AJR 2002; 179:1253-1260
© American Roentgen Ray Society
"Absent" Pulmonary Artery in One Adult and Five Pediatric Patients: Imaging, Embryology, and Therapeutic Implications
Sotiria C. Apostolopoulou1,
Nikolaos L. Kelekis2,
Elias N. Brountzos3,
Spyridon Rammos1 and
Dimitrios A. Kelekis3
1 Department of Pediatric Cardiology, Onassis Cardiac Surgery Center, 356
Syngrou Ave., Athens, GR 176 74, Greece.
2 Department of Radiology, University of Thessalia Medical School, 22
Papakyriazi St., Larissa, GR 41221, Greece.
3 Second Department of Radiology, Eugenidion University Hospital, University of
Athens, 20 Papadiamantopoulou St., Athens, GR 115 28, Greece.
Received December 5, 2001;
accepted after revision April 9, 2002.
Address correspondence to S. C. Apostolopoulou.
Abstract
OBJECTIVE. The purpose of this article is to present serial clinical
and imaging findings for the "absent" pulmonary artery. Data from
six patients with this condition (five unilateral cases and one bilateral
case) provide evidence concerning its embryology and illustrate the
therapeutic implications of surgical intervention.
CONCLUSION. In our series, we found the anatomy of the absent
pulmonary artery to be consistent with a distal ductal origin and involution
of the proximal sixth aortic arch. The absent pulmonary artery is a distinct
embryologic entity that requires early detection and detailed investigation.
Early surgical intervention may be justified in selected patients.
Introduction
Aunilateral or bilateral "absence" of a pulmonary artery may be
encountered either as an isolated entity or as an associated condition in
0.39% of the cases of congenital heart diseases, such as right-sided aortic
arch, septal defects, truncus arteriosus, and tetralogy of Fallot
[1]. Patients with an isolated
unilateral absent pulmonary artery may be relatively asymptomatic into
adulthood [2]. They may
experience recurrent respiratory infections or develop exercise intolerance
[3], hemoptysis, high-altitude
pulmonary edema, and pulmonary hypertension in the contralateral lung. They
may require surgical establishment of blood flow to the unilateral absent
pulmonary artery [4] or even
pneumonectomy in infancy [5].
Patients who have congenital heart disease and a unilateral absent pulmonary
artery usually present with symptoms that are due to their congenital heart
lesions. The distal ductal origin of the absent pulmonary artery has been
proposed as an embyologic explanation
[6]; however, discrete evidence
for this theory is hard to find.
The purpose of our retrospective study was to examine clinical and imaging
data from five patients with unilateral and one patient with bilateral absent
pulmonary arteries, present serial angiographic evidence for the embryology of
the entity, and discuss the therapeutic implications.
Materials and Methods
Our study population was composed of four females and two males who ranged
in age from 10 days to 22 years. Table
1 describes the patients' clinical characteristics in detail. The
22-year-old woman and 6-year-old girl were incidentally diagnosed with
unilateral absent pulmonary artery after chest radiographs were obtained to
confirm respiratory infections. In retrospect, they reported that they had
experienced mildly decreased exercise tolerance and bronchitis episodes twice
a year but that these conditions had not been subject to prior workup. During
infancy, the 18- and 15-month-old girls had received diagnoses of a large
ventricular septal defect with failure to thrive; in both patients, the
unilateral absent pulmonary artery was discovered at the preoperative
assessment. Truncus arteriosus communis type 1 (the main pulmonary artery
arising from the ascending aorta) had been diagnosed in the 10-day-old boy at
birth. The 2-month-old boy had received a diagnosis of heterotaxy,
dextrocardia, visceral situs inversus, single right ventricle, pulmonary
atresia, and total pulmonary venous return to the right-sided innominate vein.
The last two patients underwent angiography because of inadequate central
pulmonary artery visualization on echocardiography.
The diagnostic workup consisted of chest radiography, ECG,
echocardiography, a complete hemodynamic evaluation, and angiography in all
patients. Ventilation—perfusion lung scanning and contrast-enhanced
single-detector helical CT of the chest were performed in the three older
patients. Patients were followed up every 6 months with clinical examinations;
echocardiography; and, in the three older patients, annual exercise stress
tests. Two patients underwent repeated angiography to assess surgical
results.
Results
Chest radiographs in the two older patients showed hypoplasia in the
affected lung with mediastinal displacement
(Fig. 1A). These findings were
more subtle in the two patients in their second year of life
(Fig. 2A), whereas only mildly
decreased pulmonary vascular markings were found in the two patients during
their first year of life (Figs.
3A and
4A). Echocardiography showed
the intracardiac anatomy, hypertrophy of the right ventricle, and pulmonary
artery dilatation. Echocardiography depicted the absent pulmonary artery in
the older patients, but findings were inconclusive in the infants.
Ventilation—perfusion lung scanning showed a total absence of perfusion
to the affected lung, with no perfusion or ventilation defects in the
contralateral lung. In addition to the absent pulmonary artery,
contrast-enhanced CT showed an aortopulmonary collateral network that fed tiny
intrapulmonary vessels in all examined patients
(Fig. 1B) and small cylindrical
bronchiectasis in the 6-year-old girl.
View larger version (147K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1A. —6-year-old girl with right aortic arch, mirror-image
branching, and left-sided "absent" pulmonary artery.
Posteroanterior chest radiograph shows smaller left-sided hemithorax with
absent ipsilateral pulmonary artery shadow; mediastinal shift to left, with
mild elevation of left hemidiaphragm; and presence of right aortic arch.
|
|
View larger version (128K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 2A. —18-month-old girl with large perimembranous ventricular
septal defect, right aortic arch, and right-sided "absent"
pulmonary artery. Posteroanterior chest radiograph shows smaller right lung
with decreased pulmonary vascular markings, slight mediastinal shift to right
and elevated right hemidiaphragm, absence of ipsilateral pulmonary artery
shadow, right aortic arch, and increased vascular markings on left lung
attributable to underlying ventricular septal defect.
|
|
View larger version (131K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 3A. —10-day-old boy with truncus arteriosus, right aortic arch
with mirror-image branching, and left-sided "absent" pulmonary
artery. Posteroanterior chest radiograph shows right aortic arch with mildly
decreased pulmonary vascular markings on left.
|
|
View larger version (146K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 4A. —2-month-old boy with heterotaxy, dextrocardia, visceral situs
inversus, single right ventricle, pulmonary atresia, total pulmonary venous
return to right innominate vein, and bilateral "absent" pulmonary
arteries. Posteroanterior chest radiograph shows dextrocardia with globular
right heart border, prominent upper mediastinum, and decreased pulmonary
vascular markings with mild hyperinflation bilaterally. Central venous
catheter is in place in left superior vena cava.
|
|
View larger version (95K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1B. —6-year-old girl with right aortic arch, mirror-image
branching, and left-sided "absent" pulmonary artery. Oblique axial
maximum-intensity-projection image derived from set of axial helical CT images
(obtained during arterial phase) reveals absence of left main pulmonary artery
and aortopulmonary collaterals (arrowhead) from descending aorta
coursing through hilum to reach tiny intrapulmonary vessels
(arrow).
|
|
On hemodynamic evaluation, the 22-year-old woman and 6-year-old girl had
patent foramen ovale with a mild left-to-right cardiac shunt and mild
right-sided pulmonary artery hypertension. The 18- and 15-month-old girls as
well as the 10-day-old boy had systemic right ventricular pressure with a
large left-to-right shunt. The 18-month-old girl and 10-day-old boy had
stenosis at the origin of the normal pulmonary artery and the lower lobe
artery with normal peripheral pressure.
On angiography, all patients except the 18-month-old girl and the
2-month-old boy had a left-sided unilateral absent pulmonary artery
(Fig. 1C) and a mirror-image
right aortic arch, usually associated with left-sided ductus arteriosus from
the base of the left innominate artery. Excluding the neonate and infants,
pulmonary vein wedge injections showed a hypoplastic 1- to 2-mm peripheral
pulmonary artery, which reached only to the hilum with no antegrade blood flow
(Fig. 1D). The 18-month-old
girl was found to have a right-sided absent pulmonary artery
(Fig. 2B) and a right aortic
arch with a retroesophageal left subclavian artery without diverticulum of
Kommerell (bulbous configuration of the origin of left subclavian artery due
to embryonic ipsilateral ductus arteriosus); in other words, she had
right-sided ductus arteriosus. The 18-month-old girl and the 10-day-old boy
had stenosis at the origin of the normal pulmonary artery, multiple peripheral
stenoses, and mild peripheral pruning (Figs.
2B and
2C). In the 10-day-old boy, a
small patent ductus arteriosus was depicted from the base of the left
innominate artery, supplying an intrapulmonary left pulmonary artery of normal
caliber and distribution with no extrapulmonary portion
(Fig. 3B). Angiography
performed after 6 months showed significant regression of the ductus
arteriosus and the left pulmonary artery
(Fig. 3C). The connection
disappeared angiographically 6 months later
(Fig. 3D). Angiography in the
2-month-old boy showed a left aortic arch with normal branching and bilateral
ductus arteriosi supplying intrapulmonary right and left pulmonary arteries of
normal caliber and distribution with no extrapulmonary portion. The right
ductus arteriosus arose from the base of the right innominate artery, whereas
the left arose from the underside of the arch
(Fig. 4B).
View larger version (133K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1C. —6-year-old girl with right aortic arch, mirror-image
branching, and left-sided "absent" pulmonary artery. Angiogram of
main pulmonary artery shows single right pulmonary artery with increased
caliber, mild tortuosity of peripheral vessels, and absence of left pulmonary
artery.
|
|
View larger version (149K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 1D. —6-year-old girl with right aortic arch, mirror-image
branching, and left-sided "absent" pulmonary artery. Pulmonary
vein wedge angiogram obtained in arterial phase shows extremely hypoplastic 1-
to 2-mm peripheral vessel with pulmonary arterial distribution that reaches
only to hilum (arrow).
|
|
View larger version (144K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 2B. —18-month-old girl with large perimembranous ventricular
septal defect, right aortic arch, and right-sided "absent"
pulmonary artery. Postoperative pulmonary angiogram obtained in
anteroposterior projection shows single left-sided pulmonary artery with
normal distribution, stenosis at its origin (arrow), and absence of
right pulmonary artery.
|
|
View larger version (148K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 2C. —18-month-old girl with large perimembranous ventricular
septal defect, right aortic arch, and right-sided "absent"
pulmonary artery. Postoperative pulmonary angiogram obtained in lateral
projection shows multiple stenoses at origin (open arrow) of left
pulmonary artery as well as its major branches (solid arrows).
|
|
View larger version (118K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 3B. —10-day-old boy with truncus arteriosus, right aortic arch
with mirror-image branching, and left-sided "absent" pulmonary
artery. Angiogram of ascending aorta obtained 10 days after birth shows
patency of ductus arteriosus from base of left innominate artery (open
arrow) supplying intrapulmonary left pulmonary artery of normal caliber
and distribution but with no extrapulmonary portion. Main pulmonary trunk
originates from ascending aorta (solid arrow) and gives rise to right
pulmonary artery.
|
|
View larger version (132K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 3C. —10-day-old boy with truncus arteriosus, right aortic arch
with mirror-image branching, and left-sided "absent" pulmonary
artery. Angiogram of ascending aorta obtained at 6 months shows significant
regression of connecting patent ductus arteriosus (arrow).
|
|
View larger version (126K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 3D. —10-day-old boy with truncus arteriosus, right aortic arch
with mirror-image branching, and left-sided "absent" pulmonary
artery. Angiogram of left innominate artery obtained at 12 months shows
presence of blind stump (solid arrow) with disappearance of
previously patent ductus arteriosus. Superimposed aorto pulmonary collateral
vessel (open arrow) is supplying parts of left upper lobe.
|
|
View larger version (136K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 4B. —2-month-old boy with heterotaxy, dextrocardia, visceral situs
inversus, single right ventricle, pulmonary atresia, total pulmonary venous
return to right innominate vein, and bilateral "absent" pulmonary
arteries. Angiogram of aortic arch shows patent right ductus arteriosus from
base of right innominate artery supplying right intrapulmonary pulmonary
artery and stenosis (arrow) at their junction. Large tortuous left
ductus arteriosus (arrowhead) from underside of aortic arch supplies
left intrapulmonary pulmonary artery. Bilateral absence of extrapulmonary
pulmonary arteries is evident.
|
|
The length of the follow-up ranged from 4 months to 3 years 10 months. The
22-year-old woman and 6-year-old girl remained stable; they were not treated
surgically because of their relatively older ages and mild symptomatology.
Both the 18- and 15-month-old girls underwent surgical closure of the
ventricular septal defect at 18 months of age and remain asymptomatic. The
18-month-old girl underwent graft anastomosis of a 2-mm intrapulmonary
right-sided pulmonary artery remnant identified at surgery with the main
pulmonary artery. She was maintained on oral anticoagulants after surgery.
Angiography was performed 2 years later when the right pulmonary artery was
inadequately visualized on echocardiography. No flow to the anastomosed
remnant was seen; the left pulmonary artery stenoses remained unchanged with
mild right ventricular hypertension and normal peripheral pulmonary artery
pressure. A dobutamine challenge was performed in an attempt to mimic
exercise, which produced a decrease in aortic saturation, systemic right
ventricular pressure, and peripheral pulmonary artery hypertension. The
10-day-old boy underwent a modified left Blalock-Taussig shunt (left
subclavian artery end-to-end anastomosis to left pulmonary artery) at 12
months of age. Subsequent angiography depicted adequate antegrade flow to the
intrapulmonary left pulmonary artery despite mild narrowing at the anastomosis
(Fig. 3E). The flow allowed
the alleviation of ongoing hypoplasia of the vessel before the patient's
scheduled surgical repair (ventricular septal defect closure associating the
left ventricle with the truncal valve, main pulmonary artery dissection from
the ascending aorta, and placement of a right
ventricle—to—pulmonary artery conduit). The 2-month-old infant is
awaiting surgical intervention at this writing.
View larger version (124K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 3E. —10-day-old boy with truncus arteriosus, right aortic arch
with mirror-image branching, and left-sided "absent" pulmonary
artery. Angiogram of left-sided modified Blalock-Taussig shunt
(arrow) obtained 6 months after surgery shows mildly hypoplastic but
patent intrapulmonary left pulmonary artery with normal distribution and
adequate antegrade flow. Mild stenosis (arrow) at anastomotic site is
seen.
|
|
Discussion
Our study confirms that the term "absent pulmonary artery" is
misleading because an intrapulmonary vessel with relatively normal
distribution is present in patients with this condition. Embryologically, the
distal intrapulmonary pulmonary arteries arise from their respective lung buds
and join the proximal portion of the sixth aortic arch (extrapulmonary
pulmonary artery), whereas the main pulmonary artery is derived from the
pulmonary arterial portion of the truncoaortic sac. The dominant embryologic
explanation for the origin of the absent pulmonary artery is the involution of
the proximal sixth aortic arch (destined to become the proximal pulmonary
artery) and persistence of the connection of the intrapulmonary pulmonary
artery to the distal sixth aortic arch (destined to become the ductus
arteriosus). The ductus arteriosus connects to the primitive dorsal aorta,
which becomes the underside of the aortic arch ipsilateral to the arch or the
base of the innominate artery contralateral to the arch
[6]. If involution of the
proximal sixth aortic arch were the sole causation, the side on which the
absent pulmonary artery occurred should be independent of the side of the
fetal ductus arteriosus. Careful review of the literature reveals that all
reported cases of absent pulmonary artery with satisfactory angiographic
[7], surgical
[4,
8], or autopsy
[6] documentation had a ductus
arteriosus or ligamentum ipsilateral to the absent pulmonary artery (12 cases
in total). Even in cases with known contralateral ductus arteriosus arising
from the underside of the arch, an ispilateral ligamentum from the base of the
innominate artery to the absent pulmonary artery was discovered on angiography
[7] or at autopsy
[6]; therefore, bilateral
ductus arteriosi were present (three cases in total).
In accordance with this observation, all our patients had a ductus
arteriosus ipsilateral to the absent pulmonary artery, including the
2-month-old patient who had bilateral absent pulmonary arteries supplied by
bilateral ductus arteriosi. We therefore postulate that involution of the
proximal sixth aortic arch, which results in absent extrapulmonary pulmonary
artery, leads to persistent connection of the intrapulmonary pulmonary artery
to the distal sixth aortic arch (fetal ductus arteriosus).
Figure 5 is a diagram
depicting the primitive (seventh fetal week) anatomy as well as the final
anatomy of the aortic arch and pulmonary arteries observed in the 6-year-old
girl. The same embryology applies to all other patients except the
18-month-old girl (Fig. 6) and
the 2-month-old boy (Fig. 7).
Most of the literature on absent pulmonary artery describes the base of the
innominate artery as the origin of the ductus arteriosus of the absent
pulmonary artery, even though the ductus arteriosus connects, as do all the
aortic arches, to the dorsal aorta, which becomes part of the proximal
subclavian artery (shown schematically in Figs.
5,6,7).
Tissues of the base of the innominate and subclavian arteries are presumably
interwoven during aortic arch development, hence the connection of the ductus
arteriosus to the base of the innominate artery after birth. If the connecting
ductus arteriosus closes after birth, the ipsilateral pulmonary artery will
lose its source of blood supply, diminish in size, and not be visible on
imaging after contrast medium injections in the systemic or pulmonary
circulation. To our knowledge, ours is the only study that angiographically
depicts the progressive cessation of antegrade blood flow to the absent
pulmonary artery due to closure of the ductus arteriosus, as seen in the
images of the 10-day-old boy (Fig.
3A,3B,3C,3D,3E).
View larger version (34K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 5. —6-year-old girl with mirror-image right aortic arch with left
ductus arteriosus from base of left innominate artery and left-sided
"absent" pulmonary artery (same patient as pictured in Fig.
1A,1B,1C,1D).
Diagram shows primitive (seventh fetal week) and final arch and pulmonary
artery anatomy. Roman numerals denote six (I-VI) primitive aortic arches and
seventh intersegmental artery (VII). Truncoaortic sac is depicted in dark
gray, ventral aorta in pink, dorsal aorta in light gray, intrapulmonary
pulmonary arteries in light blue, and primitive aortic arches: III in red, IV
in yellow, and VI in dark blue.
|
|
View larger version (32K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 6. —18-month-old girl with large perimembranous ventricular
septal defect, right aortic arch with retroesophageal left subclavian artery
(persistence of caudal part of left dorsal aorta and involution of left IV
aortic arch) without diverticulum of Kommerell, right ductus arteriosus from
underside of arch, and right-sided "absent" pulmonary artery (same
patient as in Fig.
2A,2B,2C).
Diagram shows primitive (seventh fetal week) and final arch and pulmonary
artery anatomy. Roman numerals denote six (I-VI) primitive aortic arches and
seventh intersegmental artery (VII). Truncoaortic sac is depicted in dark
gray, ventral aorta in pink, dorsal aorta in light gray, intrapulmonary
pulmonary arteries in light blue, and primitive aortic arches: III in red, IV
in yellow, and VI in dark blue.
|
|
View larger version (32K):
[in this window]
[in a new window]
[as a PowerPoint slide]
|
Fig. 7. —2-month-old boy with heterotaxy, dextrocardia, visceral situs
inversus, single right ventricle, total pulmonary venous return to right
innominate vein, pulmonary atresia, left aortic arch with normal branching,
and bilateral ductus arteriosi supplying intrapulmonary right and left
pulmonary arteries with bilateral absent extrapulmonary portions. Right ductus
arteriosus originates from base of right innominate artery and left ductus
arteriosus from underside of arch. (same patient as in Fig.
4A,4B).
Diagram shows primitive (seventh fetal week) and final arch and pulmonary
artery anatomy. Roman numerals denote six (I-VI) primitive aortic arches and
seventh intersegmental artery (VII). Truncoaortic sac is depicted in dark
gray, ventral aorta in pink, dorsal aorta in light gray, intrapulmonary
pulmonary arteries in light blue, and primitive aortic arches: III in red, IV
in yellow, and VI in dark blue.
|
|
An absent pulmonary artery is rare and may escape clinical detection even
into a patient's adulthood if the condition is unilateral and isolated
[9]. Radiologists should be
aware of their potential role in early detection of this entity; even
relatively asymptomatic patients with a unilateral absent pulmonary artery may
have significant morbidity. A higher index of suspicion is advocated in the
case of infants because chest radiography may show only mildly decreased
vascular markings, especially in infants with a patent ductus arteriosus with
normal distal pulmonary arteries. This finding may be overlooked in the
presence of increased pulmonary arterial flow to the contralateral pulmonary
artery because of a coexistent left-to-right cardiac shunt, as seen in our
18-month-old patient. The principal radiologic differential diagnosis is
Swyer-James, or MacLeod's, syndrome
[10], in which the expiratory
chest radiograph shows air trapping and the ventilation—perfusion scan
reveals decreased ventilation and perfusion, as well as delayed washout in the
ventilation scan. Ventilation—perfusion scans in our three patients with
such examinations showed an absence of perfusion in the affected lung with no
delayed washout in the ventilation scan.
In the extremely rare occurrence of bilateral absent pulmonary arteries,
the patient's pulmonary blood flow and life are dependent on the patency of
the ductus arteriosi. This entity, although rare, should be included along
with various other forms of congenital heart disease in the differential
diagnosis of bilaterally decreased pulmonary vascular markings. The
clinician's awareness of the need for further workup in such patients is
critical.
Early detection of an absent pulmonary artery is desirable because it may
offer the opportunity of surgical repair. Our data are insufficient to draw
conclusions concerning surgical intervention; some investigators, however,
have advocated early intervention to preserve the affected lung vasculature
[4]. Regression and hypoplasia
of the affected pulmonary artery begin soon after the closure of the ductus
arteriosus and diminish the chances of successful surgical correction. In a
study of experimental animals with ligation of the left pulmonary artery at
birth, the hilar and axial pulmonary arteries in rapidly growing pigs became
smaller and were often obliterated within 4-6 months, despite a satisfactory
bronchial arterial supply to the intraacinar pulmonary arteries
[11].
The surgical approaches have been either the creation of an aortopulmonary
shunt [1] or connection of the
affected pulmonary artery to the main pulmonary artery
[4]. Attempted surgical
correction (at 18 months of age) in the 18-month-old girl was unsuccessful,
possibly because of her older age and the preexisting severe hypoplasia of the
vessel. Nevertheless, previous reports about patency of the affected pulmonary
artery have been encouraging
[4]. These findings agree with
our experience that provision of adequate antegrade blood flow to the
unilateral absent pulmonary artery before hypoplasia is established may
increase the probabilty of long-term patency, as seen in our 10-day-old
patient who underwent surgery soon after closure of the ductus arteriosus.
In our small series, contrast-enhanced CT of the chest noninvasively
produced with consistent quality an anatomic definition of the pulmonary
arteries, the hilum, and the aortopulmonary collaterals, as reported by other
researchers [2]. Nevertheless,
for the initial diagnosis, CT could not substitute for invasive angiography,
which provides hemodynamic data and more accurate anatomic delineation. The
superiority of angiography is especially apparent, with the use of pulmonary
vein wedge injections, in depicting hypoplastic intrapulmonary vessels in
potential surgical candidates.
Our three older patients had dyspnea on exertion and mild pulmonary
hypertension of the contralateral lung at rest or during pharmacologically
induced exercise. This finding, which may be age-dependent, is in accordance
with reports of mild dyspnea and hypoxemia on exertion in patients with
unilateral absent pulmonary artery
[3] and may help in determining
the prognosis.
Stenoses at the origin of the contralateral pulmonary artery and at
multiple peripheral sites, as seen in the 18-month-old girl and 10-day-old
boy, have been previously reported
[12]. Such stenoses may be
beneficial in patients with left-to-right cardiac shunts, delaying the
development of pulmonary vascular disease. On the other hand, stenoses
increase right ventricular pressure in patients already at risk for pulmonary
hypertension and may produce right ventricular failure, which sometimes
necessitates intervention
[13].
In conclusion, serial angiographic data in our study support the
embryologic theory that an absent pulmonary artery results from involution of
the proximal sixth aortic arch (extrapulmonary pulmonary artery) and
persistence of the connection of the intrapulmonary pulmonary artery to the
distal sixth aortic arch (fetal ductus arteriosus). Regression of the ductus
arteriosus after birth leads to hypoplasia of the affected intrapulmonary
pulmonary artery. Radiologists and clinicians should be aware that a patient
with an absent pulmonary artery might present later in life if the entity is
isolated and that an absent pulmonary artery may be associated with
contralateral pulmonary artery stenosis and pulmonary hypertension. Our study
supports the value of early and accurate detection using imaging and catheter
studies and raises the issue of possible benefit from early surgical
intervention.
References
- Kucera V, Fiser B, Tuma S, Hucin B. Unilateral absence of pulmonary
artery: a report on 19 selected clinical cases. Thorac Cardiovasc
Surg 1982;30:152
-158[Medline]
- Bouros D, Pare P, Panagou P, Tsintiris K, Siafakas N. The varied
manifestation of pulmonary artery agenesis in adulthood.
Chest
1995;108:670
-676[Abstract/Free Full Text]
- Brassard JM, Johnson JE. Unilateral absence of pulmonary artery:
data from cardiopulmonary testing. Chest
1993;103:293
-295[Abstract/Free Full Text]
- Presbitero P, Bull C, Haworth SG, DeLeval MR. Absent or occult
pulmonary artery. Br Heart J
1984;52:178
-185[Abstract/Free Full Text]
- Canver CC, Pigott JD, Mentzer RM Jr. Neonatal pneumonectomy for
isolated unilateral pulmonary artery agenesis. Ann Thorac
Surg 1991;52:294
-295[Abstract]
- Sotomora RF, Edwards JE. Anatomic identification of so-called
absent pulmonary artery. Circulation
1978;57:624
-633[Free Full Text]
- Hentrich F, Stoermer J, Wiesemann G. Unilateral proximal aplasia of
the pulmonary artery: studies on the clinical significance and embryologic
interpretation. Klin Padiatr
1984;196:311
-314[Medline]
- Moreno-Cabral RJ, McNamara JJ, Reddy VJ, Cald-well P. Unilateral
absent pulmonary artery: surgical repair with a new technique. J
Thorac Cardiovasc Surg
1991;102:463
-465[Medline]
- Currarino G, Williams B. Causes of unilateral pulmonary hypoplasia:
a study of 33 cases. Pediatr Radiol
1985;15:15
-24[Medline]
- Grum CM, Yarnal JR, Cook SA, Cordasco EM, Tomashefski JF.
Unilateral hyperlucent lung: noninvasive diagnosis of pulmonary artery
agenesis. Angiology
1981;32:194
-207
- Haworth SG, McKenzie SA, Fitzpatrick ML. Alveolar development after
ligation of left pulmonary artery in newborn pig: clinical relevance to
unilateral pulmonary artery. Thorax
1981;36:938
-943[Abstract]
- Schamroth CL, Sareli P, Dean H. Combined unilateral pulmonary
artery agenesis and contralateral peripheral pulmonary artery stenosis.
Clin Cardiol
1987;10:363
-366[Medline]
- Dalvi BV, Vora AM, Narula D, Kulkarni H. Percutaneous stent
implantation in an adult with left pulmonary artery stenosis and absent right
pulmonary artery. Eur Heart J
1997;18:700
-701[Free Full Text]
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
H. Mutlu, C. Basekim, E. Silit, Z. Pekkafali, E. Ozturk, B. Karaman, M. Kantarci, E. Kizilkaya, and F. Karsli
Gadolinium-enhanced 3D MR angiography of pulmonary hypoplasia and aplasia.
Am. J. Roentgenol.,
August 1, 2006;
187(2):
398 - 403.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. R. Trivedi, T. Karamlou, S.-J. Yoo, W. G. Williams, R. M. Freedom, and B. W. McCrindle
Outcomes in 45 Children With Ductal Origin of the Distal Pulmonary Artery.
Ann. Thorac. Surg.,
March 1, 2006;
81(3):
950 - 957.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
K. Welch, F. Hanley, T. Johnston, C. Cailes, and M. J. Shah
Isolated Unilateral Absence of Right Proximal Pulmonary Artery: Surgical Repair and Follow-Up
Ann. Thorac. Surg.,
April 1, 2005;
79(4):
1399 - 1402.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
