Proximal Interruption of the Pulmonary Artery: A Case Series

Abstract

We present a few cases of Proximal Interruption of the Pulmonary Artery, an uncommon developmental anomaly associated with congenital heart disease. The cases had varied clinical presentations. Chest radiograph showed a hypoplastic lung with an ipsilateral small hilum on the side of the interruption and hyperinflation of the contralateral lung. Contrast CT confirmed the diagnosis, demonstrating non-visualization of the left or right pulmonary artery, and other related findings.

Keywords: Computed tomography; Congenital heart disease.

[Table/Fig-1]:

(a) Chest radiograph PA view shows hypoplastic left lung (blue arrow), with small left hilum and herniation of the right lung (red arrow). (b) Contrast enhanced CT thorax shows non-visualization of the left pulmonary artery, with volume loss in the left lung (blue arrow). (c) Contrast enhanced CT thorax showed a collateral in the mediastinum, which was seen to arise from the left subclavian artery (blue arrow)

[Table/Fig-2]:

(a) Chest radiograph PA view shows loss of right lung volume, with paucity of vascular markings and right upper zone linear opacities and pleural thickening (thin red arrow). The left lung is hyperinflated with prominent left hilum (blue arrow). (b) Contrast enhanced CT thorax shows loss of right lung volume, with non-visualization of the right pulmonary artery. Few systemic collaterals are seen (blue arrow). Internal mammary artery is larger than on the uninvolved side (thin red arrow)

[Table/Fig-3]:

(a) Chest radiograph shows volume loss in the left lung, with a mass in the left upper zone (blue arrow). There is paucity of vascular markings on the left side. (b) Contrast enhanced CT thorax demonstrated a left upper lobe mass, occluding the bronchus and invading the mediastinum (blue arrow). Interruption of the left pulmonary artery is seen. Reduced left lung volume and left pleural thickening is seen (red arrow).

[Table/Fig-4]:

Schematic representation of the embryological development of the major vessels in the neck and thorax

[Table/Fig-5]:

(a) A 5-year-old boy with history of recurrent respiratory tract infections and poor growth since infancy, with subaortic VSD on ECHO. (a) Contrast enhanced CT Thorax shows interrupted right pulmonary artery (blue arrow). (b) Contrast enhanced CT Thorax shows right sided aortic arch (blue arrow) with separate origins of the right subclavian artery and right common carotid artery from the aortic arch (small red arrows). (c) Axial and coronal reconstructed images show the abnormal origin of the left subclavian artery from the proximal descending thoracic aorta (small red arrow)

[Table/Fig-6]:

A 36-year-old man with polycythaemia, pulmonary arterial hypertension and bronchiectasis(a) Chest radiograph shows volume loss in the right lung with areas of parenchymal scarring (blue arrow). (b) Contrast enhanced CT Thorax shows interrupted right pulmonary artery (blue arrow). (c) Contrast enhanced CT Thorax in lung window shows parenchymal scarring with bronchiectatic changes in the right lung (small red arrow). (d) Contrast enhanced CT Thorax shows hypertrophied intercostal and bronchial arteries (small red arrow). (e) Axial and sagittal reconstructed images demonstrate the patent ductus arteriosus (small red arrows) with dilated main pulmonary artery (small blue arrow).

[Table/Fig-7]:

A 22-year-old woman with nephrotic syndrome, presented with cerebral venous thrombosis and dyspnoea. (a) Contrast enhanced CT Thorax shows interrupted right pulmonary artery (blue arrow). (b) Patent ductus arteriosus is demonstrated on contrast enhanced CT Thorax (small red arrow). (c) Contrast enhanced CT Thorax shows a thrombus in the left internal jugular vein (small red arrow).