Grant: Travel Awards - Institute Community Support

Congenital diaphragmatic hernia patients are born with underdeveloped lungs and pulmonary hypertension. Pulmonary hypertension is a condition where the blood vessels in the lung become thickened, stiff, and constricted, which impairs breathing. Bosentan is a drug currently used to treat pulmonary hypertension in these patients, but unfortunately the effectiveness of this treatment is unknown. In order to determine how effective bosentan is we created a mouse model that has pulmonary hypertension like that of congenital diaphragmatic hernia patients. Next we evaluated the severity of the mouses pulmonary hypertension using a treadmill exercise test and echocardiographs. We then treated the mice with bosentan for three weeks. After three weeks of treatment we then reevaluated the pulmonary hypertension in the mice. We found that after three weeks of treatment the mice had improved exercise capacity and decreased pulmonary hypertension. This shows that bosentan is an effective drug in treating pulmonary hypertension. Our hope is that understanding how effective this drug is can lead to more informed use in congenital diaphragmatic hernia patients and help these patients live better healthier lives.