Familial persistent pulmonary hypertension.

Familial primary pulmonary hypertension.

O insights into a disease are gained by studying the exceptional cases, the outliers. For the population with primary pulmonary hypertension (PPH), it is the patients with the familial or hereditary form that are the outliers, representing only 6 percent of the recent NHLBI PPH Registry population.' However, with recent advances in molecular biology and genetic analysis, the familial population...

Persistent pulmonary hypertension in the neonate.

Respiratory failure is the leading cause of death in the neonatal period. The anatomic and functional basis for this, particularly in full-term infants, most often is persistent pulmonary hypertension of the neonate (PPHN). This condition is reversible but can cause very severe and unrelenting respiratory failure and ultimate death when uncontrolled. Recent technologic advances have expanded th...

Persistent Pulmonary Hypertension in the Newborn

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 al...

Persistent Pulmonary Hypertension of the Newborn

Introduction: Persistent pulmonary hypertension of the newborn (PPHN) was first described by Gersony et al 1 of persistence of the fetal circulation in 1969. It is a clinical syndrome characterized by respiratory distress, hypoxemia, elevated pulmonary vascular resistance and a right to left shunting of venous blood across the foramen ovale and/or ductus arteriosus. The etiology of PPHN is not ...

Persistent Pulmonary Hypertension of the Newborn (pphn)