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Pulmonary Artery Malformation Syndrome

We present a case to illustrate that pulmonary artery malformation is a potential diagnosis in patients with nonresolving perfusion defects. The diagnosis can usually be made by the history, physical examination, chest roentgenogram, and ventilation-perfusion scanning. If the patient's clinical symptoms are inconsistent with the scan, then pulmonary angiography is warranted. Pleuritic chest pain, hypoxemia, and a perfusion defect are nonspecific and should not be interpreted as indicative of pulmonary embolism, but only that it has not been ruled out. Anticoagulation is risky because these patients are already at increased risk for pulmonary hemorrhage and hemoptysis.