AJR 2001; 177:681-685
© American Roentgen Ray Society
Congenital Unilateral Pulmonary Vein Atresia
Radiologic Findings in Three Adult Patients
Laura E. Heyneman1,
Robert L. Nolan1,2,
J. Kevin Harrison3 and
H. Page McAdams1
1
Department of Radiology, Box 3808, Duke University Medical Center, Rm. 2523
Blue Zone South, Durham, NC 27710.
2
Present address: Department of Radiology, Queen's University at Kingston, c/o
Kingston General Hospital, Kingston, Ontario, K7L 2V7 Canada.
3
Division of Cardiology, Department of Medicine, Duke University Medical
Center, Durham, NC 27710.
Received December 6, 2000;
accepted after revision February 28, 2001.
Address correspondence to L. E. Heyneman.
Abstract
OBJECTIVE. The purpose of our study is to describe the radiologic
findings of adult patients presenting with congenital unilateral pulmonary
vein atresia.
CONCLUSION. Chest radiography in affected patients typically reveals
a small hemithorax and ipsilateral pulmonary artery as well as ipsilateral
septal thickening. CT shows, in addition, ground-glass attenuation, the
absence of a pulmonary vein connection to the left atrium, and abundant
mediastinal venous collateral vessels. MR imaging is helpful in further
characterizing the vascular abnormalities. Angiography may help to confirm the
diagnosis.
Introduction
Patients with congenital unilateral pulmonary vein atresia usually present
in infancy or childhood with recurrent episodes of pneumonia or hemoptysis
[1]. Presentation in adulthood
is rare but does occur [2,
3]. The radiographic findings
of pulmonary vein atresia include a small ipsilateral hemithorax and
unilateral reticular opacities with septal lines
[4,
5]. Given this appearance,
adult patients with this entity may be suspected to have acquired pulmonary
vein obstruction caused by fibrosing mediastinitis or lung cancer. As a
result, these patients may undergo cross-sectional imaging. To our knowledge,
the CT and MR imaging findings of unilateral pulmonary vein atresia in adults
have not been described. We therefore report the CT findings of three adult
patients with unilateral pulmonary vein atresia, one of whom also underwent MR
imaging.
Materials and Methods
We retrospectively identified three women who were diagnosed with
congenital unilateral pulmonary vein atresia over a period of 4 years. The
patients were 25, 31, and 43 years old. Two of the patients presented with
significant hemoptysis; one of the two also experienced hematemesis. The third
patient presented with recurrent episodes of pneumonia and progressive dyspnea
as a result of pulmonary artery hypertension. Both patients with hemoptysis
had a history of pneumonia as a child. Both also had a history of acyanotic
congenital heart disease (a patent ductus arteriosus requiring surgical
closure and a ventricular septal defect that spontaneously closed). The
clinical and angiographic findings of one of the patients have been previously
reported [2].
All patients underwent conventional chest radiography and CT examinations.
The CT scans were obtained on different scanners using a variety of scan
techniques (collimation, 1.5, 5, and 10 mm). Two studies were performed both
before and after the administration of IV contrast material. The patient with
hematemesis underwent axial T1-weighted, gradient-echo, and phase-contrast MR
imaging performed on a 1.5-T scanner (General Electric Medical Systems,
Milwaukee, WI) to evaluate patency of the portal vein and the intrathoracic
vasculature.
The diagnosis of unilateral pulmonary vein atresia was defined by cardiac
catheterization and pulmonary angiography in all patients. The diagnosis was
confirmed at pneumonectomy in two patients. The third patient was treated
conservatively with bronchial artery embolization and to date has required no
further therapy.
Results
Chest radiography revealed a small hemithorax with ipsilateral mediastinal
shift in all three patients. In one patient, there was a marked mediastinal
shift to the side with the small pulmonary artery; in the other two patients,
only a mild mediastinal shift was noted. The abnormality involved the right
lung in two patients and the left lung in one. The ipsilateral hilum was
diminutive in all patients. Reticular opacities with septal lines were present
in all three patients and were most marked in the lower lobes
(Fig. 1A).
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Fig. 1A. —Congenital unilateral pulmonary vein atresia in 25-year-old
woman with dyspnea and pulmonary artery hypertension. Posteroanterior chest
radiograph shows small right hemithorax and diminutive right pulmonary
artery.
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In all patients, CT scans confirmed a small hemithorax and a small
ipsilateral pulmonary artery. No soft tissue was seen adjacent to the
pulmonary artery to suggest an obstructing mass, and no evidence of bronchial
obstruction was seen to explain the mediastinal shift
(Fig. 1B).
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Fig. 1B. —Congenital unilateral pulmonary vein atresia in 25-year-old
woman with dyspnea and pulmonary artery hypertension. Contrast-enhanced CT
scan (mediastinal window settings) reveals small right pulmonary artery
(black arrows). Note confluent hilar and mediastinal soft tissue in
azygoesophageal recess (white arrows) and surrounding right main
bronchus. No evidence of bronchial obstruction is seen.
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The pulmonary veins on the affected side were not identified. The margin of
the left atrium in the expected location of the pulmonary veins was completely
smooth, without evidence of rudimentary pulmonary veins
(Figs. 2A). Confluent
low-attenuation soft tissue was present in the mediastinum adjacent to the
left atrium and extended from the region of the left atrium into a subcarinal
and paraesophageal location (Figs.
1B,
2A, and
3A). In one patient, the region
of confluent low-attenuation soft tissue extended superiorly in a paratracheal
location to the level of the great vessels. After the administration of IV
contrast material, the abnormal perihilar, mediastinal, and fissural soft
tissue showed heterogeneous enhancement. A persistent left superior vena cava
was present in one patient. In all three patients, the small affected lung
exhibited diffuse ground-glass attenuation and smooth thickening of the
interlobular septa and bronchovascular bundles
(Fig. 1C).
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Fig. 2A. —Congenital unilateral pulmonary vein atresia in 43-year-old
woman with dyspnea and hemoptysis. Contrast-enhanced CT scan (mediastinal
window settings) shows smooth left atrial wall at expected location of right
inferior pulmonary vein (small arrows). Confluent mediastinal soft
tissue extends anteriorly from azygoesophageal recess to surround, but not
narrow, right middle lobe bronchus (large arrow).
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Fig. 3A. —Congenital unilateral pulmonary vein atresia in 27-year-old
woman with hemoptysis and hematemesis. Thin-section CT scan (1.5-mm
collimation, mediastinal window settings) reveals apparent esophageal wall
thickening (black arrows). Also note transpleural collateral vessels
(white arrows). Abnormal subcarinal soft tissue was also seen on
cephalad images (not shown).
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Fig. 1C. —Congenital unilateral pulmonary vein atresia in 25-year-old
woman with dyspnea and pulmonary artery hypertension. Contrast-enhanced CT
scan (lung window) shows ground-glass attenuation throughout right lung. Note
interlobular septal thickening (arrows).
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MR imaging, performed in one patient, confirmed unilateral absence of the
pulmonary veins and revealed abnormal retrocrural collateral vessels that
corresponded to the paraesophageal soft tissue identified on CT
(Fig. 3B). Furthermore,
phase-contrast images revealed retrograde flow in the ipsilateral pulmonary
artery. High signal on gradient-echo sequences in the affected lung
corresponded to regions of interlobular septal thickening on CT. Because of
the patient's hematemesis, the portal vein was evaluated and was found to be
patent, with normal hepatopetal flow.
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Fig. 3B. —Congenital unilateral pulmonary vein atresia in 27-year-old
woman with hemoptysis and hematemesis. Axial T1-weighted spin-echo MR image
(TR/TE, 750/20) through lower hemithorax reveals multiple flow voids in
paraesophageal location (arrows), consistent with paraesophageal
varices and corresponding to apparent esophageal wall thickening on CT. On
gradient-echo images (not shown), flow-related enhancement was present in
subcarinal region. RV = right ventricle, LV = left ventricle, IVC = inferior
vena cava.
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The diagnosis of congenital unilateral pulmonary vein atresia was confirmed
by cardiac catheterization and pulmonary angiography in all patients. Several
findings suggested the diagnosis. In all three patients, markedly increased
pulmonary capillary wedge pressures were noted in the affected lungs,
consistent with pulmonary vein obstruction. The ipsilateral pulmonary arteries
were hypoplastic. Oxygen saturation in the affected pulmonary arteries was
higher than in the contralateral pulmonary arteries, suggesting the presence
of systemic artery flow into the hypoplastic pulmonary arteries. The presence
of systemic collaterals was confirmed by the visualization of reversed
pulmonary artery blood flow on the affected side, a finding that was also
noted on MR imaging. In two cases, absence of the ipsilateral pulmonary veins
was identified on venous phase imaging of the left atrium
(Fig. 2B). In the third case,
the patient with hematemesis, a definitive diagnosis was made by pulmonary
capillary wedge angiography in which contrast material was force-fully
injected into the distal pulmonary vascular bed. Peripheral pulmonary veins
were identified, but no connection to the left atrium was seen; instead the
veins drained into a paraesophageal venous plexus
(Fig. 3C).
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Fig. 2B. —Congenital unilateral pulmonary vein atresia in 43-year-old
woman with dyspnea and hemoptysis. Image from venous phase of pulmonary
angiography reveals absence of right pulmonary veins (arrows on
left). Note normal left pulmonary veins (arrows on right). LA =
left atrium, LUPV=left upper lobe pulmonary vein, LLPV=left lower lobe
pulmonary vein.
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Fig. 3C. —Congenital unilateral pulmonary vein atresia in 27-year-old
woman with hemoptysis and hematemesis. Image from venous phase of capillary
wedge angiography reveals termination of left pulmonary veins at left hilum
(open arrow) and opacification of paraesophageal plexus (solid
arrows). (Reprinted with permission from
[2])
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One patient with hemoptysis underwent percutaneous coil embolization of the
systemic arterial collateral vessels with a therapeutic result. Two patients,
one with recurrent pneumonia and one with both hemoptysis and hematemesis,
underwent ventilation—perfusion scanning as part of a workup before
pneumonectomy. The ventilation scans showed normal findings, but virtually no
perfusion to the affected lung was seen
(Fig. 2C). These two patients
subsequently underwent pneumonectomy of the nonfunctioning lung. At surgery,
the affected lungs had multiple macroscopic transpleural and mediastinal
collateral vessels. The macroscopic pulmonary veins were hypoplastic.
Pathologic examination of the pneumonectomy specimens confirmed the hypoplasia
of the macroscopic veins and revealed other areas of microscopic venous
atresia. The pulmonary veins were markedly thickened, with sclerotic walls
often resulting in obliteration of the lumina. Severe hypertensive changes
were present in peripheral veins and venules.
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Fig. 2C. —Congenital unilateral pulmonary vein atresia in 43-year-old
woman with dyspnea and hemoptysis. Posterior 99mTc macroaggregated
albumin perfusion scan reveals absence of perfusion to right lung. Right lung
ventilation was normal (not shown). L = left lung, R = right lung.
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Discussion
Unilateral pulmonary vein atresia is a rare congenital abnormality that
likely results from failure of incorporation of the common pulmonary vein into
the left atrium [6]. It may
occur in either lung, with no right- or left-sided predominance, and it
usually presents in infants [1,
4]. The most frequent
presenting complaints in infancy include recurrent infections in the
hypoplastic lung and hemoptysis due to the systemic collateral supply to the
affected lung. Other associated congenital heart defects are found in
approximately 50% of patients. Pulmonary artery hypertension is also a
frequent association [7].
To our knowledge, the CT and MR imaging findings of unilateral congenital
pulmonary vein atresia have not been described. The CT scans of all three of
patients showed a small hemithorax with ipsilateral mediastinal shift, a
diminutive ipsilateral pulmonary artery, and absence of ipsilateral pulmonary
vein drainage into the left atrium. The left atrial wall at the expected site
of insertion of the ipsilateral pulmonary vein was completely smooth, without
evidence of even a rudimentary pulmonary vein. No bronchial obstruction was
seen as the cause of the mediastinal shift. All three CT studies also revealed
abnormal confluent mediastinal soft tissue, likely representing collateral
pulmonary-to-systemic venous channels. Venous collaterals were confirmed
angiographically in one patient and surgically in two.
The presence of mediastinal collateral vessels likely relates to the
embryologic development of the pulmonary veins. The primitive pulmonary veins
initially drain into the systemic system via the vitelline and cardinal veins.
The pulmonary veins later coalesce to form a pulmonary vein confluence that
fuses with the left atrium [6].
It is postulated that failure of normal incorporation of the pulmonary veins
into the left atrium results in persistence of the connections between the
pulmonary circulation and the vitelline and cardinal venous systems, and thus
drainage into the portal vein and vena cava
[2].
Pulmonary artery and parenchymal abnormalities were also present. The
ipsilateral pulmonary artery was diminutive, likely because of preferential
pulmonary artery perfusion to the contralateral side, with resultant impaired
growth of the affected pulmonary artery
[5]. This pulmonary artery
hypoplasia likely accounts for the arterial systemic-to-pulmonary collateral
vessels. The interlobular septal thickening, bronchial wall thickening, and
ground-glass opacities likely reflected both pulmonary vein hypertension and
engorged lymphatics.
Gradient-echo and phase-contrast MR imaging further characterized the
vascular abnormalities. Reversal of flow in a small pulmonary artery implied
the presence of systemic-to-pulmonary arterial collaterals. Blood in the
systemic collateral vessels flowed retrogradely into the ipsilateral, then
contralateral, pulmonary arteries, then coursed through the contralateral lung
into the left atrium. The ipsilateral pulmonary veins were absent. Most
abnormal mediastinal soft tissue on CT was confirmed to represent vasculature
on MR imaging. MR imaging was specifically helpful in excluding the presence
of a central obstructing mass.
Despite the ability of MR imaging to characterize vascular abnormalities,
the diagnosis of unilateral pulmonary vein atresia is usually made
angiographically. The presence of systemic-to-pulmonary arterial collaterals
is suggested both by reversal of blood flow in the pulmonary artery and by an
increase in oxygenation in the ipsilateral pulmonary artery. The absence of
venous opacification on venous phase imaging is virtually diagnostic, and the
visualization of atretic pulmonary veins via capillary wedge angiography is
definitive [2].
Adult patients with this entity may present a diagnostic dilemma. The
confluent mediastinal and hilar soft tissue may suggest lung cancer or
fibrosing mediastinitis that involves the hilar vessels, resulting in a small
ipsilateral pulmonary artery and pulmonary vein obstruction. However, the
presence of a small hemithorax without evidence of bronchial obstruction
should suggest a congenital abnormality.
In summary, congenital unilateral pulmonary vein atresia is a rare
abnormality that may present in adults with signs and symptoms of hemoptysis
or respiratory infections. CT findings include a small hemithorax with
ipsilateral mediastinal shift, small ipsilateral pulmonary artery, absence of
the pulmonary vein connection to the left atrium, and parenchymal
abnormalities such as interlobular septal thickening, peribronchovascular
thickening, and ground-glass opacities. The presence of a small hemithorax
without evidence of bronchial obstruction suggests a possible congenital
abnormality. Abnormal mediastinal and hilar soft tissue is commonly caused by
pulmonary-to-systemic venous collaterals. MR imaging may confirm the presence
of vascular collaterals and may show retrograde flow in a small pulmonary
artery on the affected side. Although the diagnosis may be suggested by a
clinical history of recurrent infection, the definitive diagnosis of
hemoptysis, or coexistent congenital heart disease, usually requires cardiac
catheterization, including pulmonary angiography.
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Abstract
Introduction
