Ann Thorac Surg 2010;90:e6-e8. doi:10.1016/j.athoracsur.2010.03.097
© 2010 The Society of Thoracic Surgeons
Case Reports
Unilateral Absence of the Left Pulmonary Artery Accompanied by Right Lung Cancer
Masaoki Ito, MD,
Yoshinori Yamashita, MD, PhD*,
Hiroaki Harada, MD,
Ken-ichi Omori, MD, PhD
Department of General Thoracic Surgery, National Hospital Organization Kure Medical Center and Chugoku Cancer Center, Kure, Japan
Accepted for publication March 26, 2010.
* Address correspondence to Dr Yamashita, Department of General Thoracic Surgery, National Hospital Organization Kure Medical Center and Chugoku Cancer Center, Kure, 737-0023 Japan (Email: yamashitay{at}kure-nh.go.jp).
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Abstract
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Unilateral absence of a pulmonary artery is a rare congenital anomaly that is often accompanied by other cardiovascular anomalies. Surgical treatment for this rare disease is usually performed only on the abnormal side. We report on a case of unilateral absence of a pulmonary artery that was complicated by contralateral primary lung cancer. We performed a right middle lobectomy despite the absence of the left pulmonary artery and detected changes in respiratory function during the operation. The present case provides information on the surgical tolerability and predictable morbidity following lung resection of the normal side in unilateral absence of a pulmonary artery.
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Introduction
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Unilateral absence of a pulmonary artery (UAPA) is a rare anomaly and usually occurs in association with other cardiac lesions [1–4]. Symptoms such as hemoptysis, recurrent pulmonary infections, and dyspnea on effort are often associated with the condition. The prognosis of UAPA depends on the severity of the complicating cardiovascular anomalies or pulmonary hypertension, or both [1, 3]. Surgical treatment for UAPA is usually considered on the affected side.
After a routine medical check-up of a 57-year-old man, tumors were detected in his stomach and right lung. He was referred to Kure Medical Center and Chugoku Cancer Center for further examination. The patient's past medical history was solely tuberculosis. After the tumors had been identified as adenocarcinoma, both were resected in two operations performed 1 month apart. The pathologic diagnoses were pT1(Noguchi B)N0M0 lung cancer and pT1N0M0 stomach cancer (Fig 1). The patient was presumed to have simultaneously developed separate cancers. Five years later, a new pulmonary nodule in the right lower lobe was discovered on a periodic computed tomographic (CT) scan. Neither distant nor lymph node metastases were detected. Although no biopsies were taken, the new lesion was suspected to be a new primary lung cancer and resection was considered. Because the new tumor was located near the upper bronchial trunk and the hilar vessels had been completely ablated in the previous surgeries, we feared that resection of the upper and lower lobes may be necessary. So, perfusion scintigraphy was performed prior to surgery, and an absent perfusion in the left lung was identified. By rechecking the CT scans, we identified a unilateral absence of the left pulmonary artery. This finding was further confirmed on a CT scan that had been taken before the first series of operations (ie, 5 years previously). Pulmonary arteriography also provided evidence of interruption of the left pulmonary artery (Fig 2). After thrombosis of the pulmonary artery had been ruled out, the patient was diagnosed as having congenital absence of the left pulmonary artery. We decided not to proceed with the surgery, and at the patient's request the tumor was treated with radiotherapy using the Novalis stereotactic system, and the new lesion had shrunk and was under good control.
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Fig 1. Computed tomographic (CT) scan shows the pulmonary nodule and hematoxylin and eosin stained histology of the lung and stomach tumors. (A) The CT scan shows a solitary nodule in the middle lobe of the right lung. (B) Histology of the lung tumor indicates adenocarcinoma growing along the bronchial structures. The tumor is accompanied by adenomatous hyperplasia and focal collapse (Noguchi B). (Hematoxylin and eosin; x200.) (C) Histologic finding of the stomach tumor represents adenocarcinoma with focal submucosal invasion. The tumor does not invade beyond the submucosal layer. (Hematoxylin and eosin; x200.)
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Fig 2. Images of the newly found pulmonary lesion and representative findings of unilateral absence of a pulmonary artery. (A) The new tumor in the lower lobe of the lung was located near the upper bronchial trunk and hilar vessels. (B) Pulmonary arteriography revealed an interruption of the left pulmonary artery.
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Comment
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Unilateral absence of a pulmonary artery is a rare anomaly that was first reported by Fraentzel [5]. The UAPA is often accompanied by cardiovascular anomalies, such as tetralogy of Fallot, septal defects, and so forth, especially in pediatric patients [1–4]. In adult patients, symptoms such as hemoptysis, recurrent pulmonary infections, and dyspnea on effort are often associated with the condition, or UAPA can be diagnosed incidentally on the basis of abnormalities shown on chest roentgenograms [2, 3]. Approximately 13% to 15% of patients with UAPA remain asymptomatic [1, 2]. Harkel and colleagues [2] reported that 14 of 108 patients with UAPA had no symptoms. A diagnosis of UAPA is often difficult, and patients with few or no symptoms may remain undiagnosed until adulthood [1, 4, 6]. The diagnosis of UAPA in the present case was based on the same findings of an interrupted pulmonary artery on CT scans performed 5 years apart and the fact that thrombosis was ruled out by normal electrocardiogram and blood examination results.
Chest roentgenograms, CT scans, digital subtraction angiography, and pulmonary angiography are useful modalities for the diagnosis of UAPA [1–4, 6]. On CT scans or roentgenograms of patients with UAPA, elevation of the ipsilateral hemidiaphragm, a small hemithorax, a mosaic attenuation pattern, and displacement of the mediastinum are often seen, in addition to interruption in the pulmonary artery [2–4]. The prognosis of UAPA depends on the severity of the complicating cardiovascular anomalies or pulmonary hypertension, or both [1, 3]. Surgical treatment for UAPA is considered for patients in whom these anomalies are present or for those who are symptomatic. For example, possible therapeutic strategies are revascularization for the treatment of pulmonary hypertension or for cardiac failure and pneumonectomy or lobectomy for hemoptysis or recurrent pulmonary infection (or both of the latter two) [2, 4, 6].
We believe there have only been two other reported cases of lung cancer with UAPA [6, 7]. In both cases surgery was performed on the affected side only, and there are no guidelines as to the indications for surgery on the normal side. We believe that the present case is the first in which surgery was performed on the contralateral (normal) side. In our case, right middle lobectomy was performed by a thoracotomy, and sensitive changes in respiratory function were seen during the operation. According to the anesthesia record during the surgical procedure, repeated decreases in CO2 in the expired gas were noted before resection of the bronchus. These decreases in CO2 occurred every time hemi-lateral lung ventilation was started, necessitating the release of the bronchial clamp.
Although we could not determine the reason for this inadequate ventilation, the right middle lobectomy was performed. There were no postoperative complications, and the patient underwent total gastrectomy 1 month later.
We were able to perform standard curative surgery for primary lung cancer and to achieve a good prognosis regardless of the UAPA. To some extent, this outcome may be due to the relatively good condition of the patient initially. There were no abnormalities identified on the computed tomographic scan or radiographic scan, other than the tumor itself, interruption of the left pulmonary artery, and trivial abnormalities that could be explained by the patient's history of tuberculosis. In fact, the patient had lived asymptomatically until his late 50s, and his daily life was not restricted despite the postoperative decline in respiratory function.
This present case indicates that standard surgery for lung cancer on the normal side in UAPA may be feasible. In addition, we performed a lobectomy, which is not a limited resection. This could indicate that lesser resections (eg, segmentectomy or wedge resection) can also be performed on the normal side in patients with this rare congenital disease.
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Acknowledgments
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The authors thank Drs Kikuo Nakano and Teruya Nakamura for their useful suggestions regarding treatment in this case.
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References
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- Ten Harkel AD, Blom NA, Ottenkamp 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]
- Sakai S, Murayama S, Soeda H, et al. Unilateral proximal interruption of the pulmonary artery in adults: CT findings in eight in patients J Comput Assist Tomogr 2002;26:777-783.[Medline]
- Griffin N, Mansfield L, Redmond KC, et al. Imaging features of isolated unilateral pulmonary artery agenesis presenting in adulthood: a review of four cases Clin Radiol 2007;62:238-244.[Medline]
- Fraentzel O. Angeborener Defect der Rechten Lungenarterie Virchow Arch Pathol Anat 1868;43:420.
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- Mancebo A, Wanner A. Lung tumor in a patient with congenital unilateral hypoplasia of the pulmonary artery Chest 1975;68:846-847.[Abstract/Free Full Text]
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Abstract
Introduction
