Coarctation of the Pulmonary Artery and
Pulmonary Valvular Stenosis*
PAUL HALL, M .D .,t BENGT W. JOHANSSON, M .D ., HANS BROOK, M .D ., ARNE MALM, M .D .,
MILS-MAGNUS OLSSON, M .D ., LARS ANDR9N, M .D . and HELGE WULFF, M .D .
Malmo, Sweden
HE SKODAIC THEORY of the etiology of
T coarctation of the aorta postulates that
coarctation is produced by an extension of the
obliterating tissue from the ductus arteriosus
into the aortic wall . Since a coarctation of the
pulmonary artery has never been observed at
the pulmonary end of the ligament of the ductus
arteriosus, it has been argued that the hypothesis
is not tenable .'
Since 1953, several cases have been described
with a narrowing not only in the pulmonary
artery at the origin of the ductus arteriosus
but also peripherally in the branches of the
pulmonary artery . Sondergaard' reported
three cases, all of which had a pulmonary
valvular or infundibular stenosis and a nar-
rowing of the pulmonary artery at the bifurca-
tion . The author called the last-mentioned
anomaly "coarctation of the pulmonary ar-
tery ." Schumacher and Luries and Coles
et al 4 have each described one case of pulmonary
valvular stenosis in combination with a coarcta-
tion of the pulmonary artery . Powells de-
scribed a . similar case in which there was no
evidence of a pulmonary valvular stenosis but
this lesion could not be excluded with certainty .
Vermillion6 described two cases, one with a
ventricular septal defect and the other with a
pulmonary valvular stenosis, both having a
narrowing of the right pulmonary artery .
Similar peripheral narrowings have been de-
scribed in ever increasing number since Oppen-
heimer 7 reported the first case of multiple nar-
rowings of the pulmonary artery . Thus, Arvids-
son et al .8 contributed two cases ; Gyllensvard
et al . 9 described eight cases including one or two
with a narrowing at the bifurcation ; and D'Silva
et al ., 10 ten cases .
From the Cardiological Laboratory of the Department of Medicine, the Department of Thoracic Surgery, and
the Department of Roentgenology, Allmanna Sjukhusct, Malmo, Sweden .
j' Present address : Department of Medicine, Serafimerlasarettet, Stockholm, Sweden .
JULY 1961 109
In 1959 Shafter and Bliss" made a survey of
all published reports of cases of narrowing or
stenosis of the pulmonary artery and found
thirty-three such cases . They described an
additional five cases . In four of these the
diagnosis was made by catheterization of the
heart without angiocardiography ; because of
this it is difficult to define the exact anatomic
position of the stenosis . Trower et al ." have
recently published two cases, one of which was
successfully corrected under hypothermia .
Thus, a review of the literature has shown
that several cases have been diagnosed by
catheterization alone without angiocardiog-
raphy . We have observed one case in which
catheterization did not give the diagnosis but
angiocardiography did .
CASE REPORT
The patient was a twenty-one year old carpenter
with heart disease diagnosed at school . He had
never been cyanotic but had always complained of
breathlessness on exertion, but not during routine
work .
Examination in 1957 showed a patient of normal
body build (and normal strength) without cyanosis
or dyspnea at rest . A slight precordial bulging
was found and a systolic murmur (grade 4) over the
pulmonary area as well as a thrill and an accentuated
pulmonary second sound . The electrocardiogram
(Fig. 1) showed normal axis deviation with a QRS
duration of 0 .11 second . P waves were prominent
but not definitely increased above normal values .
In the precordial leads marked signs of right ventricu-
lar preponderance were found. The roentgenologic
examination (Fig. 2) showed slight enlargement of
the heart, especially of the right ventricle, with
a relative volume of 690 cc . per square M . of body
surface (Liljestrandu) and dilatation of the pulmonary
artery . The pulmonary vasculature was sparse .


110 Hall et al.

FIG. 1 . Electrocardiogram demonstrating 'gh

FIG . 2 .Roentgenograms (P-A and lateral views) showing increased heart size (right ventricle) and prominence of
the pulmonary artery . Note the decreased vascularity of the lungs, more on the left than on the right side .

F G. 3 . Angiocardiogram demons ra mg pulmonary valvular stenosis and coaretation of the pulmonary artery .

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Coarctation of the Pulmonary Artery
Right heart catheteication showed a mean pressure
of 7 mm . Hg in the right atrium, 181/0 in the right
ventricle and 26/8 in the pulmonary artery with a
mean pressure of 11 mm . Hg . Pressure was meas-
ured in the right pulmonary artery (8 11 mm . Hg)
but not in the left branch . Pulmonary capillary
venous pressure was 6 mm . Hg. Where were no
signs of left to right shunt and there was normal
oxygen saturation in the brachial artery .
Angiocardiography showed a narrowing of the left
pulmonary artery at the pulmonary end of the ductus
artcriosus and also a pulmonary valvular stenosis
(Fig . 3) .
Operative Findings : The patient was operated upon
with a left-sided posterolateral incision and resection
of the fourth rib_ The right ventricle was extremely
enlarged and the pulmonary artery dilated . The
coarctation of the left pulmonary artery was marked
and there was no patent ductus . Surgical relief of
the coarctation was not considered necessary . A
transventricular dilatation of the pulmonary valve
was made with Brock's dilator, and the systolic pres-
sure in the right ventricle fell promptly from 130 to
75 mm . I1g ; the mean pressure in the pulmonary
artery rose from 7 to 27 mm . Hg . The postoperative
period was uncomplicated .
Re-examination one Year later revealed that the
heart volume had diminished . The electrocardio-
gram showed no further changes and the patient was
in good health .
COMMENTS
In the literature there are to be found at
least five cases with a proved narrowing of the
pulmonary artery at the pulmonary end of the
ligament of the ductus arteriosus in combination
with a pulmonary infundibular or valvular
stenosis . The pressure gradient between the
right ventricle and the pulmonary artery in
some of these cases was extremely high . For
the diagnosis it is important to make pressure
recordings from both the right and left pul-
monary artery and to perform angiocardiog-
raphy .
The combination of these two anomalies is
remarkable . 4 Can the pulmonary valvular
stenosis have any connection with the develop-
ment of the coarctation of the pulmonary ar-
tery? If the pulmonary blood flow is low, can
it result in premature closure of the ductus
arteriosus with consequent overeontraction at
the origin of the ductus and subsequent stric-
ture of the pulmonary artery at that level?
There is no adequate answer, as we do not know
the mechanism underlying the closing pro-
cedure, 14 but if there is such a connection the
combination of a pulmonary valvular stenosis
and a patent ductus ought to be rare . Ekstrom
JULY 1961
111
found three patients with pulmonary valvular
stenosis and a pressure gradient of more than
30 rum . Hg out of 290 patients with patent
ductus . Thus, the combination of a pulmo-
nary valvular stenosis and a patent ductus with
a high pressure difference across the pulmonary
valve is rare" On the other hand, if a high
pulmonary resistance may be established at
birth, the frequency of patent ductus ought to
be high according to this theory . In 115
cases of patent ductus, Wood" found an Eisen-
menger reaction (bidirectional or reversed
shunt) in 13 .3 per cent . Thus, the combination
of a high pulmonary resistance and a patent
ductus seems to be fairly common .
We believe that we can neither exclude the
possibility of a connection between valvular pul-
monary stenosis and coarctation of the pulmonary
artery nor exclude the skodaic theory on the basis
of the fact that we have not observed any co-
arctation at the pulmonary end of the ligament
of ductus arteriosus, as such cases are ob-
served .
SUMMARY
A case is reported of coarctation of the pul-
monary artery at the pulmonary end of the
ligament of the ductus artcriosus in combina-
tion with a pulmonary valvular stenosis . The
valvular stenosis was operated on with Brock's
dilator with good result .
The combination of these two malformations
and the possibility of a high pressure difference
between the right ventricle and the pulmonary
artery as a possible cause of coarctation are
briefly discussed .
REFERENCES
1 . EDWARns, J . FL Congenital malformations of the
heart and great vessels . In : Gould, S . E . :
Pathology of the Heart, p . 453 . Springfield,
Illinois, 1959 . Charles C Thomas,
2 . S6NDERGAARn, T . Coaretation of the pulmonary
artery . Danish d4 . Bull., 1 : 46, 1954.
3 . SenuMACnvm, H . B. J . and LURIE, P. R . Polnto-
naryvalvulotomy : description of a new operative
approach with comments about the diagnostic
characteristics of pulmonic valvular stenosis .
J . Thoracic Surg_, 25 : 173, 1953 .
4 . Cones, J . B . and WALKER, J . W . coarctation of
the pulmonary artery . Arn . Heart J ., 52 : 469,
1956 .
5 . POWELL, M . L . and HILLER, H . G . Pulmonary
Excoriation . M . J. Australia, 1 :272, 1955 .
6 . VERMILLION, M . B., LEtrHT, L. and DAVts, L . A .
Pulmonary artery stenosis . Circulation, 17 : 55,
1958 .
7 . OPPENHRTMER, E. Partial atresia of the main
112 Hall et al .
branches of the pulmonary artery occurring in
infancy and accompanied by calcification of the
pulmonary artery and aorta . Bull . Johns Hopkins
Hasp ., 63 : 261, 1938 .
S . ARVIDSSON, H ., KARNELL, J . and MOLLER, T . Mul-
tiple stenosis of the pulmonary arteries associated
with pulmonary hypertension, diagnosed by
selective angiocardiography . Act radial ., 44 :
209, 1955 .
9 . GYLLENSVARD, A ., LOGIN, H ., LUNDBERG, A . and
MotLER, T. Congenital multiple peripheral
stenosis of the pulmonary artery . Pediatrics,
19 : 399, 1957 .
10 . D'SILVA, J . L ., GASUL, B . M . and DILLON, R. F .
Stenosis of the right main pulmonary artery .
Circulation, 21 : 1116, 1960 .
11 . SHAFTER, I3 . A . and BLISS, H . A . Pulmonary
artery stenosis . Ain . J. .Jled., 26 : 517, 1959 .
12 . TROW'FR, W . B ., ABELMAN, W. H . and HARKEN,
D . E . Surgical correction of coaretation of the
main pulmonary artery. Circulation, 21 :672,
1960 .
13 . LILJESTRAND, G ., LYSHOLM, E., NYLIN, G . and
ZACHRISSON, C . G. The normal heart volume in
man . Am . Heart J ., 17 : 406, 1939 .
14 . WOOD, P. Diseases of the Heart and Circulation,
2nd edition, pp. 380, 394 and 408. Philadelphia .
J . B. Lippincott Co .
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