title: Isolated unilateral absence of a pulmonary artery: influence of systemic circulation on alveolar capillary vessels.
abstract: We report a case of isolated unilateral absence of a pulmonary artery. The first clinical symptom that was manifested in the patient was recurrent hemoptysis, and subsequent angiography revealed that the main pulmonary artery was absent in the right lung, which was being fed only from the systemic circulation. Right pneumonectomy was performed, and neither the main pulmonary artery nor its remnant was detected in the resected right lung. Histologically, there were many muscular vessels in the resected lung, with intimal proliferation, or with plexiform-like lesions. The alveolar septum was moderately thickened and alveolar capillary vessels were dilated. We examined the alveolar capillary endothelial cells of the resected lung for immunoreactivity to thrombomodulin (TM) and von Willebrand factor (vWF). The endothelial cells were negative for TM and positive for vWF, while in the normal lung control group, these cells were positive for TM and negative for vWF. We considered that the hemodynamics of the systemic circulation in the resected lung caused the alteration of immunohistochemical characteristics in alveolar capillary endothelial cells.
authors.affiliation: Department of Pathology, Tohoku University School of Medicine 2-1 Seiryo-machi, Aoba-ku, Sendai 980-8575, Japan. sumiko@idac.tohoku.ac.jp
authors.forename: S
authors.initials: S
authors.lastname: Maeda
authors.forename: S
authors.initials: S
authors.lastname: Suzuki
authors.forename: T
authors.initials: T
authors.lastname: Moriya
authors.forename: T
authors.initials: T
authors.lastname: Suzuki
authors.forename: M
authors.initials: M
authors.lastname: Chida
authors.forename: H
authors.initials: H
authors.lastname: Suda
authors.forename: H
authors.initials: H
authors.lastname: Sakuma
authors.forename: T
authors.initials: T
authors.lastname: Kondo
authors.forename: H
authors.initials: H
authors.lastname: Sasano
mesh_terms: D000328:Adult; D001980:Bronchi / Q000098:blood supply* / Q000473:pathology*; D004730:Endothelium, Vascular / Q000473:pathology*; D005260:Female; D006469:Hemoptysis / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007564:Japan; D011651:Pulmonary Artery / Q000473:pathology*; D011652:Pulmonary Circulation; D018180:Thrombomodulin / Q000032:analysis; D014841:von Willebrand Factor / Q000032:analysis
chemical_list: D018180:Thrombomodulin; D014841:von Willebrand Factor