Role of TBX4 in neonatal and pediatric developmental lung disease

In the recent paper, Pulmonary Vascular Compromise is Associated with Survival in Pediatric Pulmonary Hypertension: a New Computational Model by Niccum et al. [1] presents an innovative computational model reframing pulmonary vascular disease through the concept of pulmonary vascular compromise (PVC), a quantifiable estimate of the proportion of pulmonary vascular surface area lost relative to an idealized normal pulmonary vascular capacity.

Children with single ventricle heart disease (SVHD) demonstrate decreased arginine/NO metabolism following Stage 2 (Glenn) palliation, associated with poor postoperative outcomes. It is unknown if arginine dysregulation persists at Stage 3 (Fontan). 

Pulmonary hypertension (PH) in children requires complex medical management. Health-related quality of life (HRQoL) remains understudied in this population.