Clinical Studies

Stenotic Lesions of the Pulmonary Arteries

Clinical and Hemodynamic Findings in 84 Cases*

IVAN A. D’CRUZ, M.D., MAGNUS H. AGUSTSSON, M.D., J. PEDRO BICOFF, M.D.,

MILTON WEINBERG, JR., M.D. and RENE A. ARCILLA, M.D.

Chicago, Illinois

A LTHOUGH innumerable papers have been written about various aspects of the

pulmonary circulation, very few have dealt with stenotic lesions of the pulmonary artery and its branches. Three recent monographs devoted entirely to the pulmonary circulation’-3 contained little or no mention of this entity. We have, however, encountered 84 such cases over the last 12 years. This series includes 10 cases of unilateral pulmonary artery stenosis4 and 12 cases of bilateral stenosis5 previously re- ported from this department. The object of this paper is to call attention to its incidence, the means by which it may be diagnosed and the clinical and hemodynamic significance of this group of lesions, either as an isolated entity or as a complicating factor in the various cardiac anomalies with which it is often associated.

MATERIAL AND METHODS

Out of approximately 2,000 patients subjected to diagnostic procedures, including cardiac catheteriza- tion or angiocardiography, or both, during the last 12 years, 84 patients were found to have stenosis of one or both pulmonary artery branches or of the pulmonary trunk.

The diagnosis of unilateral pulmonary artery stenosis was based on a pressure gradient between the pulmonary trunk and the right or left pulmonary artery branch during right heart catheterization. Angiocardiography was diagnostic in half of the cases (see Table IA).

The criteria used to diagnose bilateral pulmonary artery stenosis were (1) presence of a gradient be- tween the pulmonary trunk and one or both branches,

(2) angiocardiographic demonstration of the stenosis, (3) presence of characteristic pressure curves in the pulmonary trunk.” At least two of these three criteria were required to establish the diagnosis (see Table IB).

In a few cases where a left to right shunt and a mild gradient between the pulmonary trunk and one branch were observed, the possibility that the gradient was due to increased flow was entertained, and these cases were excluded from this series.

RESULTS

Pulmonary Artery Stenosis Witflout .hsociated Lesions: Table II lists the cases with unilateral stenosis without associated lesions. All 7 pa- tients in this group were asymptomatic and normal on physical examination, except for a grade 2 to 3/6, somewhat high-pitched, ejection systolic murmur at the base of the heart, which was well transmitted to the back and the axillae, particularly of the corresponding side. All had normal electrocardiograms. The chest roent- genograms in 2 cases showed hypovascular lung fields on the side corresponding to the stenotic artery. All 7 patients had pulmonary trunk pressures near the upper limit of normal. In the 3 cases where both pulmonary artery branches were entered, a gradient was demon- strated on one side but not on the other. In the other 4 cases, only one branch, the one showing a pressure gradient, was entered. The unilateral nature of the pulmonary stenosis in these cases is presumptive, since it has not been proved that no gradient exists on the other side.

Table III contains the relevant clinical and

* From the Department of Pediatric Cardiophysiology, Cook County Children’s Hospital and Hektoen Institute for Medical Research, University of Illinois College of Medicine, and Presbyterian-St. Luke’s Hospital, Chicago, Ill.

This investigation was supported in part by U. S. Public Health Service Research Grant H-3518 from the National Heart Institute, National Institutes of Health, and a grant from the Chicago Heart Association.

APRIL 1964 441

442 D’Cruz et al.

‘rAESLIt I

Diagnosis of Pulmonary Arterial Stenosis

Associated Anomalies

Catheterization Angiocardiography No. No. Opera-

Cases No. Diagnostic C%CS Diagnostic tion Autopsy --________-_ ~_ ~_._.__

--.-______ ____- __~_~

None 7 7 7 7 1 Ventricular septal defect 6 6 5 6 3 Pulmonary stenosis 9 10 7 9 7 Patent ciuctus arteriosus 4 5 5 3 1 Supravalvular aortic stenosis 2 2 2 2 2 Others 4 4 3 3 1 1 1

Total 32 34 29 30 15 1 1 -__

B. Bilateral Pulmonary Arlery Stenosis

None 20 23 19 20 16 Ventricular septal defect 8 13 10 9 5 Pulmonary stenosis 10 15 11 ‘/ 9 1 Ventricular septal defect with pul- k’ monary stenosis 3 4 4 4 3 Atria1 septal defect 5 5 5 5 4 Tetralogy of Fallot 2 2 2 2 1 Patent ductus arteriosus 2 4 1 3 2 Others 2 4 1 3 2 1

Total 52 70 51 55 43 2 1

TABLE II

Unilateral Pulmonary Artery Stenosis Without Associated Jl)efects

Case No.

Sex &

Age (yr.1 RV

Pressures Angiocardiography (mm. Hg)

Type of MPA RPA LPA SA Type RPA LPA

Stenosis

1 F8 26/2 26/9 (11) 123,‘8!, s - - 2 Fl 3112 25/10 1c;;o 90150 s - - :.: 3 M 15 27/4 24/l 2 1i;;o 110/56 * 4 M 13 42/4 38/14 25/13 40/12 145/80 S ‘* ‘_’ Locahzed .‘: 5 Fl 30/3 27/9 24/l 2 18/12 124/76 s - - 6 M7 36,‘0 37/l 2 19/8 36/10 132/70 * 7 MS 35/3 30/l 2 23110 *

* Awiocardiosrams unsatisfactory; ( ) = mean pressure; S = selective; RV = right ventricle; MPA = main pulmonky artery; RPA = right &mokry arter;; LPA = left pulmonary artery; SA = systemic artery.

hemodynamic data in 20 cases of bilateral stenosis without associated defects, an entity about which very little information is available in the literature. In all cases a grade 3/6, long, systolic ejection murmur was present, heard best at the base. Typically, this murmur was widely conducted, being heard equally loud in both parasternal regions, axillae and inter-

scapular areas. The second heart sound was usually moderately to widely split, the degree of splitting varying with respiration. The electro- cardiogram and vectorcardiogram correlated with the right ventricular pressure, as observed in isolated pulmonic stenosis. The systolic pressure in the pulmonary trunk varied from normal levels to those of severe hypertension.

THE AMERICAN JOURNAL OF CARDIOLOGY

Stenotic Lesions of the Pulmonary Arteries

Hilatrral Pulmonary Artery Sttwosis Without .\sswiatrd Irsiolls

443

-

-

RV M PA RPA LPA

39,‘4 36:‘) 17,‘6 23/o

5015 SO/16 18,‘lO 23/12

48/4 46/16

MPA ‘ressure ::urvrs

Type

!P (

-

SA

92/‘63

130/72

100/55

Min. M.,r.

H M 6 yr.

‘1 1: 3 mo.

II) 1’ fi mo.

11 M 2 yr. 6 yr.

12 F 16 mo.

I.3 F 16 mo. 7 yr.

14 F 4 mo.

15 F 13 mo.

16 F

+

: +

+ i *

+

+

+

+

+

+

+

+

+

+

+

+

+

s

s

s

V s s

S

V

S

t

s

s

I V

s

t

s

S

S

S

42/Z 44/11 20/10 21/10 122/47 3613 35/R 1216 1917 104/60 39,‘2 39/10 20/10 110/60

(I 06 0 075 IRBBB 0: 0; Unchanged

0.05 ?&wmal

47 ‘3 4313 1717 1.:

83/59 3413 33/a 15/6 105/75 32/6 38/13 20/12 100/60

0 045 0.06 RVH (mild) Normal

0.02 0.04 Normal

-

++

Localized Hypoplasia and

multiple periph- eral type

+ SeEmental

+ Segment.*1

- LocnlizPd

37/4 33/a 2519 18/9 100/60

35/R 35/5 1315 110/50

35/l 38/5

2714 2715 12/5 17/8 135/80

37/2 34/11 11/6 115/72

RVH (mild)

RVH (mild)

RVH (mild)

Normal

IRBB

RVH

0 03 0 04

0 03 0 04

0.04 0.06

0.05 O.OR

0.04 0.06

0.03 0.06

2 mo. 17 F

2 yr. 18 F

6 yr. 19 F

8 yr. 20 M

-c Hypoplasia

+ Segmental

_ Localized

Autopsy: no pulma nary stenosis but severe stenosis 01 MPA at bifurca. tion

f Loralizrd

+ Suprnvalvular sten- osis and hypo- plasia; multiple peripheral

_ .

+ Localized

15 In”. 3 yr

21 F ‘1 mo.

22 I’ 4 yr.

23 M 16 mo.

7013 70/4 100/50 6013 60/14 100/60 50/4 52/16 23/14 . 95/70

34/o 36/t? 11/4 105/65

100/7 6/l

(modrrate) tinchanged RVH

RVH (scvcrr)

24 M 15 mo.

25 F 15 yr.

50 ‘6 50/10 102,‘52

145/Z 130/151 16/5 15/s 138/76 95/202

RVH (moderate)

KVH (severe)

2712 2717 21/8 19/7 125/64

57/8 57/6 7/l 143176

0.04 0.07 RVH (mild)

. . . RVH [mild)

26 M 5 yr.

27 F 7 yr.

L

* Pressure tracings unsatisfactory. t hngiocardiograms unsatisfactory. 1 Proximal to coarctation of MPA. I Distal to coarctation of MPA. St = second heartsound; V = venous; S = selective; RVH = right ventricular hypertrophy; IRBBR = incomplete right bundle branch block.

In the majority, however, the pressure was only slightly elevated. Characteristic pressure curves5 were obtained from the pulmonary trunk in most of the cases (Fig. 1). If the sys- tolic pressure in the pulmonary trunk is only minimally elevated, the pressure curves may not be diagnostic. In the more severe cases, the characteristic pulmonary trunk pressure curves were consistently observed. In 7 cases both right and left branches were entered ; in 9 cases

only one branch was entered ; and in 4 cases neither branch was entered. Bilateral stenosis in the latter cases was diagnosed because of the typical pulmonary artery curve and the angio- cardiographic findings.

Angiocardiography demonstrated four majn anatomic types: (1) localized stenosis with poststenotic dilatation (Fig. 2), (2) segmental stenosis (Fig. 3), (3) diffuse hypoplasia (Fig. 4), (4) multiple peripheral stenoses (Fig. 5). Two

APRIL 1964

444

mm.Hg

D’Ckuz et al.

“:I: 1: ::’ 12:: ,I, :I:. ,1

0-S . . __ -.

:.. . _- __ _ _ ::.: ,1,, ::. ,... . . .

8

.,.

,... .,. I.,

: :. :,: .::’

FIG. 1. Pressure tracings in bilateral pulmonary artery stenosis. MPA = pulmonary trunk; RV = right ven- tricle. During ventricular systole the pulmonary trunk curves were identical in contour and timing to those of the corresponding right ventricular curves. The de- scending limb of the pulmonary trunk tracing is steeper than normal and is followed by a deep dicrotic notch and a low diastolic curve.

FIG. 3. Sclcctivc angiocardiogram with injection into the pulmonary trclnk (PA) showing segmental stenosis of thr right pulmonary- artcry (arrows).

Fro. 4. Selective angiocardiogram with injection into the pulmonary trunk (PA) showing diffuse hypoplasia of the pulmonary arteries. A small amount ofcontrast material regurgitated into the right ventricle.

FIG. 2. Selective angiocardiogram with injection into the pulmonary trunk (PA) showing localized stenosis of the proximal primary pulmonary artery branches (arrows).

FIG. 5. Venous angiocardiogram showing diffuse hypo- plasia of left pulmonary artery and multiple stenosis of the peripheral branches (arrows).

THE AMERICAN JOURNAL OF CARDIOLOGY

345

l’ress”rr$ lmm. Hg

51 P.\ RPA

62 2 42’13 26,‘14 6.5 “9 26 ‘8 2%‘7 17,‘R

35 ‘14 23113

25/l? 16,‘10

4;;.;4 80 ‘50

113 7x 43,‘9 148,‘Hj 25/‘7 95;‘62

90. ‘C,5

23,‘14 95 ;55

100/s 32/‘10 115,‘5 32,/10

69;0 34/B 50,‘R 36/10

58 2 28,‘lO

48 ‘2 26;12

159/9 26/l 5

56 ‘0 34,“R

56 ‘3 27/13

34 ‘2 22/9

43/Z 28112

52/4 53119

47/10 44/20

1 o/7 28/B 115/90 37/10

10/5 3417 12&‘75 19/12 36/14 130170

16/10 105;so

14/11 132/97

15/12

16/10

19/11

28/12

27/17

119:78

32/7 130,‘67

22/14 110:70

23/9 115,/70

95;60

116/65

90/60

71/52 54/15 36/20

38/10

(26) (10)

25/16

2819

34/l 0

10/6

47/l 5

30/l 0

18/B

20/10

71/52 169 166 115’80

20/l 4 95 155

96 ‘46

100165

18/9 45/l 9 142/74

l6/8 125/75

RI’.\ R\

i.l’.\

.‘X hI 4 ,110. 2 yr.

!‘I 1’ io F

2 yr. ?I M

‘I mo. 12 M

4 mo. 33 M

5 mo. 34 M

4’/2 yr. 4a;, yr. 6 yr:

35 F

62 L 43/1

\’

x

:<

s

s

V

S

s

s

V

S

S

S

S

S

*

s

*

S

s

V

V

*

+ \IS.D.; PBI’ ‘SBI’ 0 I

V.S.D.: PBF/SBl: 2 7’ 1 V.S.D.: PBF,‘SBI: 2.2’1

V.S.D. & Palm. SW” : 1’81) SRF 14: I

V.S.D ; sm:dl shunt

Shunt undetermined

43/ 1 341’3

65 ‘5

28,‘5

7617 4713 50/l

38;4

2R/2

33/2

87/O

43/3

34:‘2

+

+ -

+ L

_

i

+

-c

Localized ..,

Segmental

Locnlized

Localized

Hypoplasia

Incalized

Locnlized

-

Pulm. sten. (valvular) 3 mo. postvalvulotomy 2 yr. postval”“lotom) Pulm. sten. (wllvular~

Pulm. sten. (\alvulari

Pulm. sten. (valvular)

Pulm. sten. (infundihular~

Pulm. sten. (valvul.~r~, P.D..4. ligated

Pulm. sten. (valvulxi

Pulm. en. ivdvular’~

Pulm. sten. (\~alvulnr‘l

P.D..4.; PRk’/SBF 6.1

P.D.A

f P.D.A. Postop. P.D.A.

-

+

+

-t Localized

Localized

Segment‘ll

Supravalvular aortir skn.

Supravalvular aartir sten

Fibroelastosis

Tetralogy of Fallot; sten. KPA at autopsy

Marfan syndrome; aortic incompetence

Partial anomalous pulm. venous drain-

age

9 yr. 36 M

1’ ‘2 yr. 37 F

8 mo. 38 F

12 yr. 39 F

16 yr. 40 F

17 mo. 41 M

5 yr. 42 F

21 mo 43 F

4 yr. 44 F

15 yr. 45 M

13 yr. 14 yr.

4(> F 4 mo.

47 F 11 yr.

4X M 2’/2 yr.

4’) M 7 mo.

50 M 18 mo.

51 M 15 yr,

52 M

1 Yr

I - * Angiocardiogram unsatisfactory.

patients had supravalvular stenosis of the pul- monary trunk. The most frequent type of stenosis was the localized stenosis with post- stenotic dilatation. Three patients were re- catheterized after intervals of two, three and a half and five and a half years, respectively, and all showed a slight decrease in the right ventric- ular and pulmonary trunk pressures.

Pulmonary Artery Stenosis with Associated Lesions: The cardiac catheterization and angiocardio-

graphic findings in the patients with unilateral or bilateral stenosis and associated cardiac lesions are shown in Tables IV and V. The most frequently encountered associated lesions were ventricular septal defect and pulmonic stenosis. Pulmonary valvular stenosis of all degrees of severity and left to right shunts of varying magnitude were found. In the 2 cases of patent ductus arteriosus, the diagnosis of bilateral stenosis was first suspected following

APRIL 1964

446 IXruz et al.

Bilateral Pulmonary .4rtery Stenosis with Associatrcl Lesions

53 E 15 mo

54 F 6 yr. G’/z yl

55 F 2 yr. 3 yi-.

56 F 7 mo.

57 M 4 mo.

58 M 2 mo. 2 yr. 5 yr.

59 F 3 mo.

60 M 15 mo 3 yr.

61 M 9 mo.

62 F 14 mo

63 F 16 mo

64 F 4 yr. 4 yr. 4 yr.

65 F 1 yr. 1 yr.

66 M 3 mo.

67 M 4 yr. $2 yr

68 F 10 mo.

69 M 10 mo.

70 F 4 yr. 8 yr.

71 F I ‘/P yr.

72 M 3 mo.

73 M 10 ItlO. 2’iz yr.

74 F 14 mo.

75 F 13 mo.

76 M 18 mo.

77 F 7 yr.

78 M

5 yr. 79 M

4 YT- 6 yr.

80 M 2 mo.

81 M 4 mo.

RV MPA R P‘t

53,‘2 45/lG

35;o 32i2

78/3 47/ 3 53;t

35/2

30/a 139/R 32/t I 2t:1t 22/10 196/71

70/l 5 30/18 30/l 2 loo:% 38/l 0 20/l 3

53/12 34/12 . . . .

27i7 14/G 35/7 .

25/O 17/7 47/s 39ilO 1 z/9

51/o 42/9 17;9

54/10 52/18 20/14

50/o . . 30/4 34/R IS/8

60,‘5 38/18 20/14

54/10 42/l 5 22/14

70/Z 3Oi6 17/a

i25/7 46/10 84,‘8 76/12

,10/3 64/l 4

25/s 20,‘9 GO/10 60/24 39/o 24/l 2

17/12 .

. . . 22/12 15/10

7414 85/4 65/2

72/l

50/t2 25/10

48/l 1 26/10 . . . 22/e

.

23/5 23i7 31/5 31/5 IS/4

00/z 65/10 25110

78/3 52/10 20/l 2

92/o 58/10 16/6 75/o 48/14 18/5

70/3 68/l 0 23/10

36/O ZR./li 19/12

49/G 49i7 24/7

34/3.. 30/7 20/G

56/3 50/20 12/6

85/2 30/o . . .

65/10 65‘00 65/10

. . .

.

35/15 SO/l 0 65,‘10

171’1 lf

. . .

(15)

. .

LPA s A

34/l 4 136/7t

. . ).. . . .

120/6r 37/f 8 95/s<

. 15/7 123161 20/8 (80)

115/G{

17/5 90/4c

18/12 110;75 20/12 110/75 18/12 (90)

I 100/50 24/15 80/60

57/40

115/60 125/60

90/45

IO4/52

1313

52/16 1 Oil/60 . . 110/65

36’00 109/62 , 120/68

1 On,‘60

. IOU60

I.. 98/64

20/7 96/5G

42/21 112/60

102,‘65 . . 130/60

(15) . . 25,‘14 _,. 60/10 .

_I_ +

* f

T + + + : +

: * + +

: + + * *

: 0

0

1

:

z f + -t + + 0 0

. . .

: f

s

V V

*

. *

V I

s V

V V s

S

s

S .

S .

V

S

s

V

,

S s

V s

s

S

S

S

S

S

+

S

-I-

.-

.

.

+ .., -I- +

z f i- + i- + . . + +

..1

-I-

+

f

:

*

+

f

+

+

+

. f

t

- -

+ Segmental . + Segmental f Hypoplasia

+ Segmental + Segmental - Localized

+ Segmental

-I- Localized

+ Localized

.

* Localized . . . -f Localized

-f Localized

+ Hypopiasia Pi&n. stem.

f Hypoplasia

+ Localized

Pulm. sten. Posrvaivulotomy

V.S.D.: p&n. stew V&D.; pulm. stew

: Hypoplasia and

multiple peri- pheral

+ Localized

V.S.D.: pulm. sten.

A.S.D.; PBF,‘SBF 1.3: t

+ Localized A.S.D.; PBF/SBF 1.7~1

- Localized A.S.D.; PBF/SBF 2:l

+ Localized A.&D.; PBF/SBF 2.3:1

+ Multiple peri- pheral

+ Segmental

A.S.D.; p&n. art. “en. drain; PBF/SBF 1.8 : 1

. . . - Sllp~W&T&W

& right pulm. artery

C Localized LPA;

retralagy . .

retra1ogy

multiple bi- .1 lateral peri-

pheral

1,

-

l%cuspid atresia & pulm. sten.; postvalvulotomy; post patch on LF’A

V.S.D.; PBF/SBF 1 .U:t

V.S.C.; PBF/SBF 2:l Posrop. no shunt

V.S.D.; PBF/SBF 3.3:1 Poatop. no shunt V.S.D.: PBF/SBF 1.5:1

V.S.D.; very small shunt

V.&D.; PBF/SBF 2: 1 PBF/SBF 1.6: I PBF,‘SBF 1.3: 1 PBF/SBF 3: 1

V&D. Spontaneous closure Pulm. sten.

Pulm. sten.

Pulm. stem

Pulm. sten. At operation P0stop.

Pulm. sten. POStXdVUIOt0XIly Pulm. sten.

Puim. sten. .

Pulm. sten.

THE AMERICAN JOURNAL OF CARDIOLOGY

Stenotic Lesions of tlie Pulmonary Arteries 437

, 81 F

1) mu. ~ 81,6 31/50 t 5 VI’. 90!7 20 12 95163 5’ ‘? VT. 84, 0 86!63 C

x\ 12 9 mo. 48/6 45/4 104164 + 3’S’? yr 36 ‘1 35j16 110/70 t-

84 F 14 mo. 64,‘4 52/8 39/14 115/65 + 4 yr. 3R’h 38.‘12 ..: 26/l?. 115/75 +

* Angincardiograms nnd pressure tracings unsatisfactory.

t Pulmnnary YCIIOUS wedge. 1 Prrssure curve compatible with pulmonary insufficiency.

ligation of the ductus and was subsequently confirmed by cardiac catheterization and angio- cardiography (Fig. 6).

DISCUSSION

Schwalbe6 in 1909 described a case with absence of the right, and stenosis of the left, puhnonary arteries and referred to two pre- vious reports7 s8 of isolated stenosis of the left pulmonary artery. Monckeberg and associate9 emphasized the association of stenosis of the pulmonary artery branches with pulmonary valvular stenosis. OppenheimerlO in 1938 de- scribed a case of bilateral stenosis with calcifica- tion of the stenotic areas. Since then several small series have been reported”-l7 as well as some isolated case reports.1s-22 Occasional mention of pulmonary artery stenosis is en- countered in papers dealing with the surgical treatment of tetralogy of Fallot.23*24 The major- ity of these reported cases have been in the pedi- atric age group, and tend to fit into one of two groups : localized stenosis involving the pul- monary trunk at or proximal to its bifurcation, or multiple stenoses of the peripheral type in- volving the pulmonary artery branches.

The pulmonary trunk immediately above the semilunar cusps is probably derived from the bulbus cordis, and the remainder of this trunk arises from the truncus arteriosus. The ventral portions of the sixth branchial arches on either side form the proximal segments of the right and left pulmonary arteries. The dorsal por- tion of the right sixth arch disappears com- pletely, while that on the left persists as the ligamentum arteriosum or ductus arteriosus. Owing to the rotation of the truncus arteriosus

APRIL 1964

with respect to the right and left pulmonary arteries, the proximal portion of the left pul- monary artery becomes incorporated into the pulmonary trunk at its bifurcation. The peripheral portions of the pulmonary artery branches are derived from the “post-branchial pulmonary vascular plexus,” which lies in close relation to the growing lung buds.‘”

In our series there were 2 cases classified as supravalvular stenosis, the stenotic area being above the valve but below the bifurcation. Stenosis of the proximal right and left pulmonary arteries would signify defective development of the ventral components of the sixth branchial arches. Presumably stenosis represents an in- termediate stage between the normal and complete unilateral absence of a pulmonary artery. The uniform hypoplastic type of ste- nosis of the main pulmonary branches, which is usually bilateral and appears to involve also the secondary and tertiary branches, could be ascribed to hypoplasia of the vascular plexus arising in relation to the developing lung buds. Of 7 cases of this type described by Gyllens- wgrd et a1.16 2 had blind vascular pouch-like formations communicating with the left pul- monary artery.

It is interesting to note that all our 4 cases of patent ductus arteriosus with unilateral steno- sis had stenosis of the right branch, in this re- spect resembling the reported cases of patent ductus arteriosus with unilateral absence of the pulmonary artery of the opposite side to the duc- tus.

Hypoplasia of the pulmonary arterial tree in tetralogy of Fallot could be explained by the diminished pulmonary blood flow throughout

D’Cruz et al.

FIG. 6. A, selective angiocardiogram with injection into the pulmonary trunk (PA) showing stenosis of a secon- dary branch of the left pulmonary artcry (arrows). B, levoangiocardiogram of same patient showing delay in drainage of the corresponding pulmonary segment. This phenomenon was observed frequently in the uni- lateral pulmonary artery stenosis. LA = left atrium.

life Rich26 described thrombotic lesions in small pulmonary arteries in tetralogy of Fzllot. Keith et al.?’ have confirmed this finding. Theoretically, a possibility exists that in some instances the arterial stenosis is acquired fol- lowing recanalization of such intravascular thromboses.

Unilateral pulmonary artery hypoplasia has been described in association with a small hemi- thorax, an abnormal bronchogram on the affec- ted side and symptoms suggestive of bronchiec- tasis.28s2g None of our cases were this type.

Ciinical Dingnasis: 7’hr incidence of stenosis of the pulmonar\~ artery’ or its branches in the general population probably is higher than previously indicated. As pointed out by Luan et a .,4 some of the so-called functional murmurs in children and voun,g adults may be due to undiagnosed stenosis of the pulmonary artery. Associated lesions, such a; ventricular septal defect and pulmonary valvular stenosis, often overshadow these murmurs and the diagnosis is made unexpectedly at cardiac catheterization or angiocardiography., or both.

The auscultatory findings in stenosis of the pulmonary arteries unassociated with other lesions are usually diagnostic, especially of bilateral stenosis. The murmurs are of systolic ejection type, soft, grade 3;6 intensity, heard maximally at the pulmonic area, diffusely con- ducted, heard very well in the axillae and back. In bilateral pulmonary stenosis the second heart sound at the pulmonary area has a wide and variable splitting, resembling that of pulmo- nary valvular stenosis. In unilateral pulmonary artery stenosis it is normally split. The splitting of the second sound is consistently wider when bilateral pulmonary stenosis is associated with a significant left to right shunt.

The presence of a continuous murmur has been emphasized in previous reports. We did not find such a murmur in any of our cases, although it was particularly sought in each case. The hemodynamics involved in the production of murmurs in stenotic arteries have been in- vestigated by Myers et a1.3n Further investiga- tion is necessary to ascertain whether this applies to the pulmonary circulation.

In our experience, combined cardiac cathe- terization and angiocardiography are necessary for diagnosis of anomalies of the pulmonary artery branches. The hypoplastic and multiple peripheral type of stenosis can be diagnosed preoperatively only by angiocardiography. Se- lective angiocardiography with injection into the main pulmonary artery is preferable for di- agnostic purposes.

The characteristics of the pressure curves obtained from the pulmonary trunk in bilateral pulmonary artery stenosis have been discussed in an earlier paper 5 Similar pressure curves have been found in experimental supravalvular aortic stenosis.31

Treatment: Unilateral stenosis usually causes no adverse hemodynamic effects and needs no treatment; nor is surgical intervention indicated in cases with bilateral stenosis, in which the

THE AMERICAN JOURNAL OF CARDIOLOGY

Stenotic Lesions of the Pulmonary Arteries 349

riqlrt \.cntricular pressure is only midl!. rle- \-atc,d. In the l’ew cases with severe stenosis antI considerable elevation of right ventricular prrssurc, suryer). is indicated. We had 3 xuch patients wit11 localized stenosis, in whom the stc.notic area was incised and the lumen wid- cncd by a patch.“? Other surgical methods of clralinq with the problem have been de- scrihed.2”~‘4~“” The multiple peripheral type and the uniform hypoplastic type of stenoses are not amenable to surgery.

Cases with bilateral pulmonary artery stenosis associated with a left to right shunt at the ventric- ular or atria1 level, and a high pulmonary artery pressure, often pose a difficult problem. If the stenosis in the pulmonary arteries is not recog- nized, or not operable, and if the right ventricu- lar pressure should remain significantly elevated after closure of the septal defect, right heart failure in the postoperative period may be ca- tastrophic.

Thus also, bilaterel pulmonary artery stenosis associated with pulmonary valvular stenosis may be the cause of an elevated pu!monary trunk pressure following pulmonary valvulot- only. This pay be one of the explanations of “unrxplained pulmonary hypertension” that occasionally occurs in pulmonic stenosis24 and in tetralogy of Fallot following corrective sur- gprp.34

SUMMARY

The clinical and hemodynamic data of 32

cases with unilateral pulmonary artery stenosis

and 52 cases with bilateral pulmonary artery stenosis are reported. Twenty-five of the for- mer group and 32 of the latter were associated with other cardiac defects. The diagnosis was substantiated by cardiac catheterization or an- giocardiography, or both. The diagnosis may be suspected because of the typical distribution of the murmur, which is well heard in the axil- lae and back. This is particularly true of the group with bilateral stenosis unassociated with other lesions, which constitutes a relatively clear- cut entity with characteristic auscultatory find- ings, pulmonary trunk pressure curves and an- giocardiographic appearances. The develop- mental origins of this group of lesions and the treatment are briefly discussed.

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