weekly_question 12/3/2023

[Admin]

A 38-week gestation, 2.9-kg infant is transferred to the NICU with the diagnosis of a left congenital diaphragmatic hernia (CDH). She is intubated and on 100% FiO₂ with ventilator pressures of 28/6, IMV rate of 60/minute and a preductal ABG of pH 7.10, PaO₂ 75 and PaCO₂ 65. Head ultrasound is normal. However, her echocardiogram shows coarctation of the aorta with a large PDA and right to left flow to the aorta distal to the coarctation. The left ventricle does not appear small. Gentle ventilation is initiated, but the infant continues to deteriorate. Cardiology recommends prostaglandin E1 to maintain ductal flow. High frequency ventilation and nitric oxide are provided, but hypoxia and metabolic acidosis worsen.

The next best step in management of this patient with a CDH and aortic coarctation is

A emergent repair of the CDH.

B emergently repair the coarctation.

C continue medical management-not an ECMO candidate.

D VV ECMO.

E VA ECMO.

[Admin]

correct answer
D VV ECMO.

Congenital diaphragmatic hernia (CDH) is frequently associated with congenital cardiac anomalies (30%), making treatment of these infants a major challenge. Aortic coarctation in the setting of CDH can be associated with left ventricular hypoplasia resulting in a nearly universally fatal condition. However, infants with CDH and isolated coarctation have survived.

CDH and coarctations can be difficult to diagnose. ECHO can demonstrate what appears to be a small arch due to the high pulmonary pressures and right to left flow. It may not be until the duct has closed to increase flow across the aorta that one can confidently rule out coarctation of the aorta.

If medical management can stabilize the infant (including PGE1), early repair of the CDH followed by repair of coarctation and PDA is feasible. However, if the infant continues to deteriorate then venovenous (VV) ECMO may be preferred to venoarterial (VA) cannulation. VA ECMO can improve preductal oxygen saturation, but would not effectively improve postductal oxygen delivery and metabolic acidosis may worsen. Furthermore, if VA ECMO proved helpful in managing pulmonary hypertension, ligation of the right carotid artery at the completion of ECMO creates limitations in the subsequent repair of the coarcation where clamping the aortic arch might be required. VV ECMO has the potential to improve oxygenation including postductal, postcoarctation flow.

[eselhalaby]

E

[Sarah Magdy Abdelmohsen]

E

[Admin]

correct answer
D VV ECMO.

Congenital diaphragmatic hernia (CDH) is frequently associated with congenital cardiac anomalies (30%), making treatment of these infants a major challenge. Aortic coarctation in the setting of CDH can be associated with left ventricular hypoplasia resulting in a nearly universally fatal condition. However, infants with CDH and isolated coarctation have survived.

CDH and coarctations can be difficult to diagnose. ECHO can demonstrate what appears to be a small arch due to the high pulmonary pressures and right to left flow. It may not be until the duct has closed to increase flow across the aorta that one can confidently rule out coarctation of the aorta.

If medical management can stabilize the infant (including PGE1), early repair of the CDH followed by repair of coarctation and PDA is feasible. However, if the infant continues to deteriorate then venovenous (VV) ECMO may be preferred to venoarterial (VA) cannulation. VA ECMO can improve preductal oxygen saturation, but would not effectively improve postductal oxygen delivery and metabolic acidosis may worsen. Furthermore, if VA ECMO proved helpful in managing pulmonary hypertension, ligation of the right carotid artery at the completion of ECMO creates limitations in the subsequent repair of the coarcation where clamping the aortic arch might be required. VV ECMO has the potential to improve oxygenation including postductal, postcoarctation flow.