Rare cause of pulmonary hypertension and elevated lactate in a neonate: Scimitar Syndrome Case Report

Abstract

Scimitar syndrome, a rare congenital anomaly with an incidence of 1-3/100,000 live births, is commonly associated with hypoplasia of the right lung, pulmonary sequestration, and persisting superior vena cava (SVC). The other features are a partial anomalous pulmonary venous connection (PAPVC) with a curvilinear pattern on chest radiograph, with the pulmonary veins draining to the Inferior Vena Cava (IVC). This case report presents a two-week-old female, presented with escalating respiratory distress. Serum lactate gradually increased despite sildenafil and diuretics. Scimitar syndrome with large aortopulmonary collaterals from the celiac trunk was confirmed by contrast CT. Multiple abnormal systemic arteries (4-6 mm in diameter) were discovered during a one-month cardiac catheterization. Transcatheter embolization with Piccolo coils, PDA occlusion devices, and microvascular plugs worked well. Transcatheter embolization of aortopulmonary collateral vessels in neonatal Scimitar syndrome leads to a rapid resolution of high-output cardiac failure and pulmonary hypertension. Transient persistent hyperlactemia (resolving by 6 months of age) is likely due to ischemic changes in the embolized lung. Neonates with hyperlactemia should be screened for Scimitar syndrome.