Dear Editor,
A seven-day-old neonate presented to our institution with complaints of respiratory distress since birth. Transthoracic echocardiography suggested total anomalous pulmonary venous return with non-visualisation of the left pulmonary artery. A contrast-enhanced computed tomography angiography (CTA) was done for further evaluation, which confirmed a supracardiac type of total anomalous pulmonary venous return (TAPVR). The four pulmonary veins joined together to form a common pulmonary vein, which drained into a vertical vein, which in turn drained into the left brachiocephalic vein. Additionally, an anomalous left pulmonary artery (LPA) was observed arising from the right pulmonary artery (RPA). The LPA was observed coursing between the trachea and oesophagus to reach the left pulmonary hilum, suggesting a pulmonary artery sling (PAS). There was also a large ostium secundum atrial septal defect (ASD) [Figure 1]. The child was planned for surgical repair of the anomalies.
Figure 1.
Computed tomography angiography (CTA) maximum intensity projection (MIP) coronal images (a and b) showing the right and left pulmonary veins (RPV and LPV in a) draining into a vertical vein (arrow, a and b), followed by drainage into the left brachiocephalic vein (LT BCV in a and b) and right SVC (RT SVC in a and b). Axial CTA image (c) shows the LPA arising from the RPA. Thin volume-rendered technique (VRT) image (d) shows an atrial septal defect (white star)
TAPVR is a rare congenital heart defect, with incidence ranging from 0.6 to 1.2 per 10,000 live births.[1] TAPVR usually occurs as an isolated lesion but may be associated with other anomalies such as atrioventricular septal defect, transposition of the great arteries, pulmonary stenosis, double outlet of the right ventricle, and coarctation of the aorta.[1] PAS is an uncommon congenital vascular anomaly with a reported prevalence of 59 cases per million children.[2] In PAS, the left pulmonary artery originates from the posterior surface of the RPA, instead of the main pulmonary trunk, resulting in an anomalous long course of the LPA in between the trachea and the oesophagus.[3] Both TAPVR and PAS can lead to respiratory distress in the newborn, hence imaging plays a crucial role in identifying the underlying cause amongst the plethora of anomalies. The association of TAPVR with PAS, to the best of our knowledge, has not been previously described. Timely identification of the TAPVR and the associated anomalies is important for appropriate management. The indexed case highlights the unique association between pulmonary venous and pulmonary arterial anomalies. CTA with multiplanar and three-dimensional reconstructions allows excellent anatomical depiction and detailed evaluation of the cardiac as well as extracardiac structures, which is crucial for surgical planning.[4]
Conflicts of interest
There are no conflicts of interest.
Funding Statement
Nil.
REFERENCES
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