Scimitar syndrome with large atrial septal defect and a rare partial anomalous venous drainage in an adult: a case report.

Abstract

Scimitar syndrome is a very rare congenital cardio-pulmonary disease with anomalous right pulmonary vein draining either partially or completely into the inferior vena cava. It is called Scimitar syndrome due to the classical appearance in the chest X-ray, which resembles the curved blade of Turkish sword 'Scimitar'. It commonly associates with atrial septal defect (ASD), hypoplasia of the right lung, dextroposition of the heart, and pulmonary hypertension (PHT). A 67-year-old lady, diagnosed with atrial fibrillation and moderate PHT 3 years ago, presented with worsening bilateral ankle oedema and New York Heart Association class III shortness of breath. Chest X-ray showed the Scimitar appearance. The trans-thoracic and trans-oesophageal echocardiograms revealed a 46 mm ASD and a partial anomalous pulmonary venous drainage (PAPVD) of the right upper pulmonary vein (RUPV) into the right atrium at the junction of the atria. Three-dimensional reconstruction of the computed tomographic pulmonary angiogram confirmed Scimitar syndrome of the right lower pulmonary vein (RLPV). We managed her conservatively on her wish. After 13 months, she succumbed due to a massive stroke. We describe a very rare case of an elderly lady who has Scimitar syndrome with an ASD and evidence of PAPVD of the RUPV; thus, we intend to provide an antecedent for further cases, for prompt and accurate diagnosis and timely interventions in order to prevent life-threatening complications.