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ANOMALOUS PULMONARY VEIN AS COLLATERAL IN AORTIC STENOSIS
Anomalous Pulmonary Vein Serving
as Collateral Channel in Aortic Stenosis
with Hypoplastic Left Ventricle and
Endocardial Fibroelastosis*
Cad E. Hunt, M.D., S d y a m r a y a ~Rao, M.B., B.S.,
James H. MoUer, M.D. and Jesse E. Edwards, M.D.
In an infant mW aortic s t e n d and hypoph& left
ventricle, obstmction was PssociPted WW a pulmonary
venous collated channel to the left iDMMlinrtc v e h
Viwutlzedio~udBveIKmEdrrnnd
was atmnPr b total anomP1011~pohnonaty venous
connection.
n atresia, either of the aortic or of the mitral
Ithrough
valve, the pulmonary venous return must pass
an abnormal pathway to reach the systemic circulation. In each condition, the usual route
For editorial comment, see page 113.
for escape of blood from the left side of the heart
into the right side is through an interatrial communication. Uncommonly, as when the foramen
ovale is either sealed or unusually small, the channel for flow of pulmonary venous blood to the right
side of the heart is through an anomalous vein. The
latter may originate either from a pulmonary vein1
or from the left a t r i ~ m . In
~ , ~either instance, it
terminates in a systemic vein. (Such a vein onginating at the atrium has been termed levoatriocardinal vein.) When an anomalous pulmonary vein
serves as the main channel for flow of blood from
the lungs to the right side of the circulation,
angiographic demonstration of the anomalous channel may be obtained. The angiograms may be
falsely interpreted as representing the enti9 total
anomalous pulmonary venous connection, whereas,
in fact, the vein demonstrated angiographically is
simply a collateral channel incident to serious
intracardiac obstruction.
The purpose of this report is to describe a case
of hypoplasia of the left ventricle with endocardial
fibroelastosis and aortic stenosis (which, in some
respects, is functionally like aortic and/or mitral
atresia) in which an anomalous vein extended
from the left upper pulmonary vein to the left
innominate vein. The channel served as a principal
channel for the flow of blood from the pulmonary
'From the Departments of Pediatrics and Pathology, The
University of Minnesota, Minnea lis, Minnesota and the
Departpent of Pathology, The &rles T. Miller Hospital.
St. Paul. Minnesota.
This study was supported
Public Health Service Research Grant 5 RO1 H E O h and Research Training
Grant 5 TO1 HE05SIO from the National Heart Institute.
veins to the right side of the heart and was demonstrated angiocardiographically. We wish to indicate
the similar angiographic features between total
anomalous pulmonary venous connection and the
anomalous vein in conditions with left-sided intracardiac obstruction and means of distinguishing
them by catheterization and angiographic techniques.
The patient was a full-term boy delivered by cesarean
section. At birth, he required resuscitation. He responded
well and was discharged from the hospital at six days of
age in apparent good health. Over the next six weeks,
however, the infant exhibited rapid, noisy respirations,
poor feeding, and upon crying, peripheral cyanosis. When
six weeks old, the infant was thought to be in a state of
congestive heart failure and was referred for evaluation.
Physical examination on admission revealed a mildly
cyanotic infant in respiratory distress. The cardiac rate
was 160 per minute and respiratory rate was 80 per minute.
Values for blood pressures measured simultaneously by the
flush method were equal in an arm and leg. The lungs
were clear to auscultation. Examination of the heart revealed no thrill or heave. The first sound was split with
an ejection click along the lower left sternal border. A
grade I/VI systolic ejection murmur was present at the
base and radiated to the upper back. The second sound
was split and the pulmonic component was moderately
accentuated.
The edge of the liver was palpable 4 cm below the
right costal margin. The spleen was not palpable. Examination of the extremities revealed weakly palpable pulses with
cyanotic nail beds. No differential cyanosis was present.
The concentration of hemoglobin was 17.8 grams per
100 milliliters of blood. The electrocardiogram revealed s
normal QRS axis (+105"), right atrial enlargement, and
left ventricular hypertrophy with strain. Thoracic roentgenograms revealed marked cardiomegaly with prominent
pulmonary arterial vasculature (Fig 1). The initial clinical
impression was tricuspid valvular atresia.
Right-sided cardiac catheterization was performed. The
tip of the catheter could he advanced intn the right
FIGURE1. Roentgenogram of thorax when patient was six
weeks old. Cardiomegaly. In the original films, the pulmonary vasculature appeared prominent.
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HUNT ET AL
ventricle and then through a patent ductus into the descending aorta. The pressure in the latter compartment
was low (63/45, mean = 58 mm Hg). A significant increase in peak systolic pressure was found as the catheter
was withdrawn to the main pulmonary artery (118/57,
mean = 75 mm Hg). The right ventricular pressure was
also markedly elevated (118/0-20). From the right atrium,
the catheter was advanced across a patent foramen ovale
into the left atrium. From the latter chamber, the catheter
was advanced into the left upper pulmonary vein and,
from that vein, into an anomalous vein which coursed superiorly toward the neck. The mean pressure in each atrium
was elevated (19 mm Hg). Data for oxygen saturation indicated three features of significance as follows: (a) pulmonary venous desaturation (76 percent); (b) a right-to-left
shunt through the ductus arteriosus; and (c) no site of
significant increase in oxygen saturation in the superior
vena cava or the right atrium. A right ventriculogram
demonstrated an enlarged hypertrophied right ventricle
and a "reversing" patent ductus arteriosus. In the levogram
phase, pulmonary veins were well visualized and, in addition. a vein running vertically in the left upper mediastinurn
was seen.
Initially, this vein was thought to represent total anomalous
pulmonary venous connection, but the presence of a reversing ductus arteriosus and subsequent filling of the brachiocephalic vessels was felt to be unusual for total anomalous
pulmonary venous connection as a primary d y . Them
fore, a left atriogram was performed (Fig 2). This study
revealed the presence of a small left atrium and reflux of
contrast media into the left and right pulmonary veins,
indicating that these veins entered the left atrium in a
normal fashion. From the region of the junction of the
left upper pulmonary vein with the left atrium, an anomalous
vein arose and passed superiorly to enter the left innominate
vein. A thick left ventricular wall encased a small ventricular
cavity. The latter did not change in shape or size throughout
the remainder of the angiocardiogram.
The studies led to a diagnosis of hypoplastic left heart
syndrome with pulmonary venous collateral flow into the
left innominate vein. The patient's condition deteriorated
and he expired 12 hours after the catheterization.
Pathologic Findings:
At necropsy, the findings in the heart and vascular system were of greatest interest. The great vessels were normally related. The ascending aorta was relatively narrow,
measuring 0.8 cm in diameter, while the pulmonary trunk
was somewhat distended and tense. It measured 1.5 cm in
diameter. The aortic arch revealed a zone of tubular hypoplasia in the segment between the left common carotid and
the left subclavian arteries. In this region, the arch measured 0.4 cm in diameter. The aorta widened to a diameter
of about 1 cm distal to the entrance of a widely patent
ductus arteriosus. The left ventricle was the site of abnormalities, one of which was hypoplasia of the chamber
(Fig 3a). Tbe latter measured 3.5 cm in bight, as from
FIGURE
2. Left atriogram in frontal view. a Early phase. Shardy after injedh of conmaterial into the left atrium (LA), there is evidence of reflux into each pulmonary vein and,
in addition, an anomalous vein (AV) arises from the left upper pulmonary vein (LUPV).
This allows opacification of the
It ascends and terminates in the left innominate vein 0.
superior vena &va (SVC). No evidence of a left-to-right shunt between the atria. b. Late
phase showing opacification of the right atrium (RA) and the right (RV) and left (LV) ventricles. O p d c a t i o n of the left innominate vein 0 and the superior vena cava is still
evident. It is concluded that o p d c a t i o n of the right-sided chambers results from delivery
of blood by way of the anomalous pulmonary venous and the superior vena caval systems.
The opacification of the left ventricle i s a residual effect from direct h w from the left
atrium into the hypoplastic left ventricle. There is major hypertrophy of the left ventricular
wall around the hypoplastic chamber.
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ANOMALOUS PULMONARY VEIN AS COLLATERAL IN AORTIC STENOSIS
the aortic valve to the apex, and 1.7 cm in greatest width.
The wall of the left ventricle was hypertrophied and
measured 0.9 cm in thickness and equaled the thickness
of the hypertrophied right ventricular wall. The left ventricular endocardium was grossly thickened by white, firm
tissue. This layer measured up to 4 mm in thickness.
The aortic valve was stenotic, having the deformity of
the unicuspid, unicommissural, congenitally stenotic aortic
valve. The d t r a l valvular leaflets were essentially normal,
although the chordae were relatively short. The left atrial
chamber was of normal size. The endocardium showed
a mild degree of thickening.
Each of the pulmonary veins joined the left atrial
chamber in a normal manner (Fig 3b). From the left upper
pulmonary vein, just proximal to its junction with the left
atrium, a wide vein measuring about 9 mm in diameter
proceeded upward anterior to the left pulmonary hilus in
a somewhat medial direction to join the lateral extremity
of the left innominate vein. The foramen ovale showed a
valvular competent patency with a potential opening of
4 mm in diameter. The valve of the foramen ovale was
pressed against the atrial septum and showed minimal
herniation toward the right atrium.
The gross abnormalities are summarized in Figure 4.
Histologic examination of the left ventricular myocardium showed marked thickening of the endocardium
by collagenous and elastic fibers, while the myocardial
tissue was not remarkable. Examination of the lungs revealed thickening of alveolar septae on the basis of interstitial edema. There was moderate thickening
- of the medin
of the muscular pulmonary arteries and the proximal segments of the arterioles. Distention of capillaries was also
a feature. as was dilatation of lym~hatics
in the visceral
- pleura and interlobular septa.
In the case presented, the primary cardiac malformation was hypoplasia of the left ventricle
associated with stenosis of the aortic valve. In this
situation, although a patent route existed for the
flow of blood through the left side of the heart,
the evidence indicates that some of the pulmonary
venous return was diverted through the described
nnomalous vein into the systemic venous system
at the level of the left innominate vein. It is
assumed that the nature of the left side of the
heart constituted a significant obstruction to pulmonary venous flow. This accounted for the development of a collateral channel from the pulmonary
venous system to the systemic veins. In this way,
the circulation bears similarity to that observed in
patients with aortic andlor mitral valvular atresia.
In the present patient, left ventricular output
was reduced because of left ventricular inflow
obstruction incident to the hypoplasia of the left
ventricle with endocardial fibroelastosis and the
aortic valve was stenotir. As a result, low pressure
was found in the proximal segment of the aorta.
Support for this concept comes from the fact that
when a right ventriculograrn was performed, some
of the contrast material, passing into the aorta,
FIGURE3. Gross pathologic findings. a. Left atrium (LA)
and left ventricle (LV). The left ventricular chamber is
small. The endocardium is grossly thickened. There is
hypertrophy of the myocardium. The papillary muscles are
covered by thickened endocardium and the chordae are
relatively short. The left atrium is not remarkable. The
atrial septum is normally formed. b. Hilar aspect of the
left lung and related structures. Aorta in badcground. The
upper probe has passed from the left atrium (LA) into
the left upper pulmonary vein and then into the anomalous
pulmonary vein (AV) which ran between the left upper
pulmonary vein and the left innominate vein. The lower
probe lies in the coronary sinus, which is not remarkable.
regurgitated into the ascending aorta to opacify
the brachiocephalic vessels.
The quati& arises as to the manner of develop
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HUNT ET AL
FIGURE4. Summary of the gross abnormalities observed. a. Emphasis is made of the
hypoplastic left ventricle and stenotic aortic valve associated with moderate hypoplasia of
the ascending aorta and a patent ductus arteriosus. b. Emphasis is made on the occurrence
of an anomalous vein running between the left upper pulmonary vein (LUPV),below, and
the left innominate vein (LIV), above. This channel supplied an effective link in the route
for the flow of blood from each lung to the systemic venous system, for ultimate delivery
to the right atrium.
ment of the anomalous vein which extended between the left upper pulmonary vein and the
left innominate vein. It is recognized that in the
early stages of pulmonary venous development,
the primary connections for drainage extend into
two major venous systems, the umbilico-vitelline
system (from which the portal venous system is
developed) and the cardinal system of veins (from
which the systemic veins are developed). Only
after secondary development between a protrusion
from the heart (common pulmonary vein) and the
pulmonary venous system is established, do the
primitive connections disappear. In instances such
as the one herein reported wherein an obstruction
to flow from the pulmonary venous system exists,
one or several of the primitive connections may be
retained as a collateral channel to carry venous
blood from the lungs. It is, therefore, concluded
that the anomalous vein observed in the case
described represents persistence of a primitive
venous connection between the pulmonary venous
system and the cardinal system of veins incident to
failure of development of a free passage from the
lungs through the left side of the heart. If this
explanation is valid, it would mean that the left
ventricular problem existed from early stages of
development of the individual. Such a problem
could have resulted either from aortic stenosis
alone with secondary left ventricular failure or
from intrinsic hypoplasia of the left ventricle with
endocardia1 fibroelastosis.
Identification of the anomalous channel between
the left upper pulmonary vein and the left atrium
angiocardiographically brought to mind the possibility that the channel was the anomalous trunk
as occurs in the entity total anomalous pulmonary
venous connection to the left innominate vein. As
has been indicated in this case, this channel had a
different explanation and was simply an indication
of a serious intracardiac disease. Important points
in differential diagnosis between total anomalous
venous connection, on one hand, and a collateral
vein as that described in association with left-sided
obstructive disease as seen here need to be made.
In total anomalous pulmonary venous connection,
the levels of oxygen saturation, classically, are approximately identical in each of the four cardiac
chambers. In our case, although there was desaturation of blood of the left atrium, the values in this
position were higher than those in the right atrium
and in the right ventricle. Another important
factor is that, in total anomalous pulmonary venous
connection, reversal of flow through a patent ductus
arteriosus is present only when a patent ductus and
severe pulmonary venous obstruction exist4 In our
case there were signs of reversed flow through
the ductus arteriosus. This finding represents an
important factor suggesting the presence of an
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SUBCUTANEOUS IMPLANTATION OF CANCER
obstructive lesion either in the pulmonary veins or
beyond the pulmonary circulation. Strong evidence
for the obstruction to pulmonary venous flow beyond the pulmonary veins was supplied by the
hypoplastic left ventricle. An additional support
for disease involving the left side of the heart was
seen in the angiocardiogram which showed a
reversal of contrast material in the aortic arch from
the ductus arteriosus. As suggested earlier, the
aortic stenosis was probably an important factor
in determining the hemodynamics of the proximal
aorta that would underlie this observation.
In classk examples of total anomalous pulmonary
venous connection, the left side of the heart is
normal. Under this circumstance, it is anticipated
that a left atriogram would yield essentially normal
findings. In our case, in contrast, a left atriogram
showed the presence of pulmonary veins joining
the left atrium which, of itself, would exclude the
diagnosis of total anomalous pulmonary venous
connection and, in addition, demonstrated the
anomalous channel which is the subject of this
report.
REFERENCES
1 SHONE, J.D. AND EDWARDS,
J.E.: Mitral atresia associatecl
with pulmonary venolls anomalies, Brit. Heart J., 26:
241, 1964.
2 BUTLER,H.: Some derivations of the foregut venous plexus of the albino rat, with reference to man, 1. Anat..
86:95,1952.
3 LUCAS,R.V.. JR., LESTER,R.C.., LILLEHEI,C.W. ~ h m
EDWARDS,
J.E.: Mitral atresia with levoatriocardinal vein.
.4 form of congenital pulmonary venous ohstn~ction.
A:~rrr.J . Cardinl.. 9:607, 1962.
4 H a s m m , A.R., PA-,
M.N., MOLTHAN,M.E. AM,
MILLER,R.A.: Total anomalous pulmonary venous connection with severe pulmonary venous obstruction, a clinical
entity, Circulation, 25:916, 1962.
diagnosis of pleural disorders. Complications, chiefly
pneumothorax and intrapleural bleeding, have
been unusual and almost always of minor extent.
Subcutaneous implantation of cancer along the
needle tract has been reported only once before.=
This paper describes a second case.
A 42-year-old housewife underwent radical mastectomy
in November 1984 for scirrhous carcinoma of the right
breast. Postoperatively, she received Cobalt-@ teletherapy
which was complicated by fibrosis of the right upper lung
and pericardial e h i o n , the latter subsiding spontaneously.
She remained asymptomatic until August 1966, when
she began to note progressively severe dyspnea. A roentgenogram of the chest showed bilateral pleural e h i o n s
and densities radiating peripherally from both hilar areas
compatible with lymphatic spread of tumor. Thoracentesis
and biopsy of the right parietal pleura were performed
with a Cope needle. Both cytological and cell block
examinations of the fluid revealed malignant cells, and the
biopsy showed a cluster of similar cells compatible with
metastatic breast carcinoma. Identical malignant cells also
were noted in fluid removed subsequently by a left thoracentesis which was performed without biopsy. Thio-TEPA
was instilled in each pleural space, and therapy with
testosterone ,was begun. (~ilateraloophorectomy had been
performed elsewhere in September 1963, for a benign
disorder).
No changes occurred in the bilateral pleural densities
during the ensuing year, but there was clinical and roentgenographic evidence of increasing lymphatic spread of
tumor in both lungs. Further palliative therapy incliided
stilbestrol, radiotherapy of metastatic lesions of the spine
and pelvis, and three courses of 5-fluorouracil.
In March 1967, six months after thoracentesis and
needle biopsy, sht= developed a firm, tender nodule measur-
Reprint requests. Dr. esse E. Edwards, Charles T. Miller
Hospital, 125 West Col ege Avenue, St. Paul 55102
1
Subcutaneous Implantation of Cancer:
A Rare Complication of Pleural Biopsy
Frederick L. J M ,
Jr., M.D.,F.C.C.P..
A ease of wbcutaneous implantation and growtb of
mammary cancer fonorPing needle biopsy of the parietal
plemra is reported. Tbis is a rare complication wbieb
does not Meet the prognh of patients with m
w
pleural efhsions. Fear of such local spread of tumor
&odd not serve as a contraindication to p l d biopsy.
S i n c e DeFrancisl introduced needle biopsy of
the parietal pleura in 1955, this technique has
been universally accepted and employed in the
"From the Department of Internal ~ e d i c i n e , Geisinger
Medical Center, Dandle, Pennsylvania.
FIGURE1. Subcutaneous nodule at site of needle biapsy of
parietal pleura Arrow points to indsional scar.
CHEST, WL. 57, NO. 2, FEBRUARY 1970
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