Ann Thorac Surg 2009;87:312-314. doi:10.1016/j.athoracsur.2008.06.015
© 2009 The Society of Thoracic Surgeons
Case Reports
Coiling of Major Aortopulmonary Collateral Arteries With Agenesis of the Left Pulmonary Artery Prior to Pneumonectomy
Karen C. Redmond, MD, FRCS(CTh)a,
Lucy Mansfield, MBBSa,
Nyree Griffin, MDb,
Michael Mullen, MDc,
Michael Dusmet, MDa,*
a Department of Thoracic Surgery, Royal Brompton Hospital, London, United Kingdom
b Department of Radiology, Royal Brompton Hospital, London, United Kingdom
c Department of Cardiology, Royal Brompton Hospital, London, United Kingdom
Accepted for publication June 2, 2008.
* Address correspondence to Dr Dusmet, Consultant Thoracic Surgeon, Royal Brompton Hospital, Sydney St, London, SW3 6NP, United Kingdom (Email: m.dusmet{at}rbht.nhs.uk).
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Abstract
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The case of a patient with a left upper lobe lung tumor and unilateral agenesis of the left pulmonary artery with multiple aortopulmonary collaterals is discussed. Preoperative angiographic localization with embolization of the dominant multiple aortopulmonary collaterals allowed pneumonectomy to be performed safely with acceptable blood loss.
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Introduction
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Although frequently accompanied by cardiovascular anomalies, such as tetralogy of Fallot or septal defects [1], unilateral agenesis of the left pulmonary artery (UAPA) can present as an isolated finding first detected by an abnormal chest roentgenogram showing a hypo-perfused lung field [2]. As there is no pulmonary artery on the affected side, all blood flow to the lung is derived from hypertrophied bronchial vessels or multiple aortopulmonary collaterals (MAPCAs) [3, 4]. Typically there are also diffuse vascular adhesions between the lung and chest wall. Thirty percent of patients are asymptomatic [2], but in a review of 108 cases, the mortality of untreated unilateral agenesis of the left pulmonary artery was 7% [5]. Dyspnea can occur as either a result of pulmonary hypertension or increased dead-space ventilation [5]. Massive hemoptysis from the hypertrophied bronchial vessels can occur [5]. Bronchiectasis can develop in the affected lung and can be responsible for recurrent infection or even lung gangrene [6]. Rare cases of tumor pathology in the affected lung have been described [7].
This article discusses a novel treatment strategy for a patient with a tumor in the left lung and unilateral agenesis of the left pulmonary artery and multiple systemic to pulmonary collateral vessels arising from the left brachiocephalic, the left internal mammary artery, the descending aorta, and the intercostal vessels.
A 70-year-old retired builder presented with a 1-month history of shortness of breath associated with decreased exercise tolerance and productive cough (New York Heart Association functional class II). He smoked a pipe with approximately 25 grams of tobacco per week for the last 60 years. He had no significant past medical history. His physical examination was unremarkable.
A chest roentgenogram was noted to be abnormal with a right-sided aortic arch and a left suprahilar mass. A contrast enhanced computed tomographic chest scan (Fig 1) demonstrated all of these anomalies, as well as absence of the left pulmonary artery and multiple large systemic vessels arising from the left brachiocephalic and internal mammary arteries, the aorta, and the intercostal arteries going to the left lung. The venous anatomy was normal. In addition, there was a well-circumscribed 5.1-cm left upper lobe mass. A fusion positron emission tomographic, computed tomographic scan showed moderate fluorodeoxyglucose (FDG) uptake by the mass. Pulmonary function tests showed moderate airflow limitation with normal lung volumes and normal gas transfer per unit lung volume. This was believed to be consistent with an agenesis of the left pulmonary artery and not suggestive of significant emphysema: Forced expiratory volume in 1 second of 1.29 (47% of predicted), forced vital capacity of 2.24, forced expiratory volume in 1 second and forced vital capacity ratio of 58%, diffusion capacity for CO, corrected for hemoglobin (DLCOc) of 4.52 (58%). An echocardiogram confirmed that there were no associated cardiac malformations and that pulmonary artery pressure was normal. A needle biopsy of the left upper lobe mass was not undertaken due to the significant risk of hemorrhage, with systemic vessels in close proximity to the lesion.
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Fig 1. A contrast enhanced chest computed tomographic scan showing a right-sided aortic arch, the absence of a left pulmonary artery and large, multiple aortopulmonary collaterals (black and white arrows).
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It was decided that preoperative coil embolization of the predominant feeding MAPCAs should be carried out to reduce the risk of perioperative hemorrhage. Angiography confirmed the right-sided aortic arch and collaterals arising from the left brachiocephalic artery, from a large left internal mammary artery, from the descending aorta, and from intercostal vessels (Fig 2). These all drained into the left atrium through the pulmonary veins. The three largest collaterals, which came off of the left brachiocephalic artery, the left internal mammary artery, and the descending aorta were embolized with Cook MReye coils (Cook Medical Inc, Bloomington, IN; Fig 3). The patient sustained a transient ischemic attack after the procedure (partial hemianopia). Surgery, which was initially scheduled on the day after the embolization, was delayed 1 week on advice from a neurologist because of the transient ischemic attack.
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Fig 2. An aortogram revealed collaterals arising from the left brachiocephalic artery in addition to a large left internal mammary artery with significant branches (arrow).
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Fig 3. The three largest collaterals were embolized with Cook MReye coils (Cook Medical Inc, Bloomington, IN; arrows).
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We then proceeded to perform a left thoracotomy and pneumonectomy. The expected innumerable vascular adhesions between the lung and the chest wall were divided during the mobilization of the lung and a large systemic feeding vessel was found and divided on the superior aspect of the hilum. There was no blood flow through this considerable artery. Multiple hypertrophied bronchial arteries were also found and divided. There was no pulmonary artery. Blood loss during the procedure totaled 900 mL, which was significantly less than expected if the embolization had not been performed. A cell saver was used and 300 mL of recovered blood (equivalent to one unit) was auto-transfused to the patient. A biopsy was taken from the tumor and sent for frozen section. This biopsy and the final pathology demonstrated a hamartoma. The patient was discharged and is well at 18-month follow-up.
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Comment
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In cases in which the pulmonary artery fails to develop, small transitory branches of the dorsal aorta persist as enlarged supra-diaphragmatic and infra-diaphragmatic aortic branches or as bronchial arteries [3]. With growth, these develop into MAPCAs, which typically originate from the aorta or its major branches, or both. As there is no pulmonary circulation, the bronchial arteries are also hypertrophied. At surgery, a myriad of vascular adhesions is typically found. Identification and control of the major MAPCAs can be difficult, especially when they are transdiaphragmatic, as the operative field can be very hemorrhagic with MAPCAs supplying many of the smaller collaterals. That is why we sought to exclude the blood supply from the dominant MAPCAs with preoperative coil embolization. There is no robust data in the literature to prove that we effectively reduced blood loss, but we never encountered particularly troublesome or torrential bleeding during the procedure. There is also the possibility that blood loss would have been further reduced had we been able to proceed with surgery on the day after the embolization procedure as planned, because this delay could potentially allow other collateral channels to develop to compensate for the loss of major MAPCAs. However, there were no identifiable complications as a result of this delay.
The second issue in this case was the indication for surgery. If a diagnosis of hamartoma had been secured prior to surgery we could have observed the patient. However, two radiologists did not wish to proceed with any sort of a computed tomographic-guided biopsy because of the risk of severe hemorrhage from the MAPCAs, and we had a sizeable, moderately avid positron emission tomographic positive lesion so that resection seemed to be the only reasonable option with diagnostic and therapeutic intention.
We believe that this is the first report of preoperative preparation for pneumonectomy with embolization of the dominant vessels in a patient with large MAPCAs due to unilateral agenesis of the left pulmonary artery. We believe that it could have significantly contributed to control of perioperative blood loss and we would recommend this approach.
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References
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- Krishnan US, Lamour JM, Hsu DT, Kichuk MR, Donnelly CM, Addonizio LJ. Management of aortopulmonary collaterals in children following cardiac transplantation for complex congenital heart disease Heart Lung Transplant 2004;23:564-569.[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]
- Ellis K. Developmental abnormalities in the systemic blood supply to the lungs AJR Am J Roentgenol 1991;156:669-679.[Abstract/Free Full Text]
- Prasad SK, Soukias N, Hornung T, et al. Role of magnetic resonance angiography in the diagnosis of major aortopulmonary collateral arteries and partial anomalous pulmonary venous drainage Circulation 2004;109:207-214.[Abstract/Free Full Text]
- Harkel DJT, Blom NA, Ottencamp J. Isolated unilateral absence of a pulmonary artery: a case report and review of the literature Chest 2002;122:1471-1477.[Abstract/Free Full Text]
- Canver CC, Pigott JD, Mentzer RM. Neonatal pneumonectomy for isolated unilateral pulmonary artery agenesis Ann Thorac Surg 1991;52:294-295.[Abstract/Free Full Text]
- Roman J, Jones S. Case report: congenital absence of the left pulmonary artery accompanied by ipsilateral emphysema and adenocarcinoma Am J Med Sci 1995;309:188-190.[Medline]
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Abstract
Introduction
