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Ann Thorac Surg 2002;74:255-257
© 2002 The Society of Thoracic Surgeons

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Case report

Hemoptysis resulting from unilateral pulmonary artery agenesis

^a Division of Thoracic and Cardiovascular Surgery, University of Louisville School of Medicine, Louisville, Kentucky, USA
^b Department of Chest Diseases, Minia University, Minia, Egypt

Accepted for publication February 18, 2002.

* Address reprint requests to Dr Bousamra II, 201 Abraham Flexner Way, #1200, Louisville, KY 40202 USA
e-mail: bousamra{at}louisville.edu

	Abstract

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Unilateral pulmonary artery agenesis is a rare congenital anomaly often associated with other cardiovascular abnormalities. It is usually diagnosed and surgically treated in childhood. Subjects without associated cardiac anomalies (isolated unilateral pulmonary artery agenesis) may be asymptomatic or have recurrent respiratory infections. We report a case of left pulmonary artery agenesis in a 35-year-old man complicated by hemoptysis and treated by pneumonectomy. Physicians should be aware of unilateral pulmonary artery agenesis presenting later in life as a source of chronic respiratory symptomatology or hemoptysis.

	Introduction

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Most patients with unilateral pulmonary artery agenesis (UPAA) have coexisting cardiac defects and present in infancy or childhood with symptoms related to the associated cardiac abnormality. Isolated UPAA often goes unrecognized and adults with this malformation usually have chronic symptoms of recurrent respiratory tract infection and dyspnea. Hemoptysis as a presenting symptom of UPAA is uncommon. There have been approximately 150 reported cases of UPAA and fewer than 10% have been associated with hemoptysis [1]. Our patient presented with major hemoptysis requiring urgent radiographic assessment and treatment.

The patient is a 35-year-old man with a long history of recurrent respiratory tract infections and exertional dyspnea. The patient noted throughout his life an inability to engage in vigorous physical activity and was frequently prescribed antibiotics for presumed bronchitis and pneumonia. In the 2 years before admission, the patient experienced several mild episodes of hemoptysis, but with this presentation he coughed up approximately 1 cup of blood. He was seen at 2a local hospital where he was found to be in moderate respiratory distress. Supplemental oxygen was administered. Computed tomography identified the absence of the left pulmonary artery, a right aortic arch, and thickened pleura on the affected side. Bronchoscopy localized the bleeding to the left lung.

The patient was transferred to our institution for further management. The patient continued to have minor hemoptysis. He was in no acute distress. Vital signs were normal and he was afebrile. The patient had symmetrical expansion of the chest and the trachea was midline. There were rhonchi and rales noted posteriorly in the left chest. Cardiac examination revealed normal heart sounds without murmur.

Pulmonary angiography and aortography were performed. The pulmonary angiogram demonstrated complete absence of the left pulmonary artery (Fig 1). The right pulmonary arterial anatomy and bilateral venous drainage was normal. Aortography identified multiple collaterals supplying the left pulmonary parenchyma. These included branches from the distal left subclavian artery, midintercostal arteries, and the inferior phrenic artery. Embolization of the intercostal and inferior phrenic vessels was performed. After the embolization procedure, the patient’s hemoptysis abated.

View larger version (110K): [in this window] [in a new window]   Fig 1. Pulmonary angiogram demonstrating absence of the left pulmonary artery.

Pneumonectomy was performed through a lateral thoracotomy. Pleural adhesions were moderate but were relatively devascularized by the embolization procedure. The pleurae within the inferior portion of the chest were particularly thickened and the lung and the diaphragm were fused with a rich collateral vasculature. No left pulmonary artery was identified. There were two draining veins and the bronchus was normal. The hilar structures were isolated, stapled, and divided. The lung was removed from the diaphragm by application of a linear stapling device after attempts at conventional dissection resulted in increased blood loss. Multiple bleeding points remained along the diaphragm, which were controlled with suture ligature and cautery. The chest was closed and the patient initially recovered well. A hemothorax developed on postoperative day 4 and required reexploration. The bleeding site arose from the diaphragm and was again addressed with hemostatic measures. The subsequent hospital course was uneventful.

In the 18 months after his operation, the patient has not required hospitalization or experienced recurrent respiratory infections. Six months after resection he underwent a maximal oxygen consumption study, which revealed a maximum oxygen consumption rate of 24.4 mL · kg^-1 · min^-1 (59% predicted).

	Comment

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Unilateral pulmonary artery agenesis was first reported by Fraentzel in 1868 [2]. In 1962, Poole and colleagues [3] gathered 98 cases of UPAA of which 32 patients were isolated. In a recent review by Bouros and associates [4], approximately 150 cases of UPAA have been accumulated. Absent left pulmonary artery occurs more frequently in association with other cardiac malformations than right pulmonary artery agenesis [5]. Cardiac malformations occurring in patients with left pulmonary artery agenesis include tetralogy of Fallot, ventricular septal defect with pulmonic stenosis, truncus arteriosus, and total anomalous pulmonary venous drainage [4]. Left pulmonary artery agenesis has been reported with peripheral pulmonary artery stenosis with normal cardiac anatomy [5]. Absent right pulmonary artery is often associated with anomalous pulmonary blood supply originating from the ascending aorta and patent ductus arteriosus [6]. Unilateral pulmonary artery agenesis is thought to result from failure of the sixth aortic arch to form the main branch pulmonary artery. In the absence of a single source of inflow, the pulmonary arterial circulation is derived from segmental vessels arising from the aorta, including a subphrenic contribution from the celiac axis [7]. These vessels hypertrophy, become ectatic, and may rupture within the bronchial submucosa to produce hemoptysis.

The diagnosis of isolated UPAA is often overlooked. Symptoms, when they occur, are related to recurrent respiratory infections. Long-standing mild dyspnea and exercise intolerance are reported [8]. Clinical examination may show asymmetry of the chest with ipsilateral deviation of the trachea, and decreased breath sounds on the involved side [8].

The hemodynamic response of patients with isolated UPAA is variable. Moderate levels of pulmonary hypertension have been encountered. In the report by Poole and colleagues [3], 32 patients had isolated UPAA and 6 of these (19%) had pulmonary hypertension. Unilateral pulmonary artery agenesis can be suspected radiographically by ipsilateral absence of the pulmonary artery shadow. Lung markings may be diminished on the involved side or there may be a reticular pattern from the systemic collaterals [8]. A right aortic arch is commonly associated. Erroneous diagnoses have included tuberculosis, Swyer-James syndrome, tetralogy of Fallot, and lung tumor [4].

Our patient presented with a long history of recurrent respiratory tract infections and a recent history of major hemoptysis. Angiography was performed to define the lesion. Repeated embolization effectively devascularized the pleural space and contributed to a reduction in blood loss during the pulmonary resection. We particularly anticipated a difficult dissection of the pleural space due to the patient’s history of multiple respiratory tract infections and the thickened pleura identified on computed tomographic scan. Pneumonectomy was indicated due to the patient’s recurrent hemoptysis. In addition, repeat embolization carried the theoretical risk of pulmonary infarction as occluding these vessels eliminated the only source of blood supply to the lung. In long-term follow-up, the patient appears to have benefited from resection with a marked decrease in his respiratory symptomatology and improvement in his exercise tolerance.

	Acknowledgments

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Dr. Farghly’s work is supported by a grant from the Embassy of the Arab Republic of Egypt Cultural and Educational Bureau.

	References

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1. Cogswell T.L., Singh S. Agenesis of the left pulmonary artery as a cause of hemoptysis. Angiology 1986;37:154-159.
2. Fraentzel O. Ein fall von abnormer communication der aorta mit der anterior pulmonalis. Virchow Arch Path Anat 1868;43:420.
3. Poole P.E., Vogel J.H., Blount S.G. Congenital absence of a pulmonary artery, the importance of flow in pulmonary hypertension. Am J Cardiol 1962;10:706-732.[Medline]
4. Bouros D., Pare P., Panagou P., et al. The varied manifestation of pulmonary artery agenesis in adulthood. Chest 1995;108:670-676.[Abstract/Free Full Text]
5. Shakibi J.G., Rastan H., Nazarian I., et al. Isolated unilateral absence of the pulmonary artery: review of the world literature and guidelines for surgical repair. Jpn Heart J 1978;19:439-451.[Medline]
6. Ko T., Gatz M.G., Reiss G.R. Congenital unilateral absence of a pulmonary artery: a report of two adult cases. Am Rev Respir Dis 1990;141:795-798.[Medline]
7. Mehta A.C., Livingston D.R., Kawalek W., Golish J.A., O’Donnell J.K. Pulmonary artery agenesis presenting as massive hemoptysis—a case report. Angiology 1987;38:67-71.
8. Bouros D., Panagou P. Agenesis of the lung. Eur Respir J 1990;3:379-380.[Medline]

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This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Michael Bousamra, II Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Farghly, E. Articles by Bousamra, M. Search for Related Content PubMed PubMed Citation Articles by Farghly, E. Articles by Bousamra, M., II Related Collections Lung - other