The nitrofen/bisdiamine murine model of congenital diaphragmatic hernia has a pulmonary hypertension vascular phenotype consistent with human CDH.

Congenital diaphragmatic hernia (CDH)-associated pulmonary hypertension (CDH-PH) has severe implications for the survival of patients with CDH; however, CDH-PH is often refractory to pulmonary vasodilators, rendering it difficult to treat. As such, models are necessary to study the etiology, mechanism, onset, and progression of pulmonary vascular remodeling in CDH. Despite several established murine models of CDH, no characterized CDH-PH or CDH-associated pulmonary vascular remodeling murine model exists. In this work, we assessed the nitrofen/bisdiamine (N/B) murine CDH model for pulmonary hypertension (PH) hallmarks to establish its usefulness as a model for studying mechanisms leading to CDH-PH. To do so, we evaluated key metrics of vascular PH at two different gestational time points and compared the results to sex- and age-matched human CDH tissue sections and results from a meta-analysis of published data of human CDH samples. We found that vessel rarefaction, smooth muscle hypertrophy, and adventitial extracellular matrix deposition were present in the N/B CDH murine model at