Premature differentiation of vascular smooth muscle cells in human congenital diaphragmatic hernia

► Vascular smooth muscle cells (VSMC) vary morphologically from synthetic to contractile cells ► Congenital Diaphragmatic Hernia (CDH) patients suffer from pulmonary hypertension (PH) ► PH shows thickening of adventitia, VSMC layer and vascular neo-muscularization ► CDH lungs contain more synthetic VSMCs than controls ► We show that VSMCs are phenotypically different at the onset of CDH.