Download Anomalous Origin of Left Pulmonary Artery from Ascending Aorta

ANOMALOUS ORIGIN OF LEFr PULMONARY ARTERY
and others,2s3but not as often as Coxsackie viruses.
Since carditis due to ECHO viruses has been associated with serious consequences, including life-threatening arrhythmias and even death, the diagnosis of
asymptomatic or truly benign pericarditis or myocarditis
occurring with ECHO viruses is obviously i m p ~ r t a n t . ~ - ~
Therefore, the so-called "benign" pericarditis associated
with enterovirus disease may be a somewhat misleading
classification.
The electrocardiographic changes in our patient were
thought to be consistent with viral pericarditis and myocarditis. There were no historical or clinical grounds to
associate them to coronary artery disease, hypokalemia,
alkalosis or severe central nervous system disease.
The elevations of the serum glutamic pyruvic transaminase pointed to liver involvement. There was a sustained rise in the alkaline phosphatase. Confirmation of
the hepatic origin of these admittedly small enzyme
changes were furnished by the simultaneous elevation of
the patient's serum guanase and his ornithine carbamyl
transferase. These two latter enzymes, particularly
guanase, are specific for parenchymatous liver disease.
The patient's appetite remained depressed for a month,
and he was found to be hypoglycemic on several occasions despite reduction of insulin dosage. This anorexia
might possibly have been due to hepatic involvement.
Hepatitis associated with ECHO 9 virus disease has
been reported in a previous communication by
A recent editorialR in the Journal of the American
Medical Association appears pertinent in regard to this
case. The editorial writer states that isolation and identification of ECHO virus from the spinal fluid is preferable
for definitive diagnosis in patients with this type of
meningitis as compared to isolation from the throat and
feces. The former was accomplished in our ~ a t i e n t . ~
The pathophysiology of cardiac involvement in
enterovirus disease is a subject for a recent editorial by
L e r n e r . V e points out the occurrence of an early acute
infectious phase and prolonged auto-immune and recovery phase of enteroviral myocarditis. Accordingly,
our patient was not permitted to return to work until his
electrocardiogram had become completely normal and
his fatigue had subsided.
REFERENCES
1 Melnick JL, Aagren K: Poliomyelitis and Coxsackie viruses
isolated from normal infants in Egypt. Proc Soc Exper Biol
Med 81 :621-624, 1952
2 Kibrick S: Current status of Coxsackie and ECHO viruses
in human disease. Prog Med Virol6:27-70, 1964
3 Bell EJ, Grist NR: ECHO viruses, carditis and acute
pleurodynia. Am Heart J 82:133-135, 1971
4 Mendex-Cashion D, Sanchez-Longo LP, Valcarcel MI, et
al: ECHO virus type 1 and aseptic meningitis. J Pediat
63:432-436, 1963
5 Taytsch FZ: Wirusy Echo izolowane z przypadkow
zachorowan z objawarni zapalenia opon mozgowych.
P ~ e g l a dEpidemiologicmy ROK XV: 179-187, 1981
6 Johnson RT, Portnoy B, Rogers NG, et al: Acute benign
pericarditis. Arch Intern Med 108:823-832, 1961
7 Schleissner LA, Portnoy B: Hepatitis and pneumonia associated with ECHO virus, type 9, infection in two adult
siblings. Ann Intern Med 68:1315-1319, 1968
8 Editorial: Echovirus as a cause of meningism. JAMA
212:1206, 1970
9 Lemer AM: Coxsackie virus myocardiopathy. (Editorial) J
Infect Dis 120:496-499, 1969
Anomalous Origin of Left Pulmonary
Artery from Ascending Aorta, Right
Aortic Arch and Right Patent Ductus
~rteriosus*
Walter H. Herbert, M.D., F.C.C.P.;" Michael Rohman,
M.D., F.C.C.P.;? Peter Farworth, M.D.;$ and Saraswathi
Swamy, M.D.5
A case of anomdous left pulmonary artery (ALPA) arising from the ascendiog aorta, a right aortic arch and a
right patent ductus arterims, a combination not previously reported, is described. Current concepts regarding
the genesis of an ALPA also predict main pulmonary
artery hypoplasia and defects of the ventricular septum.
These associated defects were not present io our patient.
Their absence suggests that the developmental faults associated with these abnormalities are even more complex
than previously suspected. The need for early diagnosis
and the efficacy of surgical correctioo are emphasized.
I
n 1969 Caudill and co-workers' described a case of
anomalous origin of the left pulmonary artery arising
from the aorta and added the 35th such report to the
world literature. Anomalous origin of the right or left
pulmonary artery (ALPA, ARPA) from the aorta or its
primary divisions (ie innominate) is a rare phenomenon
although it was first recognized over 100 years ago.2 It
was not until 1949, however, that Bopp3 demonstrated
an ARPA at postmortem examination.
The importance of angiographic studies to demonstrate the origin of both pulmonary arteries has been
emphasized by several authors1.4-'2 as an ALPA or
ARPA is a correctible lesion and successful surgical
It is, of
intervention has been reported.1~e~7*10-1Z
course, most important to meticulously seek out associated lesions which are so frequently p r e ~ e n t . ~ ~ ~ - ' ~ * ' ~
This presentation describes the association of an
ALPA arising from the ascending aorta, a right aortic
arch and a right patent ductus arteriosus, a combination
which has not, to our knowledge, been previously reported. This association of defects is particularly noteworthy as it does not include pulmonary outflow anomalies or defects of the ventricular septum. These latter
deficiencies would be anticipated by the current and
'From the Cardiopulmonary Laboratory and Departments
of Surgery and Pediatrics, Grasslands Hosnital, Valhalla;
and De artments of Medicine and Surgery, New York Medical coRege, New York.
''Assistant Professor of Medicine.
tProfessor of Surgery.
:Director of Pediatrics, Grasslands Hospital.
$Fellow, Westchester Heart Association.
Reprint requests: Dr. Herbert, Grasslands Hospital, Vahalh,
New York 10595
CHEST, VOL. 63, NO. 3, MARCH, 1973
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HERBERT ET AL
FIGURE1. The catheter passes up from the right saphenous
vein to enter the right atrium, right ventricle (RV), main
pulmonary artery ( MPA), right pulmonary artery ( RPA) and
the descending aorta (DAo) in the right chest via a right
duchls artenosus.
FIGURE2. Contrast media is delivered to the descending aorta
( DAo) via the right ductus arteriosus ( D ) . The proximal
aorta also fills demonstrating the large left pulmonary artery
( LPA ) arising from the ascending aorta ( AAo ) .
widely held concepts of developmental faults which
cause main trunk pulmonary arteries to arise from the
aorta.ll.lS
ly unsaturated.
Cineangiocardiographic studies demonstrated the left pulmonary artery to arise from a left lateral position on the
ascending aorta (Fig 2,3). It rose steeply to approximately
the same height as the aortic arch and then ramified normally
to supply the left lung. The ventricular septum was intact
(Fig 3). A large right pulmonary artery rose from a large
main pulmonary trunk ( Fig 4 ) .
A six-week-old white boy was referred with a history of
cyanotic spells beginning after a few days of life. These
episodes were related to crying and feeding.
The infant was delivered normally at term with a birth
weight of 5%Ib. A diagnosis of preeclampsia was made in
the third trimester.
The pertinent physical findings were: weight, 7%lb and
slight facial cyanosis. The respiratory rate was 36 per minute.
The lungs were clear. The heart was enlarged to percussion
and a grade 111 rough systolic murmur was heard best at the
left sternal border. The liver edge was palpable below the
right costal margin. There were bilateral talipes equinovarus
deformities of the legs.
A venous catheter was advanced to the heart via the right
saphenous vein. The catheter passed easily into the right
atrium, right ventricle, right pulmonary artery and then into
the descending aorta in the right chest via the right ductus
arteriosus (Fig 1 ). It was also possible to manipulate the
catheter from the right to the left atrium and then into the
left ventricle.
There were no demonstrable systolic gradients across
either the pulmonary or the aortic valves. Systolic pressures
in the mid portions of both ventricles, the pulmonary artery
and the aorta were similar. The left ventricular end-diastolic
pressure was abnormally elevated (Table 1 ).
Left atrial, left ventricular and aortic blood was moderate-
On February 2, 1972, surgical exploration confirmed the
catheterization findings. The left main pulmonary artery had
a diameter of 1 cm and originated from the left side of the
ascending aorta, approximately 2 cm distal to the origin of
the left coronary artery. The ascending aorta turned posteriorly toward the right and descended behind the right main
pulmonary artery. There was a patent ductus arteriosus 1 cm
in length and 0.4 cm in diameter between the proximal portion of the descending aorta and the right main pulmonary
artery.
After ascertaining that temporary interruption of the ALPA
could be tolerated, this structure was transected and anas-
Table 1 4 a t h e t e r i z a t i o n Data
-
-
Site
Right atrium
Left atrium
Right ventricle
Left ventricle
Pulmonary artery
Aorta
Saturation
56
86
63
88
59
87
70
Pressure mm Hg
4
4
85/3
87/15
82/34
79/33
FIGURE3. A left ventriculogram (LV) was exposed in a left
anterior oblique projection. The large left pulmonary artery
arising from the ascending aorta is clearly seen ( Catheter, C ) .
CHEST, VOL. 63, NO. 3, MARCH, 1973
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ANOMALOUS ORIGIN OF LEFT PULMONARY ARTERY
and a ventricular septal defect, overridden by a large
aorta, ensues."
The ontogenetic theory of Cucci and co-workerslS
reasonably explains the usual anatomic alignment of
patients with anomalous origin of a main pulmonary
artery. However, the findings in our case, demonstrated
in the catheterization laboratory and at surgical exploration, of a large pulmonary artery with no evidence of
pulmonary trunk hypoplasia or ventricular septal defects
and yet ALPA and right aortic arch suggest that the
developmental fault may be still more complex.
The successful surgical correction in a six-week old
infant underscores the need for early angiographic diagnosis. Since cyanosis, severe pulmonary hypertension
and progressive pulmonary vascular bed changes are an
integral part of this syndrome, early diagnosis and surgical correction are warranted.
FIGURE
4. Contrast material was delivered to the main pulmonary artery (MPA). Only a right distribution is apparent.
tomosed to the side of the rather large main pulmonary
artery. The ductus arteriosus was then isolated and obliterated. The procedure was well tolerated and the infant made
a rapid uncomplicated recovery.
Cucci and colleagues13 discussed the various theories
which have been suggested to account for the absence of
a primary division of the pulmonary trunk. They concluded that the theories invoking developmental errors
of the sixth arch did not account for the combinations of
abnormalities observed and that defective truncoconal
septation did. Specifically, they noted that prior theories
accounted for neither the association of ALPA and
tetralogy of Fallot1."11J3 nor the location of the aortic
arch on the side opposite that of the absent pulmonary
artery. They postulated that dorsorotation of the left or
right truncoconal ridge caused the ipsilateral sixth arch
to be incorporated into the ascending aorta. Either rotational fault would cause the resultant aorta to be large at
the expense of the pulmonary trunk. In addition, either
would (due to spiral rotation) cause blood to flow
preferentially into the contralateral fourth arch. This
would then cause this arch to persist as the aortic arch
while its paired branch would resorb. In the event of
dorsorotation of the right truncoconal ridge, the right
pulmonary artery will then arise from the ascending
aorta and the left fourth arch will persist normally. The
degree of rotation needed to produce this combination is
slight and is therefore usually an isolated finding. Left
dorsorotation sufficient to cause the left pulmonary
artery to arise from the ascending aorta and a persistence
of the right fourth arch is substantially greater. It is to
this striking degree of rotation that Cucci and
associates13 attribute the association of Fallot type abnormalities. These authors state, "The incorporation of
the left sixth arch into the ascending aorta requires a
degree of dorsorotation so pronounced as to cause hypoplasia of the pulmonary trunk and of the right ventricular outflow tract. The truncoconal and the muscular
ventricular septa lying on different planes cannot fuse
ACKNOWLEDGMENT: We would like to express our appreciation for the efforts of Deborah Telesco, RN, Philomena
Adinaro, PN, Margaret Teague, RN, Pattie Baskette, Catherine Doyle, anet Waldie and Alberta Varble in the preparation
of this wor .
L
1 Caudill DR, Helmsworth JA, Daoud C, et al: Anomalous
origin of left pulmonary artery from ascending aorta. J
Thorac Cardiovasc Surg 57:493-506, 1969
2 Fraentzel 0:Ein fall von abnormer communication der
aorta mit der arteria pulmonalis. Arch Anat Physiol
43 :420-426, 1868
3 Bopp F: Anonnale arterielle Gef'iissversorgung der rechen
lunge (Abgang der rechen arteria pulmonalis aus der
aorta bei norrnaler versorgung der linken lunge durch die
pulmonalarterie) . Zentralbl Path Path Anat 85: 155-160.
1949
4 Cam C, Lermanda VC, Lyons HF: Aortic origin of the
right pulmonary artery. Br Heart J 19:345-352, 1957
5 Dushane JW, Weidman WH, Ongley PA, et al: Clinicalpathologic Conference. Am Heart J 59:782-788, 1960
6 Armer RM, Schumacker HB, Klatte EC: Origin of the
right pulmonary artery from the ascending aorta. Report
of a surgically corrected case. Circulation 24:662-668.
1961
7 Criffiths SP, Levine OR, Andersen DH: Aortic origin of
the right pulmonary artery. Circulation 25:73-84,1962
8 Pool PE, Vogel JHK, Blount SG Jr: Congenital absence of
a pulmonary artery. The importance of flow in pulmonary
hypertension. Am J Cardiol 10:706-732, 1962
9 Czarnecki SW, Hopeman AR, Child PL: Tetralogy of
Fallot with aortic origin of the left pulmonary artery.
Radiographic and arteriographic considerations. Dis
Chest 46:97-101, 1964
10 Weintraub RA, Fabian CE, Adarns DF: Ectopic origin of
one pulmonary artery from the ascending aorta. Radiology 86:66-676,1966
11 Winship WS, Beck W, Schrire V: Congenital "absence"
and anomalous origin of the main pulmonary arteries. Br
Heart J 29:34-42, 1967
12 Flege JB Jr, Durnin RE, Rossi NP: Aortic origin of the
right pulmonary artery and ventricular septal defect. J
Thorac Cardiovasc Surg 59:468-473, 1970
13 Cucci CE, Doyle EF, Lewis EW Jr: Absence of a primary
division of the pulmonary trunk. An ontogenetic theory.
Circulation 29: 124-131. 1964
CHEST, VOL. 63, NO. 3, MARCH, 1973
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