Abstract
Pulmonary venous connections may be infrequently abnormal in patients with tetralogy of Fallot (TOF). A special subgroup of partial anomalous pulmonary venous return,"scimitar cyndrome", and its coexistence with TOF is less frequently reported. It may proceed unnoticed, as cyanosis already predominates in the clinical picture. This uncommon association must be kept in mind for patients with TOF who have an accessory flow in the inferior vena cava, especially when all pulmonary venous return to the left atrium is not clearly seen.
Highlights
Either total or partial, may accompany tetralogy of Fallot (TOF) in some patients, which is rarely reported in the literature[1,2]
The patient was diagnosed with scimitar syndrome, after the suspicion of an abnormal flow connecting to the inferior vena cava on echocardiography, together with a radiological demonstration of right lung hypoplasia, disoriented right lower pulmonary vein, sequestrating artery originating from the descending aorta
In the light of our case report and the current literature, the detection of an abnormal flow connection to the inferior vena cava on echocardiography may raise the suspicion that scimitar syndrome lies underneath in those patients with TOF[1,4,5,6]
Summary
Either total or partial, may accompany tetralogy of Fallot (TOF) in some patients, which is rarely reported in the literature[1,2]. Scimitar syndrome represents a partial anomalous pulmonary venous connection which is associated with systemic arterial supply from the abdominal aorta to some part of the lungs, along with concurrent lung hypoplasia. This anomalous pulmonary vein often drains into the inferior vena cava, drainage into hepatic vein, right or left atrium or portal vein can occur. Cardiac computed tomography, which was used to clarify the anatomical connections, enlightened a partially abnormal venous drainage from the right lower pulmonary vein to the inferior vena cava and an anomalous arterial blood supply from the descending aorta to the sequestrating pulmonary segment, in addition to the abovementioned echocardiographic findings (Figure 1).
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