weekly_question 12/6/2022

[Admin]

You are seeing a mother carrying a fetus diagnosed with a left sided CDH. The O/E LHR using tracing method at 24 weeks is 28%. Follow-up measurement at 26 weeks is 32%. The parents conceived this baby after significant difficulty and are asking about the outcomes of fetal tracheal occlusion (FETO) therapy for their baby. You advise the parents that

A Pregnancy should be terminated.

B FETO is definitely recommended.

C Delivery is safe at a community hospital.

D Delivery should be at a hospital with ECMO resources.

[Admin]

correct answer
D Delivery should be at a hospital with ECMO resources.


In 2021, Deprest et al. published their results on the Tracheal Occlusion to Accelerate Lung Growth (TOTAL) trial. The accrual of patients occurred over 11 years. The patients were categorized as “moderate” (defined as the quotient of observed-to-expected lung-to-head ratios of 25.0 to 34.9%, irrespective of liver position, or 35.0 to 44.9% with intrathoracic liver herniation) or “severe” (the observed-to-expected lung-to-head ratios of less than 25.0%, irrespective of liver position). The intervention was Fetal Endoluminal Tracheal Occlusion (FETO) performed at 27-29 weeks’ gestation and removed at 34 weeks. The primary outcomes were infant survival to discharge and survival without oxygen supplementation at six months.


In the moderate group, 63% survived in the FETO group and 50% in the expectant care group (relative risk, 1.27; 95% confidence interval [CI], 0.99 to 1.63; two-sided P = 0.06). At 6 months of age, 53 of 98 infants (54%) in the FETO group and 43 of 98 infants (44%) in the expectant care group were alive without oxygen supplementation (relative risk, 1.23; 95% CI, 0.93 to 1.65). However, the incidence of preterm rupture of membranes was higher among women in the FETO group than among those in the expectant care group (44% vs. 12%; relative risk, 3.79; 95% CI, 2.13 to 6.91). Preterm birth (64% vs. 22%, respectively; relative risk, 2.86; 95% CI, 1.94 to 4.34) was higher in the FETO group as well.


In the severe group, 40% of infants in the FETO group survived to discharge, as compared with 15% (6 of 40) in the expectant care group (relative risk, 2.67; 95% confidence interval [CI], 1.22 to 6.11; two-sided P=0.009). Survival to 6 months of age was identical to the survival to discharge (relative risk, 2.67; 95% CI, 1.22 to 6.11). The incidence of preterm, rupture of membranes was higher among women in the FETO group than among those in the expectant care group (47% vs. 11%; relative risk, 4.51; 95% CI, 1.83 to 11.9), as was the incidence of preterm birth (75% vs. 29%; relative risk, 2.59; 95% CI, 1.59 to 4.52).


Based on these data, the authors concluded that infants with moderate CDH should be treated expectantly while those with severe CDH be offered FETO. Critics of the studies have cited a few concerns with the studies. While trials were indeed randomly assigned, the studies took 11 years to complete. During the last 11 years, there were many changes to the treatment of patients with CDH and the ECMO utilization rate in this cohort is lower than typically reported. Further, the 15% survival seen in the expectantly managed “severe” patients is lower than the mortality quoted by the North American Fetal Treatment Network (NAFTnet) which is closer to 40%, with or without FETO. The authors responded by citing that postnatal survival of severe CDH babies may be overestimated due to inability to account for the hidden mortality in this condition.


In this particular scenario, based on the published data, expectant management after delivery is recommended. However, once born, the baby would be well served by being in a level IV NICU with systems and protocols to care for a baby with CDH and pulmonary hypertension, including the ability to provide ECMO, if necessary.


NAFTNet consists of centers in the US and Canada that perform advanced in-utero fetal therapeutic procedures. Recent publications support standardization of prenatal measurements in fetuses with CDH.

[Radwan suleiman abukarsh]

D

[Admin]

correct answer
D Delivery should be at a hospital with ECMO resources.


In 2021, Deprest et al. published their results on the Tracheal Occlusion to Accelerate Lung Growth (TOTAL) trial. The accrual of patients occurred over 11 years. The patients were categorized as “moderate” (defined as the quotient of observed-to-expected lung-to-head ratios of 25.0 to 34.9%, irrespective of liver position, or 35.0 to 44.9% with intrathoracic liver herniation) or “severe” (the observed-to-expected lung-to-head ratios of less than 25.0%, irrespective of liver position). The intervention was Fetal Endoluminal Tracheal Occlusion (FETO) performed at 27-29 weeks’ gestation and removed at 34 weeks. The primary outcomes were infant survival to discharge and survival without oxygen supplementation at six months.


In the moderate group, 63% survived in the FETO group and 50% in the expectant care group (relative risk, 1.27; 95% confidence interval [CI], 0.99 to 1.63; two-sided P = 0.06). At 6 months of age, 53 of 98 infants (54%) in the FETO group and 43 of 98 infants (44%) in the expectant care group were alive without oxygen supplementation (relative risk, 1.23; 95% CI, 0.93 to 1.65). However, the incidence of preterm rupture of membranes was higher among women in the FETO group than among those in the expectant care group (44% vs. 12%; relative risk, 3.79; 95% CI, 2.13 to 6.91). Preterm birth (64% vs. 22%, respectively; relative risk, 2.86; 95% CI, 1.94 to 4.34) was higher in the FETO group as well.


In the severe group, 40% of infants in the FETO group survived to discharge, as compared with 15% (6 of 40) in the expectant care group (relative risk, 2.67; 95% confidence interval [CI], 1.22 to 6.11; two-sided P=0.009). Survival to 6 months of age was identical to the survival to discharge (relative risk, 2.67; 95% CI, 1.22 to 6.11). The incidence of preterm, rupture of membranes was higher among women in the FETO group than among those in the expectant care group (47% vs. 11%; relative risk, 4.51; 95% CI, 1.83 to 11.9), as was the incidence of preterm birth (75% vs. 29%; relative risk, 2.59; 95% CI, 1.59 to 4.52).


Based on these data, the authors concluded that infants with moderate CDH should be treated expectantly while those with severe CDH be offered FETO. Critics of the studies have cited a few concerns with the studies. While trials were indeed randomly assigned, the studies took 11 years to complete. During the last 11 years, there were many changes to the treatment of patients with CDH and the ECMO utilization rate in this cohort is lower than typically reported. Further, the 15% survival seen in the expectantly managed “severe” patients is lower than the mortality quoted by the North American Fetal Treatment Network (NAFTnet) which is closer to 40%, with or without FETO. The authors responded by citing that postnatal survival of severe CDH babies may be overestimated due to inability to account for the hidden mortality in this condition.


In this particular scenario, based on the published data, expectant management after delivery is recommended. However, once born, the baby would be well served by being in a level IV NICU with systems and protocols to care for a baby with CDH and pulmonary hypertension, including the ability to provide ECMO, if necessary.


NAFTNet consists of centers in the US and Canada that perform advanced in-utero fetal therapeutic procedures. Recent publications support standardization of prenatal measurements in fetuses with CDH.