Pulmonary hypertension in the newborn- etiology and pathogenesis

A disruption in the well-orchestrated fetal-to-neonatal cardiopulmonary transition at birth results in the clinical conundrum of severe hypoxemic respiratory failure associated with elevated pulmonary vascular resistance (PVR), referred to as persistent pulmonary hypertension of the newborn (PPHN). In the past three decades, the advent of surfactant, newer modalities of ventilation, inhaled nitric oxide, other pulmonary vasodilators, and finally extracorporeal membrane oxygenation (ECMO) have made giant strides in improving the outcomes of infants with PPHN. However, death or the need for ECMO occurs in 10-20% of term infants with PPHN. Better understanding of the etiopathogenesis of PPHN can lead to physiology-driven management strategies. This manuscript reviews the fetal circulation, cardiopulmonary transition at birth, etiology, and pathophysiology of PPHN.