Criss-cross pulmonary arteries with a plethora of other anomalies in an adult

Dear Editor,

A 35-year-old male presented to the out-patient department with complaints of dyspnea on exertion (NYHA grade II) since childhood. There was recent onset of productive cough and low-grade fever for the past 1 month. Transthoracic echocardiography (TTE) was suggestive of a double-outlet right ventricle with mild pulmonary stenosis and an ostium secundum atrial septal defect. Contrast-enhanced computed tomography angiography (CTA) was done for further evaluation and showed the double outlet of the right ventricle [Figure 1a]. There was a criss-cross configuration of the pulmonary arteries [Figure 1b]. The arch was left-sided with an aberrant right vertebral artery, arising as the last branch of the arch, with a retroesophageal course causing mild esophageal compression [Figure 1c and f]. The origin of the left circumflex coronary artery was anomalous, arising from the right coronary cusp with a retro-aortic course [Figure 1d]. There was an ostium secundum atrial septal defect [Figure 1e]. Additionally, multiple centrilobular nodules with cavitatory changes were noted in both the lung parenchyma, suggestive of infective changes, with sputum examination positive for acid-fast bacilli [Figure 1f and g].

Figure 1.

Contrast-enhanced computed tomography angiography sagittal oblique image (a) showing the double outlet of right ventricle (white arrow) with ventricular septal defect (black asterisk); volume-rendered images (b and c) showing the criss-cross pulmonary artery configuration (white asterisk-left pulmonary artery, (b) and the aberrant origin of the right vertebral artery (arrow, c), with brachiocephalic trunk giving rise to right common carotid (arrow head, c) and right subclavian (asterisk, c) artery. Axial oblique maximum intensity projection image (d) showing the anomalous origin of left circumflex artery (black arrow head) from right coronary cusp (black arrow) with normal origin of right coronary (white arrow head) and left anterior descending coronary (black asterisk) arteries. Note the ostium Secundum ASD (asterisk, e), mild esophageal (asterisk) compression by right vertebral artery (arrow, f). Cavitatory consolidation (asterisk) with centrilobular nodules were noted in both the upper lobes suggestive of infective lung changes (g). Sputum examination was positive for acid-fast bacilli

Criss-cross pulmonary arteries, also known as crossed pulmonary arteries (CPAs), are a rare anatomical variant with a poorly understood underlying embryological basis.[1] It is characterized by the superior and right-sided origin of the left pulmonary artery in relation to the right pulmonary artery. Identification of this variant is important due to its association with various other structural cardiac anomalies, genetic syndromes, and surgical and interventional implications.[2,3] CTA with three-dimensional and volume-rendered reconstructions can provide excellent anatomical delineation of the findings, including the extra-cardiac findings. Criss-cross pulmonary artery configuration with an extremely rare arch branch pattern (aberrant right vertebral artery), anomalous coronary anatomy, and changes of tuberculosis in the lung were well depicted with high-resolution CTA images in our case, which have important management implications. Although criss-cross pulmonary arteries have been described previously with various other structural cardiac diseases, to the best of our knowledge, a unique combination of anomalies as in our case, along with pulmonary tuberculosis, has not been reported before. A timely surgical repair for double outlet right ventricle, pulmonary stenosis, and ostium secundum atrial septal defect is warranted to prevent further circulatory insufficiency and cardiac dysfunction. While the criss-cross pulmonary arteries and anomalous left circumflex coronary artery may in themselves not cause any symptoms, they are important for planning an appropriate therapeutic approach.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.