Successful management of the persistent pulmonary hypertension of the newborn with transposition of the great arteries by restricted patency of the ductus arteriosus: a simple and rational novel strategy.

Persistent pulmonary hypertension of the newborn (PPHN) complicated with transposition of the great arteries (TGA) and an intact ventricular septum (IVS) often is therapy resistant and associated with an extremely poor prognosis. For this condition, opening of the ductus arteriosus (DA) by prostaglandin-E1 in conjunction with balloon atrial septostomy and nitric oxide inhalation therapy has been applied as an initial treatment strategy. A case of PPHN with TGA/IVS is presented together with a strategy of intentionally restricted DA to increase pulmonary blood flow successfully used in treating PPHN that otherwise was resistant to conventional therapy. The DA restriction strategy may help to improve the prognosis of patients who have PPHN complicated with TGA/IVS.