Congenital diaphragmatic hernia is a rare entity in childhood carrying a high mortality rate of 30% to 50% . Ipsilateral pulmonary hypoplasia,increased pulmonary vascular resistance,and potential cardiac failure compl...Congenital diaphragmatic hernia is a rare entity in childhood carrying a high mortality rate of 30% to 50% . Ipsilateral pulmonary hypoplasia,increased pulmonary vascular resistance,and potential cardiac failure complicate early postnatal life. Surgical correction can either be performed on the first day of life or be deferred to a time after stabilization of the infant. Our patient presented with a left-sided Bochdalek’s hernia containing large and small bowel. She required intubation and resuscitation on day 1 of life,and surgical repair had to be postponed. Further respiratory deterioration required commencement of inhaled nitric oxide and high-frequency ventilation. Pulmonary artery pressure rose to suprasystemic level. Closure of the ductus arteriosus on day 8 resulted in imminent right-sided heart failure. Commencement of alprostadil (prostaglandin E1) reopened the ductus and stabilized the patient. Surgical repair was successful 3 days later. Alprostadil should be considered as an important component of therapy in severe cases of congenital diaphragmatic hernia,where deterioration of right-sided heart function occurs.展开更多
文摘Congenital diaphragmatic hernia is a rare entity in childhood carrying a high mortality rate of 30% to 50% . Ipsilateral pulmonary hypoplasia,increased pulmonary vascular resistance,and potential cardiac failure complicate early postnatal life. Surgical correction can either be performed on the first day of life or be deferred to a time after stabilization of the infant. Our patient presented with a left-sided Bochdalek’s hernia containing large and small bowel. She required intubation and resuscitation on day 1 of life,and surgical repair had to be postponed. Further respiratory deterioration required commencement of inhaled nitric oxide and high-frequency ventilation. Pulmonary artery pressure rose to suprasystemic level. Closure of the ductus arteriosus on day 8 resulted in imminent right-sided heart failure. Commencement of alprostadil (prostaglandin E1) reopened the ductus and stabilized the patient. Surgical repair was successful 3 days later. Alprostadil should be considered as an important component of therapy in severe cases of congenital diaphragmatic hernia,where deterioration of right-sided heart function occurs.
