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Isolated Unilateral Absence of a Pulmonary Artery*: A Case Report and Review of the Literature FREE TO VIEW

A. Derk Jan Ten Harkel, MD, PhD; Nico A. Blom, MD; Jaap Ottenkamp, MD, PhD
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*From the Department of Pediatric Cardiology (Dr. Ten Harkel), Sophia Children’s Hospital, Rotterdam, the Netherlands; and Willem Alexander Kinder en Jeugdcentrum (Drs. Blom and Ottenkamp), Leids Universitair Medisch Centrum, Leiden, the Netherlands.

Correspondence to: A. Derk Jan Ten Harkel, MD, PhD, Pediatric Cardiologist, Sophia Children’s Hospital, Department of Pediatric Cardiology, Dr. Molewaterplein 60, 3015 GJ Rotterdam, the Netherlands; e-mail: Tenharkel@alkg.azr.nl



Chest. 2002;122(4):1471-1477. doi:10.1378/chest.122.4.1471
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Objective: The purpose of the present study was to determine the symptomatology, diagnostic procedures, and therapeutic strategies of patients with an isolated unilateral absence of a pulmonary artery (UAPA).

Background: Isolated UAPA is a rare anomaly. Some case reports exist, but the best diagnostic and therapeutic approaches to these patients remain unclear.

Methods: A retrospective analysis was made of 108 cases reported between 1978 and 2000. The database of the National Library of Medicine (MEDLINE) was used to identify cases that were published in any language from 1978 onward.

Results: Of the 108 patients identified, 14 were asymptomatic. The median age was 14 years (range, 0.1 to 58 years). Most patients had symptoms such as frequent pulmonary infections (37%), dyspnea or limited exercise tolerance (40%), or hemoptysis (20%). Pulmonary hypertension was present in 44% of the patients. Surgical procedures were performed in 17% of patients, and the overall mortality rate was 7%.

Conclusion: Only a few patients with isolated UAPA remain asymptomatic during follow-up. The diagnosis can be made by chest radiograph, echocardiography, CT scan, and MRI. Hilar arteries can be shown by cardiac catheterization and pulmonary venous wedge angiography. This is important since revascularization may improve pulmonary hypertension. The avoidance of high altitudes and pregnancy may further improve outcomes.

Figures in this Article

Congenital unilateral absence of a pulmonary artery (UAPA) is a rare anomaly, most frequently accompanied by cardiovascular anomalies such as tetralogy of Fallot19 or septal defects.2,1012 Other congenital heart defects that have been associated with UAPA are the following: coarctation of the aorta, either isolated13or in combination with a ventricular septal defect14; subvalvular aortic stenosis1,15; transposition of the great arteries,16 either isolated or in combination with ventricular septal defect1 or pulmonary stenosis10; Taussig-Bing malformation and coarctation17; congenitally corrected transposition and pulmonary stenosis18; and scimitar syndrome.1922 Although less frequent, UAPA also may occur as an isolated finding. Although some patients with isolated UAPA are completely asymptomatic,23others may have severe pulmonary hypertension and congestive heart failure.2425

After the first case report in 1868,26many case reports and a few reviews have been published,2728 but we have found no review of isolated UAPA published after 1978. Since that time, however, major technological advances have been made, and the role of echocardiography, especially in the diagnostic workup of patients with congenital heart defects, has dominated. Because many congenital heart defects associated with UAPA can have the same signs and symptoms as isolated UAPA (eg, recurrent respiratory infections and congestive heart failure), we decided to study patients with isolated UAPA only.

In the present study, we reviewed all cases in which isolated UAPA had been reported from 1978 onward, as well as including a recent case report from our own institute. We hypothesize that, at present, the diagnosis of isolated UAPA is usually made before the onset of pulmonary hypertension or severe symptoms of hemoptysis. We also propose a diagnostic workup for patients with suspected UAPA.

All patients reported in the literature since 1978 with isolated UAPA (left or right pulmonary artery) were included. More specifically, patients with associated congenital cardiac anomalies were excluded, except those with minor (clinically insignificant) left-to-right shunt without pulmonary hypertension.

The database of the National Library of Medicine (MEDLINE) was used to identify cases that had been published in any language from 1978 onward. The key words “pulmonary artery agenesis” or “absent pulmonary artery” were used. The reference lists of all identified journal articles also were reviewed.

From the case reports, we extracted the diagnostic procedures, the symptomatology, and the therapeutic strategies applied. The most frequently performed diagnostic procedures were the following: ventilation-perfusion scanning; and cardiac catheterization, including pulmonary venous wedge angiography, echocardiography, and CT scanning or MRI. We also extracted more details about the presence or absence of recurrent pulmonary infections, dyspnea or decreased exercise capacitance, hemoptysis, and pulmonary hypertension. Since not all reports gave sufficient detail about the symptoms or procedures performed, the percentages reported here refer only to those cases with sufficient details.

A 3-year-old girl was apparently completely healthy on hospital admission. There were no signs of recurrent respiratory infections, no dyspnea, no hemoptysis, and a normal exercise tolerance. A chest radiograph, which had been performed for routine tuberculosis screening, showed a decreased pulmonary vasculature of the left lung and a displacement of the heart to the left. A soft continuous murmur was heard at the upper left sternal border. Otherwise, the findings of the physical examination were normal. Echocardiography showed a right descending aortic arch, small persistent ductus arteriosus, and the absence of the left pulmonary artery. No left pulmonary venous return was detected by color Doppler echocardiography. A perfusion scintigram showed the absence of perfusion in the left lung. Cardiac catheterization confirmed the diagnosis of an absent left pulmonary artery, a small hemodynamic insignificant ductus from the right pulmonary artery, and a right descending aortic arch (Fig 1 ). There was no pulmonary hypertension. On venous wedge angiography, no left pulmonary arteries were found. Since there were no symptoms, we decided to carefully follow this child and not perform any interventions at that time.

Including our own case, we found a total of 108 patients with UAPA of the right or left pulmonary artery without associated cardiac anomalies. These cases were described in 62 different articles.12,10,1618,20,2325,2980 The median age of these patients was 14 years (range, 0.1 to 58 years). Thirteen patients (12%) were infants < 1 year of age.

In 37% of patients (40 of 107 patients), the left pulmonary artery was absent, while the right was absent in 63% of patients (67 of 107 patients). One report29 was inconclusive about the involved side. In only three cases, the aortic arch was on the same side as the absent pulmonary artery.16,30

Symptoms were chest pain,3536 pleural effusion,35 and recurrent pulmonary infections in 37% of patients (35 of 94 patients), dyspnea or exercise limitations in 40% of patients (36 of 92 patients), and hemoptysis in 20% of patients (18 of 92 patients). Pulmonary hypertension was found in 44% of patients (25 of 57 patients), while high-altitude pulmonary edema (HAPE) was present in 12% of patients (7 of 57 patients) [Table 1] .

In 14 asymptomatic patients, the diagnosis of UAPA was made after abnormal results from a chest radiograph, which had been made for the following reasons: a tuberculosis survey23,48; a routine chest radiograph for induction into the army16; routine examination before an operation17; or for scoliosis.32 Six patients received diagnoses after experiencing HAPE,4547 and in three patients the diagnosis was made during pregnancy37or immediately after pregnancy3839 because of respiratory difficulties.

In all patients, different investigations were made, including CT scans,3133 MRI scans,3234 and bronchography.16,23 A perfusion scan of the lungs was performed in 59% of patients (55 of 94 patients), echocardiography in 34% of patients (34 of 100 patients), and cardiac catheterization in 90% of patients (98 of 108 patients) [Table 1]. In four patients, a pulmonary venous wedge angiogram was made to identify hidden pulmonary arteries. The time period had a marked influence on the usage of echocardiography. From 1990 onward, echocardiography was used in 65% of cases compared with only 15% of cases between 1978 and 1990. The same trend was seen for the use of CT scanning or MRI. From 1990 onward, CT scanning or MRI were used in 52% of cases compared with only 6% of cases between 1978 and 1990.

Therapeutic approaches also differed. In nine cases (8% of patients), a pneumonectomy or lobectomy was performed for reasons of recurrent hemoptysis or intractable pulmonary infections (Table 2 ). The revascularization of hidden pulmonary arteries was performed in eight patients (7%) [Table 2].

One of the three patients with pulmonary hypertension during pregnancy died after delivery.38 Overall, seven patients (7%) died of the following causes: massive pulmonary hemorrhage4041; right heart failure4243; respiratory failure44; pulmonary hypertension38; or HAPE.45

The first report on UAPA was published in 1868.28 The first review of 98 cases, of which 32 were isolated UAPA, was published in 1962.28

Since many patients with UAPA can remain asymptomatic for a long period, the actual prevalence of UAPA is difficult to establish. Some reports state that 30% of patients are asymptomatic.16,49 In the present review, only 14 patients were found to be asymptomatic, while another 9 patients were unmasked by predisposing factors for pulmonary hypertension such as pregnancy or high altitude.3739,4547 In studying the case records, there may, however, be some bias toward the most symptomatic patients. One of the reasons for UAPA remaining unrecognized for a considerable time is that symptoms are not always specific, leading to a considerable delay between the onset of symptoms and the final diagnosis.28 In the present study, we found patients with a time delay of > 30 years. For example, one patient, a 58-year-old woman, had experienced episodes of fever, hemoptysis, and pulmonary infiltrates for almost 30 years before the diagnosis was made.35 In another patient, multiple protracted pulmonary infections since childhood had preceded the final diagnosis at the age of 39 years.50

The best estimation of prevalence came from a study by Bouros and colleagues16 that included 600,000 participants in whom chest radiographs had been made. Of these participants, only six patients were found to have UAPA, of whom three patients had no other cardiac anomalies. This means that one isolated UAPA was found among 200,000 men.

Recurrent pulmonary infections, decreased exercise tolerance, and mild dyspnea during exertion are the most common symptoms. Our finding that 37% of the patients had pulmonary infections is comparable with the results of earlier reports (39%).27 Although the infections are usually mild, they may have devastating effects, such as necrotizing bronchopneumonia, leading to neonatal pneumonectomy.51 In some patients, the frequent respiratory infections may be explained by bronchiectasis.1617 It has been suggested that alveolar hypocapnia can cause bronchoconstriction, while impaired mucociliary clearance and the diminished delivery of appropriate inflammatory cells may contribute to the high incidence of infections in patients with UAPA.16

Less frequently found are hemoptysis and signs of pulmonary hypertension. We found hemoptysis as the presenting symptom in 20% of the patients, which is comparable to the 18% reported earlier by Shakibi et al.27

Hemoptysis in patients with UAPA is caused by excessive collateral circulation.50 The systemic collaterals to the affected lung in patients with isolated UAPA come from the bronchial, intercostal, subclavian, or subdiaphragmatic arteries.52 Before the development of the pulmonary artery, the lung is supplied by small transitory branches of the dorsal aorta that later disappear. These branches persist as enlarged supradiaphragmatic and infradiaphragmatic aortic branches, or as bronchial arteries in cases in which the pulmonary artery does not develop.1,34,52 Hemoptysis may be self-limiting for many years35,52 but also can lead to massive pulmonary hemorrhage and death.4041

In the present review, in 25 of the 57 patients (44%) in whom pulmonary arterial pressure was measured, the pressure had increased. However, when related to the total group of 108 patients, the percentage of patients with pulmonary hypertension was comparable to those found previously (20 to 25%).2728 Which factors determine the presence or absence of pulmonary hypertension in patients with UAPA remain to be elucidated. Pulmonary hypertension in patients with UAPA also may be unmasked by predisposing factors such as HAPE and pregnancy.3739,4547 When HAPE develops at modest altitudes (ie, < 3,000 m) predisposing factors are usually present.,45 Why some patients are prone to HAPE remains to be elucidated.8182 Some patients have pulmonary hypertension before the onset of pulmonary edema, and the combination of exercise and hypoxia lead to further exacerbation and HAPE.45The reactivity of the pulmonary vascular bed to hypoxia differs between individuals who are susceptible to HAPE and those not subject to it.46 This also may be the case in subjects with an absent pulmonary artery, in whom a mild degree of pulmonary hypertension may be present.45

Various diagnostic possibilities are available for patients with UAPA. As a first step, a chest radiograph is usually performed. This may show an absent hilar shadow, a shrunken affected lung, and a shift of the mediastinal structures to the affected side.35 In addition, the absence of the left or right pulmonary artery, ipsilateral grossly diminished pulmonary vascular markings, a small hemithorax and intercostal bone space, ipsilateral cardiac and mediastinal displacement, ipsilateral hemidiaphragm elevation, and contralateral lung hyperinflation52 may be present. Subsequently, an echocardiogram may confirm the diagnosis and exclude other cardiovascular abnormalities. Pulmonary hypertension also can be diagnosed. In this review, in only a minority of the patients (34%) was echocardiography performed. However, from 1990 onward echocardiography as well as CT scanning and MRI were more extensively used in the diagnostic workup of patients with UAPA. Both MRI and high-resolution CT scanning have proven valuable in the evaluation of congenital heart defects.8384 In UAPA patients, the distal pulmonary arteries can be visualized by these techniques, while high-resolution CT scanning also can assess the presence of bronchiectasis in cases of recurrent pulmonary infections.16 Ventilation-perfusion scintigraphy can elucidate the anatomy of the affected side. When revascularization is proposed, cardiac catheterization is necessary, including pulmonary venous wedge angiography to discover hilar arteries. In this review, catheterization was performed in 90% of the patients, but pulmonary venous wedge angiography was performed in only four patients. It is not clear why pulmonary venous wedge angiography was performed in so few patients. Previous studies8586 in patients with pulmonary atresia or the occlusion of one pulmonary artery showed that pulmonary venous wedge angiography is a relatively safe and effective tool with which to visualize hidden pulmonary arteries. In those patients who had undergone surgery, a good correlation with preoperative findings was reported.8586 However, the need for cardiac catheterization may decrease with the more widespread use of echocardiography, CT scanning, and MRI (Table 3 ).

When pulmonary hypertension is present in a patient with UAPA, their condition may be improved by revascularization of the side with the absent artery (Table 2). In most cases, there is an identifiable artery at the hilum that may be used for revascularization.87 In anatomic postmortem studies87 and in surgical studies, hilar arteries usually are found.2 The effect of revascularization has been described. In one case,24a 3-month-old child with severe pulmonary hypertension had normal pulmonary artery pressure after anastomosis of the hidden pulmonary artery. The child’s condition remained stable during 7 years of follow-up. Another report25 described a 21-month-old child with pulmonary hypertension and congestive heart failure in whom a hilar artery was reconstructed with a conduit (GORE-TEX; W.L. Gore and Associates; Flagstaff, AZ), leading to the normalization of pulmonary pressure and improvement of congestive heart failure. This condition remained stable during a follow-up period of 2 years.

If revascularization is not possible, or when pulmonary hypertension does not improve, therapeutic measures such as those described for patients with primary pulmonary hypertension may be helpful. Both in patients with primary pulmonary hypertension and in those with pulmonary hypertension secondary to congenital heart disease, long-term vasodilator therapy may improve survival.8889 Orally administered calcium channel blockers and continuous IV infusion of prostacyclin have been applied. However, we found no patient with UAPA and pulmonary hypertension in whom one of these therapies was applied. The therapy for HAPE consists of transport to a lower level as soon as possible and supplemental oxygen or nitric oxide if available.8182 Hemoptysis may be self-limiting over many years,35,52but massive hemoptysis can be treated only by selective embolization of the systemic collaterals,53 or even pneumonectomy of the affected side.30,54

Many patients with UAPA can remain asymptomatic for a long period. However, the development of a pulmonary hemorrhage or pulmonary hypertension may preclude long-term survival. We found an overall mortality rate of 7%. Pulmonary hypertension may have devastating effects, especially when it develops during pregnancy. In this review, one of the three patients with pulmonary hypertension during pregnancy died. The high mortality rate of patients with pulmonary hypertension during pregnancy frequently has been reported,9091 leading to the advice to prevent pregnancy or terminate pregnancy at an early stage in cases of previously known pulmonary hypertension.91If the pregnancy is too advanced, extensive evaluation and therapy may help to improve outcome. Therapy may include treatment with vasodilators such as nifedipine and continuous NO inhalation.9293

Only a few patients with UAPA remain asymptomatic. UAPA may be suspected by the presence of recurrent respiratory infections, hemoptysis, or pulmonary hypertension. The diagnosis of UAPA can be made by chest radiography and echocardiography. Anatomic details and the presence of bronchiectasis can be discerned by CT scanning and MRI. The presence of hilar arteries can be demonstrated by pulmonary venous wedge angiography. If sufficiently large hilar arteries are found, revascularization may significantly improve outcome. In the case of large collateral arteries leading to pulmonary hypertension or hemoptysis, embolization techniques can be used. The early diagnosis of UAPA thus can prevent further deterioration and can prevent the potentially devastating effects of high altitude or pregnancy.

Abbreviations: HAPE = high-altitude pulmonary edema; UAPA = unilateral absence of a pulmonary artery

Figure Jump LinkFigure 1. Pulmonary angiogram in the anterior view showing the absence of the left pulmonary artery.Grahic Jump Location
Table Graphic Jump Location
Table 1. Summary of the Clinical Findings, Diagnostic Procedures, and Therapies Performed*
* 

Values given as No. of patients/total No. of patients (%).

V̇/Q̇ = ventilation perfusion; Echo = echocardiography; HC = cardiac catheterization; Inf = recurrent respiratory infections; Dys = dyspnea at rest or diminished exercise capacity; Hem = hemoptysis; PHT = pulmonary hypertension; Pneu = pneumonectomy performed; Rev = revascularization of hilar arteries performed.

Table Graphic Jump Location
Table 2. Reasons for Revascularization or Pneumonectomy
Table Graphic Jump Location
Table 3. Reasons for Not Performing a Heart Catheterization
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Putov, NV, Shiriaeva, KF, Libov, SL Isolated unilateral agenesis of the pulmonary artery.Grudn Khir1978;,41-47
 
Thevenin, P, Guillot, B, Pinet, F Case of the month: agenesis of the pulmonary artery with associated abnormalities. J Radiol. 1981;;62 ,.:176, 190. [PubMed]
 
Bonnet, D, Rey, P, Verrot, D, et al Unilateral agenesis of the pulmonary artery: diagnosis and therapeutic management.Rev Pneumol Clin1995;51,83-86. [PubMed]
 
Schmit, P, Grimfeld, A Iconographic rubric: congenital absence of the left pulmonary artery.Arch Fr Pediatr1982;39,835-836. [PubMed]
 
Levine, SJ, White, DA, Fels, AO An abnormal chest radiograph in a patient with recurring high altitude pulmonary edema.Chest1988;94,627-628. [PubMed]
 
Lynch, DA, Higgins, CB MR imaging of unilateral pulmonary artery anomalies.J Comput Assist Tomogr1990;14,187-191. [PubMed]
 
Kucera, V, Fiser, B, Tuma, S, et al Unilateral absence of pulmonary artery: a report on 19 selected clinical cases.Thorac Cardiovasc Surg1982;30,152-158. [PubMed]
 
Morgan, PW, Foley, DW, Erickson, SJ Proximal interruption of a main pulmonary artery with transpleural collateral vessels: CT and MR appearances.J Comput Assist Tomogr1991;15,311-313. [PubMed]
 
Sosa, ES, Ba, M, Kratz, C, et al Congenital unilateral absence of a pulmonary artery.Chir Pediatr1987;28,204-208. [PubMed]
 
Lin, HI, Kao, SJ, Shen, CY, et al Congenital absence of the right pulmonary artery: a case report.J Formosan Med Assoc1984;83,1315-1315
 
Anand, IS, Prasad, BA, Chugh, SS, et al Effects of inhaled nitric oxide and oxygen in high-altitude pulmonary edema.Circulation1998;98,2441-2445. [PubMed]
 
Scherrer, U, Vollenweider, L, Delabays, A, et al Inhaled nitric oxide for high-altitude pulmonary edema.N Engl J Med1996;334,624-629. [PubMed]
 
Rebergen, SA, de Roos, A Congenital heart disease: evaluation of anatomy and function by MRI.Herz2000;25,365-383. [PubMed]
 
Choe, KO, Hong, YK, Kim, HJ, et al The use of high-resolution computed tomography in the evaluation of pulmonary hemodynamics in patients with congenital heart disease: in pulmonary vessels larger than 1 mm in diameter.Pediatr Cardiol2000;21,202-210. [PubMed]
 
Nihill, MR, Mullins, CE, McNamara, DG Visualization of the pulmonary arteries in pseudotruncus by pulmonary vein wedge angiography.Circulation1978;58,140-147. [PubMed]
 
Freedom, RM, Pongiglione, G, Williams, WG, et al Pulmonary vein wedge angiography: indications, results, and surgical correlates in 25 patients.Am J Cardiol1983;51,936-941. [PubMed]
 
Sotomora, RF, Edwards, JE Anatomic identification of so-called absent pulmonary artery.Circulation1978;57,624-633. [PubMed]
 
Barst, RJ, Maislin, G, Fishman, AP Vasodilator therapy for primary pulmonary hypertension in children.Circulation1999;99,1197-1208. [PubMed]
 
Rosenzweig, EB, Kerstein, D, Barst, RJ Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects.Circulation1999;99,1858-1865. [PubMed]
 
Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises: case 4–1999; a 38-year-old woman with increasing pulmonary hypertension after delivery.N Engl J Med1999;340,455-464. [PubMed]
 
Weiss, BM, Zemp, L, Seifert, B, et al Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996.J Am Coll Cardiol1998;31,1650-1657. [PubMed]
 
Lust, KM, Boots, RJ, Dooris, M, et al Management of labor in Eisenmenger syndrome with inhaled nitric oxide.Am J Obstet Gynecol1999;181,419-423. [PubMed]
 
Robinson, JN, Banerjee, R, Landzberg, MJ, et al Inhaled nitric oxide therapy in pregnancy complicated by pulmonary hypertension.Am J Obstet Gynecol1999;180,1045-1046. [PubMed]
 

Figures

Figure Jump LinkFigure 1. Pulmonary angiogram in the anterior view showing the absence of the left pulmonary artery.Grahic Jump Location

Tables

Table Graphic Jump Location
Table 1. Summary of the Clinical Findings, Diagnostic Procedures, and Therapies Performed*
* 

Values given as No. of patients/total No. of patients (%).

V̇/Q̇ = ventilation perfusion; Echo = echocardiography; HC = cardiac catheterization; Inf = recurrent respiratory infections; Dys = dyspnea at rest or diminished exercise capacity; Hem = hemoptysis; PHT = pulmonary hypertension; Pneu = pneumonectomy performed; Rev = revascularization of hilar arteries performed.

Table Graphic Jump Location
Table 2. Reasons for Revascularization or Pneumonectomy
Table Graphic Jump Location
Table 3. Reasons for Not Performing a Heart Catheterization

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Schamroth, CL, Sareli, P, Dean, H Combined unilateral pulmonary artery agenesis and contralateral peripheral pulmonary artery stenosis.Clin Cardiol1987;10,363-366. [PubMed]
 
Ohta, T Congenital absence of the right pulmonary artery: a case report and review.Tokai J Exp Clin Med1983;8,97-104. [PubMed]
 
Putov, NV, Shiriaeva, KF, Libov, SL Isolated unilateral agenesis of the pulmonary artery.Grudn Khir1978;,41-47
 
Thevenin, P, Guillot, B, Pinet, F Case of the month: agenesis of the pulmonary artery with associated abnormalities. J Radiol. 1981;;62 ,.:176, 190. [PubMed]
 
Bonnet, D, Rey, P, Verrot, D, et al Unilateral agenesis of the pulmonary artery: diagnosis and therapeutic management.Rev Pneumol Clin1995;51,83-86. [PubMed]
 
Schmit, P, Grimfeld, A Iconographic rubric: congenital absence of the left pulmonary artery.Arch Fr Pediatr1982;39,835-836. [PubMed]
 
Levine, SJ, White, DA, Fels, AO An abnormal chest radiograph in a patient with recurring high altitude pulmonary edema.Chest1988;94,627-628. [PubMed]
 
Lynch, DA, Higgins, CB MR imaging of unilateral pulmonary artery anomalies.J Comput Assist Tomogr1990;14,187-191. [PubMed]
 
Kucera, V, Fiser, B, Tuma, S, et al Unilateral absence of pulmonary artery: a report on 19 selected clinical cases.Thorac Cardiovasc Surg1982;30,152-158. [PubMed]
 
Morgan, PW, Foley, DW, Erickson, SJ Proximal interruption of a main pulmonary artery with transpleural collateral vessels: CT and MR appearances.J Comput Assist Tomogr1991;15,311-313. [PubMed]
 
Sosa, ES, Ba, M, Kratz, C, et al Congenital unilateral absence of a pulmonary artery.Chir Pediatr1987;28,204-208. [PubMed]
 
Lin, HI, Kao, SJ, Shen, CY, et al Congenital absence of the right pulmonary artery: a case report.J Formosan Med Assoc1984;83,1315-1315
 
Anand, IS, Prasad, BA, Chugh, SS, et al Effects of inhaled nitric oxide and oxygen in high-altitude pulmonary edema.Circulation1998;98,2441-2445. [PubMed]
 
Scherrer, U, Vollenweider, L, Delabays, A, et al Inhaled nitric oxide for high-altitude pulmonary edema.N Engl J Med1996;334,624-629. [PubMed]
 
Rebergen, SA, de Roos, A Congenital heart disease: evaluation of anatomy and function by MRI.Herz2000;25,365-383. [PubMed]
 
Choe, KO, Hong, YK, Kim, HJ, et al The use of high-resolution computed tomography in the evaluation of pulmonary hemodynamics in patients with congenital heart disease: in pulmonary vessels larger than 1 mm in diameter.Pediatr Cardiol2000;21,202-210. [PubMed]
 
Nihill, MR, Mullins, CE, McNamara, DG Visualization of the pulmonary arteries in pseudotruncus by pulmonary vein wedge angiography.Circulation1978;58,140-147. [PubMed]
 
Freedom, RM, Pongiglione, G, Williams, WG, et al Pulmonary vein wedge angiography: indications, results, and surgical correlates in 25 patients.Am J Cardiol1983;51,936-941. [PubMed]
 
Sotomora, RF, Edwards, JE Anatomic identification of so-called absent pulmonary artery.Circulation1978;57,624-633. [PubMed]
 
Barst, RJ, Maislin, G, Fishman, AP Vasodilator therapy for primary pulmonary hypertension in children.Circulation1999;99,1197-1208. [PubMed]
 
Rosenzweig, EB, Kerstein, D, Barst, RJ Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects.Circulation1999;99,1858-1865. [PubMed]
 
Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises: case 4–1999; a 38-year-old woman with increasing pulmonary hypertension after delivery.N Engl J Med1999;340,455-464. [PubMed]
 
Weiss, BM, Zemp, L, Seifert, B, et al Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996.J Am Coll Cardiol1998;31,1650-1657. [PubMed]
 
Lust, KM, Boots, RJ, Dooris, M, et al Management of labor in Eisenmenger syndrome with inhaled nitric oxide.Am J Obstet Gynecol1999;181,419-423. [PubMed]
 
Robinson, JN, Banerjee, R, Landzberg, MJ, et al Inhaled nitric oxide therapy in pregnancy complicated by pulmonary hypertension.Am J Obstet Gynecol1999;180,1045-1046. [PubMed]
 
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