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Histology of Pulmonary Arterial Supply
in Pulmonary Atresia with Ventricular
Septal Defect
GAETANO THIENE, M.D., CARLA FRESCURA, M.D., ROBERTA M. BINI, M.D.
MARIALUISA VALENTE, M.D., AND VINCENZO GALLUCCI, M.D.
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SUMMARY A histologic study was performed on 22 specimens of pulmonary atresia with ventricular septal defect to 1) ascertain the existence of the main pulmonary artery; 2) distinguish the ductus arteriosus from
the systemic collateral arteries (SCA); 3) establish the nutritive or functional nature of collateral circulation;
and 4) evaluate the morphology of the distal pulmonary bed. Three cases had absent main pulmonary artery,
one with and two without signs of infundibular septation. We suggest that absent main pulmonary artery may
exist with both infundibular pulmonary atresia and persistent truncus arteriosus. SCAs have been found to
have similar histological features as systemic muscular arteries of the same size their medial muscular layer
merges gradually into an elastic one at different depth inside the lungs. Injection of contrast material allowed
us to demonstrate that these vessels are functional, since they inosculate into efficient pulmonary arteries ending in the respiratory units. When the distal pulmonary vascular bed is perfused by large SCAs, proliferative
lesions like those found in large left-to-right shunts may occur. Early in infancy, banding of large, nonstenotic
SCAs could protect the distal pulmonary vasculature. Moreover, total surgical repair should be associated with
ligation of the SCA to avoid residual left-to-right shunt, if the pulmonary arteries can carry the full pulmonary
blood flow.
A HISTOLOGIC STUDY of the pulmonary arterial supply in pulmonary atresia with ventricular
septal defect (VSD) is important for several reasons.
First, the macroscopic dissection cannot always reveal
the existence of a pulmonary trunk arising from the
heart.' This is essential in distinguishing cases without
from cases with atresia of the main pulmonary artery,
and may suggest whether they represent embryologically a form of persistent truncus arteriosus
or a pulmonary atresia with VSD.2 Second, only by
histology it is possible to recognize whether a
collateral branch that arises from the aortic arch is a
ductus arteriosus or a systemic collateral artery.8-10
Third, debate exists on whether the collateral arterial
supply should be considered functional or nutritive.
These vessels are generally reported as a compensatory dilatation or hypertrophy of bronchial
arteries."-14 Stenosis of the collateral vessels may be
visualized by angiocardiography,"-21 but their
morphologic substrate is unknown. Finally, some
patients with pulmonary atresia actually have an increased pulmonary blood flow and eventually become
cyanotic.'2, 16, 22 The question arises whether pulmonary vascular disease occurs in this congenital malformation as much as in other defects with left-toright shunt.
Definition of Terms
Pulmonary atresia with VSD is the malformation in
which both ventricles eject only into the aorta. A
pulmonary trunk usually arises from the cardiac base
at least as a fibrous stem. The pulmonary circulation
is supplied by a patent ductus arteriosus or by systemic collateral arteries originating from the descending aorta.
Truncus type IV is also a cardiac anomaly in which
the pulmonary arterial supply comes through systemic
collateral arteries or a ductus arteriosus, but in which
neither a main pulmonary artery nor an infundibular
septum is identifiable.
Material and Methods
Twenty-two hearts were selected from our anatomic
collection according to the above mentioned
definitions. All specimens had visceroatrial situs
solitus, levocardia and atrioventricular concordance.
The great arteries were normally related when the
pulmonary trunk was identifiable. Cases with
posterior atretic pulmonary trunk (so-called
transposition with pulmonary atresia and VSD)24 have
been excluded from our study.
All patients died within the first year of life.
According to the pattern of pulmonary arterial
supply, the specimens were divided as follows: 11 cases
in which the pulmonary arterial supply was solely
ductus-dependent; seven cases without ductus
arteriosus in which the pulmonary arterial circulation
depended on small systemic collateral arteries arising
from the descending aorta; three cases without ductus
arteriosus and with large systemic collateral arteries;
and one case with a left ductus arteriosus that supplied
the left lung and two large systemic collateral arteries
that supplied the right lung.
From the Departments of Pathology, Cardiology, Pediatrics and
Cardiovascular Surgery, University of Padova Medical School,
Padova, Italy.
Supported in part by contract 78.02306.05, Consiglio Nazionale
delle Ricerche, Rome, Italy.
Address for correspondence: Dr. Gaetano Thiene, Department of
Pathology, Via Gabelli 61, 35100 Padova, Italy.
Received January 30, 1979; revision accepted April 30, 1979.
Circulation 60, No. 5, 1979.
1066
PULMONARY ATRESIA WITH VSD/Thiene et al.
1 067
Segments from the systemic collateral arteries and
the ductus arteriosus and blocks from multiple areas
of the lungs were embedded in paraffin, sectioned 7y
thick and stained with hematoxylin and eosin and
elastic Van Gieson. The great vessels at the cardiac
base were examined histologically when the pulmonary trunk was not seen on gross examination. In
three patients, postmortem angiography of the pulmonary arterial supply was performed before fixation
and histologic examination.
pulmonary arteries; the intracardiac anatomy had an
extremely deviated infundibular septum. The fourth
heart showed a similar extracardiac anatomy, but we
did not see an infundibular septum in the right ventricular outflow tract. On histologic examination the
presence of a vessel with an elastic media was
demonstrated only in the last case. This vessel, which
lay on the left side of the aortic root, was the atretic
pulmonary trunk (fig. 1).
Results
Histology of the Ductus Arteriosus
and the Systemic Collateral Arteries
The ductus arteriosus had the features of a closing
Histology of the Great Vessels at the Cardiac Base
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In four specimens a fibrous strand arising from the
heart base as a vestige of the pulmonary trunk could
not be positively identified by gross examination. In
two of these, derivatives of the sixth aortic arches, i.e.,
ductus arteriosus and pulmonary arteries, were not
observed either inside the pericardial cavity or at the
pulmonary hila. The pulmonary arterial circulation in
these cases was supplied by systemic arteries that
arose from the descending aorta; the intracardiac
anatomy did not show any signs of infundibular septation. The third heart had a right aortic arch and a left
ductus arteriosus connected with two confluent
ductus with intimal proliferation, fragmented internal
elastic lamina, mucoid lakes and cytolitic necrosis of
the media, which appeared musculoelastic.10
The systemic collateral arteries had a muscular
media with absent external elastic lamina and a
thick adventitia, like any other systemic artery of the
same diameter. The media merged gradually into a
thin elastic layer; large systemic arteries lost their
muscular features near the hilum, while small
systemic arteries maintained their muscularity up to
the periphery of the lungs (fig. 2). Sections from
stenotic segments of systemic collateral arteries disclosed focal fibrous intimal thickenings (fig. 3).
N
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FIGURE 1. Histology of the root of the great arteries in a case with apparent absence of the main pulmonary artery. A small
vascular structure with elastic medial layer is visible aside the aorta. A = aorta; P = pulmonary artery. Elastic-Van Gieson
stain; original magnification A) X 12, B) X 48.
l1068
CIRCULATION
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VOL 60, No 5, NOVEMBER 1979
PULMONARY ATRESIA WITH VSD/Thiene
et
al.
1069
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FIGURE 3. Pulmonary atresia with ventricular septal defect: segments of large
collateral arteries with fibrous intimal
proliferations (A and B) and rupture of the
internal elastic lamina (A). Elastic-Van
Gieson stain; original magnification X 60.
Histology of the Lungs
Histologic examination of the lungs was performed
in 15 cases. Figure 4 shows the degree of the
pulmonary vascular disease25 plotted against age, according to the pattern of pulmonary arterial supply.
Medial hypertrophy, intimal proliferation and even
plexiform lesions were observed only in pulmonary
segments perfused by large, nonstenotic systemic
collateral artries (fig. 5). Progressive thinning of the
media of the small pulmonary arteries and arterioles
occurred with age only in those cases with pulmonary
arterial supply that depended on a ductus arteriosus
and/or small or stenotic systemic collateral arteries.
In one patient with multifocal pulmonary arterial
supply, different degrees of pulmonary vascular disease were detected. Serial sections, including
collaterals and their lung segments, showed that the
contrast material injected postmortem passed from
systemic arteries with muscular media, through elastic
FIGURE 2. Pulmonary atresia with ventricular septal defect and pulmonary circulation supplied by collateral arteries. A large
systemic-pulmonary artery together with its origin from the aorta (A) and its branching inside the lung has been dissected and
serially cut histologically. The systemic muscular media (B) merges gradually into a pulmonary elastic layer (C and D). ElasticVan Gieson stain; original magnification B) X 48, C and D) X 190.
CIRCULATION
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PULMONARY ATRESIA AND VENTRICULAR SEPTAL DEFECT
PULMONARY VASCULAR DISEASE IN 15 PATIENTS
12
.
.
9
in
~6-
VOL 60, No 5, NOVEMBER 1979
histologic examination to have a small elastic vessel
next to the aortic root. Among the remaining three,
two had an intracardiac anatomy typical of truncus
arteriosus and one showed an anteriorly deviated infundibular septum that completely obstructed the intracardiac pulmonary outflow as seen in typical
pulmonary atresia with VSD.
A
Nature of the Collateral Circulation
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GRAOE OF PULMONARY VASCULAR DISEASE
4
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ARTERIAL SUPPLY DEPENDING BY:A oUCTUS ARTERIOSUS
* SMALL OR STENOTIC ARTERIES
* LARGE ARTERIES WITHOUT STENOSIS
* SAME PATIENT IN DIFFERENT AREAS
FIGURE 4. Pulmonary atresia with ventricular septal
defect. Grades of pulmonary vascular disease according to
the Heath and Edwards classification25 are plotted against
age in 15 patients with reference to the pattern ofpulmonary
arterial supply.
arteries, to small arteries, arterioles and capillaries of
the respiratory units (fig. 6).
Discussion
Absence of the Main Pulmonary Artery:
A Form of Persistent Truncus Arteriosus
or Pulmonary Atresia with VSD?
Many cardiovascular anomalies cannot be considered either a classic truncus arteriosus communis or
a truncus solitarius aorticus or pulmonalis. Whether
the single trunk is a persistent truncus or a normal
aorta is controversial. This malformation has been
considered a pulmonary atresia with VSD16, 17, 19, 26 or
a solitary aortic trunk with agenesis of the pulmonary
arteries,3 8 or as a persistent ventral aorta with
agenesis of both the sixth aortic arches and the aorticopulmonary septum.27 Manhoff and Howe28 first recognized the absence of the main pulmonary artery as a
separate nosographic entity and described two main
forms: 1) single trunk with lungs supplied by arteries
arising from the descending aorta or by other
anomalous arteries; 2) single trunk with lungs supplied
by ductus arteriosus. The first arterial pattern was
classified by Collett and Edwards as persistent truncus
arteriosus type IV,23 while the latter was described
by Edwards' group as ductal origin of the pulmonary arteries.29 30 We believe that both intraand extracardiac features are essential in distinguishing these two anomalies. In fact, absence of
the main pulmonary artery may be present in
pulmonary infundibular atresia (pseudotruncus) as
well as in persistent truncus arteriosus (truncus type
IV). Among our four cases with absent pulmonary
trunk on gross examination, only one was found on
It is generally believed that the development of the
collateral circulation takes place after birth, due to
hypertrophy and dilatation of the nutritive circulation
of the bronchial arteries, while the ductus arteriosus
undergoes its natural closure. However, in 1947
Taussig observed that in pulmonary atresia with VSD
the ductus arteriosus may often be absent.31 Bharati et
al. confirmed this observation in autopsy material.32
We observed collateral arteries even in newborns,
suggesting that collateral circulation must be present
before birth and may represent the persistence of the
primitive arterial connections between the aorta and
the pulmonary plexus.6 Absence of the ductus
arteriosus has been described in persistent truncus
arteriosus type I and II,4 in tetralogy of Fallot with
absent pulmonary valve,34 in dogs with pulmonary
atresia and VSD,35 and it has been illustrated by Van
Mierop et al. in a dog embryo with persistent truncus
arteriosus.7 These collateral arteries have the same
histologic features as any other systemic muscular
artery of the same size. Distally the muscular media
merges gradually, at different depth inside the lungs,
into an elastic media. The injection of the contrast
material allowed us to visualize these vessels inosculated into efficient pulmonary arteries, ending at
the respiratory units where gas exchange occurs. Since
this circulation is functional and not nutritive,36 the
term "bronchial arteries" is inaccurate to describe the
collateral vessels.
Pulmonary Vascular Disease
A histologic study of the lungs in pulmonary atresia
has rarely been performed and no special attention has
been paid so far to the pattern of the pulmonary circulation or to the difference between cases with or
without VSD.9 7
Despite the complete obstruction of the pulmonary
outflow, large systemic collateral arteries may be
responsible for increased pulmonary blood flow. In
these instances, the pulmonary arterial vascular bed
may have pathologic features similar to those in large
left-to-right shunts." 25 Medial hypertrophy, intimal
proliferations and even plexiform lesions were
observed in our cases. In such patients, cyanosis may,
therefore, worsen as a consequence of pulmonary
vascular disease.
We did not perform a quantitative analysis of structural pulmonary features, although such an investigation would be of value also in pulmonary atresia with
VSD. There is evidence that the high pulmonary
vascular resistance may be due to insufficient develop-
PULMONARY ATRESIA WITH VSD/Thiene et al.
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FIGURE 5. Pulmonary alresia with ventricular septal defect. obstructive pulmonary
vascular disease with medial hypertrophy, intimal proliferation (A) and plexiform lesions
(B) in pulmonary segments perfused by
large, unobstructed collateral arteries.
Hematoxylin and eosin stain; original
magnification A) X 190, B) X 120.
ment of the intra-acinar pulmonary circulation in addition to the hypertensive vascular disease.40
Surgical Considerations
Our histologic findings show that the peripheral
vessels function for gas exchange, regardless of
the source of blood supply. If repair is to be attempted, true pulmonary arteries must be demonstrated.15-2, 41, 42, 45 Surgical reconstruction can be
performed when at least one well-developed true
central pulmonary artery can be identified at the pulmonary hila.'14 41 Both central pulmonary arteries
were absent in only two of our cases. A previously
reported gross examination of our specimen6 disclosed
that in 40% of the cases the pulmonary arteries were
too diminutive to be used for total repair. Progressive
enlargement of hypoplastic pulmonary arteries may
be obtained surgically by reconstructing right
ventricle-to-pulmonary artery continuity with a
pericardial patch graft, leaving the VSD unrepaired.43
If no stenoses occur in the systemic collateral
arteries before the peripheral distribution, the ultimate pathologic changes will result in severe
pulmonary vascular disease. Banding of large, nonstenotic collateral vessels could be indicated early in
infancy to protect the lungs. Nevertheless, the
obliterative lesions may be unequally distributed, depending on the pattern of pulmonary arterial supply.
In one patient in whom the left lung was perfused entirely by the ductus arteriosus and the right lung by
Voi 60, No 5, NOVEMBER 1979
CIRCULATION
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PULMONARY ATRESIA WITH VSD/Thiene et al.
1073
FIGURE 6. Pulmonary atresia with ventricular septal defect and large collateral arteries. Contrast material injected into a
large collateral artery opacifies part of the superior lobe of the right lung, with regular peripheral ramification (A). Histologic
sections reveal contrast material inside large elastic arteries (B), musculoelastic arteries (C), small muscular arteries with intimal proliferation, and inside the alveolar capillaries of the respiratory units (D). Elastic-Van Gieson stain; original magnification B) X 60, C and D X 190.
two large systemic collateral arteries, the pulmonary
vascular disease was confined in the right lung; the
whole left lung was spared and could be used for surgical reconstruction. This case suggests that very accurate hemodynamic assessment is mandatory before
making a decision of inoperability. To avoid residual
left-to-right shunt, total surgical repair should be
associated with ligation of the collateral vessels if the
pulmonary arteries are capable of carrying the full
pulmonary blood flow.'4
References
19.
20.
21.
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1. Sotomora RF, Edwards JE: Anatomic identification of socalled absent pulmonary artery. Circulation 57: 624, 1978
2. Lampertico P: Persistent truncus arteriosus comunis. Folia
22.
Hered Path (suppl IV), Sapil, Milan, 1964
3. Van Praagh R, Van Praagh S: The anatomy of common
aortico-pulmonary trunk (truncus arteriosus comunis) and its
embryologic implications. A study of 57 necropsy cases. Am J
Cardiol 16: 406, 1965
4. Thiene G, Bortolotti U, Gallucci V, Terribile V, Pellegrino PA:
Anatomical study of truncus arteriosus comunis with embryological and surgical considerations. Br Heart J 38: 1109,
1976
5. Crupi G, Macartney FJ, Anderson RH: Persistent truncus
arteriosus. A study of 66 autopsy cases with special reference to
definition and morphogenesis. Am J Cardiol 40: 569, 1977
6. Thiene G, Bortolotti U, Gallucci V, Valente ML, Dalla Volta
S: Pulmonary atresia with ventricular septal defect. Br Heart J
39: 1223, 1977
7. Van Mierop LHS, Patterson DF, Schnarr WR: Pathogenesis of
persistent truncus arteriosus in light of observations made in
dog embryo with the anomaly. Am J Cardiol 41: 755, 1978
8. Stone FM, Amplatz K, Lucas RV, Fukida T, Edwards JE:
Ventricular septal defect, solitary aortic trunk and ductal origin
of pulmonary arteries. Am Heart J 92: 506, 1976
9. Van Praagh R, Ando M, Van Praagh S, Senno A, Hougen TJ,
Novak G, Hastreiter AR: Pulmonary atresia: anatomic considerations. In The Child with Congenital Heart Disease after
Surgery, edited by Langford and Rowe, New York, 1976
10. Gittemberger De Groot AC: Persistent ductus arteriosus: most
probably a primary congenital malformation. Br Heart J 39:
610, 1977
11. Wagenvoort CA, Heath D, Edwards JE: The pathology of the
pulmonary vasculature. Springfield, Ill, Charles C Thomas,
1964
12. Stuckey D, Bowdler JD, Reye RDK: Absent sixth aortic arch: a
form of pulmonary atresia. Br Heart J 30: 258, 1968
13. Edwards JE, McGoon DC: Absence of anatomic origin from
the heart of pulmonary arterial supply. Circulation 47: 393,
23.
1973
14. McGoon DC, Baird DK, Davis GD:
Surgical management of
large bronchial collateral arteries with pulmonary stenosis or
atresia. Circulation 52: 109, 1975
15. Chesler E, Beck W, Schrire V: Selective catheterization of pulmonary or bronchial arteries in the preoperative assessment of
pseudotruncus arteriosus and truncus arteriosus type IV. Am J
Cardiol 26: 20, 1970
16. Jefferson K, Rees S, Somerville J: Systemic arterial supply to
the lungs in pulmonary atresia and its relation to pulmonary
artery development. Br Heart J 34: 418, 1972
17. Macartney FJ, Deverall P, Scott 0: Haemodynamic
characteristics of systemic arterial blood supply to the lungs. Br
Heart J 35: 28, 1973
R, Beck W: The assessment of the arterial
supply to the lungs in pseudotruncus arteriosus and truncus
arteriosus type IV in relation to surgical repair. Am Heart J 88:
542, 1974
Macartney FJ, Scott 0, Deverall P: Haemodynamic and
18. Chesler E, Matisonn
24.
25.
anatomical characteristics of pulmonary blood supply in
pulmonary atresia with ventricular septal defect including a
case of persistent fifth aortic arch. Br Heart J 36: 1049, 1974
McGoon MD, Fulton RF, Davis GD, Ritter DG, Neill CA,
White RI Jr: Systemic collateral and pulmonary artery stenosis
in patients with congenital pulmonary valve atresia and ventricular septal defect. Circulation 56: 473, 1977
Soto B, Pacifico AD, Luna RF, Bargeron LM Jr: A
radiographic study of congenital pulmonary atresia with ventricular septal defect. Am J Roentgenol 129: 1027, 1977
Danilowicz D, Ross J Jr: Pulmonary atresia without cyanosis.
Report of two cases with ventricular septal defect and increased
pulmonary blood flow. Br Heart J 33: 138, 1971
Collett RW, Edwards JE: Persistent truncus arteriosus: a
classification according to anatomic types. S Clin North Am
29: 1245, 1949
Marcelletti C, Mair DC, McGoon DC, Wallace RB, Danielson
GK: Complete repair of transposition of the great arteries with
pulmonary atresia. J Thorac Cardiovasc Surg 72: 215, 1976
Heath D, Edwards JE: The pathology of hypertensive
pulmonary vascular disease. A description of six grades with
special reference to congenital cardiac septal defects. Circula-
tion 18: 533, 1958
26. Somerville J: Management of pulmonary atresia. Br Heart J
32: 641, 1970
27. Angelini P, Leachman RD: Trunco-conal septal defects. An
anatomic and embryologic discussion of common truncus and
related malformations. Eur J Cardiol 2: 11, 1974
28. Manhoff LJ, Howe JS: Absence of the pulmonary artery: a new
classification for pulmonary arteries of anomalous origin. Arch
Pathol 48: 155, 1949
29. Murray CA, Korns ME, Amplatz K, Edwards JE: Bilateral
origin of pulmonary artery from homolateral ductus arteriosus.
Chest 58: 310, 1970
30. Todd EP, Lindsay WG, Edwards JE: Bilateral ductal origin of
the pulmonary arteries. Systemic-pulmonary arterial
anastomosis as first stage in planned total correction. Circulation 54: 834, 1976
31. Taussig HB: Clinical and pathological findings in cases of truncus arteriosus in infancy. Am J Med 2: 26, 1947
32. Bharati S, Paul MH, Idriss FS, Potkin RT, Lev M: The surgical anatomy of pulmonary atresia with ventricular septal
defect. Pseudotruncus. J Thorac Cardiovasc Surg 69: 713, 1975
33. Bharati S5 McAllister HA Jr, Rosenquist GC, Miller RA,
Tatooles CJ, Lev M: The surgical anatomy of truncus
arteriosus comunis. J Thorac Cardiovasc Surg 68: 501, 1974
34. Emmanouilides GC, Thanopoulos B, Siassi B, Fishbein M:
Agenesis of ductus arteriosus associated with the syndrome of
tetralogy of Fallot and absent pulmonary valve. Am J Cardiol
37: 403, 1976
35. Patterson DF, Pyle RD, Van Mierop LHS, Melbin J, Olson M:
Hereditary defects of the conotruncal septum in Keeshond
dogs: pathologic and genetic studies. Am J Cardiol 34: 183,
1974
36. Daliento L, Stritoni P, Chioin R, Frescura C, Thiene G:
Systemic-pulmonary arterial supply in pulmonary atresia and
ventricular septal defect. Postmortem angiography and
histological survey in a case. Chest 74: 685, 1978
37. Wagenvoort CA, Edwards JE: The pulmonary arterial tree in
pulmonic atresia. Arch Pathol 71: 56, 1961
38. Ferencz C: The pulmonary vascular bed in tetralogy of Fallot.
Bull J Hopkins Hosp 106: 81, 1960
angiography. Circulation 58: 140, 1978
43. Gill CC, Moodie DS, McGoon DC: Staged surgical management of pulmonary atresia with diminutive pulmonary arteries.
J Thorac Cardiovasc Surg 73: 436, 1977
44. Kirklin JW, Bargeron LM Jr, Pacifico AD: The enlargement of
small pulmonary arteries by preliminary palliative operations.
Circulation 56: 612, 1977
45. Singh S: Demonstration of pulmonary arteries by contrast injection into a pulmonary vein. Presented at the 15th Annual
General Meeting of the Association of European Paediatric
Cardiologists, Gent, Belgium, 1977
39. Haworth SG, Reid L: Quantitative structural study of
pulmonary circulation in the newborn with pulmonary atresia.
Thorax 32: 129, 1977
40. Haworth SG, Sauer U, Buhlmeyer C, Reid L: Development of
the pulmonary circulation in ventricular septal defect: a quantitative structural study. Am J Cardiol 40: 781, 1977
41. Davis GD, Fulton RE, Ritter DG, Mair DD, McGoon DC:
Congenital pulmonary atresia with ventricular septal defect:
angiographic and surgical correlates. Radiol 128: 133, 1978
42. Nihill MR, Mullins CE, McNamara DG: Visualization of the
pulmonary arteries in pseudotruncus by pulmonary vein wedge
Effects of Acute Hemodynamic Alterations
on Pulmonic Valve Motion
Experimental and Clinical Echocardiographic Studies
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RICHARD E. KERBER, M.D., JAMES B. MARTINS, M.D., ROBERT BARNES, M.D.,
WALTER J. MANUEL, AND MICHAEL MAXIMOV, M.D.
SUMMARY The purpose of this study was to assess the effects of acute alterations of the pulmonary circulation on the pulmonic valve echocardiogram. We measured the pulmonic valve opening velocity (PVOV) and
right-sided systolic time intervals (right ventricular preejection period-to-right ventricular ejection time ratio
[RPEP/RVET] ) in 22 open-chest dogs subjected to acute hemodynamic alterations produced by inferior vena
cava constriction, atrial pacing, isoproterenol infusion and microsphere embolization of the pulmonary artery.
We found only fair correlations between PVOV and peak pulmonary artery flow (r 0.56), right ventricular
dp/dt (r = 0.43), stroke volume (r = 0.42), pulmonary artery systolic pressure (r = 0.33) and peak pulmonary
artery acceleration (r = 0.31). RVET was shortened by reduced venous return (caval constriction) and by increases in heart rate (atrial pacing and isoproterenol), which resulted in increases in RPEP/RVET that did not
correspond well to simultaneous changes in pulmonary artery pressure. In seven patients breathing 10% 02 to
raise pulmonary artery pressure acutely, we found no change or a fall in PVOV. Thus, the pulmonic valve
echocardiogram is influenced by multiple factors relating to parameters of pulmonary flow and right ventricular contractility, and may be of limited clinical usefulness in predicting pulmonary artery pressures.
=
CHARACTERISTIC ALTERATIONS of echocardiographic pulmonic valve motion in the presence of
pulmonary hypertension have been reported.'
These include rapid pulmonic valve opening velocity
(PVOV),' attenuation or abolition of the normal presystolic pulmonic valve opening ("a" dip),'. 2 and
prolongation of the right ventricular preejection
period-to-right ventricular ejection time ratio (RPEP/
RVET).3 4 Several authors have suggested, on the
basis of these studies, that pulmonic valve echocardiography may be useful in the serial assessment of the
pulmonary circulation and permit noninvasive detection of developing pulmonary hypertension.1' 4
However, all the studies reported have been performed on patients with chronic pulmonary hypertension. Little information is available on the effects
of acute changes in pulmonary artery pressure on
pulmonic valve motion. In fact, the determinants of
pulmonic valve motion in general are not known.
Therefore, to evaluate the effects of various acute
hemodynamic alterations on the pulmonic valve
echocardiogram and to determine what factors influence pulmonic valve motion, we performed experiments in both animals and patients.
Methods
Animal Studies
Twenty-two dogs were anesthetized with intravenous chloralose-urethane, intubated and ventilated with a Harvard respirator. A mid-sternal
thoracotomy was performed. The pericardium was incised anteriorly and sutured to the chest wall so that
the heart was suspended in a pericardial cradle. A #7F
Swan-Ganz catheter was inserted into the right ventri-
From the Departments of Internal Medicine and Thoracic
Surgery, The University of Iowa Hospital, Iowa City, Iowa.
Supported in part by NHLBI grant HL-014388, and Iowa Heart
Association grant 76-G-16.
Address for correspondence: Richard E. Kerber, M.D., Department of Medicine, University of Iowa Hospital, Iowa City, Iowa
52242.
Received February 2, 1979; revision accepted April 17, 1979.
Circulation 60, No. 5, 1979.
1074
Histology of pulmonary arterial supply in pulmonary atresia with ventricular septal
defect.
G Thiene, C Frescura, R M Bini, M Valente and V Gallucci
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Circulation. 1979;60:1066-1074
doi: 10.1161/01.CIR.60.5.1066
Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231
Copyright © 1979 American Heart Association, Inc. All rights reserved.
Print ISSN: 0009-7322. Online ISSN: 1524-4539
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