Neonatal hyperreninemia and hypertensive heart failure relieved with resection of an intralobar pulmonary sequestration

Intralobar pulmonary sequestrations are congenital lung malformations sometimes known to cause symptoms of heart failure in the neonate because of shunting of blood flow from an anomalous systemic feeding artery into the pulmonary venous system. These are cured by pulmonary lobectomy. We report the case of a 37-weeks' gestational age infant who presented with bilateral pleural effusions perinatally, followed by hypertensive heart failure and hyperreninemia 2 weeks postnatally. Computed tomography revealed a large artery from the infradiaphragmatic aorta proximal to the celiac axis supplying a right lower lobe intralobar pulmonary sequestration and 4 small nonstenosed renal arteries. Subsequent to a right lower lobectomy, the patient recovered with a precipitous drop in plasma renin assay level and was discharged from the neonatal intensive care unit in good condition 12 days postoperatively. This is the first reported case of hyperreninemia treated by lobectomy. We hypothesize that the aberrant feeding vessel resulted in a relative deficiency in renal perfusion via a “steal” effect that was relieved by surgical ligation of the artery.