Congenital diaphragmatic hernia-associated pulmonary hypertension

In brief

This article addresses pulmonary hypertension in neonates with congenital diaphragmatic hernia, a major determinant of morbidity and mortality. It reviews pathophysiology, diagnostic approaches, and management strategies for CDH-associated pulmonary hypertension in the perioperative period.

• CDH-associated pulmonary hypertension results from pulmonary hypoplasia and abnormal vascular remodeling in utero.
• Echocardiography is the primary tool for diagnosing and monitoring pulmonary hypertension severity in CDH patients.
• Management includes optimizing ventilation, oxygenation, and targeted pulmonary vasodilator therapy (inhaled NO, sildenafil).
• ECMO may be required for refractory pulmonary hypertension with severe right ventricular failure despite medical therapy.
• Long-term follow-up is essential as pulmonary hypertension can persist or develop after initial CDH repair.

Written by the GCMD Library team from the article.