Prenatal diagnosis |
Ultrasound measurement of O/E LHR should be used between 22 and 32 w of gestational age to predict the severity of pulmonary hypoplasia in isolated CDH. | ●●●● | B-NR |
In left-sided CDH, an O/E LHR < 25% predicts poor outcome. In right-sided CDH, an O/E LHR < 45% may predict poor outcome. | ●●●○ | B-NR |
Fetal magnetic resonance imaging should be used (where available) for the assessment of lung volume and liver herniation in moderate and severe CDH. | ●●●● | B-NR |
Ventilation |
Newborns with CDH and immediate respiratory distress should be preferentially intubated at birth. Bag-valve-mask ventilation should be avoided. | ●●●● | C-EO |
Sedation should be provided to all mechanically ventilated newborns with CDH. Deep sedation and neuromuscular blockade should be provided selectively to those with greater ventilation or oxygen requirements. | ●●●● | B-NR |
A T-piece should be used with the ventilator to avoid a peak inspiratory pressure > 25 cm H2O. | ●●●● | B-NR |
An arterial pCO2 between 45 and 60 mm Hg and a pH between 7.25 and 7.40 should be targeted in all newborns with CDH. | ●●●● | B-NR |
Supplemental oxygen should be titrated to achieve a preductal saturation of at least 85%, but not > 95%. | ●●●● | B-EO |
Gentle, intermittent mandatory ventilation should be the initial ventilation mode for newborns with CDH who require respiratory support. High-frequency oscillatory ventilation or high-frequency jet ventilation should be used when the peak inspiratory pressure required to control hypercapnia using intermittent mandatory ventilation exceeds 25 cm H2O. | ●●●● | B-NR |
Hemodynamic support |
Treatment of poor perfusion (capillary refill > 3 s, lactate > 3 mmol/L, urine output < 1 mL/kg/h) and blood pressure below norms for age should include:
judicious administration of crystalloid, generally not exceeding 20 mL/kg; inotropic agents such as dopamine or epinephrine; and hydrocortisone. If poor perfusion continues, assessment of cardiac function (i.e., echocardiogram, central venous saturation) should be performed | ●●●● | B-NR |
Echocardiography |
Two standardized echocardiograms, one within 48 h of birth and one at 2–3 w of life, are needed to assess pulmonary vascular resistance, as well as left ventricular and right ventricular function. Additional studies may be conducted as clinically indicated. | ●●●● | C-LD |
Management of pulmonary hypertension |
iNO is indicated for confirmed suprasystemic pulmonary arterial hypertension without left ventricular dysfunction, provided lung recruitment is adequate. In the absence of clinical or echocardiographic response, iNO should be stopped. | ●●●○ | C-EO |
Sildenafil should be considered in patients with refractory pulmonary hypertension (i.e., unresponsive to iNO) or as an adjunct when weaning iNO. | ●●●○ | B-R |
Milrinone should be used to treat cardiac dysfunction, particularly if it is associated with pulmonary hypertension. | ●●●● | B-NR |
Prostaglandin E1 can be used to maintain ductus arteriosus patency and reduce right ventricular afterload in patients with pulmonary hypertension with right ventricular failure, or in the presence of a closing ductus. | ●●●○ | C-LD |
Extracorporeal life support |
The possibility of extracorporeal life support should be discussed during prenatal counselling for CDH, and should disclose that available evidence does not suggest a survival benefit to its use. | ●●●○ | B-R |
Surgery |
The following physiologic criteria should be met before surgery:
urine output > 1 mL/kg/h FiO2 < 0.5 preductal oxygen saturation between 85% and 95% normal mean arterial pressure for gestational age lactate < 3 mmol/L estimated pulmonary artery pressures less than systemic pressure. Failure to meet these criteria within 2 w should prompt consideration of either attempted repair or a palliative approach. | ●●○○ | C-EO |
Patch repair: For diaphragmatic defects that are not amenable to primary repair, oversized, tension-free polytetrafluoroethylene/GORE-TEX patches should be used. | ●●●● | C-LD |
Open repair v. minimally invasive surgery: A minimally invasive surgical approach or technique should not be used in the repair of neonatal CDH because of the high rates of recurrence. | ●●●● | B-NR |
For patients on extracorporeal life support: Surgery should be avoided until after decannulation. If the patient cannot be weaned off extracorporeal life support, consideration should be given for either surgery or palliation, as appropriate. | ●●●● | C-LD |
Long-term follow-up |
| ●●●● | B-NR |
| ●●●● | B-NR |
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