delayed appearance of echo targets in the right
atrium and ventricle (Fig. 2).
Similarly, a first pass radionuclide flow study from the
left arm disclosed no evidence of right to left shunting (Fig.
3A). The left superior vena cava flow appeared to enter the
medial region of the right atrium, probably through the
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AL.
SCHICK ET AL
ACY ANOTIC VENA CAV AL DRAINAGE TO LEFr ATRIUM
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A
Figure l. A, Chest X-ray film after insertion of the pulmonary
artery catheter from the left subclavian vein. The arrows highlight
the course of the catheter from the persistent left superior vena
cava to the pulmonary artery by way of the coronary sinus. B,
Chest X-ray film after replacement of the catheter from the right
arm. The arrows again trace the pathway of the catheter from the
right superior vena cava to the aorta.
coronary sinus. A flow study from the right arm showed
prompt direct visualization of the left ventricle, again consistent with peripheral venous drainage into the left atrium
(Fig. 38). Faint, late right heart opacification was also believed to be present.
Cardiac catheterization and subsequent course. After
discharge, the occurrence of frequent anginal episodes eventually led the patient to undergo diagnostic cardiac catheFigure 2. Contrast echocardiograms in the apical four chamber
view. A, Baseline. RA = right atrium; RV = right ventricle; LA
= left atrium; LV = left ventricle. B, Opacification of the right
heart by contrast after left antecubital venous injection of agitated
indocyanine green dye solution. C, Echo contrast completely fills
the left heart chambers after right antecubital venous injection.
Less dense contrast.
contrast, evident in the right atrium and right ventricle.
followed left heart opacification by 2 to 3 beats.
terization. Right heart catheterization was accomplished from
the right femoral vein without difficulty. Right-sided pressures were mildly elevated, but no intracardiac oxygen stepup was detected and the catheter could not be advanced
directly into a superior vena cava. From the right median
basilic vein, a catheter was easily advanced to the left atrium
where the hemoglobin saturation of 96% equaled the systemic arterial values. Saturations were not measured along
the course of this vein proximal to the atrium. A left atrial
angiogram provided no evidence of an atrial sep.d defect.
Selective coronary angiography demonstrated total occlusion of the proximal right coronary artery and a 50% narrowing of the left anterior descending artery; diaphragmatic
hypokinesia was noted on ventriculography. Surgical therapy was considered, but the patient declined and was followed up elsewhere.
In May 1981, he reentered University Hospital because
of excruciating right frontal headache. Computed tomography localized a ringlike lesion, consistent with an abscess,
in the right medial frontal lobe. Rapid deterioration occurred
despite surgical drainage of the abscess and subsequent right
frontal pole resection. Numerous blood cultures were negative.
Postmortem findings. At autopsy, there was evidence
of previous transmural inferior infarction and biventricular
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SCHICK ET AL.
ACYANOTIC VENA CAVAL DRAINAGE TO LEFT ATRIUM
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no evidence of atrial septal defect or patent foramen ovale,
no innominate bridge or other intrathoracic venous collateralization was detected and the inferior vena cava was
normal. A large brain abscess occupied nearly the entire
remaining right cerebral hemisphere.
Discussion
t t t
Figure 3. First pass radionuclide flow studies in the anterior projection. A, After left ann
arm injection of technetium, proceeding left
to right, the isotope may be followed through the left superior
vena cava and the coronary sinus into the right atrium, with subsequent filling of the right ventricle and pulmonary artery. 8,
B,
Radionuclide injected into the right arm is seen first in the superior
chambers .
vena cava, followed by opacification of the left heart chambers.
Less intense activity is also apparent in the right ventricle in the
lower three frames (arrows).
hypertrophy. The right coronary artery was totally occluded,
with moderate atherosclerosis of the left anterior descending
and the circumflex arteries. A left superior vena cava was
present and communicated with the right atrium through a
dilated comary sinus. A right superior vena cava was present, but it terminated in the right superior pulmonary vein
(Fig . 4).
4) . There was
and thus emptied into the left atrium (Fig.
Noninvasive diagnosis. The dissemination of noninvasive technology in recent years, particularly the use of
radionuclide flow and echocontrast studies, has improved
the ability to detect and define anomalies of venous drainage
as exemplified by this case (16,17). The echocardiographic
hallmark of the persistent left superior vena cava, a dilated
coronary sinus (16), was present in our patient and certainly
would have been detected on a routine echocardiogram without previous knowledge of the unusual pathway of the pulmonary flotation catheter. In the absence of cyanosis, however, the anomaly would probably not have been pursued
further, and the potential systemic venous-arterial connecmissed . In other reported cases of this
tion might have been missed.
disorder in cyanotic adults (10,15), the discovery was made
in a similarly fortuitous fashion when radioisotope failed to
appear in the lungs after a right arm vein injection for a
lung scan. Thus, lacking evidence of right ventricular disease
ease,, it is recommended that echocardiographic evaluation
of the cyanotic adult always include contrast injection from
both arms.
Embryologic considerations. A review of venous ontogeny elucidates the probable basis for the anomalies observed. Ordinarily during fetal development, the left of the
paired anterior cardinal veins involutes after the development of the innominate vein. The remaining right precardinal and common cardinal veins eventually become the
(18) . Failure of the innominate vein to
superior vena cava (18).
either enlarge to normal caliber or assume a normal orientation appears to favor persistence of the left superior
vena cava (2,19). Left caval drainage under these circumstances is usually into the right atrium by way of the coronary sinus, which is frequently accompanied by obliteration
of the right superior vena cava. The right cava in our case
was noted to adjoin the right superior pulmonary vein, thus
establishing communication with the left atrium.
During early lung development, the primordial pulmonary venous system is in direct communication with the
system,, and relatively late atrial continuity
cardinal venous system
is established by fusion of the pulmonary venous channels
with a dorsal evagination from the left atrium (20). The
persistence of the primitive systemic pulmonary venous collateral vessels probably accounts for the generally high prevalence of anomalous pulmonary venous drainage with superior vena cava anomalies and for the specific findings in
our case. SemanticaIly,
Semantically, although a distinct right superior
vena cava was present in our patient and the patient reported
lACC Vol. 5. No.
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February 19X5:374-8
4. Postmortem
Postmortem findings. Left, Photograph
Photograph of
of the
the posterior
posterior
Figure 4.
surface of
of the
the heart.
heart. Right, Diagram
Diagram of
of the
the pathologic
pathologic specimen
depicting the
the pathways
pathways of
of probes
probes from the
the righl
right superior vena
vena cava
(RSVC) through
(RSYC)
through the
the right
right superior pulmonary
pulmonary vein
vein to
to terminate
terminate in
in
lhe
the left atrium, and from the left superior
superior vena cava
cava (LSYC)
(LSVC)
through the coronary sinus,
which had
had been
been opened,
opened, into
into the
the right
right
sinus. which
atrium.
atrium.
by Park et al. (15), it may be more appropriate to refer to
the communication between a systemic vein and the left
atrium via a pulmonary vein as a levo-atrial cardinal vein.
vein .
This term has been applied by Edwards and coworkers (21,22)
(2\ ,22)
to embryologically analogous systemic-pulmonary venous
continuity
continuity.. Other cases of persistent right superior vena cava
are probably the result of an initial systemic-pulmonary
venous anastomosis more proximal to the left atrium. Evidence of this communication is ultimately lost, and the caval
orifice appears separate because of incorporation of the pulmonary veins into the left atrial wall. This explanation seems
more likely than the malposition of the right horn of the
coronary sinus proposed by Kirsch et al. (7).
Acyanosis. The puzzling aspect of our case was the
desaturation, which
complete absence of systemic arterial desaturation.
has been present in all previously reported instances of right
superior vena cava drainage into the left atrium. There are
two reports (23,24)
(23.24) of left superior vena cava termination
in the left atrium without arterial desaturation associated
with aortic coarctation. It was suggested that a communication between the left and right superior vena cava through
a so-called innominate bridge resulted in preferential flow
from the left superior vena cava to the right atrium because
of the greater impedance to left ventricular filling. This is
similar to the explanation for the left to right shunt
shunl with an
atrial septal defect. As a result, no systemic venous flow
reached the left atrium and cyanosis was absent. A case in
which abnormal inferior vena cava drainage into the left
atrium was "masked" by a shunt in the presence of an atrial
septal defect has also been reported (25). Neither an in-
SCHICK ET AL.
AC YANOTIC VENA CAVAL DRAINAGE TO LEFT ATRIUM
ACYANOTIC
377
nominate bridge nor an atrial septal defect was identified at
catheterization or autopsy in our patient. Since retrograde
venous angiography was not performed, we cannot directly
address the only remaining possibility; namely, that extrathoracic systemic venous collateralization shunted venous
flow from the right to left superior vena cava in the manner
just described. Pressurized bolus injection of echo contrast
medium or radioisotope under these circumstances might
temporarily reverse the flow balance, accounting for echo
contrast opacification of the left heart chambers as well as
the right heart opacification suggested by the first pass radio's
nuclide scan. The report (26)
Ebstein's
(26) of a patient with Ebstein
anomaly with a left to right shunt through a left superior
vena cava in the presence of a normal mitral valve supports
this hypothesis.
Complications and management. Despite the absence
of cyanosis in our patient and the tolerance of cyanosis in
many other reported cases, patients with direct continuity
of the systemic venous and arterial circuits are at risk for
the complications of brain abscess or paradoxical embolization
zation.. Brain abscess with both left and right superior vena
cava-left atrial communication has been previously reported
(15,27) . Paradoxical embolization with transient right hemi(15.27).
paresis and aphasia was associated with the flushing of an
intravenous catheter in a patient whose right superior vena
cava drained into both atria (13). That potential or intermittently functional communications of this type confer the
same susceptibility is amply illustrated by our case. For
these reasons, detection of a systemic venous-left atrial connection is an indication for surgical closure.
We g.ralefully
g.ratefully recog.nize Ihe
the secrelarial
secretarial contribution
conlribulion of Mary Lou Caslillo
Castillo
In
to Ihe
the rreparation
rreparalion of Ihis
this manuscript.
manuscripl.
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