Management of pulmonary sequestration and scimitar syndrome presenting in infancy

Abstract

Anomalous systemic arterial supply to the lung, either isolated or in association with partial anomalous pulmonary venous drainage (scimitar syndrome, SS), is uncommon in infancy. Traditionally the management has been surgical, but more recently the technique of arterial embolisation (AE) has been used. We report 11 infants seen over 11 years with anomalous systemic arterial supply to the lung. All patients presented by 10 months of age, 10 with persisting tachypnoea and 1 with pneumonia. Seven had isolated anomalous vascular supply to the lung and 4 associated SS. Six had pulmonary hypertension (PH) at presentation and all of these had associated cardiac defects. Five patients were managed surgically (ligation 3, resection 2) and 5 by AE. All infants managed by AE and 1 of the 3 with ligation have had excellent outcomes; 2 patients with SS had reduction of PH following AE. Three patients with isolated anomalous systemic vascular supply to the lung are now asymptomatic. Two patients with anomalous arterial supply to the lung and significant cardiac lesions had cardiac surgery performed but had persistant cardiac failure. Ligation of the anomalous vessel was then undertaken, but both died. AE is an effective means of initial management of anomalous systemic arterial supply to the lung. It precludes the immediate need for surgery and may lead to control of cardiac failure and PH. In patients with associated cardiac anomalies, AE of the anomalous systemic arterial supply should be considered prior cardiac surgery. Reduction in PH following embolisation of the anomalous systemic arterial connection to the lung occurs in SS, and AE should be considered early in the management of these patients.