title: Persistent pulmonary hypertension of the newborn.
abstract: Persistent pulmonary hypertension of the newborn (PPHN) is characterized by elevated pulmonary vascular resistance resulting in right-to-left shunting of blood and hypoxemia. PPHN is often secondary to parenchymal lung disease (such as meconium aspiration syndrome, pneumonia or respiratory distress syndrome) or lung hypoplasia (with congenital diaphragmatic hernia or oligohydramnios) but can also be idiopathic. The diagnosis of PPHN is based on clinical evidence of labile hypoxemia often associated with differential cyanosis. The diagnosis is confirmed by the echocardiographic demonstration of - (a) right-to-left or bidirectional shunt at the ductus or foramen ovale and/or, (b) flattening or leftward deviation of the interventricular septum and/or, (c) tricuspid regurgitation, and finally (d) absence of structural heart disease. Management strategies include optimal oxygenation, avoiding respiratory and metabolic acidosis, blood pressure stabilization, sedation and pulmonary vasodilator therapy. Failure of these measures would lead to consideration of extracorporeal membrane oxygenation (ECMO); however decreased need for this rescue therapy has been documented with advances in medical management. While trends also note improved survival, long-term neurodevelopmental disabilities such as deafness and learning disabilities remain a concern in many infants with severe PPHN. Funded by: 1R01HD072929-0 (SL).
authors.affiliation: Department of Pediatrics (Neonatology), Hennepin County Medical Center, 701 Park Avenue, Shapiro Building, Minneapolis, MN 55415 USA.
authors.forename: Vinay
authors.initials: V
authors.lastname: Sharma
authors.affiliation: Department of Pediatrics, Women and Children's Hospital of Buffalo, 219 Bryant St, Buffalo, New York 14222 USA.
authors.forename: Sara
authors.initials: S
authors.lastname: Berkelhamer
authors.affiliation: Department of Pediatrics, Women and Children's Hospital of Buffalo, 219 Bryant St, Buffalo, New York 14222 USA.
authors.forename: Satyan
authors.initials: S
authors.lastname: Lakshminrusimha
keywords: Fetal circulation; Meconium aspiration; Milrinone; Natriuretic peptide; Nitric Oxide; Prostaglandin E1; Pulmonary vascular remodeling; Sildenafil; Surfactant