Connection between the right upper pulmonary vein to the superior vena cava in a patient with a secundum-type atrial septal defect.

The Editor,

A 23-year-old man was referred to our echocardiography department for further evaluations of right ventricular enlargement. His chief complaint was dyspnea on exertion (New York Heart Association's functional class II). Transthoracic echocardiography demonstrated severe right ventricular enlargement and a large secundum-type atrial septal defect (ASD). The defect was confirmed by 2D and 3D transesophageal echocardiography (TEE). Furthermore, 2D TEE with an agitated saline injection and 3D TEE revealed a connection between a vein and the superior vena cava [Figure 1a-c and Videos 1, 2]. Computed tomographic angiography of the pulmonary veins confirmed the connection between the superior pulmonary vein and one half of the distal superior vena cava [Figure 1d and e]. The patient was, therefore, referred for corrective surgery.

Figure 1

(a) En face view of the interatrial septum from the right atrium, obtained via 3DTEE shows a secundum type ASD (*). (b) Agitated saline injection in 2D TEE shows a negative contrast (*) in the SVC, suggestive of a connection between a vein and the SVC. (c) 3DTEE, obtained via the full-volume mode and subsequently cropped demonstrates a connection between a pulmonary vein and the SVC. Computed tomographic angiography in (d) the transverse, and (e) coronal planes shows a connection between the RUPV to one half of the distal SVC. AO: Aorta; ASD: Atrial septal defect; RUPV: Right upper pulmonary vein; SVC: Superior vena cava

ASD is the most congenital cardiac disease in adulthood that can be accompanied with partial anomalous pulmonary vein connection (PAPVC).[1] PAPVC is a condition defined as abnormal connection of one, two, or three pulmonary veins(s) (PV) to right circulation. This abnormal connection in more prevalent in right PV(s) than left PV(s).[2] The sinus venosus-type ASD accompany PAPAVC more often than secundum-type ASD, i.e., 85% and 10–15% respectively.[3] The coexistence of secundum and sinus venosus-type ASD has been reported in 7% ASD cases.[4] In secundum-type ASD, abnormal connection of left PVs is more common than right PVs,[5] so the presence of an anomalous connection between the right PV and a secundum-type ASD is occasionally seen, and any experienced cardiologist should consider it while evaluating a patient with a secundum-type ASD because of detection of PAPVC may lead to change in treatment plan.

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Conflicts of interest

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