Diagnosis & management of pulmonary hypertension in congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) contributes to neonatal morbidity and mortality worldwide. Pulmonary hypertension (PH) is a key component of CDH pathophysiology and critical consideration for management and therapeutic options. PH associated with CDH has traditionally been attributed to pulmonary vascular maldevelopment and associated lung parenchymal hypoplasia, leading to pre-capillary increase in pulmonary vascular resistance (PVR). However, there is increasing recognition that left ventricular hypoplasia, dysfunction and elevated end diastolic pressure may contribute to post-capillary pulmonary hypertension in CDH patients. The interplay of these mechanisms and associated dysfunction in the right and left ventricles results in variable hemodynamic phenotypes in CDH. Clinical assessment of individual phenotype may help guide personalized management strategies, including effective use of pulmonary vasodilators and extra-corporeal membrane oxygenation. Ongoing investigation of the underlying mechanisms of PH in CDH, and efficacy of physiology-based treatment approaches may support improvement in outcomes in this challenging condition.