Successful salvage of the left pulmonary artery in a neonate with isolated unilateral absence of the pulmonary artery.

ABSTRACT

Isolated unilateral absence of the pulmonary artery (UAPA) is a congenital anomaly where involution of the extrapulmonary PA is insufficient and the intrapulmonary PA is only fed by the ductus arteriosus. Affected lung disorder causes complications years after ductus closure; thus, early diagnosis is of importance to avoid these complications. Here, we present the case of a male infant who was admitted to the neonatal intensive care unit because of transient tachypnea of the newborn and absence of the left PA (LPA) was indicated. Intensive echocardiography could detect neither the LPA nor the aortopulmonary collateral arteries to the left lung. Although the ductus was orthotopic with the right aortic arch, use of prostaglandin (PG) E1 unmasked the diagnosis of UAPA with bilateral ductus arteriosus. After ductal closure, delineation of the anatomy is not necessarily easy even with catheterization, whereas early use of PGE1 facilitates anatomical understanding by echocardiography, particularly early after birth. <Learning Objectives: While identification of disrupted pulmonary artery is often unsuccessful after ductal closure in the patients with unilateral absence of pulmonary artery, use of prostaglandin in the neonate with right aortic arch and large orthotropic right ductus arteriosus allowed successful recirculation of the disrupted pulmonary artery by reopening closed contralateral ductus arteriosus. Prostaglandin infusion early after birth delineated anatomical diagnosis of unilateral absence of pulmonary artery due to bilateral ductus, and it also made it possible to perform single stage uni-focalization.>.