Children with congenital diaphragmatic hernia (CDH), a condition characterised by herniation of the abdominal contents into the thoracic cavity, also develop pulmonary hypertension. Angiopoietin-1 (Ang-1), a central mediator of angiogenesis, is involved in the development of non-familial pulmonary hypertension, but its role in CDH-associated pulmonary hypertension is unclear. Grzenda et al. now examine the expression patterns of Ang-1 and its receptor Tie-2 during lung development in normal mice and in a nitrofen-based murine model of CDH. Their results suggest that the Ang-1–Tie-2 pathway is important during normal lung development and that alterations in the pathway might be responsible for the development of pulmonary hypertension in CDH. Investigation of the role of other components of the Ang-1 pathway during normal and pathological lung development is therefore warranted. Page 106

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