Agenesis of the Left Pulmonary Artery, With Patent Ductus Arteriosus and Hypoplasia of the Left Lung

Ashu Sharma, MD; Frank Bieuei, MD; Evan H. Dillon, MD

Disclosures

Appl Radiol. 2004;33(6) 

In This Article

Discussion

Congenital absence of a pulmonary artery can affect either the right or left side; it was first described by Fraentzel in 1868.[1]In his initial report, he indicated that 30% of patients with this finding could remain asymptomatic until adult life. To date, 168 to 170 patients with this disease have been described in the literature.[2]This case is unusual because imaging observations have been correlated with autopsy findings.

Left pulmonary artery agenesis has been reported with a variety of cardiac malformations and usually presents with symptomatic disease. The associated cardiac malformations include tetralogy of Fallot, atrial septal defect, coarctation of aorta, right aortic arch, and Eisenmenger's syndrome.[3] Rarely, this abnormality has been reported in combination with patent ductus arteriosus.[4] Absent right pulmonary artery is more commonly present as an isolated, asymptomatic finding without associated congenital cardiac malformations. In the case reported here, an adult patient was diagnosed with asymptomatic left pulmonary artery agenesis in combination with hypoplasia of the left lung, right aortic arch, and patent ductus arteriosus on the basis of the CT scan.

According to previously published reports, isolated pulmonary artery agenesis can present clinically in a variety of ways. In a recent series, Bouros et al[5] described 6 cases of isolated pulmonary artery agenesis in adulthood. Only 1 case was asymptomatic, while the others had recurrent respiratory tract infections. Additionally, 1 patient had hemoptysis and dyspnea.

Chest radiography findings may include absence of pulmonary markings on the affected side, displacement of cardiac and mediastinal shadow, elevation of the diaphragm on the affected side, and contralateral lung hyperinflation. Ventilation-perfusion scans usually reveal absence of lung perfusion on the affected side. On CT scans, absence of the affected pulmonary artery is observed. The affected lung is usually supplied by systemic vasculature including bronchial, subclavian, intercostal, and subdiaphragmatic branches. In this case, the collateral circulation involved bronchial arteries.

Other evaluation techniques, including physiologic measurements, cardiac catheterization, pulmonary angiography, and magnetic resonance imaging, have also been used to diagnose this condition.[5] However, as this case illustrated, a CT scan is quite useful in revealing the features of this disorder.

Rarely, left pulmonary artery agenesis in combination with patent ductus arteriosus can present as an asymptomatic condition in adult life. Characteristic findings on contrast-enhanced CT allow accurate characterization of the anomaly.

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