Objective:The objective of the study was to examine the size and growth of fetal pulmonary artery diameters in congenital diaphragmatic hernia and assess their correlation with postnatal survival and morbidity outcome...Objective:The objective of the study was to examine the size and growth of fetal pulmonary artery diameters in congenital diaphragmatic hernia and assess their correlation with postnatal survival and morbidity outcomes. Study design:Prospective antenatal echocardiographic examination of fetal branch pulmonary arteries were correlated with postnatal survival and respiratory morbidity in cases of congenital diaphragmatic hernia. Receiver operator curves were developed to assess their utilization as predictors of respiratory morbidity and survival. Results:Twenty-one cases were diagnosed antenatally over 3 years. Fifteen subjects survived postnatally. The ipsilateral fetal branch pulmonary artery diameter was smaller throughout gestation in left-sided congenital diaphragmatic hernia (P = .008). The small left pulmonary diameter did not correlate with survival but correlated strongly with morbidity outcomes. Progressive ipsilateral fetal pulmonary artery hypoplasia was demonstrated throughout gestation in subjects who died or had severe morbidity. Conclusion:Fetal pulmonary artery diameter measurements correlate with respiratory morbidity in postnatal congenital diaphragmatic hernia and may assist with prediction of outcome. Survival has improved,possibly because of improved postnatal management of congenital diaphragmatic hernia,limiting this measurement in assessing survival.展开更多
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Objective:The objective of the study was to examine the size and growth of fetal pulmonary artery diameters in congenital diaphragmatic hernia and assess their correlation with postnatal survival and morbidity outcomes. Study design:Prospective antenatal echocardiographic examination of fetal branch pulmonary arteries were correlated with postnatal survival and respiratory morbidity in cases of congenital diaphragmatic hernia. Receiver operator curves were developed to assess their utilization as predictors of respiratory morbidity and survival. Results:Twenty-one cases were diagnosed antenatally over 3 years. Fifteen subjects survived postnatally. The ipsilateral fetal branch pulmonary artery diameter was smaller throughout gestation in left-sided congenital diaphragmatic hernia (P = .008). The small left pulmonary diameter did not correlate with survival but correlated strongly with morbidity outcomes. Progressive ipsilateral fetal pulmonary artery hypoplasia was demonstrated throughout gestation in subjects who died or had severe morbidity. Conclusion:Fetal pulmonary artery diameter measurements correlate with respiratory morbidity in postnatal congenital diaphragmatic hernia and may assist with prediction of outcome. Survival has improved,possibly because of improved postnatal management of congenital diaphragmatic hernia,limiting this measurement in assessing survival.
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.