Background:

Scimitar syndrome consists in a rare malformation characterized by a partial abnormal connection in one or both right pulmonary veins to the inferior vena cava, right lung hypoplasia and systemic circulation from the descending aorta. Scimitar syndrome is occasionally associated to other congenital malformations, such as patent ductus arteriosus (PDA).

Case report:

We report a 4-year-old patient with ‘‘adult’’ variety of scimitar syndrome associated to symptomatic PDA, which was successfully occluded using retrograde guidewire-established femoral arteriovenous loop with an Amplatzer® PDA occluder, without complications.

Conclusions:

Scimitar syndrome is complex and requires a complete hemodynamic study for the determination of the appropriate treatment. Pulmonary arterial hypertension is a factor associated with poor prognosis.