persistent pulmonary hypertension of newborn
Failure of normal postnatal decrease in pulmonary vascular resistance, resulting in right-to-left shunting and hypoxemia.
Full Definition
Persistent pulmonary hypertension of the newborn (PPHN) occurs when pulmonary vascular resistance fails to decrease normally after birth, maintaining fetal circulation patterns with right-to-left shunting through the foramen ovale and ductus arteriosus. This results in severe hypoxemia despite adequate ventilation. PPHN can be idiopathic or associated with conditions such as meconium aspiration syndrome, sepsis, or congenital diaphragmatic hernia. Treatment includes optimizing oxygenation and ventilation, inhaled nitric oxide to selectively reduce pulmonary vascular resistance, and in severe cases, ECMO support. The condition carries significant morbidity and mortality, particularly when associated with underlying lung disease.
Usage
Usage note: PPHN preferred over older term 'persistent fetal circulation'. Specify underlying cause when known.
In Context
- "Echocardiography confirmed severe persistent pulmonary hypertension of the newborn with suprasystemic pulmonary pressures." — Cardiology consultation
- "PPHN secondary to meconium aspiration syndrome was treated with inhaled nitric oxide and high-frequency ventilation." — NICU progress note