Unilateral Hyperlucent Lung. Non-Invasive Diagnosis of Pulmonary Artery Agenesis

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Abstract
Congenital absence of a pulmonary artery is a rare condition. The patient who survives to adulthood usually presents with an abnormal chest roentgenogram, few symptoms and a variety of diagnostic possibilities. The chest roentgenogram demonstrates an ipsilateral small, contracted, poorly vascularized lung (frequently attributed to fibrosis) and a contralateral enlarged, hyperinflated (compensatory emphysema), well-vascularized lung, that frequently herniates across the midline. There is usually ipsilateral tracheal deviation, diaphragm elevation and narrowed intercostal spaces. The clinician, presented with such a roentgenogram, must first determine which is the abnormal lung to differentiate it from lobar or lung collapse, chronic pleural thickening, unilateral obstructive emphysema or the Swyer-James syndrome. An orderly selection of potentially diagnostic procedures is essential. Two cases of pulmonary artery agenesis (1 right and 1 left) are presented. The appropriate use of pulmonary function testing, laminography, computerized axial tomography, bronchoscopy, bronchography and pulmonary angiography are discussed. The use of conventional ventilation/perfusion lung scanning and Tc nuclear vascular perfusion scanning is discussed. A protocol is presented for the non-invasive diagnosis of this interesting entity.

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