We illustrate the case of a newborn with dyspnea after delivery. Echocardiography established the diagnosis of severe coarctation of the aorta (CoA). The patient underwent surgical correction (resection of CoA and end-to-end anastomosis). At the age of 5 years, he showed hypertension at follow-up. Due to unclear outpatient echocardiographic findings of increased gradient across the aortic valve and aortic isthmus (55mmHg and 85mmHg respectively), the patient underwent cardiothoracic (CT) angiography (informed consent was obtained), revealing an incidental finding of an unusual type of partial anomalous pulmonary venous drainage (PAPVD).

The right upper pulmonary vein (RUPV) (figure 1, arrows; figure 2, arrows) showed a course crossing the midline behind the right main bronchus, and anterior to the left bronchus and descending aorta (figure 2A, arrows: Ao, aorta), into the left venous angle (figure 1A, arrows; figure 2B, arrows: SVC, superior vena cava). Supravalvar aortic stenosis was confirmed by computed tomography angiography, which excluded CoA.

Figure 1

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Figure 2

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The prevalence of PAPVD is reported up to 0.7% of newborns. The most common variant of a right-sided PAPVD is drainage of the right upper or middle PV into the superior vena cava. This type is associated with a superior sinus venosus septal defect.

The most common form of left-sided PAPVD is the partial return of the left upper PV to the left venous angle of the left innominate vein (IV) (figure 1A, arrows; figure 2B, arrows). This case illustrates a rare form of PAPVD that combines the 2 most common variants of PAPVD. The right upper pulmonary vein crosses the midline and drains into the left venous angle of the innominate vein. CT angiography plays a crucial role in imaging these types of anomalies.