Download Meandering Right Pulmonary Vein Simulating the Scimitar Syndrome*

GOODMAN, JAMSHIDI, HIPONA
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paralysis in left ventricular failure. Am J Med Sci 188:
691-697, 1934
Tashneck AB: Left recurrent laryngeal nerve paralysis in
patient with arteriosclerotic heart disease. Southern Med
J, 46:718-719, 1953
Zelman S, Nice GW: Recurrent left laryngeal nerve
paralysis in atherosclerotic heart disease. JAMA 149: 12911292, 1952.
Huppler EG, Schmidt H, Devine KD, et al: Causes of
vocal cord paralysis. Proc Staff Meet Mayo Clin 30:518521, 1955
Erlanger H, Levine SA: Atrial septa1 defect: A report of
two cases in which there was recurrent laryngeal nerve
paralysis. Am Heart J 26:520-527, 1943
Dolowitz DA, Lewis CS: Left vocal cord paralysis associated with cardiac disease. Am J Med 4:856-862, 1948
Mead KC: Persistent patency of the ductus arteriosus.
JAMA 50:2205-2210, 1910
Talley JD, Fowler K: Tetralogy of Fallot with hypoplasia
of dextroposed aorta. Am J Med Sci 191:618-626, 1936
Camishion RC, Gibbon JH, Pierucci L, et al: Paralysis of
the left recurrent laryngeal nerve secondary to mitral
valve disease: Report of two cases and literature review.
Ann Surg 163:818-827, 1960
Fife R, Miur A: Laryngeal paralysis associated with
mitral stenosis. ( Ortner's syndrome ) : Report of two cases.
Glasgow Med J 36: 184-167, 1955
Malcomson K, Hillman LM: Ortner's syndrome. Guy
Hosp Rep 105:307-319, 1956
Fetterolf G , Nonis GW: The anatomical explanation of
the paralysis of the left recurrent laryngeal nerve found in
certajn cases of mitral stenosis. Am J Med Sci 141:625638, 1911
Ari R, Harvey WP, Hufnagel CA: Etiology of hoarseness
associated with mitral stenosis: Improvement following
mitral surgery. Am Heart J 50: 153-160,1955
Brinton WD: Primary pulmonary hypertension. Br Heart
J 12:305-311, 1950
Soothill JF: A case of primary pulmonary hypertension
with paralyzed left vocal cord. Guy Hosp Rep 100:232237, 1951
Bohme Von H, Bohme G: Das Ortner-Syndrome. Munchen Med Wschr 104:681-887, 1962
Pamell FW, Brandenburg JH: Vocal cord paralysis: A
review of 100 cases. Laryngoscope 80: 1036-45, 1970
Clerf LH: Unilateral vocal cord paralysis. JAMA 151:900903, 1953
Goff WF: Letter to the Editor. JAMA 212:1378-1379,
1970
Meandering Right Pulmonary Vein
Simulating the Scimitar Syndrome*
Lawrence R. Goodman, M.D.,OV Ahmd Jamshidi, M.D.,t and
Florencio A. Hipona, M.D.1
'From the Department of Medicine, Cardiovascular Laboratory, Bridgeport Hospital, Bridgeport, Connecticut and
the Department of Radiology, The Boston City Hospital,
Boston.
''Resident in Radiology, Boston University Medical Center.
tAssistant Clinical Professor in Pediatrics, Yale University
School of Medicine; Director, Cardiovascular Laboratory,
Bridgeport Hospital.
:Associate Professor of Radiology, Harvard Medical School.
Reprint requests: Dr. Hipona, Department of Radiology.
Boston City Hospital, Boston 02118
A 51-year-old woman presented with clinical and chest
x-ray film findings simulating partial anomalous pulmonary venous rebelow the diaphragm (scimitar syndrome). Angiography, however, revealed the scimitm
vein turned cephalad at the diaphragm and draiied into
the left side of the left alrium. It is postulated that the
abnormal left atrial drainage is via persistent thebestan
veins.
0
n frontal chest x-ray film, a large pulmonary vein
running caudad and medially along the right heart
border to the diaphragm is referred to as a scimitar sign.
This commonly indicates partial anomalous pulmonary
venous return to the inferior vena cava or low right
atrium. Associated findings in the scimitar syndrome
include displacement of the heart to the right, abnormal
segmentation of the right lung, hypoplastic right hemithorax and hypoplastic right pulmonary artery. Varicosities of the pulmonary veins or arteriovenous malformations may cause a scimitar-like vein adjacent to the right
This communication describes a patient whose condition simulated the classic scimitar syndrome except that
the right pulmonary vein, after a meandering course,
drained into the left atrium rather than the inferior vena
cava.
A 51-year-old woman was admitted to the Bridgeport
Hospital for episodes of coughing small amounts of bright red
blood every several weeks over a 20-year period. The last
episode had been three weeks prior to admission. These
episodes were associated with difficult breathing when lying
in the left lateral decubitus position. For the last 15 years, the
patient had had multiple episodes of right-sided pleurisy,
fever and productive cough. Recurrent asthmatic attacks
required use of bronchodilators. X-ray films of this period
described a large tubular density over the right mid-lung, as
well as right pleural thickening.
Past medical history revealed one bout of hematemesis
about 12 years previously. This was believed due to a
"stomach ulcer."
Physical examination was unremarkable except for dullness
and decreased breath sounds at the right base.
Frontal chest x-ray 6lm (Fig 1A) revealed dextroposition
of a normal-sized heart. There was pleural thickening at the
right base. A 9 mm diameter vessel was seen adjacent to the
right heart border. The main right pulmonary artery a p
peared small. On the lateral film (Fig 1B) the 9 mm vessel
was seen posteriorly overlapping the vertebral column. A p
parently, continuous with this vessel was a 12 mm vessel
coursing upward and forward to the upper posterior heart
border. Right anterior oblique examination c o n h e d their
continuity.
Angiography (Fig 2A) showed the right main pulmonary
artery to be smaller than the left. A lingular artery was
present on the right (mirror image circulation). The levophase (Fig 2B, C ) demonstrated the posterior vessel (described above) drained the entire right lung toward the
diaphragm. Instead of piercing the diaphragm, it was continuous with the larger mid-thoracic vessel. This vein drained
cephalad, crossed the mediastinum and entered the left side
CHEST, VOL. 62, NO. 4, OCTOBER, 1972
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MEANDERING RIGHT PULMONARY VEIN
FIGURE1A. Frontal chest film shows dextroposed normal-sized heart. The right hemithorax and
pulmonary artery are small. A 9 m m vessel is seen adjacent to the right heart border (arrow).
B. Lateral chest film shows the 9 m m vase1 running posteroinferiorly over the vertebral column
(posterior arrow). It is continuous with a 12 mm vessel running anteriosuperiorly (anterior arrow).
of the left atrium.
Right-sided cardiac catheterization, including bilateral
pulmonary wedge pressures and oxygen saturations, was
normal.
With the exception of the left atrial drainage, this
case has all the features of the scimitar syndrome. There
is hypoplasia of the right main pulmonary artery and a
mirror image lung. The initial course of the draining vein
also mimics the scimitar vein. Clinically, dyspnea and
recurrent bouts of the right chest pain are commonly
associated symptoms. Although the cause of the hemoptysis is uncertain in this case, hemoptysis is common
in patients with pulmonary venous vari~osities.~
Embryologically, the trachea and lungs arise as a
midline structure from the primitive f o r e g ~ t . A
~ .plexus
~
of channels course through the loose mesenchyme and
communicate with both the cardinal and umbilicomesenteric veins. As the pulmonary veins enlarge, the
other connections atrophy. If the cardinal or umbilicomesenteric channels are abnormally large, they may
persist as abnormal vessels with various aberrant venous
connections. It would seem to follow that abnormally
FIGURE
2A. Dextro-phase of angiogram shows normal-sized right atrium and ventricle with minimal rotation to the right. The right pulmonary artery is small and shows a lingular artery ( arrow).
B and C. Levo-phase shows the entire right lung draining via a posteroinferiorly oriented vein.
At the diaphragm, it takes a meandering course upward, crosses the midline and drains into the
left side of the left atrium (arrows outline direction of flow).
CHEST, VOL. 62, NO. 4, OCTOBER, 1972
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large but normally draining vessels might persist in a
similar manner.
During fetal life, the superior vena cava is continuous
with the coronary sinus. Small thebesian veins drain
from this area into the left atrium. If the coronary sinus
should be atretic or 0ccluded,2.~.gblood drains retrogradely into the left superior vena cava. If the latter
atrophies too, dilated thebesian veins drain into the left
atrium (foramen of Bochdalek). Aberrant pulmonary
veins may join this system at any point. Usually the
coronary sinus or left superior vena cava, or occasionally
both,*^^*^^ drain the abnormal vessel. If neither is patent, blood drains via the thebesian system into the left
atrium. We postulate this type of drainage to explain the
aberrant left-sided insertion into the left atrium.
Becu et ale and Nelson et a14 emphasize the importance of distinguishing abnormal connections from abnormal drainage. The latter causes the return of oxygenated pulmonary venous blood to the right side of the
heart. These left-to-right shunts are often surgically correctable. With abnormal connections, the route back to
the left atrium may be circuitous, but hemodynamically
is normal.
This case illustrates the value of cardiac catheterization in ruling out left-to-right shunts and the value of
angiography in assessing the direction of flow.
1 Bender F, Hilgenberg F, Jungehulsing C: Dextrokardie
mit Pulmonalvenentransposition bei Partieuer Lungenagenesie. Ztschr Kreiglaufforch 46: 172-179,1957
2 Blake HA, Hill RJ. Manion WC: Anomalous pulmonary
venous return. Circulation 32:406-414, 1965
3 Gottesman L, Weinstein A: Varicosities of the pulmonary
veins. Dis Chest 35:322-327, 1959
Isolated Lobular Pulmonary Artery
Atresia with Systemic Collaterals in
Tetralogy of allot*
Robert Klint, M.D.; Alexis Hartmnn, Jr., M.D.;
Nickohuss Schad, M.D., F.C.C.P.; C h Weldon, M.D.;
and D a d Coldring, M.D.
Thishad
report describes a patient with tetralogy of Fallot
absence or hypoplasia of the right upper
who
lobe pulmonary artery and unusual collateral blood supply to that lobe. The collateral vessel was used to create
a systemic-pulmonary artery shunt.
The patient was the full-term product of a normal pregnancy, labor and delivery. The child was asymptomatic until
13 months of age when he became cyanotic while crying. He
experienced six similar episodes during the next nine months.
Physical examination revealed a faintly cyanotic boy in no
distress. He demonstrated cardiomegaly, a prominent thrill in
the third left intercostal space, a grade IV/VI pansystolic
murmur maximal at the second and third left intercostal
spaces with radiation into the back, and a diminished second
heart sound in the pulmonic area. The nailbeds were faintly
cyanotic; clubbing was absent.
The electrocardiogram demonstrated right axis deviation
'From the Edward Mallinckrodt Department of Pediatrics
Division of Cardiology, Edward Mallinckrodt Institute ol
Radiology and the Mary Culver Department of Surgery,
Division of Cardiothoracic Surgery, Washinqton University
School of Medicine, and St. Louis Children s Hospital, St.
Louis.
Aided in part by the Arthur Fund, St. Louis Children's Hospital Heart Mothers' Fund, Scott Centsch Memorial Fund,
William T. Beauchamp Memorial Fund, and the John Clay
Seier Fund.
Reprint requests: Dr. Klint, St. Louts Children's Hospital, St.
Louts 63110
4 Nelson WP, Hall RJ, Garcia E: Varicosities of the pulmonary veins simulating arteriovenous fistulas. JAMA
195:103107, 1966
5 Vengsarker AS, Kincaid OW, Weidman WH: Selective
angiocardiography in diagnosis of varicosities of the pulmonary veins. Am Heart J 88:296-298,1963
6 Becu LM, Tauxe WN, D u Shane JW, et al: Anomalous
connection of pulmonary veins with normal pulmonary
drainage. Arch Path01 59:463-470.1955
7 Patten B: Human Embryology (3rd ed). New York.
McCraw Hill, 1968
8 Fieldstein LE, Pick J: Drainage of the coronary sinus into
the left atrium. Am J Clin Path 12:66-89,1942
9 Reed AF: Left superior vena cava draining the blood
from a closed coronary sinus. J Anatomy 73:195-197,
1938
10 Nakib A, Moller JH, Kanjuk VI, et al: Anomalies of the
pulmonary veins. Am J Cardiol20:77-90, 1967
FIGURE1. PA projection. This roentgenogram shows cardiomegaly, left aortic arch, and inferior displacement of the right
main pulmonary artery.
CHEST, VOL. 62, NO. 4, OCTOBER, 1972
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